Egyptian Journal of Surgery Vol. 31, No. 2, April 2012
ORIGINAL ARTICLE
OUTCOME OF SURGICAL EXCISION OF CERVICO FACIAL
LYMPHATIC MALFORMATIONS IN CHILDREN: A PROSPECTIVE STUDY
Kamal A. M. Hassanein
Maxillofacial/Head and Neck Surgical Unit, Sohag Faculty of Medicine, Sohag, Egypt
Email: [email protected]
Abstract
Introduction: Surgical treatment of lymphatic malformations (LMs) in the cervicofacial area is challenging for
surgeons because of their poorly defined borders and the potential for damaging neighboring structures. The
aim of the study: was to evaluate the outcome of surgical treatment of cervicofacial LMs in children to
improve preoperative counseling.
Methods: A prospective study included cases with LMs in the cervicofacial area that had been treated in the
Maxillfacial/Head and Neck Surgical Unit, Sohag University Hospitals, Egypt, in the period from June 2002
to May 2011. All cases were treated with surgical excision as a primary treatment modality and were
evaluated for recurrence and complications. Cases were followed for a period from 8 to 69 months (mean of
28 ± 13 months).
Results: Recurrence and overall complications occurred in 4 (9%) and 11 (25.5%) cases, respectively.
Satisfaction was described as excellent or good by 41 (93%) parents of the cases. Permanent nerve weakness
was not encountered. Recurrence was higher in cases with age group < 2 years, when the lesion located in or
close to the area of the facial nerve and/or the suprahyoid region, and in the microcystic and mixed types (P
= 0.001). Overall rate of complications was higher, when the lesion located in or close to the area of the facial
nerve and/or the suprahyoid region (P = 0.02), and in the microcystic and mixed types (P = 0.003).
Conclusions: Surgical excision of LMs, especially the macrocystic type in infants and children as the primary
treatment modality, especially when it is complete is safe and highly successful technique and has satisfactory
outcome.
Keywords: Lymphangiomas, cystic hygroma, surgical management, complications.
INTRODUCTION
Lymphatic malformations (LMs), also known cystic
hygroma and lymphangiomas, are rare congenital
tumours affecting mainly the head and neck area.
64
Embryologically, they are thought to originate from the
sequestration of lymphatic tissue during the
development of lymphaticovenous sacs, which fail to
communicate with the remainder of the lymphatic or
venous system. Later on the sequestered lymphatic
Egyptian Journal of Surgery
tissues dilate, which results in the cystic morphology of
the lesions.(1) Recently, the Vascular Endothelial Growth
Factor C gene has been found to be a critical factor for
normal lymphatic development.(2,3)
The majorities of LMs are located in the head and neck
area and of equal incidence in both genders. (4) They are
classified, according to the size, into macrocystic,
microcystic, or mixed type (macrocystic lesions, spaces
≥ 2 ml; microcystic lesions, spaces < 2 ml). (5,6) Another
classification of LMs into different stages according to
the extent of the lesion in the cervicofacial area has been
considered by some authors.(7,8) LMs usually are
symptomless apart from painless disfiguring mass with
concerns regarding cosmesis. Sometimes may be present
with difficulties in breathing and/or swallowing, or
infection, which may be life threatening. (4)
Management of LMs is often difficult and depends on
size, extent, and presenting symptoms of the lesion.
There have been different methods of treating this
condition
such
as;
observation,
aspiration,
sclerotherapy, and surgical excision. A period of
observation may be warranted to optimize the timing of
treatment of the LMs. However, spontaneous regression
should not be expected as the reported involution is rare
and reported to be less than 5%.(6,9) Aspiration may
provide temporary relieve in urgent cases as when there
is a compromise of the upper airways by an enlarging
lymphangioma but, it should not be an option of a
definitive treatment as it is followed with recurrence
and may induce infection10. Sclerotherapy using
different materials have been reported to be effective
with satisfactory overall results and it is gaining wide
acceptance. Of these materials are OK-4325,(11-13) Fibbrin
sealant,(14) and ethanol. (15) Some consider sclerotherapy
will always leave residual malformation,(16) and more
seriously it may result in stridor due to upper airway
obstruction as a result of swelling of the lesions after
injection.(17)
Complete surgical excision still the standard option to
treat LMs by many surgeons.(18) Complete surgical
excision carries a low rate of recurrence, while
incomplete surgical removal is associated with very
high rate of recurrence. (10,19) Recurrence usually appears
shortly after incomplete surgical resection of the LMs,
however rarely it may be delayed after years.(20) Most
LMs of the head and neck area impinge on the nearby
neurovascular bundle. This renders their management
tedious and carries a significant risk of the surrounding
important structures. The aim of the study: was to
evaluate the outcome of surgical treatment of
cervicofacial LMs to improve preoperative counseling.
PATIENTS AND METHODS
This prospective study included patients with LMs in
the cervicofacial area and had been treated initially by
surgical excision in the Maxillfacial/Head and Neck
Surgical Unit, at Sohag University Hospitals, in the
period from June 2002 to May 2011. Exclusion criteria
EJS, Vol. 31, No. 2, April 2012
included cases with treatment modalities other than
surgery as primary treatment modality, those with
intrathoracic extension that needed thoracotomy or
mediastinotomy or cases with isolated tongue, and
intraoral mucosal involvement, or cases lost for follow
up. The parents of all eligible cases for the study had
consented and the study protocol was planned and
approved by the Research Ethics Committee of the
Faculty, before the start of the study.
The following data were collected; age at treatment,
gender, presenting symptoms, extent of the lesion
(according to the staging system of de Serre et al., (1995)
7 (Table 1), radiologic investigations performed, and
extent of surgery. Diagnosis was made clinically and by
ultrasound scan and computed tomography (CT), which
is essential prior to surgery as accurate study of the type
of the cyst and its extension into deeper tissue planes is
more superior by using CT and hence it helps in
planning for the surgical procedure and even may
predict the outcome. Final diagnosis was confirmed by
histopathological evaluation after excision of the lesion.
The aim of surgery was to achieve complete resection of
the disease whenever possible. A subtotal excision was
performed if it was felt that complete excision may lead
to neurological complications or functional impairment.
During surgery, wide surgical exposure was adopted.
Careful attention was given to protect and preserve the
essential neurovascular bundle (Figs. 1,2).
Table 1. Degrees of extension of LMs.
Stage I
Unilateral infrahyoid disease
Stage II
Unilateral suprahyoid disease
Stage III
Unilateral infra and suprahyoid disease
Stage IV
Bilateral suprahyoid disease
Stage V
Bilateral infra and suprahyoid disease
This staging system is according to de Serre et al.,
(1995)(7)
All cases were evaluated for: recurrence; complications
that included neurovascular derangement, wound
healing, and seroma formation; and overall satisfaction
as rated by the parents in response to a single question
about their satisfaction of the results of the surgery. The
response ranged as excellent, good, fair, or
unsatisfactory. The studied cases were followed for a
period from 8 to 69 months (mean 28 ± 13 months).
Statistical Methods: Statistical analysis between
treatment groups was performed by Chi-square test.
P-value less than 0.05 were considered significant.
65
Fig 1a. Macrocystic LM, occupying the whole
left side of the neck.
Fig 1d. Operative picture showing the preserved
important neck structures; SMM: sternomastoid
muscle; CCA: common carotid artery; VN: vagus
nerve; IJV: internal jugular vein; SAN: spinal
accessory nerve; TD: thoracic duct; TrM:
trabezius muscle
Fig 1b. CT revealing the extent of the lesion
bicavitary, pushing and compressing the treachea.
Fig 1e. Operative picture of the specimen
after complete excision of the cyst.
Fig 1c. Operative picture demonstrating the cyst
deep to the sternomastoid muscle (SMM)
and the omohyoid muscle (OHM).
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Egyptian Journal of Surgery
Fig 2a. Massive lymphatic cyst, in
a 7 day old new born.
Fig 2d. Three weeks postoperative picture.
Fig 2b. Operative picture showing dissection
of the cyst with excision of the excess skin.
Fig 2e. No recurrence five years
after surgical procedure.
Fig 2c. Operative picture showing the preserved
neurovascular bundle with excision of the internal
jugular vein; CCA: common carotid artery; HN:
hypoglossal nerve; FN: facial nerve.
EJS, Vol. 31, No. 2, April 2012
67
RESULTS
This study included 43 cases (Table 2) and they were 20
(46.5%) males and 23 (53.5%) females and their age
ranged from 7 days to 14 years. The majority of the
cases (65%) presented and treated before the age of 2
years. Suprahyoid region and area of the facial nerve
were involved in 31 cases (72%). Some of these lesions
were extended into the parotid gland (4 cases), the
submandibular salivary gland (4 cases), and the face
(3 cases). The majority of the cases were of the
macrocystic type 30 (70%). Complete excision of the
lesion was achieved in the vast majority of the cases, 40
out of 43 cases (93%). All the cases with incomplete
excision were located in the area of the facial nerve and
were of the microcystic and mixed type.
Recurrence occurred in 4 patients (9%). Of them were
the 3 cases that ended up with incomplete surgical
excision. Three of these cases were treated with ethanol
injection (three sessions each case) later on, which
resulted in reasonable response in 2 cases and minor
response in the remaining one. The parents of the
remaining case refused to have any further
management. Recurrence was statistically significant
higher (Table 3) in cases less than 2 years of age
(P=0.001), extensive lesions located in or close to the
area of the facial nerve and or the suprahyoid region
(P=0.001) and the microcystic and mixed types
(P=0.001).
Overall complications rate was encountered in 11
patients (25.5%). Of these, temporary neurological
complications occurred in 8 cases (18.5%). Marginal
mandibular branch of the facial nerve paresis was
observed in 6 cases (14%), hypoglossal nerve paresis in
one case (2.3%), and cervical sympathetic chain paresis
was observed in another case (2.3%). All these
neurological complications were improved within 3
months. Two cases were complicated with seroma that
regressed spontaneously and one case with mild wound
dehiscence, which needed simple suturing of the skin.
Complications were statistically higher, when the lesion
located in or close to the area of the facial nerve and/or
the suprahyoid region (P=0.02), and in the microcystic
and mixed types (P=0.003). Overall satisfaction was
described as excellent or good by the majority of the
parents of the cases (93%).
Table 2. Medical characteristics and surgical outcome of the 43 cases of the study.
Age
< 2 years
>2 years
28 (65%)
15 (35%)
Sex
Males
Females
20 (46.5%)
23 (53.5%)
Staging
I
II
III
VI
V
12 (28%)
14 (32.5%)
13 (30%)
2 (4.7%)
2 (4.7%)
Presenting symptoms
Mass
Difficulties in swallowing
Difficulties in breathing
Inflammation
43 (100%)
2 (4.7%)
3 (7%)
5(11.5%)
Type of LMs
Macrocystic type
Microcystic type
Mixed type
30 (70%)
7 (16%)
6 (14%)
Recurrence
4 (9%)
Overall complications
11 (25.5%)
Overall satisfaction
Excellent
Good
Fair
27 (63%)
13 (30%)
3 (7%)
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Egyptian Journal of Surgery
Table 3. Prognostic factors in outcome evaluation.
Recurrence
(4 cases)
Factors
P-value
Complications
(11 cases)
P-value
Age
< 2 years
>2 years
4/28 (14%)
0/15(0%)
0.001
Sex
Males
Females
2/20 (10%)
2/23(8.7%)
0.35
Staging
I
( II-V)
0/12 (0%)
4/31(13%)
0.001
1/12(8.3%)
11/31(35%)
0.02
1/30(3.3%)
3/13(23%)
0.001
3/30(10%)
8/13(61.5%)
0.003
Type of LMs
Macrocystic
Microcystic and Mixed type
DISCUSSION
Traditionally, surgical excision of LMs has been
considered the gold standard of treatment. (18,21,22)
Maximal surgical reduction of the LMs, while
preserving important neurovascular structures and
cosmesis considered the treatment of choice by Bjaj et
al.(16)
More recently some authors have advocated the use of
other methods in wide range of cases, especially in
children. (8,23) Of these are sclerosant materials and the
aim of this is to avoid the complications of surgery and
the procedure may be less invasive. The disadvantages
are being that immediately after the procedure, the
lesion swells up markedly, the lesion slowly regresses
over 3 months (long duration of treatment), possibility
of recurrence and the long term toxicities of the
treatment are not known.(24) Sclerotherapy for lesions
around or pressing on the trachea is not a favorable
option, as post treatment marked swelling may result in
airway obstruction. (17) It is of importance to stress that
old sclerosing materials such as bleomycin, ethibloc,
tetracyclin, dextrose, and sodium morrhuate are known
of causing scarring and contraction of the surrounding
tissues, rendering subsequent surgery more difficult.
More recently, OK-432 is gaining wide acceptance as a
new emerging sclerosing material and has the
advantage that following administration there is no
peripheral fibrosis and therefore subsequent surgery for
residual lesions is not made more difficult nor
hazardous.(23)
In this series, complete resolution of the disease was
achieved in 91% of patients with one operation as the
first and only treatment. This is comparable to or
slightly better than the results of other literatures
reporting on surgery as the first and only treatment in
cervicofacial LMs in children.(10,20-22) Amongst these,
EJS, Vol. 31, No. 2, April 2012
8/28(28.5%)
3/15(20%)
5/20(25%)
6/23(26%)
0.09
0.87
patients with macrocystic type complete response was
achieved in all of them. This is explained by the success
of complete excision of these lesions. However, the
recurrence rate was 9% and attributed to incomplete
resection of the lesion as all these cases were of
microcystic or mixed type, which has more tendency to
interdigitate into tissue planes, enveloping neural and
vascular structures and its complete excision is
extremely difficult and hazardous. (25) Recurrence rate in
our cases was significantly higher in patients less than 2
years of age, which is parallel to the study of Chen et
al. (22) However, several authors found that age was not a
significant factor in recurrence or complications
following surgical excision of the cervicofacial LMs.(26-28)
Recurrence was also significantly higher in extensive
lesions, involving more than one anatomical site, and
those located in the suprahyoid region and extending to
the facial area, and cases with microcystic and mixed
types. These observations have been addressed by many
authors. (16,22,27,28)
Of paramount importance in surgical resection of the
LMs in the head and neck area is to avoid
complications, especially neurological deficits. Nerve
palsies were encountered in 8 cases (6 were the marginal
branch of the facial nerve). This may seem to be high in
comparison with other studies16, but all cases were
improved in 3 months with no permanent deficits. The
most probable reason behind this is complete excision of
the lesions was attempted and done more frequently in
our series (91%) than that reported by Bjaja et al.,(16)
study, which was 79.3%. In our study the rate of
complications was significantly higher in extensive
lesions and those involving the suprahyoid region and
facial nerve area and cases with microcystic and mixed
types. This is comparable to the findings of other
studies.(7,26,28)
Overall satisfaction was described as excellent or good
by the vast majority of the parents of the cases (93%),
69
and it was fair in the remaining cases (7%) but none was
unsatisfied. This is close to the results reported by Lei et
al.(28)
Surgical excision has the advantage of usually a single
procedure to excise the disease. It offers potential
complete eradication of the disease and a lifetime cure
in the vast majority of cases. Some children with
extensive disease of the microcystic and mixed types
will need multiple treatments or may need another
combination therapy as scelerosing materials. Further
studies are needed to develop the area more fully, but
the diverse nature of the lesions will make any
prospective randomised trials difficult to manage.(29)
Conclusions: Surgical excision of cervicifacial LMs is a
golden option for their management, especially the
macrocystic type and those located away from the facial
nerve area. It is effective and safe and has a satisfactory
outcome.
9.
Ninh TN, Ninh TX. Cystic hygroma in children: a report of
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