1. science
2. medicine
3. surgery

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Faculty
Oficial Seminar Coordinators:
Dr B. Sudarshan, Ms, Mch.
(Senior Professor having 20 years experience)
Professor of Plastic Surgery
Kurnool Medical College
Dr M. Bhanu Murthy, Ms, Mch.
Consultant Plastic Surgeon
Faculty:
Dr. Don Laub
Volunteer Clinical Associate Professor of Surgery, Stanford University,
Founder of Interplast and retired Chief of Plastic Surgery, Stanford University.
Dra. Debra Johnson
Chief of Surgery for the Sutter Community Hospitals,
and Co-Director of the Sutter Cleft Lip and Palate Panel.
Member of the Board of Directors for Interplast, Inc.
Dr. Richard Jobe
Professor of plastic surgery at Stanford University
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Cirujanos
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Dra. Andrea Jobe
Executive Director and Director of Speech Programs
RSF-EARTHSPEAK
Dra. Marlene Long
Leprosy Reconstructive Surgeon/AMREF
Flying Doctors
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Dr. Fco. Javier Beut
Presidente y Fundador Fundación Cirujanos Plástikos Mundi
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Dr. Alberto Mussolas
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Coordinador Médico Fundación Cirujanos Plástikos Mundi
Delegado de Catalunya
Dra. Pilar Alloza
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Jefe Clínico de Anestesiología (Hospital de Princeps, Barcelona)
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Preface
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Cirujanos
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Vicente Ferrer
Executive Director
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Rural Development Trust (Fundacion Vicente Ferrer) is working in Anantapur
district in the rural areas since 35 years. Rural Development Trust is now working
in 1,500 villages. It has got extensive experience in all fields of rural development
and building of people‚s institutions. Rural Development Trust works mostly in the
areas of community health, including rural hospitals, development of disabled
persons & women, education of children, environment & ecological programs,
cultural activities and special programs for economic improvement of very poor
families.
Anantapur district is approximately the size of Aragon in Spain and has a population
of almost 4,000,000. In the first 20 years of RDT‚s work not even 1% of persons
affected with cleft lip & cleft palate even knew that a surgical treatment was available
to correct the defect. Anantapur district like all districts in India was full of children
and persons suffering with cleft lip & cleft palate, untouched by available modern
techniques.
With the rise in the number of plastic surgeons in India, the facility of plastic
surgery became available close by to Anantapur, in a town 150 kms away. Having
this facility close by, a few years ago, Rural Development Trust decided to take the
challenge that, with the help of plastic surgery, there should be no child suffering
from cleft lip and cleft palate in Anantapur district. From that time, we have
motivated people in the village and explained them the advances in plastic surgery
and that their children can have a normal life like any other child. Up till today, we
have managed to get operated 200 children in this district suffering with this
problem.
Two years ago, we had a visit from Cirujanos Plastikos Mundi and as a result of that
visit, Rural Development Trust (Fundacion Vicente Ferrer) & Cirujanos Plastikos
Mundi agreed to have some collaboration between their organizations. With their
visit, came the idea to have an international workshop with specialists from other
countries to come to India to our hospital in Bathalapalli, Anantapur and to host a
workshop especially on cleft lip and cleft palate in the field of plastic surgery.
In India, international workshops are normally hosted in big specialty hospitals /
hotels in big cities. This international workshop between Rural Development Trust
(Fundacion Vicente Ferrer) and Cirujanos Plastikos Mundi is hosted in a rural
hospital in South India.
We hope this workshop will serve to improve the skills and techniques of visiting
Indian plastic surgeons and we hope the visiting professors of Plastic Surgery from
abroad will benefit from exchange and discussions with our Indian Surgeons.
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First East Indian International Cleft Surgery Workshop
Dr Y Bala Subbaiah
Director - Hopitals
Plastic Surgery is one of the fascinating branches of medical science which challenges
the very verdict of nature by remodelling the shape of tissue / structure. It equally
gives the same benefit to those who acquire structure abnormality due to accident or
any other cause. In other words, plastic surgery can be termed as RECREATION.
It gives the opportunity for an individual to RELIVE. In the context of our country,
one can even say that the effects of plastic surgery are akin to REINCARNATION
and allow a person to live a new life with human dignity.
The above is more so true in the vast rural areas of India where many children are
born with deformities they have to carry throughout the life, discriminated in many
ways by society on account of those deformities. Children, particularly girls with
cleft lip and palate and other such deformities are often unable to get married or
lead normal lives.
Not only deformities by birth but, women are often the victims of harassment in the
husband‚s home, due to which they become also victims of attempted suicide by
burning with kerosene, or family members conspire to kill them also by burning
with kerosene. If they survive, they then become victims of discrimination on
account of the horrendous injuries due to burns.
The above type of cases are commonly found in rural areas and parents and the
community in general have never heard of the advances in medical science and
plastic surgery, which can literally change the lives of these persons.
In Anantapur, in our program, which works in many different ways for the
development of disabled people, we make a point to find out all children having
cleft-lip / cleft-palate and it is our objective that in Anantapur district in future,
there should be no child with this problem who does not receive the benefit of
plastic surgery. In the last 10 years, Rural Development Trust has conducted 200
cleft lip / palate surgeries through the services of plastic surgeons in our locality.
And this represents 200 children who can now have the same hopes and aspirations
of any other child.
In the beginning, it was not easy to explain parents that this problem of cleft lip and
cleft-palate could be rectified. Many people believe that this problem is due to the
fact that pregnant mothers have been exposed to the rays of the Sun during solar
eclipse or due to exposure to Moonlight during lunar eclipse. Therefore, they feel
that this abnormality is a curse of God and should be received as such.
The main idea of this workshop is to bring together surgeons from abroad and
surgeons from India who are dealing with cleft-lip, cleft-palate and other such cases
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to share their experiences. We also hope that this workshop will impart new or
improved surgical techniques and skills to those who are participating in this
workshop. We hope that this workshop will further help all people in both rural
and urban areas who suffer from this problem to have the advantage of improved
treatment from the doctors who are attending this workshop.
In this context, we express our gratitude to the team members of ŒCirujanos
Plástikos Mundi‚ who have travelled thousands of miles to give their valuable time
and knowledge for the benefit of rural people.
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I also express our sincere thanks to the doctors and other professionals who have
come here to attend this workshop despite their busy schedules.
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Acknowledgements
The planning and organizing of an International Cleft Surgery Workshop requires
the diligent expertise and efforts of many individuals.
We would like to extend our gratitude and appreciation to all individuals involved in
organizing and coordinating this Workshop. A special thanks to the Vicente Ferrer
Foundation for offering the opportunity and the space to perform this First East
Indian International Cleft Surgery Workshop.
Finally, we thank our outstanding Faculty for their conscientious effort in taking
time out of their busy schedules and personal lives to share their knowledge. We also
thank you, the participant, and hope you have found the workshop intellectually
stimulating and clinically useful. We hope this is just the beginning of many
workshops here at the Vicente Ferrer Foundation.
Fco. Javier Beut Cabrera
President & Founder
Cirujanos PlástiKos Mundi
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Introduction
HISTORY
390 A.D. First recorded lip repair by unidentified Chinese physician
1561 Pane Obturation of palatal defect
1764 Le Monnier First palatorrhaphy
1843 Malgaigne Lip closure by local flaps
Von Langenbeck Bilateral relaxing incisions; first anatomical repair
1884 Hagedorn Z-plasty and rectangular flap
1952 Tennison Triangle flap
1958 Millard Rotation flap
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INCIDENCE/EPIDEMIOLOGY
Clefting in U.S. 1:750 births
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Asians
2.1:1000
Caucasians
1:1000
Blacks
0.41:1000
Males:Females
2:1
Isolated clefts
1:2000 without racial influence
Isolated palates
Female:Male
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2:1
Site of clefting
Left:Right:Bilateral 6:3:1
Most common on left
ETIOLOGY
Heredity, probably a dominant gene with limited penetrance, is the main etiologic
cause. Another theory is a combination of inheritance and multiple other factors.
There is no genetic connection between cleft lip + palate .
COUNSELING OF FAMILY
1. If a person has a cleft lip, his/her child faces a risk of 2%
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2. If a person with cleft lip already has a cleft lip child, the following child faces a
risk o f 14%
3. A non cleft parent with a cleft lip child faces a risk of 4% for the following child
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MORPHOGENESIS AND GENETICS
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“Failure of fusion of palatal shelves, septum, and primary palate, which normally
takes place between the 8th and 17th week of embryologic development.”
“The actual etiology (cause) of a cleft lip and/or palate is largely unknown, and yet
at the same time we know that there are many different things that can contribute to
or create a cleft.
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The face and facial structures are
formed out of three plates, each
migrating toward a meeting point in
the middle area of the face.
The facial structures of the
orbicularis muscle form the lip.
They are joined at the philitrum
lines.
“If you rub your finger above your
top lip, you will feel those two
ridges. Those are in fact the “cleft
scars” of a non cleft-affected
person.”
Those tissues naturally join by the
fourth week of pregnancy.
The palate is then formed out of the
structure that begins as the tongue and palate. Between the fourth and the eighth
weeks of gestation, the tongue drops down and the palatal segments then move from
the sides and toward the middle, fusing in the center.
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“Run your tongue across your hard palate from side to side and you will feel the
seam where the two sides fused.”
A cleft, therefore, is not something that is formed, so much as it is something that
does not form. Everyone began life with a cleft. For 699 out of 700 of us, the cleft
fuses before birth. For that one in 700, it fails to fuse. So we are not talking about
something that happens so much as something that fails to happen.
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Normal Process:
T
6 weeks
Maxillary process
Lateral nasal process
Median nasal process
These three processes join and fuse to form the primary palate
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Current thought is cleft will result with no mesodermal migration across site
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7 weeks
Median nasal process and maxillary process have fused creating upper lip and
anterior maxillary alveolus
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8 weeks
Complex totally fused and mesodermal migration completed
Tongue, which has been postured superiorly between lateral palatal shelves of
maxilla, moves inferiorly allowing palatal processes to grow toward midline and
fuse, form nasopalatine foramen to uvula
11 weeks
Total palatal closure
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Why does it fail to happen? Lots of theories, and lots of known reasons. If the
tongue, for instance, fails to drop down because the baby is tucked too tightly at that
critical time, the result will be a very wide, horseshoe-shaped cleft of the palate. If
the blood flow to through the placenta is disrupted at a critical time, the fusion may
not occur. Drugs, alcohol or medications may disrupt normal patterns of
development. Or some genetic code may simply dictate that it will not happen.
Women who smoke are twice as likely to give birth to a cleft-affected child. Women
who ingest large quantities of Vitamin A or low quantities of folic acid are more
likely to have children with cleft. A parent with a cleft has a minimum 5% chance of
passing the cleft along. A parent with an autosomal dominant genetic condition that
results in clefting will have a 50% chance of passing along the condition.
The human embryo has a recognisable face at around eight weeks, when it measures
28mm from head to rump. At this stage the nose and lips have already been formed,
probably as a result of cells migrating from the direction of the forehead and cheeks
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into the face. The primary palate is formed at the end of week seven, and results
from growth from the inner sides of the upper jaw towards the midline, and their
subsequent fusion. Fusion proceeds from front to back and is not completed until
the 11th week. The primary deformity in clefting of lip or palate occurs if this
process of fusion is not completed.
Cleft lip and palate occur among all races and has been recognised for many years.
In the general population, i.e. from couples with no history of clefts on either side of
the family, the incidence varies with populations, between 1/5000 and 1/1000 for
cleft lip and/or cleft palate, and about 1/2500 for cleft palate alone. Asians are at
higher risk than Caucasians or Blacks.
The causes of cleft lip and palate remains unclear, although there is agreement that
heredity plays a major role as a cause. The most recent surveys indicate that in some
families there may be just one change in one of the units of heredity, or genes;
however, cleft lip or palate in different families may be due to different changes, or
mutations, in different genes that have very similar effects in the development lip. In
these cases genetic risks should be reasonably easy to determine. In other families,
cleft lip may be due to the combined effects of more than one gene. Possibly more
than 3% of families. Lastly, animal models suggest that maternal exposure to
substances that alter the normal composition of the hereditary material during the
critical period of development cooperate with the genetic make up of the developing
embryo in producing the cleft lip. Manifestation of the trait would depend then, not
only on genetic make up, or genotype, but also on exposure to these unspecified
harmful substances called mutagens.
The genetics of cleft lip and/or cleft palate is complex to say the least, and an
accurate clinical diagnosis is essential before giving recurrence risk figures. The first
thing to be considered is that cleft palate as an isolated malformation behaves as an
entity distinct from cleft lip with or without cleft palate. One line of evidence
suggesting that this is the case comes from twin studies, one third to one half of
identical twins are concordant, i.e. if one shows clefting the other one shows it also,
although the degree of severity observed may be quite different between both twins,
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however, one twin with cleft lip and the other with cleft palate have never been
observed.
A second consideration is that cleft lip, with or without cleft palate, may occur as
one of a group of malformations in many syndromes of complex appearance. A
syndrome is a group of small abnormalities that are often seen together in children
of unusual appearance, giving them a resemblance or “family air” as it were. So cleft
lip and/or palate are often associated with other abnormalities, forming part of
several syndromes in a person of otherwise normal appearance fig 1). In some of
the syndromes where cleft lip and/or palate are seen, the other abnormalities may be
obvious and more threatening, as in the case of some chromosomal syndromes. It
could be worthwhile to make here some brief comments about chromosomes.
Chromosomes are like packages where the cell organises the hereditary material
when it is going to divide. These packages are normally found in an even number,
and can be matched in pairs, reflecting the fact that half of the hereditary material of
the cell comes from the father and the other half from the mother. There are 46
chromosomes (23 pairs) in the human cells, except in the germ cells where the
members of each pair have separated and are found now in “single dose”. Twenty
two of these are called “autosomes”, and they contain the information for many
varied traits, some of which will be noticeable in the offspring. For the purpose of
identification, these chromosomes are assigned a number, from 1 to 22, so that when
we refer to chromosome 6 for instance, we all know what chromosome we are
talking about. The remaining chromosome is called the sex chromosome, because
among other genes it contains the genes that determine the sex of the offspring.
Sperm carrying the Y sex chromosome is bound to produce a male offspring, while
sperm carrying the X sex chromosome will produce a female.
The chromosomal syndromes are associated with abnormalities in the distribution of
the chromosomes among the
germ cells, so that some of these
germ cells or gametes end up
having more hereditary material
than normal, and others less.
These events, when they
happen, cause very characteristic
abnormalities, easy to identify,
like for instance Downs
syndrome. In other cases where
cleft lip and/or palate is
observed, the accompanying
clinical features may be more
subtle and could go unnoticed
in a superficial examination. For
instance, in Van der Woude
syndrome the clefting of the
palate or upper lip is
accompanied by symmetrical
lumps or pits on the lower lip.
Van der Woude synfrome is
diagnosed in the presence of
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First East Indian International Cleft Surgery Workshop
cleft lip, or cleft palate, or both, together with the lower lip pits, and it is inherited
from one of the parents to the child with the probability of one in two.
In some families, clefting of the secondary palate or bifid uvula are inherited
following an “X-linked recessive” pattern, i.e. the problem is observed in 50% of the
male offspring of unaffected couples, where the mother is in most cases the carrier.
In these families, the daughters are hardly ever affected, but 50% of them on average
are carriers. In one of these families from Iceland the single gene has been mapped to
the long arm of chromosome X.
In its “pure form”, i.e. unaccompanied by any other malformation (the so called
non-syndromic cleft lip with or without cleft palate) it appears to be an autosome;
dominant in some families, where it has been located on the short arm of
chromosome 6. Other pedigrees show an autosome; recessive pattern of inheritance,
not mapped so far. A detailed family history may help to establish the mode of
segregation, and hence the recurrence risk factor. However, most families may not
show a pedigree congruent with the autosomal dominant model, and if other
diagnoses have been excluded, it would be proper to quote recurrence risk figures
based on empirical data compatible with a multifactorial model. The following table,
compiled by Peter harper, may be useful in these cases.
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GENETIC RISKS IN CLEFT LIP AND PALATE
RELATIONSHIP TO INDEX CASE
Siblings (overall risk)
Siblings (no other affected)
Siblings (2 affected siblings)
Siblings & affected parents
Children
Second degree relatives
Third degree relatives
General population
CLEFT LIP/PALATE
CLEFT PALATE
4.00%
2.20%
10.00%
10.00%
4.30%
0.60%
0.30%
0.10%
1.80%
—
8.00%
—
6.20%
—
—
0.04%
Notice that if it can be established that there are no other affected relatives, the risk
to siblings (2.2%) is less than the overall risk (4%). The higher figure should be used
if the history is unreliable or unavailable.
From a subjective point of view, it is essential to emphasize that cleft lip occurs as an
accident of nature, and not through any fault of any of the parents..
We can not but speculate on the reasons why the genetics of cleft lip and palate are
so frustrating. It could well be that we are looking at the wrong trait, that is to say, at
a trait that is too far removed from the error in the development of the embryo.
There are many processes which could play a role in the formation of the lip, and
each of these requires different key molecules to be made normally. Many genetic
mutations could alter the function of any of these molecules, and any of these could
lead to cleft lip. This model would explain the high incidence of cleft lip and the low
recurrence in the same family. But several other models are possible.
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In brief, when counselling a family with cleft lip and palate it is essential to ascertain
whether this is the only malformation (non-syndromic cleft lip) by examining the
patient in minute detail. Then a careful family history should be taken to ascertain
whether there is a recognisable pattern of segregation, i.e. whether it can be said that
in a particular family the clefting is inherited as a sex-linked or an autosomal trait,
etc., and if all of these have been excluded the figures given in the table above could
be mentioned. The therapeutic requirements and possibilities should be mentioned
in the same session, if possible with illustration of real life corrections.
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Fig 1
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CLEFT RELATED GENETIC SYNDROMES/DISEASES
The following table was extracted from a table on Embryogenesis by Dr. Harold C.
Slavkin; Director, National Institute of Dental & Craniofacial Research (NIDCR) :
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TYPE
(1)
GENE
SYMBOL
ECM
Collagen, COL11A2 6p21.3 120290 Stickler
184840 AD
type XI,
syndrome,
alpha-2
type II
chain
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GENE
SYMBOL
CHROMOSOMAL
LOCATION
OMIM
NUMBER
FOR
GENE
SYNDROME
OMIM INHERINUMTANCE
BER
(2)
FOR
SYNDROME
Osmed
215150 AR
syndrome
Shprintzen- 182212 AD
Goldberg
syndrome
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ECM
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Glypican GPC3
Xq26 300037 Simpson 312870 X
dysmorphia
syndrome
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DESCRIPTION OF
CRANIOFACIAL
FEATURES (3)
cleft palate, micrognathia, glossoptosis, severe myopia, flat facies,
dental anomalies,
deafness
saddle nose, cleft palate, progressive deafness
craniosynostosis, microcephaly, maxillary
and mandibular hypoplasia, palatal shelf soft
tissue hypertrophy,
cleft palate, prominant
nose, narrow palpebral
fissures
disproportionately large
head, coarse facies, large
protruding jaw, wide
nasal bridge, upturned
nasal tip, large mouth,
thickened lips, central
cleft of lower lip, midline groove of tongue
and inferior alveolar
ridge, enlarged tongue,
short neck
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First East Indian International Cleft Surgery Workshop
TYPE
(1)
GENE
SYMBOL
ENZ
Phenyla- PAH
lanine hydroxylase
IS
SEC
TM
Retinoblastoma-1
GENE
SYMBOL
RB1
Sonic
SHH
hedgehog
Fibroblast FGFR2
growth
factor
receptor-2
CHROMOSOMAL
LOCATION
OMIM
NUMBER
FOR
GENE
SYNDROME
OMIM INHERINUMTANCE
BER
(2)
FOR
SYNDROME
12q24.1 261600 Phenylke- 261600 AR
tonuria
13q14.1- 180200 Retinoblas- 180200 AD
q14.2
toma
7q36 600725 Holopros- 142945 AD
sencephaly,
type 3
10q26 176943 Crouzon 123500 AD
craniofacial
dysostosis
Jackson- 123150 AD
Weiss syndrome
Apert syn- 101200 AD
drome
Pfeiffer
101600 AD
syndrome
Beare-Ste- 123790 AD
venson cutis gyrata
syndrome
TM
Peroxiso- PXMP3
mal membrane protein-3
8q21.1 170993 Zellweger 170993 AD
syndrome-3
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DESCRIPTION OF
CRANIOFACIAL
FEATURES (3)
microcephaly, occasional
cleft palate, long simple
philtrum, thin upper lip,
flattened nasal bridge,
epicanthus, upturned
nose
cleft palate, high forehead,
prominent eyebrows,
broad nasal bridge, bulbous tip of the nose, large
mouth with thin upper
lip, long philtrum, prominent earlobes
cyclopia, ocular hypotelorism, proboscis, midface
hypoplasia, single nostril,
midline cleft upper lip,
premaxillary agenesis
craniosynostosis, parrotbeaked nose, short upper
lip, hypoplastic maxilla,
relative mandibular prognathism, shallow orbit
craniosynostosis, midfacial
hypoplasia
craniosynostosis, brachysphenocephalic acrocephaly, flat facies, high narrow
palate
mild craniosynostosis, flat
facies, acrocephaly
craniosynostosis, cloverleaf
skull, cleft palate or uvula,
craniofacial anomalies
high forehead, dolichoturricephaly, large fontanels, flat face, round face,
hypoplastic supraorbital
ridge, epicanthus, cleft
palate
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TYPE
(1)
GENE
SYMBOL
TM
Diastrophic DTDST
dysplasia
sulfate
transporter
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GENE
SYMBOL
CHROMOSOMAL
LOCATION
OMIM
NUMBER
FOR
GENE
SYNDROME
OMIM INHERINUMTANCE
BER
(2)
FOR
SYNDROME
5q32- 222600 Diastrophic 222600 AR
q33.1
dysplasia
hypertrophic auricular
cartilage, cleft palate, micrognathia
Neonatal 256050 AR
osseous
dysplasia I
micrognathia, cleft palate,
flat nasal bridge, mid-face
hypoplasia, neonatal
osseous dysplasia, lethal
chondrodysplasia
9q22.3 601309 Basal cell 109400 AD
nevus syndrome
(Gorlin
syndrome)
macrocephaly, broad facies, frontal and biparietal
bossing, mild mandibular
prognathism, odontogenic
keratocysts of jaws,
misshapen and/or carious
teeth, cleft lip and palate,
ectopic calcification of falx
cerebri
3p14.1- 156845 Waardenp12.3
burg syndrome,
type IIA
193510 AD
wide nasal bridge, short
philtrum, cleft lip or
palate, deafness
PallisterHall syndrome
146510 AD
short nose, flat nasal bridge, multiple buccal frenula, microglossia, micrognathia, cleft palate,
malformed ears
193500 AD
wide nasal bridge, short
philtrum, cleft lip or palate, occasional deafness,
dystopia canthorum
R
TM
Patched
PTC
TF
Microph- MITF
thalmiaassociated
transcription factor
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DESCRIPTION OF
CRANIOFACIAL
FEATURES (3)
TF
Paired box PAX3
homeotic
gene-3
2q35 193500 Waardenburg syndrome,
type I
TF
Sry (sexdetermining region Y)box 9
SOX9
17q24.3- 211970 Campome- 211970 AR
q25.1
lic dysplasia
small chondrocranium,
large neurocranium,
occasional platybasia,
cleft palate, retroglossia,
micrognathia, flat nasal
bridge, malformed ears
TF
Twist
TWIST
7p21 601622 Saethre- 101400 AD
Chotzen
syndrome
craniosynostosis, acrocephaly, brachycephaly,
flat facies, thin long pointed nose, cleft palate, cra-
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TYPE
(1)
UNK
GENE
SYMBOL
GENE
SYMBOL
CHROMOSOMAL
LOCATION
OMIM
NUMBER
FOR
GENE
SYNDROME
OMIM INHERINUMTANCE
BER
(2)
FOR
SYNDROME
DiGeorge CATCH22 22q11 188400 DiGeorge 188400 AD
syndrome
syndrome
chromosome region
Velocardio- 192430 AD
facial syndrome
UNK
Treacle
TCOF1
5q32- 154500 Treacher 154500 AD
q33.1
Collins
mandibulofacial dysostosis
DESCRIPTION OF
CRANIOFACIAL
FEATURES (3)
nial asymmetry, ptosis,
malformed ears
low-set ears, short ears,
small mouth, submucous
or overt palatal cleft, cleft
lip, bulbous nose, square
nasal tip, short philtrum,
micrognathia,
Pierre Robin syndrome,
cleft palate, small open
mouth, myopathic facies,
retrognathia, prominent
nose with squared-off
nasal tip
malar hypoplasia, cleft palate, mandibular hypoplasia, macrostomia, malformed ears, sensorineural
deafness, coloboma of
lower eyelid
NOTES:
1. CS; cytoskeletal protein, ECM; extracellular matrix protein; ENZ; enzyme, IS, intracellular
signalling protein, NP; nuclear protein, SEC; secretory protein, TM; transmembrane protein, TF;
transcription factor, UNK; unknown
2. AD; autosomal dominant, AR; autosomal recessive, X; X-linked, XD; X-linked dominant, XR; Xlinked recessive
3. The following description is only a summary of the craniofacial features of the diseases and
disorders. For detail information regarding defects in other affected tissues and organs, refer to the
Online Mendelian Inheritance in Man (OMIM) at http://www.ncbi.nlm.nih.gov
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Others Factors
•
•
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•
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Facial mesenchyme
Facial width
Persistent high tongue position during development
Inadequate shelf force
Enzimes
– Although cleft lip and palate is by far the most common major facial
malformation, the mechanisms underlying its pathogenesis are only now
beginning to be understood. In this regard, the pathogenesis of hypoxia-induced
cleft lip and palate has been extensively studied,1 with the most vulnerable aspect
of craniofacial development related to hypoxia appearing to be the morphogenetic
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movements of facial prominences. On this premise, it may be postulated that a
metabolic impairment in the facial mesenchyma might be the cause of the failure
of mesenchymal reinforcement of the facial processes in cleft lip and palate
fetuses. In a recent study, 2 to better evaluate the role of a possible topical
metabolic alteration in the pathogenesis of cleft lip, we have histochemically and
ultrastructurally evaluated mitochondrial functionality in the orbicularis oris
muscle obtained from unilateral cleft lip patients. Our findings, while consistent
with evidence excluding an inherent myopathy of orbicularis oris muscle, evinced
both an increased oxidative metabolism and pathologic aggregates of glycogen
and mitochondria. On this premise, it is intriguing to speculate that enzymic
anomalies, reflecting the
above-mentioned local
metabolic impairment, might
be detected in the amniotic
fluid in the presence of fetal
cleft lip and palate. Indeed, it
has been recently confirmed
in a rat model that several
enzymic anomalies may be
detected in the amniotic fluid
of cleft palate fetuses.3 Aims
of this study were to evaluate
whether it is possible to
determine, by means of
isoelectric focusing, an
enzymic differentiation in
human amniotic fluid and
whether the onset of fetal
cleft lip and palate is
accompanied by a pathologic
enzymatic differentiation
pattern in amniotic fluid
(Fetal Unilateral Cleft Lip and Palate: Detection of Enzymic Anomalies in the Amniotic Fluid
Edoardo Raposio, M.D., Ph.D., F.I.C.S.; Paola Panarese, M.D.; PierLuigi Santi, M.D.
PLASTIC & RECONSTRUCTIVE SURGERY 1999;103:391-394)
• Infections (rubella, toxoplasmosis)
• Growth hormone deficiency
• Drugs and vitamins
– Low levels of FOLIC ACID has definitely been linked with causing cleft lip and
palate. Cleft lip and/or cleft palate occurs in the developing fetus between the 4th
and 8th week. After it happens, nothing can change it.
There is a new nationwide (U.S.) push to increase folic acid intake to prevent
birth defects. Low folic acid levels cause other birth defects as well.
Since January 1, 1998, most grain products, including brad, pasta, flour and rice,
will be fortified with folic acid, a B vitamin that reduces the risk of spina bifida, .
. [listing several others]. (because of a new FDA ruling)
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Women of reproductive age are advised to consume at least 400 micrograms of
folic acid daily.
Foods naturally rich in folic acid include leafy dark green vegetables, citrus fruits
and juices, kidney and lima beans, and liver.
It is important to also take a daily multivitamin-mineral pill containing at least .4
milligrams of folic acid.
– Women who take excessive amounts of vitamin A early in pregnancy can cause
serious birth defects in their unborn children, according to a Boston University
School of Medicine study. Researchers found that the babies of women who daily
consumed more than 10,000 international units (IUs) of vitamin A from
supplements (nearly four times the recommended amount) were more likely to be
born with malformations of the head, face, heart and brain.
In addition to supplements, vitamin A is found in most animal foods and
especially large amounts in liver. A three-ounce serving, for example, may have
more than 30,000 IUs. Even if women took no supplements, those who frequently
ate liver could exceed safe vitamin-A levels. Beta carotene, which the body can
convert into vitamin A, is not associated with an increased birth-defect risk.
When taken correctly during pregnancy, vitamin A is an essential nutrient in the
baby’s development. But several national surveys suggest that two to five percent
of women of childbearing age may be consuming more than 10,000 IUs daily.
Given the Boston University study’s findings, co-author Lynn L. Moore
recommends that these women consult with their physicians before taking
vitamin-A supplements exceeding 8000 IUs
•
•
•
•
•
Steroids
Diazepam
Aminopterin
Anticonvulsants (- incidence 10X)
Smokers (2X incidence)
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CLASSIFICATION AND RECORDS
CRANIOFACIAL ANOMALIES
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Cleft Types (Veau)
Cleft Lip = the severity of cleft lip can range
from microform (very small defect)
to complete clefts, and be unilateral or bilateral.
• Cleft lip will usually result in minor deformity of the nose characterized by a
flattened nostril on the affected side and flaring of the base on the affected side.
• Cleft lip can involve the alveolus, in which case it has involved the primary
palate.
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• If there is a bilateral cleft of the lip, there may be extension of the premaxillary
segment.
• Cleft palate can occur with cleft lip or less often by itself.
• Some patients will present with submucous clefts where the mucosal lining of the
oral cavity roof is present without appropriate supportive bone and muscle
structure.
• Signs of submucous clefting include a bifid uvula, diastasis of soft palate
musculature (division of muscles along midline), and notch in hard palate.
The Veau classification system:
CLASS
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T
I
II
III
IV
DESCRIPTION
Soft palate only
Hard & soft palate to the incisive foramen
Complete unilateral of soft, hard, lip, & alveolar ridge
Complete bilateral of soft, hard, and/or lip and alveolar ridge
These descriptions can be modified with the words incomplete, right, left, one/third,
and so on.
The striped-Y classification system:
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SECTIONS
1&5
2&6
3&7
4&8
9 & 10
11
12
13
AFFECTED AREAS
Floor of nose on right & left sides
Lip
Alveolar ridges
Premaxilla to incisive foramen
Each half of the hard palate
Soft palate
Congenital velopharyngeal incompetence without obvious clefts
Protrusion of premaxilla
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Lip
Alveolus
Primary
palate
Foramen
incisivum
Vomer
The variation in clefts is considerable.
A good way to record a cleft lip is by
photography. A better way to record a
palatal cleft is to fill in the following
figure with stripes and dots.
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Spinae
Soft
pa late
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Fig. 2
Cleft palate
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Fig. 3
Left-sided unilateral complete
cleft lip and palate
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Fig. 4
Bilateral complete cleft lip
and palate
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Fig. 5
Bilateral-right incomplete, left
complete-cleft lip and primary
palate
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PHILOSOPHY
OF TREATMENT
AND TIMING
OF SURGERY
In palate surgery speech
is the main concern.
Early surgery, before one
year of age, gives better
speech results. No
additional risk of growth
disturbance has been
proven with surgery at
an early age. The
inhibited growth of the
middle face is mainly a
consequence of lip and
palate surgery. Minor
disturbance is inherent
in the cleft. In a child,
this inhibited growth is
masked and the full
truth will not be learnt
until your patient
reaches adult age. Tight
lips, aggressive
undermining and
handling of tissues, and early bone grafting will contribute to disturbance of
circulation and scarring, leading to diminished growth. Growth disturbance due to
lip and palate surgery produces pseudoprognatism, dishface . No single method of
palate closure has proven to give the ideal combination of good speech and
growth. A more important factor is the technique of the individual surgeon.
Be gentle!
The lip can be operated at any time between 3 months and 90 years!. However, the
psychological trauma of growing up with a cleft makes early surgery desirable. Also,
correction of the distorted nose in a unilateral cleft is best done together with the lip;
it will not be detrimental to growth. The chance of correction will otherwise be
missed. Patients DO NOT USUALLY come back in developing countries. We have
to do as much as we can.
Normally start with the lip surgery, then palate. Doing both in one session will pose
an added risk in a child that is often not in optimal condition anyhow. (sometimes
both operations are done in one session see the Mexican operation) Closing the lip
and creating continuity of muscle will mould the alveolar cleft of the hard palate
and make anterior palate closure easier.
Palatal closure in a child older than 2 years is difficult: hard tissues, no muscles
functions… He won’t speak intelligibly with his palate, and leakage of food and
fluids to the nose is present. (see Dr. Jobe’s lecture)
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PREOPERATIVE CONSIDERATIONS AND EXAMINATIONS
Only operate when:
> 10 HB > 10 weeks old > 10 pounds*
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(Randall advocated repair in first ten days of life, as soon as health permitted)
Do not operate:
• an anemic child,
• a malnourished child, or a child with upper respiratory tract infection, which is
more common in these children.
• Listen to the lungs and check temperature!!! Look in the ears and throat. And
remember:
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“The surgery of cleft lip and palate is elective”
FEEDING OF CLEFT PALATE CHILDREN
A cleft palate child in a developing country is often malnourished. The
mother needs advice and training to feed it properly and maintain
hygiene. Mortality is high among cleft palate infants.
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Fluid Intake Needed to Prevent Dehydration after and before surgery
• Bottle feed with a regular nipple. Enlarge nipple hole and remove one corner of
cross cut area.
• Remove bottle after 1-2 swallows so as not to overfill the oral cavity.
• Feeding takes longer and demands understanding and cleanliness.
Breast feeding is only sufficient for two weeks. After this, expressing milk and
supplement is necessary. Spoon feeding is an alternative to bottle feeding. It is often
necessary to admit the mother and the child until the feeding problem has been
solved and weight gain is satisfactory. A child with double cleft lip and palate is
especially vulnerable. Check weigh gain: + - 8 ounces a week.
* Fluid and Weight Measurement Conversions:
To convert weight (kg) or fluid (cc or ml) to pounds or ounces, use the following formulas.
To convert Kilograms to pounds: Multiply by 2.205
- 10 kg = 10 x 2.205 = 22.05 lbs.
cc’s stands for cubic centimeters.
ml’s stands for milliliters
One cc = one ml They are equivalent.
One teaspoon = 5 cc = 5 ml
One Tablespoon (Tbl) = 15 cc = 15 ml
One Tablespoon = 3 teaspoons
One ounce = 30 cc = 30 ml = 2 Tablespoons = 6 teaspoons
8 ounces (The common size for a formula bottle) = 240 cc = 240 ml
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After surgery, our babies go on something of a hunger strike. They refuse any fluid
intake at all. Dehydration becomes a concern, and often times, a striking baby ends
up back in the hospital for IV rehydration.
The basic formula use for essential or “maintenance” fluids are the following:
4 cc/hr/kg for each of the first 10 kg
2 cc/hr/kg for each kg between 10 and 20 kg
1 cc/hr/kg for each kg above 20 kg
T
This is actually an IV dosing schedule, but it would be applicable to oral intake as
well. Any fever or other reason to lose fluid (drains, bleeding, etc) can GREATLY
increase the amounts stated above.
R
One ounce of water is about 28 cubic centimeters (cc); 1 Kg is about 2.2 pounds.
Thus a baby who is 10 Kilograms is about a 22,5 pound baby. So the baby would
need: 4cc x 10 kg or 40 cc of fluids.
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WATCH OUT FOR DIARRHEA, VOMITING,
FEVER, EXCESSIVE SWEATING, AND ANY OTHER
CONDITION THAT WILL DRAW OFF MORE THAN
THE NORMAL AMOUNT OF FLUID. IN THOSE
CASES, FLUID INTAKE MUST INCREASE!
TEN WAYS TO ENHANCE YOUR CHILD’S SELF-IMAGE
1 Do not allow your child’s craniofacial condition to define him as an individual.
C
“My son has a cleft”. But he also has bright, sparkling eyes, a winsome personality,
an active imagination, and many more attributes. Some parents actually refer to
their child as “a cleft”. In reality, you have “a child”, and your child has a cleft.
T
2 Love your child unconditionally. Do not allow your child to think that your love
I
is dependent on anything he or she has done or can do. Do not let your child
think that love is dependent upon personal beauty. If a child feels that your love
is dependent on something, then losing that something can, in your child’s mind,
make you stop loving him or her.
3 Cultivate a home environment in which each person’s worth as an individual is
O
affirmed. Share feelings, experiences, etc. Enjoy life together as a whole family
unit.
4 Help children to experience good feelings about themselves. Instead of saying, “I
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feel so proud of you for that.” say, “Do you feel proud of yourself for that?” or
“How does that make you feel?”
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5 Provide a good example. Children must feel that it is not conceited to feel good
about themselves. Let your child know that you, in fact, feel good about who you
are. That gives him permission to feel good about who he is.
6 Cultivate friendships with many diverse people. Your child must be given the
opportunity to experience the notion that there is not a very narrow focus of
what is “acceptable”. The people in your child’s life should portray the rich
diversity that is available to us in this big, wonderful world. In recognizing a
wider band of what is “acceptable and positive”, your child will be more likely to
find himself within that band.
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7 Be aware of and tone down your own attitudes based on “looks-ism”. Do you
often point out people who are “good looking”, or who have flawless bodies? Do
you make negative comments about persons who are not beautiful? Do you
comment on the physical beauty of TV or movie personalities? Every time you do
that in your child’s presence, you are, in effect, saying to your child, “Physical
perfection is all that matters in this world”.
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8 Always point out positive attributes about others that do not involve the physical.
Rather than identifying people by race, hair color, height, weight, etc, try finding
some other way to describe a person. Maybe a person can be defined by
something he or she has done, or by some personality trait, or by a particular
talent.
D
9 Encourage your child’s autonomy. Give your child the freedom to make
appropriate decisions, take appropriate risks, and foster a sense of competency.
Let your child’s own accomplishments give him a sense of worth and personal
value.
U
10 Join a support group. Let your child know that he or she is not the only child in
C
the world who was born with a craniofacial condition, and that others with the
same condition are lovable, likable people as well. A support group will also give
your child access to kindred spirits with whom he or she may discuss some of the
issues that only another who has “been there” can truly understand.
T
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Preliminaries to cleft surgery:
Anesthesia and equipment
(basics)
Cleft lip and palate surgery is facilitated and blood loss kept low if all areas to be
incised and undermined are injected with a 1: 200 000 adrenaline and local
anesthetic solution prior to surgery. In addition, local anesthesia allows the
anesthesiologist to keep the patient ‘lighter’ during surgery.
Always alert anesthesiologist before injecting ‘jungle juice’ (adrenaline). Inject with a
small needle to distribute the solution evenly. Inject and then wait 5 minutes by the
clock to get maximal vasoconstrition. It is advantageous to mix your own solution as
ready mixed lidocaine with adrenaline are often outdated or have been stored at too
high a temperature.
RECIPE JUNGLE-JUICE
80 ml normal saline
20 ml lidocaine 1%
0,5 ml 1:1000 adrenaline
———————————
100 ml 1:200 000 adrenaline
0,2% lidocaine
After preparing it, divide the 100 ml jungle juice into several containers, one for
each patient. Beware of AIDS HIV.
When used together with halothane, the maximum dose of adrenaline is 5 - 10
microgram/kg. This corresponds to 1-2 ml/kg when jungle juice is used. A 3kg child
can have only 3-6 ml of jungle juice. Insufficient ventilation and a high PaCO2
lowers threshold of arrythmia.
In grown ups, jungle juice gives sufficient anesthesia to be able to operate without
using general anesthesia. If you are a beginner, start your surgery on grown ups with
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incomplete unilateral
lips. Bilateral cleft lips
always bleed more.
When your lip surgery is
fast and secure and you
are able to control
bleeding it is possible to
operate cleft lips under
ketamine anesthesia.
KETAMINE
ANESTHESIA
The child be positioned
with a pillow under the
shoulders. The neck
should be extended. The
operator uses hands to
advance mandible and
keep airway free in
between. Suction is used
freery to crear airway
from blood and mucous.
Premedicate with valium
and atropine 1/2 hour
pre-op. Ketamine is given 5-10 mg/kg bodyweight in a single intramuscular
injection; 1/2 of this dose could be repeated after 30 minutes. Intubation should be
available. Mouth to mouth breathing always is. It is better to perform ketamine
surgery from 6 months of age as ketamine often gives insufficient anesthesia in
smaller babies and even a maximum dose leaves you with a very mobile patient.
With increasing age ewen lower doses 2,5-5 mg/kg body weight is sufficient to give
relaxation. Valium can be repeated at the end of surgery, which should not exceed 45
minutes. The risk is apnea and a taped stethoscope should be used to monitor the
child.
GENERAL ANESTHESIA
Cleft palate surgery should be performed only with endotracheal intubation. Cleft
palate surgery or lip surgery with intubation can only be performed when you have
an experienced anesthesiologist and proper equipment: tubes of all sizes 3 - 3,5 - 4 4,5 - 5 - 5,5 - 6 precurved are used. Refer to figure 9. Use a circuit with limited dead
space when dealing with infants. Cleft palate surgery is elective and should not be
performed if these conditions are not met.
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Patients of all ages are operated. The older patient often has teeth. Padding between
the teeth and tube in the form of a gauze help to keep the airway open. Gauze or
packing mixed with some lidocaine kept to prevent leakage from tube and to collect
blood and mucous shold be kept long and protrude outside the oral opening.
Forgotten gauzes have caused deaths in palate surgery. Postoperative, a long tied
suture through the tip of the tongue can help to keep a free airway.
The surgeon should not leave the theatre until patient breathes properly. Possible
airway problems that may occur during surgery are clogging of the tube by mucous,
tube into one bronchus, dislocation of tube, or tube in oesophagus. The surgeon
sould observe and report if blood gets darker. Watch out for hypothermia. Cover
patient properly in cold room.
EQUIPMENT FOR LIP AND PALATE SURGERY (AT LEAST)
2 knife handles 3
1 needle holder like Crile-Murray
1 Kilner scissors straight
1 Kilner scissors curved blunt
8 Halstead mosquito artery forceps curved
2 Gillie’s skin hooks
1 Adson dissecting forceps
1 suture scissors
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EQUIPMENT FOR PALATE SURGERY
1 Kilner-dott mouth gag (you can cut tongue blades smaller if they are too
large for early surgery)
3 cleft palate sharp hook and rasparatory (different sizes)
2 cleft palate rasparatory (hockey-sticks)
1 Metzenbaum or McIndoe scissors
1 Gillie’s dissecting forceps toothed
2 knife handles 5
1 needleholder Crile-Murray
2 Gillie’s skin hooks
1 long handled suture scissors
1 digman mouth separator
DISPOSABLES
jungle-juice
syringe 2ml or 5ml
small needles
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sterilized tooth picks for marking
15-blades
11-blades
3/0 4/0 5/0 absorbable material like , Dexon, , PDS on cutting needles, 5/8
curve 3/0 + 4/0 is ideal for palate
5/0 6/0 monofilament nylon cutting needle for skin or lip. Or vicryl rapid
(you won’t need to remove steaches)
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RECIPE FOR BONNEY’S BLUE MARKER
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ANATOMY OF NOSE AND LIP
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I
II
III
IV
Brilliant green 1/2 g
Chrystal violet 1/2 g MIX
Alcohol 96% 46 ml
Water for injection 53ml add.
After making your design and tattooing the important landmarks, you can inject the
whole area with jungle juice. WAIT 5 MINUTES!! It also helps to grip lip firmly
between thumb and index finger to avoid bleeding while incising
1
2
3
4
5
6
7
8
12
14
13
9
10
11
16
15
Surface anatomy on a normal nose and lip
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2
3
4
5
6
7
8
10
11
12
14
13
16
9
15
Surface anatomy of a cleft nose and lip
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
Procerus m.
Orbicularis owli m.
Levator labii superioris aleque
Nasalis
Levator labii superioris
Zygomaticus minor
Levator anguli oris
Zigumaticus major m.
Orbicularis oris m .
Risorius m.
Depresor anguli ori
Nasalis alar part
Depresor septi
Platysma
Depressor labii inferioris
Mentalis
Anatomic landmarks
• Width of cleft is impressive, but vertical height between cleft and non-cleft side is
most important.
• Principle is to lengthen cleft side of lip to equal vertical height on non-cleft side
• Key elements: midpoint cupid’s bow and peaks of cupid’s bow
• Obicularis oris muscle bundles parallel cleft margins and insert abnormally into
skin and mucosa
• Nasal septum deviated to the side of cleft and base is on no-cleft side
• Alar base widely flared
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Unilateral lip closure:
Millard method
MARKINGS
Dry skin before marking.
Medial Element
• Point (1) is the midpoint of lip and cupids bow.
• (2) is the non cleft end of cupids bow on the myocutaneus ridge (white roll).
• (3) is a point on white roll with a distance the same as (1) -> (2). Measure with
eye or caliper. This point is important and fixed!! It should be tattooed into skin
so that it will not get lost. (Deep needle into bonney’s blue; puncture white roll.)
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• (4) is a point below the non cleft nostril.
• (5) is non cleft side columella. Mark it with tatoo.
• (6) is non cleft comisure
Lateral Element
• (7) is the cleft comisure The distance from (7) to (8) should equal the distances
between (3) to (4), (4) to (5), and (5) to (6). (When you get more experienced, it
becomes easier to judge.) Tatoo some points along white roll lateral to (7).
Actually all lips have a red and white lip mucosa on vermilion..
• (8) and (9) are points to form the upper edge of the advancing cleft side (9) is the
point where ala joins lip. (8) is on the junction of skin and mucosa.
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Millard’s Method
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Now measure the followings distances :
R
1. 2 to 5
2. 3 to 5 to x (=new 8 to 9)
3. 2 to 6 = 8 to 7
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Medial Element
U
Draw straight-convex line from between (3) and (5) following the non cleft side
columella and complete the distance with the back cut (W). DON’T CROSS THE
CUPIDS BOW LINE
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Draw a line 90º 2 to 3 mm cranially to point 3. The length of this line should equal
the thickness of the lip. You can always add a small Z plasty to better fit with the
lateral side
Draw a line along the mucocutaneous junction from point 3 to the internal base of
septal mucosa, becoming FLAP M. This flap will be rectangular +/- and it’s very
important to bare l raw edges to repair the internal aspect of the lip and the floor of
the nostril. (in conjunction with the lateral L-FLAP)
The mucosa must be freed further to join the lip. Frenulum on the medial side
should be cut througth
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Draw a line from point 3 into the internal lateral aspect of columella and continue
like a marginal excision to deliver the lower lateral cartilages. This flap will elongate
the columella and help to relocated the distorted lateral alar cartilages, freeing them
up from the skin and mucosa
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Lateral element
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Remember:
R
2 to 6 = 7 to 8
8 to 9 = 3 to 5 to x
2 to 4 = 8 to 10
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Draw a line along mucocutaneous junction from point (7) as far as possible into
nose without incorporating a ‘dipping’ area. A straight line is made from (8) to (9)
on lower side of cleft nostril sill. It is easier to identify nostril sill if you push nose
medially with a finger. Mucosal L-FLAP will be rectangular flap to fill and to raw
edges and repair lip. Width of L-FLAP corresponds to thickness of lip. Save some
mucosa along the edge of lip cranial to point 8) and 9), on lateral side, 2-3 mm.
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INCISIONS
You can start from the medial or lateral aspect. Our suggestion is to begin from the
medial side. The main reason is the visualization of the lengthening of the lip
Medial
Hold with your hand the lip, a skin hook pull the lip down from point 3.
incise from 3 to 5 to W through skin with 15 or 11 blade. Also incise the line along
mucocutaneous ridge into lateral columella to form
C-FLAP. The mucosa has to be freed further to joint the lip. Frenulum on medial
side should be cut through
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Turn lip up towards you, and incise mucosa to form M-flap.
Don’t forget to cut along the mucosa through frenulum to allow rotation of medial
element. Grip medial element firmly between thumb and index to allow secure
incisions and always use a support for the hand holding the knife. Switch to 11blade. Cut free a thin rectangular M-flap while assistant gives traction on tip of flap.
Try to get base of flap slightly thicker to deep circulation intact.
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Lateral
Pack raw edges of medial element in wet saline gauze. Grip lateral lip between
fingers. While turning lip towards you, incise 8 to 9 and also incise the line along
mucocutaneous ridge into the lateral aspect of the piriform aperture L-flap . Use 15
and 11 blade.
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Skin hook gives traction on mucosal L-flap, cut it thinly all the way down to
maxilla, with base slightly thicker. Rectangular flap! Don’t forget to save mucosa
cranial to point 8) in the form of rectangle with the same width as L-flap. Use 11
blade.
In a complete cleft, free nose from maxilla. Save base of L-FLAP .Incise the line
between inner lining of nostril and the more shining mucosa further up while
assistant holds hook to expose this area. You are cutting along the pyriform opening.
The incision is usually 8-10 mm long. Use 15 blade. This will allow you to
reconstruct nasal floor and rotate nostril and ala medially.
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Free lateral lip mucosa from maxilla laterally to L flap. A back cut should be made
on lateral side after freeing mucosa from maxilla. Incise mucosa in the buccal sulcus
up to the opening of the parotid duct. This incision is necessary in complete and
incomplete clefts. Make this back cut in front of the opening of parotid duct. Make
a large back cut in a wide cleft lip.
In incomplete lips, this incision and saving of M and L is not ALWAYS necessary.
Cut line from points 9 to 10 from inside of lip, saving as much muscle as possible.
Free the lateral element from maxilla point 9 to 10 in a complete cleft. Pack this area
with wet gauze. Clamp bleeders. Tie or sizzle larger ones.. M and L flap can be
discarded in most incomplete cleft lips! An incomplete cleft does not need wide
undermining, but always free abnormal insertion of muscle under ala into pyriform
opening.
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Free the muscle from skin and mucosa. Do this all around for about 2mm with 15
or 11 blade. The tip of the lateral element should be freed for some 4mm allow a
generous leading bulk of muscle.
Also free vermilion with a horizontal cut at level of white roll.
Area of skin is freed medially.
IF nose seems distorted, as is always the case in a complete cleft, free lower lateral
cartilage from skin and other cartilage before stitching lip together. A small curved
scissors is inserted from mid point columella and later from lateral side, freeing the
cartilage from skin up on the nasion (striped area). Also free dome of normal
cartilage. If the cartilage is distorted, freeing has to be done radically.
C
SUTURING
L
1. C-FLAP has already been insert to length the columella
O
S
2. Pull L-FLAP back to feel the gap of piriform aperture and medially to attach it to
the M-FLAP over the gap between alveoli, raw side up, with some absorbables
sutures 4/0 5/0 .
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3. Key stitch into back cut of the medial element. It’s important to check that when
you pull this stitch the columella doesn’t move laterally. So a bit of periosteum and
muscle is preferable. Use a double armed dexon or vicryl suture from the muscle
pennant lateral element and tie the suture. This is a landmark stitch to obtain
balance and symmetry of the lip.
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4. continue down muscle suture with inverted 4/0
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5. Key sticht into lateral and medial vermilion (white roll point 3 to 8). This is a
landmark stitch to obtain balance and symmetry of the lip
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6. turn lip and join lateral and medial mucosa.
U
7. Remove all excess of musosa from lateral, spare muscle. Spend some extra time at
this point. Sometimes you’ll need a small Z-plasty to fit the anterior mucosa.
Remenber any irregularities at this point are visible.
N
Suture the skin of the lateral to the medial cupid’s bow giving some extra bulging.
This will creates the illusion of a Join skin edges with 6/0 nylon or 5/0 rapid
absorbles sutures without tension.
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NOSE:
1. Suture dome to dome to create better symmetry
E
2. Suture the skin edges with 4/0 absoble suture.
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3. Lift lateral cartilage with stitches tied over small rolls of gauze. This will maintain
the position of the cartilages.
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4. A small piece of silicone will help to maintain the airway clean,
Keep the wound clean!
No tension or protection is apply over the lip.
To prevent damage from the child, splint the elbow joint gently.
If avaible use Logan’s bow
Remove all nylon stitches after one week,
including the ones tied over gauzes.
Postoperatively, use an ointment and clean gently
with saline+ H20/02 to protect against formation
of crusts and delayed healing.
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In summary keep in mind these four components of the lip and nose repair
1. Rotate down Cupid’s bow and fill secondary defect.
2. Primary nasal repair
3. Correct lateral lip and alar base position
4. Repair the lip
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Bilateral cleft lip
Complete bilateral lips have no muscle in prolabium. This has to be brought in
from lateral elements.
MARKINGS:
Dry skin before marking.
Medial element:
• Mark point (1) at the midpoint of the prolabium on the mucocutaneous junction.
• Using the calliper, measures a distance of 2mm. from point (1) to (2) and (1) to
(3). This will become the future cupid.
• Points (4) and (5) are the base of columella.
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Lateral element:
• Points (6) and (7) laterally to white roll where you can see (in case) a triangle of
white vermilion and Points (8) and (9) mucocutaneous junction.
• Points (10) and (11) are the base of the ala.
L
Now measure the followings distances :
A
T
1. Points (1) to (2) to (3) are the same. No more than 2mm
2. Points (2) to (4) = (6) to (8).
3. Points (3) to (5) = (7) to (9)
4. Points (1) to (2) to (3) are = to turn downs flaps
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INCISIONS AND DISSECTION
A
Inject with jungle juice all areas to be incised and undermined. Wait 5 minutes. You
can start either prolabium or lateral. Grab between fingers.
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Incise (1) to (2) to (3) trough skin, and (2) to (4) and (3) to (5), as far as possible
into nose creating the M-FLAP (like in unilateral page 26)
• Dissect the mucosa away from the prolabium and premaxilla
• Take the thin mucosal flap
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Incise (6) to (8) and (7) to (9).. You are creating the followings flaps:
• Small two lateral turn down flaps will create the bottown of central vermillion
• Draw a line along mucocutaneous junction from point (6) to (8) and (7) to (9) in
the other side, as far as possible into nose. You are creating the L-FLAP (like in
unilateral).
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Raise bilaterally two mucosal L-FLAPS, thin and rectangular, down to maxilla. Base
should be slightly thicker. Assistant holding hook into ala will help you. Cut lateral
element free along maxilla up to the opening of the parotid duct. Back cut should be
very large in a wide cleft! Save base of L-FLAP.
Free inside nose along pyriform opening to be able to construct nasal floor and
advance the sill medially. Keep base of L-FLAP intact!
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Now undermine an area similar to that of a unilateral lip. Cut line (8) to (10) and
(9) to (11) from inside, saving muscle for tip of lateral element flap (). With a protruding premaxilla you will often have to continue along (8) to (10) with scissors,
freeing orbicularis from the mimic muscles to enable you to close the lip.
Pack all raw areas with gauze. Clamp bleeders. Continue the dissection of the prolabium at the membranous septum in continuity with the prolabium flap. Care is
take to bring the medial crura going up with the flap. If you dissect subcutaneously
you will decrease blood supply to the tip. The branches of the colummellar arteries
follow a subcutaneous plane.
You are doing a retrograde dissection to obtain whatever you want to do at the
medial, middle crura and lateral genu.
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Retrograde dissection
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Blood supply upper lip and nose
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• F.A: Facial artery
• S.L.A. Superior labial artery
• L.N.A: Lateral nasal artery
• I.A.B : Inferior alar branch
• S.A.B: Superior alar branch
• A.A: Angular artery.
• I.O.A: Infraorbital artery
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D: deep ascending branch
S: superficial ascending branch
I.A: inferior alar branch
S.A: sphenopalatine artery
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SUTURING
You should now be ready to put it all together. In a bilateral cleft, stitch both sides
simultaneously.
Start constructing a bridge to close the nasal floor
• L-FLAP fills the piryform aperture and join into M-FLAP ( + S-FLAP or septal
continuity flap) raw side up.. Use 4/0 absorbable sutures.
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• Advance lateral elements into midline and suture with the advanced thin mucosal
flap .Suture to the periosteum at the desire height of the gingivobuccal sulcus, setting the height of the sulcus and covering the bone with this thin mucosal flap.
Stitch with 3/0 or 4/0 absorbable sutures, moving to and from left to right.
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• Now join muscle with 3/0 absorbable, attaching the pennants to PERIOSTEUM
at the region of the nasal spine . If it is hard to reach, free muscle further out
along cheek in a continuation of line (8) to (10) and (9) to (11), detaching the
orbicularis from the levators of the lip. See unilateral lip
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• Muscle is joined with inverted 3/0 or 4/0 absorbable sutures. You can make a
central cut in philtrum from raw side and attach a stitch from this cut to the
muscle to create a philtrum dimple.
• Dissect througth retrograde approach the tip of the nose, without removing the
fat, obtain domes simetries and suture the foot of the medial crura at the base
region of the nasal spine. Flip philtrum down and tie the stitch. Make some subcutaneous stitches to adjust skin edges of lateral element to philtrum.
• Then suture with 6/0 nylon or vicryl rapid .You may need to excise a cranial edge
of skin from lateral elements if you have a surplus.The flaps may need trimming
to fit perfectly. Remove excess mucosa carefully. Spare muscle
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In summary.
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Keep the wound clean !
No tension or protection is apply over the lip.
To prevent damage from the child, splint the elbow joint gently.
If avaible use Logan’s bow
Remove all nylon stitches after one week,
including the ones tied over gauzes.
Postoperatively, use an ointment and clean gently with
saline+ H20/02 to protect against formation
of crusts and delayed healing.
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Flaps involved in unilateral and bilateral cleft lip reconstruction
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•
•
•
•
•
•
•
C-FLAP , columella advancement flap
M-FLAP, medial mucosal flap
S-FLAP, septal flap. It continues from M-FLAP. Sometimes it’s called M-FLAP
L-FLAP, lateral mucosal flap
Premaxilla mucosa flap,
Prolabial mucosa flap (central vermillion)
Prolabium skin flap (retrograde approach)
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Palate closure
Main objectives are :
• Complete closure of the entire palate cleft in a single operation.
• Construction of an adequately functioning soft palate
Palatoplasty should be performed on children at early age between 6-18 months.
This is important to the early speech development.
Anatomy:
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Anatomy of mouth and landmarks:
A
6
6
5
7
4
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9
3
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11
2
11
12
1
5
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4
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1 Palatopharyngeus m.
2 Pterygomandibular raphe and
superior constrictor m.
3 Palatoglossus m.
4 Horizontal plate of palatine bone
5 Palatine process of maxilla
6 Incise folla & foramen
Greater palatine nerve and vessels
Greater palatine foramen
Posterior nasal dine
Pterygoid hamulus & tensor veli
palatine
11 Levator veli palatini m.
12 Uvulae m.
4 muscles groups
• 10 Tensor veli palatini (TVP)
Sphenoid bone and membranous wall of eustachian tube
Slings around hanulus and fuses with contralateral TVP in midline aponeurosis
Function-stiffens soft palate and opens eustachian tube
Innervation-Cranial nerve V3
• 11 Levator veli palatini (LVP)
Temporal bone and eustachian tube inserts into soft palate posterior to TVP
Function-elevates soft palate in speech and swallowing
Innervation-Cranial nerve IX and X
• 12 Uvulus
Arises from palatal aponeurosis and posterior nasal spine with vertical fibers
to the tip
Function-elevates uvula
Innervation-Cranial nerve IX and X
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• 1 Palatopharyngeus
Two origins on soft palate
Anterior inferior joining uvulus
Superior posterior attaching to mucosa on nasal side of palate
Forms arch of soft palate. Inserts in pharyngeal wall and posterior rim
of thyroid cartilage
Function-narrow and seal nasal pharynx
Innervation-Cranial nerve IX and X
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Neurovascular bundle
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Greater palatine nerves and vessels, at the foramen.
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PALATE CLOSURE:
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Insert tongue retractor with tongue in midline position. Inject generously with
jungle juice prior to surgery. Wait five minutes!
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Incisions and dissection
Palatoplasty is done under general anesthesia with an endotracheal tube positioned at
midline.
Local anesthesia 0,5 lidocaine to 1/200.000 epinephrine and some controlled
hypotension minime blood loss.
Trendelenburg position obviates the need for a throat pack, but we recommend to
do it anyway.
A Dingman mouth gag is recommended: it provides excellent exposure of the
operating field.
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Principles :
1. Palatoplasty is a given techniche
2. Complete closure of the entire palate cleft in a single operation if possible
3. Construction of an adequate functioning soft palate. Soft palate is incised to
produce three distinct layers: nasal, oral mucosa, and muscle
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Rules in any palatoplasty :
1. Should be performed at early age (6/18 months). Bardach and Salyer recommend
when the soft palate is only affected it can be performed at 10/12 months
(always possible to close the cleft without leaving bare bone exposed)
2. Single stage Two- flaps palatoplasty is the surgery of choice (with all the differents
variations).
3. Two-layers closure of the entire hard palate with approximation of the oral and
nasal layers to avoid dead space between them, securing the position of the
mucoperiosteal flaps on the oral side.
4. Complete closure of the anterior palate to prevent oronasal fistulas.
5. The muscles of both sides must be dissected from the posterior edge of the hard
palate and from the nasal periosteum, reoriented, suturing together creating a
functional muscle sling
6. Approximation of mucoperiosteal flaps on the oral nasal side must be achieved
without tension. The amount of bone exposed lateral to the mucoperiosteal flaps
must be equal to or greater the witdh of the palatal cleft to achieve tension-free
closure.
Method:
We have selected, two procedures will be presented:
1. Cleft of the soft palate
2. Cleft of the soft and Hard palate
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1-A Cleft of the soft palate
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You need good light and suction. Make medial incision of soft palate slightly
towards oral side.
• Incisions are carried along a line between the oral and nasal mucosa: Nasal
mucosa is darker red
• Exposure of the soft palate musculature (avoid deep incisions into the muscle)
• Lateral incisions start 1 to 1.5 cm posterior to the maxillary tuberosity. They are
anterior (tuberosity) and medial (alveolar process
Steps
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1.
2.
3.
4.
5.
Incisions design
Raising the mucoperiosteal flaps
Left V-shaped area in the hard palate is left intact.
Mucoperiosteum on the nasal side is dissected from hard palate
Muscles dissection from post. Edge of the hard palate and neurovascular bundles
are dissected from mucoperiosteum flaps
6. Suturing the nasal layer
7. Muscles and the oral layer are sutured in place
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C
In a cleft that is not too wide, you incise only for a short distance around the
tuberosity of the maxilla.
A palate with marked conocavity towards oral cavity will close without tension, but
with a large dead space between nasal and oral layers. The medial incision is as usual.
Hockey stick palate rasparatories will facilitate freeing muco-periosteal flaps medially
from both sides.
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2-A Cleft of the hard and soft palate
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General steps:
1. Incision design
2. Raising the mucoperiosteals flaps
3. Muscles of the soft palate are dissected from the posterior edge of the hard palate
4. Closing the nasal layer. No bare bone is exposed
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2-B Cleft of the hard and soft palate with vomer attached at the midline
Steps :
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1. Incisions design
2. Mucoperiosteals flaps undermining from hard palate, vomer and ant. Portion of
the palate.
3. Closure of the nasal layer using flaps from the vomer.
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2-C Complete unilateral cleft palate
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Steps :
1. Incisons design
2. Mucoperiosteal flap from the hard palate (Woodson elevator)
3. Both flaps are dissected from the underlying bone.Identification of neurovascular
bundles and attachments of the muscles to post edge of soft palate
4. On the cleft side a flap is raised from the nasal side
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5. On the non-cleft side a flap is raised from the vomer. Both are used for nasal
closure.
6. Complete Muscles dissection from the post. edge of the bony palate on both sides
with the neurovascular bundle.
7. Nasal layer sutured in the area of the hard and soft palate.
8. Muscles of the soft palate are sutures together.
9. Mucoperiosteals flaps are sutured in place, using vertical mattress sutures in the
nasal layer (dead space)
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2-D Complete bilateral cleft palate
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In a bilateral palate, vomer flaps based cranially could be used. The longer lateral
incision is often necessary to close palate.
Steps:
1.
2.
3.
4.
5.
6.
7.
8.
9.
Incisons design –palate, premaxilla, lower edge of the septum.
Mucoperiosteals flaps raised from hard palate
Muscles freed from post. edge of the hard palate
partially dissection of neurovascular bundles.
Flaps elevation on both sides of the vomer and preamaxilla
Flaps elevation from the nasal side of the hard palate.
Closure of the nasal layer
Closure of the muscle and oral layers
Mattress sutures are used to closely approximate the oral and nasal layers, in the
area of hard palate.
10. Lateral areas of bare bone remain exposed.
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Suturing General rules
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1. Use inverted 4/0 absorbable dexon or vicryl stitches (with knot nasaly) to close
nasal layer. Start with small bite into muscle. Move posterior to uvula. Area
between soft and hard palate is difficult and a small tear could easily occur. Suture
this area last.
2. Use similar nasal closure on bilateral cleft palate. Two vomer flaps are used
3. Oral layer is closed with mattress sutures. Use 3/0 absorbable. You can also start
with a separate muscle stitch. Move to uvula; then continue anteriorly.
4. A fistula, large or small, is deliberately left anteriorly around alveolus. Your earlier
lip surgery will have narrowed the distance between alveoli. Wide palates will give
a large lateral raw area that closes by itself in three weeks. Don’t forget to remove
packs around tube. Tongue suture will help to keep airway free.
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Superiorly based pharyngeal flap
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Indication
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The indication for a pharyngeal flap in a civilized setting would be nasal escape as
diagnosed by the speech therapist. In the “bush”, a wide cleft palate in an older child
or grown up might be an indication for a primary pharyngeal flap during palatal
closure. Repaired patients that come back due to nasal speech is of course another
indication for a pharyngeal flap.
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PHARYNGEAL FLAP-PUSHBACK PROCEDURE
BY
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DR. RICHARD JOBE M.D.
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Perhaps 35 years ago when he was at Yale and I was starting a practice in Palo Alto,
Bob Chase and I were discussing a problem patient of mine over the phone, and we
came up with what we though was a marvellous idea – the pharyngeal flap-pushback
procedure. We did the procedure a few times on each side of the country. He moved
to Stanford and we asked David Dibell, then a resident, to put together a paper
describing our great idea.
In the research for a second paper giving results for many more patients, it was
found that C.A. Honig of the Netherlands had described the procedure in a thesis
and properly credited it to Sanvanero-Roseill, in Italy, who had presented the idea at
a meeting in 1954. We learned again to be careful about claiming credit. Good
minds often come up with the same good ideas when faced with the same problems.
Since that time, Chase has become a hand surgeon, a medical educator (which he
always was), and an anatomist and I have continued to use the Pharyngeal FlapPushback procedure4,5 when faced with patients who had mobile palates and the
need for a pharyngeal flap. The Cleft Palate Clinic (now Craniofacial Clinic) at
Stanford has never had a good data base. One is currently in development. As a
result, a careful statistical analysis of our results is not possible. The high frequency
with which patients have stopped having linguistic problems after the surgical
procedure has led us to continue our work without seeking alternative techniques,
although more recently I have become an enthusiast for Leonard Furlow’s double
opposing Z-plasty, and, where appropriate, I have added that technique to reduce
the circumference of the velopharyngeal sphincter, to elongate the soft palate, and
rearrange the muscles to a more normal posture and function.
The procedure of pushback with pharyngeal has been very effective in treating a
broad range of patients with velopharyngeal insufficiency (VPI) according to my
own experiences and those of other surgeons. The pharyngeal flap, which is put into
the nasal pushback raw area between the hard palate and the soft palate to keep the
velum from being pulled forward by scar contraction, is not expected to serve as an
obturator and should not block soft palate motion. Postoperative airway obstructive
problems have been encountered much less frequently than after standard
pharyngeal flaps. The operation should not be done for a patients with a paralyzed
palate. The procedure, which is not difficult to perform, is described below.
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Evaluation
In patients with velopharyngeal incompetence, in addition to the usual historical and
physical examination and appraisal of the prognosis by a speech-language
pathologist, the dynamic potential of the palate should be determined by more
objective means such as videofluoroscopic voice studies and nasopharyngoscopy. The
operation depends more on intrinsic palage motion than a pharyngeal falp procedure
that is designed primarily to obstruct the velopharynx in which valving depends on
motion of the lateral pharyngeal walls.
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The procedure
With Dingman or Dott gag in place and the patient under oral endotracheal
anesthesia, the palate and posterior pharynx are infiltrated with 0.5% lidocaine
containing l/200,000 epinephrine. A pharyngeal flap is designed using approximately
80% of the width of the posterior pharynx, extending up to a base at the top of the
nasopharynx. It is a little over 4cm long, perhaps lcm longer than the distance from
the posterior pharynx to the back of the hard palate. The longitudinal incisions are
first made with a knife and then deepened through the transverse fibers of the
superior constrictor to the prevertebral fascia with a blunt scissor, such as a
Metzenbaum. The flap is then elevated from the fascia by spreading the tissue with a
right angle scissors or clamp. When the flap has been elevated throughout its length,
it is divided inferiorly with the right angle scissor. The flap is then elevated to the base
of the skull, and bleeding is controlled in the donor area. A suture through the tip of
the flap, left long, will assist in retrieving the flap later. It is wise to elevate the flap
before doing anything more than injecting the palate, so that blood from the palate
will not obscure the development of the flap. The donor area need not be closed.
Once the flap has been developed, if any surgical correction of the soft palate is
planned, it should then be done, before the pushback, because it is easier to dissect
the soft palate when it is stable. In recent years, I have often done a Furlow Z-plasty
palate repair as an accompaniment to the pushback-pharyngeal flap procedure to
enhance the opportunity for significant improvement of the velopharyngeal
incompetence. The soft palate closure then is best postponed until the pushback is
completed and the pharyngeal flap attached, because insetting the flap into the
pushback defect is easier through the open palate.
Hard palate flaps are then raised. By now, the epinephrine can be expected to have
done its work. Dorrance flaps are used for incomplete clefts (Fig. 1), and Wardill
flaps for complete clefts (Fig. 2). The flaps are elevated posteriorly until the nasal
mucosa and muscle and/or palatal aponeurosis can be divided from the hard palate
and the soft palate pushed away. The pushback is aided by fracture of the hamulus
or release of the tensor tendon, stretching the greater palatine vessels, and lateral
dissection into the space of Ernst. The back of the palatine foramen can be resected
with an osteotome, if desired, although this is not often necessary to achieve and
adequate pushback. A centimeter of posterior displacement is not unusual.
Fig 1. Placement of the pharyngeal flap in combination with a Dorrance pushback.
(A) Palatal incision. (B) Suturing the flap into the raw area created by the pushback. (C)
Central through-and-through sutures hold the pharyngeal flap in place, lateral sutures
maintain mucoperiosteal flaps in pushed back position. (D) Lateral view of completed
operation. Note that flap covers raw area between back of bony palate and front of soft palate, not the
top of soft palate. Reprinted with persimission.
Unless something is done to maintain the retrodisplacement of the soft palate, it will
be lost to scar contracture in the healing process. Therefore, the pharyngeal flap is
then brought forward, over the dorsum of the velum through the defect of the
pushback, from the nasopharynx to the dorsal side of the hard palate
mucoperiosteum, which has been elevated and set back. If the soft palate has been
opened, it is easy to maneuver the pharyngeal flap into this position. If it has not,
pass a small Robinson catheter into the nasopharynx through the opening at the
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back of the hard palate beneath the elevated hard palate mucoperiosteum (Fig. 3).4
The end of the catheter is then retrieved from the pharynx and a suture, which has
been put into the tip of the pharyngeal flap, is passed through the holes in the
catheter. Withdrawing the catheter will bring the end of the pharyngeal flap into
contact with the underside of the retroposed hard palate mucoperiosteum where it is
secured by at least 3 sutures through the hard palate mucosa, in and out through the
flap, snubbing it against the overlying mucoperiosteum and back through the palate
mucosa, and tied in the mouth. Be certain that the flap has been advanced
sufficiently such that there is ample contact between the flap and the pushed-back
hard palate mucoperiosteum. This contact is often assisted by additional sutures,
placed to spread out the pharyngeal flap by suturing the corners of the flap to the
underside of the elevated hard palate mucosa. Chromic sutures (4-0) are used in
anchoring the flap.
At this point, the Wardill or Dorrance flaps may appear to be free, flapping from the
roof of the mouth. The tongue will soon correct this problem, but nurses and
parents are less concerned if several sutures are placed loosely to keep the flaps up
against the palatal bone.
If the soft palate has been opened, it should now be repaired, and the wounds
should be carefully inspected to control any bleeding, which concludes the
operation. At the end of the procedure, the mouth is rinsed with the
lidocaine/adrenalin solution to reduce immediate postoperative pain.
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Postoperative
After the procedure, there will be some blood staining of the saliva, which is likely to
drain externally more than expected in palate surgery because swallowing is
apparently quite uncomfortable at first. Usually on the day after surgery, the saliva is
clear and swallowing is easier. It may be a couple of days before the patient can take
enough fluid to stop the intravenous feeding, and be discharged. A stiff, painful neck
can be expected for a few days because of the proximity of the surgery to the cervical
spine. Otherwise, the early postoperative course is similar to other palate surgery. It
usually takes more than 2 weeks for all the wounds to heal. I require a liquid or
pureed diet for 3 weeks.
The hypernasality that justified the surgery is frequently replaced by denasality while
postoperative swelling is present. This denasality will disappear, and some of the
hyernasality will return after the swelling goes down. The occasional happy result is the
permanent loss of hypernasality, but more frequently some therapy is necessary. The
results of our x-ray studies have shown the palate to be thick and stiff up to a year after
surgery, so we believe that it is not desirable to start early speech therapy, because we
expect that there will be better progress after the palate is a bit more flexible
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Combining the pharyngeal flap push back with primary palate repair
We know that patients who have had palate repair after the development of speech
have a more difficult time correcting the hypernasality. Therefore, I have usually
done a pharyngeal flap/pushback along with the palate repair in older patients. I
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have used this combined procedure most frequently in the third world, where
followup is difficult, and where speech therapy is often unavailable. In the few
circumstances where I have performed this procedure in the United States, it has
seemed justified. I recall a 5-year old girl who had a flap constructed during her
palate repair. She had no suggestion of cleft palate speech immediately after the
wounds had healed.
Complications
The usual litany of complications in palate surgery apply. I can recall only one
occasion where it was necessary to take a patient back to surgery for bleeding. I
know of no deaths, and I have not seen patients harmed or their speech worsened as
a consequence of this procedure.
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Contraindications
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This operation should not be done on patients who have no motion in the soft
palate. Better results can be expected if, on videofluoroscopy, velar motion is
appropriate in direction and timing during speech.
Clinical impression
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Having done this operation more than 200 times, I believe that it can solve the
problem of hypernasal speech in any child who is intelligent and has appropriate
motion on videofluoroscopy. Having become an enthusiast for Furlow’s Z-plasty ()
that reduces the velopharyngeal diameter and circumference, corrects the muscle
abnormality, and lengthens the palate, and supported by recent reports of successful
correction of VPI after palate repair by this operation alone,7,8,9 I believe that I
would probably limit the use of the pharyngeal flap/pushback operation to those
who have failed after the Z-plasty, to those with gaps on videofluoroscopy in excess
of 4 mm, and particularly to those whose palates are centrally scarred and shortened
by surgery and its complications.
Fig 2.
(A) Beginning with a V-Y pushback. (B) Posterior through-and-through sutures hold
flap in place, anterior sutures close mucoperiosteal flaps. (C) Lateral view of completed
operation. Reprinted with permission.
Fig 3. A method of retreiving and position the flap. (A) Pharyngeal flap elevated,
pushback completed. Robinson catheter passed through pushback defect into
nasopharynx, retrieved from posterior pharynx and flap suture placed through catheter
holes. (B) Pharyngeal flap pulled into place by withdrawing catheter, through-andthrough suture placed. (C) Pharyngeal flap inset into the pushback defect between back
of hard palate and front of soft palate. Reprinted with permission.
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Further reading
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BARDACH, J. and SLAYER K. Surgical techniques in cleft lip and palate. Mosby Year
Book 1991.
HOGAN, V. A clarification of the surgical goals in cleft palate speech and the introduction
of the lateral port control pharyngeal flap. Cleft Palate J. 1973 vol. 10: 331
MCCOMB, H. Primary correction of unilateral cleft lip nasal deformity: a 10-year
review. Plast. Rec. Surgery 1985, Vol. 75: 791
MILLARD, R. Cleft craft: the evolution of its surgery, Vol I, II, III; Boston, Little,
Brown & Co. 1976
NOORDHOFF, M. Reconstruction of vermillion in unilateral and bilateral cleft lips.
Plast. Rec. Surgery 1984 vol. 73: 52
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DIBELL DG LAUB DR. JOBE RP, CHASE RA: A modification of the combined pushback
and pharyngeal flap operation. Plast Reconstr Surg 36:165, 1965
BUCHHOLZ RB, CHASE RA, JOBE RP, SMITH H: The use of the combined palatal
pushback and pharyngeal flap operation: A progress report. Plast Reconstr Surg
39:554-561, 1967
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HONIG CA: Secondary corrections of the palate, in Schuchardt K (ed). Treatment of
Patients with Clefts of Lip, Alveolus and Palate. Stuttgart, Germany, Thieme Verlag,
1966, pp 207-209
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JOBE R: The combined pharyngeal flap and palate pushback procedure: Improvements in
technique. Br J Plast Surg 26:384, 1973
A
OUSTERHOUT DK, JOBE R, CHASE RA: Combined Palate Pushback and Superiorly
Based Pharyngeal Flap. Transactions of the Fifth International Congress of Plastic
and Reconstructive Surgery. Sydney, Australia, Butterworths, 1971, pp 241-26
D
WEBER JC, CHASE RA, JOBE RP: The restrictive pharyngeal flap. Br J Plast Surg
23:347-351, 1970
I
CHEN PK, WU JTH, CHEN Y, NOORDHOFF MS: Correction of secondary
velopharyngeal insufficiency in cleft palate patients with the furlow palatoplasty. Plast
Reconstr Surg 94:933, 1994
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HUDSON DA, GROBBELAAR AD, FERNANDES KB, LENTIN R: Treatment of
velopharyngeal incompetence by the Furlow z-plasty. Ann Plast Surg 34:24-27, 1995
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FURLOW LT: Secondary cleft palate surgery, in Grotting JC (ed): Re-operative Aesthetic
and Reconstructive Plastic Surgery, vol 1. St Louis, MO, Quality Medical
Publishing, 1995, pp 799-846
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DIXON-WOOD VL, WILLIAMS WN, SEAGLE MB: Team acceptance of specific
recommendations for the treatment of velopharyngeal insufficiency as provided by
speech pathologists. Cleft Palate J 28:285, 1991
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DIXON VL, BZOCH KR, HABAL MB: Evaluation of speech after correc-tion of rhinolalia
with pushback palatoplasty combined with pharyngeal flap. Plast Reconstr Surg
64:77, 1979
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ALBERY FH, BENNETT JA, PIGGOTT RW, SIMMONS RM: The results of 100 operations
for velopharyngeal incompetence selected on the findings of endoscopic and radiological
examination. Br J Plast Surg 35:118, 1982
PEAT EG, ALBERY EH, JONES K, PIGGOTT RW: Tailoring velopharyngeal surgery: The
influence of etiology and type of operation. Plast Reconstr Surg 93:948, 1994
ADAMS-RAY WE, GRANSTROM B: Cleft lip and palate manual. Netherlands.
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Lectures
Dr. Richard Jobe
Professor of plastic surgery at Stanford University
WHAT DO WE DO WHEN THE REPAIRED
PALATE DOESN’T WORK
Occasionaly after a palate repair the palate still does not function correctly to
accomplish speech. The palate must be long enough to reach the back of the throat
to close off the nose. It must be mobile enough to do this quickly and intermittently
for correct function.
Sometimes a normal appearing palate lacks function as well. Some of these normal
looking palates are intact but have poor muscle positions. Others have palates too
short to reach across a large nasopharynx. Function can also be impaired after
adenoids have been removed, making the nasopharynx too large for the palate to
reach. Neurologic defects can also limit palate motion.
Often failure is a matter of lack of speech correction. We must be certain that this is
not the case before suggesting surgical correction. Where available, it is common
practice to send patients for a course of speech therapy, before advising surgery.
However, this is not necessary if the palate is clearly too short or moves poorly.
There are sophisticated ways to measure the function of the palate using flouroscopy
during speech and nasopharyngoscopy. A less sophisticated and easy approach can
also be to see if a patient can be taught to blow out of the mouth without air
escaping through the nose. If this is not possible, poor palate function might well be
the cause.
There are surgical procedures to correct these deficiencies. Surgically the deficiencies are:
palate too short
nasopharynx too large
poor muscle structure in the palate
immobile palate from deficient innervation
failure of previous surgery.
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The surgeon must select the best operation for the problem the patient presents.
Surgical corrections
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The first three of the following methods leave the palate looking relatively normal
and depend upon enhancing normal function.
The palate may be lengthened by use of a Z plasty or with a “push back” procedure.
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The palate can be opened as in repairing a cleft and the abnormally placed muscles
rearranged. This is done in th e correction of a submucous cleft palate
The nasopharynx can be reduced in depth by placing an implant or cartilage graft
behind the nasopharyngeal mucosa.
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In the more severe deficiencies abnormal situations are created which compensate for
the difficulty. The most common of these is a pharyngeal flap in which a vertical flap
of the posterior pharyngeal mucosa is attached to the back of the palate. This
partially obstructs the nasopharynx, reducing nasal escape, and divides the
nasopharynx into two spaces allowing the lateral pharyngeal musculature to help
close the nasopharynx. These can be narrow or wide. Wider flaps are particularly
useful where limited palate motion pre-exists.
There are a number of varieties of pharyngeal flap operations. For active palates,The
author prefers a superiorly based flap that holds the palate back done at the same
time as a push back procedure to lengthen the palate. This flap is high enough to
allow normal palate movement below it. For relatively inactive palates the flaps
should be wider and partially obstruct the nasopharynx.
Another popular approach is the pharyngoplasty which reduces the size of the
nasopharyngeal opening by taking flaps of muscle and mucosa from the sides of the
nasopharynx and turning them across the back of the nasopharynx. This creates a
bulge on the back of the nasopharynx, and the closure of the areas from which the
flaps were taken reduces the size of the orifice. The result is a much smaller orifice
which is closed appropriately by the rearranged muscles.
Patients who have had these procedures are generally significantly improved. They
will still have the abnormal speech mechanisms habituated and will require further
speech training. The surgical corrections make such training much easier and more
effective.
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Dra. Andrea Jobe
Executive Director and Director of Speech Programs / RSF-EARTHSPEAK
REHABILITATION IN PATIENTS
WITH CLEFT PALATE SPEECH
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Three Major Factors in the Rehabilitation of Cleft Palate Speech
The rehabilitation of cleft palate speech ideally requires an integrated, team approach
incorporating the professional efforts of:
• plastic and reconstructive surgery
• speech and language pathology
• dentististry and orthodontia
The impact a cleft palate has upon speech production cannot be overstated. A
number of the major organs of speech have interrupted function due to this
deformity. Even with surgical correction, speech may not proceed normally without
therapeutic help.
If surgical correction of the cleft lip and/or palate is done before 1 year of age, there
is a good likelihood that speech development will be normal. However, if such
correction occurs after 1 year of age or the age of speech onset, a significant number
of children may still require speech therapy in order to overcome their incorrect
method of sound production. Even with children who have had cleft lip and palate
repair before the onset of speech, as many as 25% of them may have the need for
some speech therapy
Historically, such correction has been problematic in developing nations. There are
many reasons for this: economic, geographical and availability of speech therapy
services. The conventional methods of speech therapy require that an individual be
brought to the professional’s office 2 to 3 times a week for individual or group
lessons that can last up to 1 hour. The fee can be high for such service where it is
available.
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Poor teeth alignment or missing teeth due to complete cleft lip and palate can also
contribute to the speech problem
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For the past 18 years, a method to provide speech correction for cleft palate speech
to patients who have no access to speech therapy has been sought by the individuals
of RSF-EARTHSPEAK. Exploration of ways that dental and orthodontic assistance
can be brought to these individuals is also being done.
Over the years a method called Corrective Babbling has been developed. This
method is delivered through educating parents and caregivers to become the speech
teachers their children need. This bypasses the geographical, economic and
availability barriers that currently exist. Corrective Babbling is a scientifically based
approach that uses what we know about the organs of speech, speech sound
production, developmental stages of speech learning, speech teaching and how the
problems of cleft lip and palate challenge normal speech development.
How Speech is effected by a Cleft Palate
There are no special organs devoted to speech in mankind. Organs that
are used for respiration and eating are adapted for speech use.
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Shortly after birth, children begin to use the organs of respiration and eating to learn
the speech sounds of their native language. This process begins at birth with the first
cry, proceeds through stages of “cooing” and proceeds on to babbling.
Crying begins the development of oral and nasal airflow distinction. Cooing forms
the motor basis for learning the vowel sounds and babbling teaches the consonants
and vowel combinations that later form words. This development proceeds and
intensifies until the child is about 1 year of age. At this time, the stored soundmotor patterns will then be further refined and employed in word development as
expressive language begins.
The developing child first learns the sounds of his native language by observing
sounds that he sees, feels and hears. He is constantly bathed in an environment of
these sounds as his mother and others “talk” to him
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Child— Sees
Feels Hear
About 4 months of age the
developing child begins to try to
imitate the sounds that he has
seen, felt and heard using his
own organs of speech.
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Child— Imitates what is seen,
felt and heard
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The mother and other speakers of
the child’s language reinforce this
constant cycle of practice until he
builds a stored sound-motorpattern in his brain for each
sound of his native language.
It is these stored sound-motor
patterns that will later be used
in different syllable combinations
to make up the words of the
child’s language.
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This cycle, external reinforcement and storage is what permits us to habituate sounds
and make them readily available to use when we begin speaking.
Child— sees a “cat” and says cat
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With or without a cleft palate this process occurs normally in all children and is
essential to speech development
Children with unrepaired cleft palates during this vital speech learning time will
engage in this cycle as well. However, these children are handicapped by the
inability to feel the sounds they see and hear or to reproduce them with intact
organs of speech. This in turn leads to the habituated storage of incorrect sound
motor patterns for each speech sound.
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Each sound has 4 characteristic features that make it different from each other
sound. These features are created by using the organs of speech in different
combinations. Each sound has its own sound-motor pattern just like a musical note.
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The 4 features of each sound are:
– Place (where in the oral or nasal cavity the sound is made)
– Manner (what the organs of speech do to the air flow coming from the lungs)
– Air Direction (whether the air exits from the nose or mouth)
– Voicing (whether the vocal cords vibrate or not)
Lacking an intact hard and/or soft palate, the child with a cleft will be unable to
create some of these features or combine them in a conventional way. He will adopt
incorrect ways of making each sound in his attempts to override the open palate.
This results in cleft palate speech. Speech that contains incorrect sound-motor
patterns and speech that is firmly habituated and resistant to change even after
surgical correction of the origin of the problem has been done.
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Effective speech therapy is needed to change this problem. RSF-EARTHSPEAK
teams are working as volunteers in developing nations to teach parents and others
about the nature of the cleft palate speech problem and how to correct it. Speech
seminars and weeklong speech camps to train parents in the Corrective Babbling
approach are held. This approach is meeting with success and can be an effective
tool in helping overcome the speech deficits of children born with cleft palate.
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Dr. Debra Johnson
Chief of Surgery for the Sutter Community Hospitals,
and Co-Director of the Sutter Cleft Lip and Palate Panel.
Member of the Board of Directors for Interplast, Inc.
THE GENETICS OF CLEFT LIP AND PALATE
Clefts occur in approximately 1:700 births.
1:750 births in Caucasians
1:500 births in Asians
1:1200 in Africans
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Clefts can occur as cleft lip alone, cleft lip/palate, or cleft palate alone
Cleft lip alone: 21% of clefts
Cleft lip/palate together: 46% of clefts
Cleft palate alone: 33% of clefts
Bilateral cleft lip is associated with a cleft palate in 86% of cases
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Clefts occur in boys more commonly than in girls
Ratio of cleft lip: left 6:right 3: bilateral 1
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Causes of clefting:
For most, no single factor can be identified as the cause.
Isolated clefts are those that have no other birth anomaly.
Syndromic clefts are those associated with other birth disorders.
Clefts are a feature of over 300 Syndromes, and most are rare.
More common syndromes: Pierre-Robin sequence, Crouzon,
Apert, Pfeiffer, Van der Woude, Treacher Collins,
Velocardiofacial
Syndromal clefts make up 15% of cleft lip +/- palate
50% of isolated cleft palate
75% of VPI are syndromic
Isolated clefts: caused by an interaction between an individual’s genes and certain
environmental factors (often impossible to identify). Phenytoin, Accutane, alcohol,
tobacco, folic acid and pyridoxine deficiencies have been associated with clefting.
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Increasing parental age, especially an older father, is associated. About 35% of clefts
have a positive family history.
Human Genome Project: identifying genes related to clefting. Genes related to the
production of tissue growth factor alpha, and fibroblast growth factor receptors have
shown an association with clefts.
Genetic testing may soon allow us to predict those families that may have a higher
risk of cleft babies, although, as Dr. Millard says, “There is little chance that the
molecular geneticist will arrive in an operating room with a cleft repair gene attached
to a retrovirus anytime soon.”
What are the risks that another family member will be born with a cleft?
For parents of one cleft child: 2-5% risk of a second child with a cleft
If additional family members have clefts: 10-12% risk
For a person born with a cleft: 2-5% risk of having a cleft child
If additional family members have clefts: 10-12% risk
For siblings of a person with a cleft: 1% risk
If additional family members have clefts: 5-6% risk
If a syndrome is involved: risk can be as high as 50%. These
patients should have a genetic evaluation.
Cleft lip and/or palate implies a risk of recurrence that ranges from
incomplete CL alone to bilateral CL and P.
Cleft palate alone implies a risk for cleft palate only; these families
are not at risk for cleft lip.
Genetic Evaluation:
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Obtain a detailed family history, a medical history, and physical examination of the
cleft patient, and laboratory testing.
1. Verify that the cleft is “isolated” and not part of a syndrome.
2. Evaluate whether other relatives have similar conditions, andhow closely related
they are. The greater the number of relatives known to have clefts, and the closer
their biologic relation, the greater the risk of recurrence.
3. Consider the type and severity of the cleft. Clefts tend to be consistent within
families, although severity can vary.
4. Testing: chromosomal testing is a syndrome is suspected; radiographs; molecular
testing is available for some specific conditions; photographs.
5. Counseling: discussion of treatment necessary; risk of recurrence; discussion of
strategies for future prenatal diagnosis.
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Donald R. Laub, M.D., FACS
Adjunct Clinical Associate Professor of Surgery,
Stanford Medical School Founder, Interplast
Welcome
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You are attending a workshop on helping the cleft patient. This work is the most
noble and most important and most effective work of mankind. You don’t require to
be congratulated. Because you are—
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Professional person
A professional person; each one of you is a professional person, that is a person who
has devoted his or her life to a skill and a science, and you practice that skill for the
good of the other person. This act produces a very comfortable feeling
—immediate gratification if you will— when you have helped someone with a
severe deformity and significantly changed their fortunes for the rest of their lives.
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Psychic Income
This is the Psychic Income. A professional person does the work for the good of the
other person, which is in contrast to the non-professional person who performs his
skill for the good of himself primarily. For example, the infamous CEOs of large
corporations.
This Psychic Income is so powerful that it possibly produces a chemical affecting the
brain that addicts us to do this type of work. Psychic Income is the motor which
runs all Plastic Surgery Voluntary Foundations, and it is the motor for CPM
(Cirujanos Plastikos Mundi), and it is what makes the world go around as far as the
Plastic Surgery organizations designed to help the developing world are concerned.
For example, the founders of most plastic surgery helping foundations and most of
the foundations devoted to orthodontia, speech therapy, ENT, dentistry, to help the
cleft child, both the founders and the members, relate a similar personal experience
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on the occasion of taking care of the first child they have had the privilege of helping
with their skill. Many or most of these people relate that at the moment of receiving
this special psychic income that it caused a change in their value system to be more
humanitarian. In a way it was the child that helped the professional, to change their
life to be more humanitarian.
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It is as if the “tail is now wagging the dog”, the tail being the Psychic Income and
the Dog being the Physician and the paramedical person, who receives impetus to do
this type of work or to form to a professional foundation. Here the patient becomes
the one who produces the result in the surgeon. This phenomenon of the Doctor
needing the patient reverses or erases our preoperative mind set that the surgeon is
the almighty one and the patient is entirely a recipient of the surgeon’s skill and
benevolence.
We are all on a horizontal relationship: doctor + patient both, not a vertical
relationship
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The team of a foundation must also consist of businessmen, accountants, lawyers
and fundraisers; and all of these are vital to our life.
In this instance, people with these skills are also professionals because they are
working for the other person and they derive the same Psychic Income as the
physicians do, because they are acting as surrogate surgeons or surrogate medical
professionals. After all, it is because of these surrogate physician professionals that all
of this is made possible.
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When these two types of skills work together, we form a new way of practicing
medicine. It is in these foundations where there is a true teamwork, where medicine
and business truly work together for the patients benefit and for each other’s
betterment of their individual specialty.
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A true combination of business and medicine is produced by the commonality of
humanitarianism.
In this case, one + one equals three: it is obvious that medicine and business working
together is a very powerful unit.
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All of the elements in this process evolved at many places simultaneously. My
experience is for example at Stanford where, as at the other places, it was
recognized that the needs of the students and the residents in training would be
better solved in the setting of these programs. The acquisition of the psychomotor
or surgical skills by trainees in these programs was excellent. This method of
teaching is second to no other method. The residents who were there for the
reason of learning the clinical or surgical parts of this process were delighted with
what was going on; they were learning what is or was the very epitome of the field:
the cleft lip repair and the cleft palate repair, and the nasal reconstruction for the
cleft: for them this was the highest achievement toward their goal of learning
surgery.
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Peak Experience
As they learned skills, they were sort of in a state of ecstasy because this constitutes a
peak experience for the trainee.
Value Change
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A Peak Experience occurs at birth, when we are married, or are divorced, or
graduate, or receive an award—or perform our first cleft lip repair. At the time of a
peak experience, our value system can change. For example, at the conclusion of this
landmark operation for them, they might say, “This was the best experience I have
ever had in my life; I want to do this for the rest of my life.” At that moment in the
training stage, the imprinting is apt to be much more permanent, meaningful, longlasting, and appropriate, than if these values of humanitarianism are transferred or
acquired at a post-residency level. This process easily occurs for all practicing
physicians and surgeons at any point in their careers; and it happened to me in early
residency, and I assume it occurs for you at every cleft surgery. The imprinting is
best done at the time in our careers set aside for learning “to the max”, which is
residency or fellowship training.
When the trainee states, “I want to do this for the rest of my life,” she or he has
indicated that an attitude has been transferred to that “student” as well as a skill.
Because the operation causing such psychic income was on a child where no fee was
charged, this was a humanitarian act, so in this way the attitude of helping others
was also transferred and imprinted. And I have heard, on 40 occasions, those
words—at the end of the 1st cleft lip repair (repeat), “This was the best experience I
have had in my life, I want to do this for the rest of my life,” a value change had
occurred, and the skill and the social attitude became part of that person’s life from
that time of the peak experience on.
In Dr. Richard Jobe’s survey of the practicing surgeons who had undergone these
experiences, as a resident, plus or minus 35% had continued practicing this work,
and had retained this value.
Interdisciplinary contact is necessary
The patient changed the surgeon in a profound way; the “tail wagged the dog”; a
good outcome resulted.
Now turning to another aspect of our professional life, let me discuss the danger of a
few in a University who achieve great excellence in a small field, and achieve power
and notoriety within their system. The tendency to pout and cry may occur if each
of your objectives is not immediately met by the Dean or the ruling class or your
peers. The tendency is to say, “I quit, and I will form my own institute in order that
I can practice excellence.”
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What happens in this case is that interdisciplinary contact is eliminated. The crossfertilization from different methods of thinking and from different related fields is
eliminated. It’s probably more difficult to form multi-disciplinary efforts with
equal emphasis given to all of the “ancillary” fields when your practice is with an
institute without pluralism, even though the institute is seemingly the ideal
setting.
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The advancements in that particular field may then wither.
The multidisciplinary approach has helped develop certain fields of surgery:
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MULTIDISCIPLINARY INITIATIVES
THAT HAVE WORKED:
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• Plastic Surgery
• Cleft Surgery and Humanitarian Surgery
• Cosmetic Surgery
• Maxillofacial Surgery (both cosmetic and reconstructive).
• Surgery for Craniofacial anomalies
• Hand Surgery
• Skin Physiology and aesthetic improvement based in this science
• Microsurgery itself
• Joint replacement Surgery
• Neurosurgery for Epilepsy
• Microsurgery including tissue tolerance and limb transplantation.
• Bariatric Microsurgery
• Ophthalmic surgery for retinal disease and for nearsighted patients.
• Oncologic Surgery
AREAS THAT HAVE NOT BENEFITED AS MUCH
FROM THE MULTIDISCIPLINARY INITIATIVES:
• Breast Surgery
• Parts of Oncologic Surgery
The theory behind Residents on Trips
And in the future, the equilibrium between oral surgery and ENT and plastic
surgery will require the skill and diplomacy that only occurs in a University
environment and does not occur in an institute.
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Hijo de tigre nacio pintado. The nourishment of our young, our own progeny in each
of our “narrow” fields is important, and is on a par with the importance of the
multidisciplinary effort.
No doubt about it, the Teaching of the Residents in developed countries is as vital as
teaching in other lands, because if the young (students and residents and
impressionable youngsters) are not nurtured and induced to gain the excitement and
pleasure of Psychic Income, the field will not reproduce itself. Withholding this
process is similar to practicing birth control on your own professional discipline.
Advances in development of our fields
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If the professionals do not teach in this way, they are sterilizing themselves. They
prevent reproduction their own kind.
Furthermore, medicine is not a static science, new operations and new advances are
occurring at regular intervals.
The inclusion of our residents in intense training experiences is a link in the chain,
which advances our field.
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For example, in cleft lip and palate surgery, think of what should happen in the five
years from now, and also think what will not happen in five years if you do not
bring in new brains and new skills?
For example:
Intrauterine fetal surgery will not be developed to repair cleft lip.
New operations will not have been developed, e.g., Mike Carsten’s (a resident)
embryologic concept of moving the bone and the soft tissue as one neurovascular
unit to repair cleft lip and craniofacial anomalies.1
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The Most Important Thing
Enthusing young people to enter our field is clearly vital to ourselves and to the
patients, who are the most important thing.
Repeat, Humanitarianism should be taught at the age when teaching is done, is best
done, is most effective.
Carsten has shown that Clefting occurs when one neurovascular unit of bone and soft tissue unit
become deficient during fetal development and that the repair of cleft should be based on this
concept.
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“Enlightened self-interest”
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In the greater perspective, we can consider the effect of our work on our world,
which will occur only through our children, and our residents. Our children and our
residents will be needed in the formation of public opinion in the next few years.
Just think how the world would change if our governments and our corporations
and our politicians were to regard this way of life as a thing of value. If our very own
children felt that helping others in these ways were a thing of value they might
counter-balance their conditioning toward their aspirations to acquire he latest
clothing styles, the latest entertainment, and their latest idea about what they should
acquire in life.
But both aspirations seem necessary.
Governments might divert a small billion or two toward these projects for their own
good. Their own political projects would enjoy easier success. They would gain favor
in the foreign countries and gain votes here in the developed world.
Our corporations could actually make more money by being nice to people in
foreign countries. This concept might mature into more than “enlightened selfinterest” (a phrase taken from history), but into a sincere value change.
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A value change would increase the espirit of the employees in the large corporation;
their company newspaper would tout the companies’ social conscience to the pride
and delight of their more socially conscious employees, would raise the profits of the
company and their “host” country; perhaps the actual expressed goals for the
corporation would perhaps include the good of the employees; the shareholders and
officers might see it this way, and all countries would benefit. This mindset is not
really “thinking different” or O.O.T.B.2, but it is thinking in the longer term in an
educated way, a bit more professional way.
This thing of value should be regarded as equal to the tangible income of making
money and parlaying that money into more money.
The field moves forward.
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Speaking of a modern, more educated method of “developing the field” of Plastic
and Reconstructive Surgery: both in regard to the educational process and even the
research, the innovative progression should include:
Developed
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&
Developing
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}
Country Professionals working together.
Out Of The Box
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In effect, we almost double the amount of brainpower brought into use by
increasing exchange of ideas between disciplines and between countries by sharing
research at an earlier level, and by assuming commonality of purpose.
This new concept leads to virtual centers of excellence because some professionals
may be geographically separated but united by a web-based center of excellence (See
Dingman PRS 2002). Again, to repeat, we are securing the development of new
operations, of research, and of new surgeons. We see the development of
multidisciplinary affiliated disciplines in both countries.
Example of personal “Modern” History of theories discussed
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Consider some Stanford medical initiatives.
Multidisciplinary humanitarianism, and Residents on trips
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In 1963 at Stanford, Dr. Chase, my mentor and professional father, began our
program with:
a) Skill
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b) The other person in mind.
This combination led to the performing of a free surgery on a single patient,
Antonio Victoria from Mexicali, Mexico and then later this led to the formation of
Mexico Medical Project, Inc. and subsequently Interplast. Our first initiatives used
for teaching, for obtaining patients with advanced pathology in our new hospital
system, are examples of being committed to a goal. They were as follows:
When challenged by the official opinion of the Mexican Government to not return
to their fair country for further surgical work on children with developmental
deformities, and adults with acquired deformity (burns, hand injuries, tumors), we
pouted for only a short time. And then for some unknown reason conceived a “Plan
B” for that situation. The government of Mexico had not initiated a more intense
program to help their own citizens. At that relatively naïve time for us we “collaged”
our assets (our group had 5 assets interlocking goals): Reconstructive Surgery
Training, a knowledge of ships and the sea, a leadership experience with the U.S.
Navy, an idealist in the law enforcement department of Los Altos, California, a
veteran with personal experience in pre-juvenile delinquency rehab., a University
faculty position, and a Rockefeller Foundation connection.
We identified yard freighter 879 in the San Diego Reserve (Mothball) fleet and then,
a Culinary Instructor, and also a retired Navy Captain for command, policemen to
refer 14 year-old pre-juvenile delinquents for rehabilitation, a medical corpsman
instructor, teaching faculties, textbooks (Lange Medical Synopses), David Werner’s
paramedical volume, and a retired Naval electronics Veteran, and educators in
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seamanship. The yard freighter has 35 feet of clear room with no internal cross
member supports to allow an operating room, a post-op care unit, preparation room
and a supply place. Quarters for the patients and crew were above deck. A lounge
was to be identified. The kitchen was new, never used. The ship had been built in
Philadelphia and towed to San Diego at the end of the war; it was kept air
aconditioned for 25 years and maintained perfectly.
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It drew only 3 ft of water and was ready for work in the shallow harbors of Western
Mexico and South America. We visited the pentagon and arranged for the ship to be
released at a certain moment, at which time we were to have our application on
Capt. Banan’s desk. Any University has second priority to military material declared
surplus. Dean John Wilson asked his nice friend at Rockefeller Foundation to
arrange a grant for the project based on U.S. Navy experience and the experience
gained.
In Norway, where socially deviant persons learned self-worth, ability to not be selfcentered, and the necessity to work together as a team when working onboard a ship.
Their programs resulted in less recidivision (back to jail or to the “Brig.”).
Using the yard freighter in the San Diego Reserve Fleet, we had a method to train
pre-juvenal delinquents in
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a) Culinary arts
b) Electronic skills
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c) Seamanship
d) Medical corpsman
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Our second abortive attempt to do the type of work we are addressing in this
symposium was the DC-3 airplane. The DC-3 was purchased by Interplast as safe
and inexpensive transportation. It increased esprit du corps, higher and higher, until
an unscheduled landing occurred, which of course led to another plan, to the
formation of an Interplast “air force”, consisting of volunteer pilots and their
airplanes, for transportation to nearby countries. It was another multidisciplinary
initiative “assured” of success. These aircraft were used for transportation to clinics
and hospitals in other countries, including parts of the U.S. The plan was
inexpensive, running at 1/2 cost of commercial transport.
Innovative funding from the community
Initial funds were derived from:
San Mateo Chope Hospital Surgical Medical funds
Gender Surgery Fees
Clair Elgin (a patient with the worst diagnosis and deformity who had “struck it
rich” with her invention).
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Our initial PRS patients were derived from:
Physiatry Department (paraplegic and quadriplegic)
Jail (Lombroso’s Theory that improving appearance improves social behavior)
Menlo Park Veteran’s Administration Psychiatric Hospital (2,000 patients, many
with skin cancer, Carpal Tunnel Syndrome, or Visual Field Defects, i.e. need for
large scale blepharoplasty)
And of course, Interplast—actually, Interplast was part of the educational process
emanating from Stanford to provide pathology for residents and students.
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Success
This initiative over the years lead to 2,000 patient surgeries from Mexicali, 4,000
patients and surgeries from San Pedro Sula, Honduras, 50,000 patients total from
various countries and 40,000 patients from developing countries for Interplast
Germany.
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Dear colleagues—the personal example of commitment to Plastic Surgery and a
multidisciplinary effort for those in need is related to you not for what sounds like
self-interest, but I write it to you so that we might share the commonality of our
humanitarian efforts, all of which are both personal and also in the name of all of us.
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“Even if we are occupied with most important things, even if we attain to high
honor, or fall into great misfortune, still, let us remember how good it was once here
when we were all together united by a good and kind feeling which made us…
better, perhaps than we are.”
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-Fyodor Dostoevsky, The Brothers Karamazov
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Upwards and Onwards.
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Cirujanos
Plástikos
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T
E
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First East Indian International Cleft Surgery Workshop
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Cirujanos
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