1. No category

Surgical Treatment of Craniosynostosis: Outcome Analysis of 250 Consecutive
Patients
Gerald M. Sloan, Karin C. Wells, Corey Raffel and J. Gordon McComb
Pediatrics 1997;100;e2
DOI: 10.1542/peds.100.1.e2
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pediatrics.aappublications.org/content/100/1/e2.full.html
PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1948. PEDIATRICS is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 1997 by the American Academy
of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.
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Surgical Treatment of Craniosynostosis: Outcome Analysis of 250
Consecutive Patients
Gerald M. Sloan, MD*; Karin C. Wells, MD‡; Corey Raffel, MD, PhD§; and J. Gordon McComb, MD‡
ABSTRACT. Objective. Surgery for craniosynostosis
has evolved rapidly over the past two decades, with
increased emphasis on early, extensive operations. Older
published series may not accurately reflect more recent
experience. Our study was designed to analyze outcome
in a large series of consecutive patients treated recently at
a single center.
Methods. We reviewed 250 consecutive patients who
underwent surgical treatment of craniosynostosis between January 1, 1987 and December 31, 1992. They were
divided into nine groups by suture involvement: sagittal,
unilateral coronal, bilateral coronal, unilateral lambdoid,
bilateral lambdoid, metopic, multiple suture, the Kleeblattscha¨del deformity (cloverleaf skull), and acquired
craniosynostosis. Outcome was analyzed in terms of residual deformities and irregularities, complications, mortality, as well as the need for additional surgery.
Results. There were 157 males (62.8%) and 93 females
(37.2%), with most of the male preponderance accounted
for by the large sagittal synostosis group, which consisted of 82 males and 25 females. Median age at first
operation was 147 days. A named syndrome was present
in 23 patients (9.2%) and was more common than expected with bilateral and unilateral coronal synostosis,
the Kleeblattscha¨del deformity, and multiple suture synostosis. There were two deaths (0.8%), both with Kleeblattscha¨del patients, and 17 other complications (6.8%).
Morbidity and mortality were significantly associated
with secondary vs primary operations and syndromic vs
nonsyndromic patients. Outcome analysis revealed the
best surgical results with metopic synostosis and significantly less good results with the Kleeblattscha¨del deformity, multiple suture synostosis, and bilateral coronal
synostosis.
Conclusions. Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and
relatively low morbidity and mortality, particularly for
otherwise healthy, nonsyndromic infants. Pediatrics
1997;100(1). URL: http://www.pediatrics.org/cgi/content/
full/100/1/e2; craniosynostosis, craniofacial anomalies,
craniofacial surgery, facial deformities.
From the *Division of Plastic Surgery, University of North Carolina School
of Medicine, the §Department of Neurosurgery, Mayo Clinic and Foundation, Rochester, Minnesota and the ‡Divisions of Plastic Surgery and Neurosurgery, Childrens Hospital Los Angeles, Los Angeles, California.
Presented in part at the 73rd annual meeting of the American Association of
Plastic Surgeons, St Louis, MO, May 1– 4, 1994.
Received for publication Mar 13, 1996; accepted Oct 8, 1996.
Reprint requests to (G.M.S.) Professor and Chief, Division of Plastic and
Reconstructive Surgery and Surgery of the Hand, University of North
Carolina School of Medicine, Chapel Hill, NC 27599 –7195.
PEDIATRICS (ISSN 0031 4005). Copyright © 1997 by the American Academy of Pediatrics.
S
urgery for craniosynostosis has evolved rapidly
over the past two decades, with increased emphasis on early, extensive operations.1– 8 Because
of changes in surgical timing and techniques, earlier
series may not accurately reflect more recent experience. Furthermore, most previous reports do not
quantitate results in a way that would allow objective analysis and comparisons. An exception is the
four-category classification of operative results introduced by Whitaker et al,6 which has subsequently
been used by others.7,8 In that classification system,
category I includes those patients in whom no surgical revisions were considered advisable or necessary by the surgeon, patient, or family. In category II,
soft tissue or minor bone contouring revisions were
desirable, whether or not they were actually performed. Category III consisted of patients in whom
major secondary osteotomies or bone grafting procedures were needed or performed. These procedures
were not as extensive as the original surgery. Category IV was composed of those patients in whom a
major craniofacial procedure, duplicating or exceeding the extent of the original surgery, was or would
be necessary. However, that classification system has
significant limitations. Categories II, III, and IV all
represent patients believed to require further surgery. Good or fair results, with residual deformities,
but not requiring further surgery, are not distinguished from excellent results.
To examine results of recent craniosynostosis surgery, we analyzed the outcome in 250 consecutive
patients treated at a single center during a 6-year
period beginning January 1, 1987 and ending December 31, 1992. We developed a seven-category outcome classification system (Table 1) to allow recognition of more subtle differences in surgical results.
The patient numbers are sufficient to permit detailed
statistical analysis of results for a well-defined group
of patients. With increasing emphasis on clinical outcome analysis at the national level, such data will be
increasingly important in developing multidisciplinary treatment protocols.
MATERIALS AND METHODS
A total of 260 patients underwent a surgical procedure for
craniosynostosis at Childrens Hospital Los Angeles between January 1, 1987 and December 31, 1992. Seven patients were excluded
because their first operation for craniosynostosis had been performed before January 1, 1987. Three other patients were excluded
because their first operation, although falling within the 6-year
study period, was performed elsewhere. The remaining 250 patients had undergone initial surgical treatment of craniosynostosis
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PEDIATRICS Vol. 100 No. 1 July 1997
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TABLE 1.
Classification of Surgical Result After Reconstruction for Craniosynostosis
Class 1
Class 2
Class 3
Class 4
Class 5
Class 6
Class 7
Good to excellent correction, with no visible or palpable irregularity
Good to excellent correction with palpable but not visible irregularity (eg, a palpable, but not
visible, surgical wire, plate, or bony irregularity), not requiring reoperation
Good to excellent correction with visible irregularity (eg, a visible prominence from a surgical
wire or plate, or a visible bony spicule or defect that does not compromise the overall
correction), not requiring reoperation
Good to excellent correction with visible or palpable irregularity requiring reoperation (eg, a
surgical plate requiring removal)
Compromised overall correction, but not severe enough to require reoperation (eg, slight
forehead asymmetry)
Compromised overall correction requiring reoperation
Compromised overall correction, believed to require reoperation by the surgeon, but family
declines further surgery
at Childrens Hospital Los Angeles between January 1, 1987 and
December 31, 1992.
The medical records of the 250 patients were reviewed. Data
were collected, including name, medical record number, date of
birth, gender, involved sutures, other medical diagnoses, dates of
all surgical procedures performed for craniosynostosis, complications, dates of follow-up visits, findings at follow-up, and the most
recent assessment of outcome. Any other relevant data were also
noted.
Data were analyzed for the entire patient group, as well as for
nine subgroups based on suture involvement: sagittal, unilateral
coronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid,
metopic, multiple suture, Kleeblattscha¨del, and acquired. The
Kleeblattscha¨del group consisted of those patients with the classic
cloverleaf skull and total sutural synostosis. The multiple suture
synostosis group was defined as those patients with synostosis of
more than one suture, excluding bilateral coronal, bilateral lambdoid, and Kleeblattscha¨del patients.
To be able to analyze surgical results, we developed a sevencategory classification system (Table 1). In this system, classes 1
through 4 represent good to excellent overall correction of the
deformity, but with varying degrees of minor visible and/or
palpable irregularities: none in class 1, palpable but not visible
irregularities in class 2, visible irregularities in class 3, and requiring reoperation in class 4. Examples would include a palpable but
not visible surgical wire in the temporal region (class 2), a visible
and palpable bone spicule in the forehead (class 3), or a visible and
palpable surgical plate requiring surgical removal because of impending exposure (class 4). Classes 5 through 7 represent patients
with significantly compromised correction: not requiring further
surgery in class 5, requiring further surgery in class 6, and believed by the surgeon to require further surgery but with the
family declining further surgery in class 7. Examples would include noticeable brow asymmetry after correction of unilateral
coronal synostosis but not severe enough for reoperation (class 5),
or residual brachycephaly after surgery for bilateral coronal synostosis requiring repeat frontal-orbital advancement (class 6).
RESULTS
Suture Involvement
Distribution of patients by suture involvement is
shown in Table 2. By far the largest group was sagTABLE 2.
Suture Involvement for 250 Consecutive Surgical
Patients With Craniosynostosis
Sutural Involvement
No.
% of Total
Sagittal
Unilateral coronal
Bilateral coronal
Unilateral lambdoid
Bilateral lambdoid
Metopic
Multiple suture
Kleeblattscha¨del
Acquired
107
28
21
30
7
20
30
6
1
42.8
11.2
8.4
12.0
2.8
8.0
12.0
2.4
0.4
Total
250
100.0
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ittal synostosis, 107 patients (42.8%). Next most frequent were multiple suture synostosis (12.0%), unilateral lambdoid synostosis (12.0%), and unilateral
coronal synostosis (11.2%).
Gender
Gender distribution, presence of an identified syndrome, and median age at first operation are shown
in Table 3. There was a clear male preponderance
among patients with sagittal synostosis (76.6%) (P ,
.0001). However, the bilateral coronal synostosis patients were 76.2% female (P , .05). Taking the entire
group of 250 craniosynostosis patients, there were 64
more males. Almost the entire difference can be explained by the sagittal synostosis group, which had
57 more males than females.
Syndrome Diagnosis
More than half of the bilateral coronal synostosis
patients, 12 of 21, or 57.1%, carried the diagnosis of a
specific named syndrome (P , .001). Six of the
twelve had Crouzon, four Apert, one Antley-Bixler,
and one Pfeiffer syndrome. A named syndrome was
present in 4 of 30 multiple suture synostosis patients
or 13.3% (P , .001), with three having Crouzon and
one Apert syndrome. Two of the six Kleeblattscha¨del
patients, or 33.3% (P , .01), carried a syndrome
diagnosis, one Antley-Bixler and one Pfeiffer. Two
unilateral coronal synostosis patients had syndrome
diagnoses, one Baller-Gerold and one Saethre-Chotzen. Additionally, there was one metopic synostosis
patient with Klippel-Feil syndrome, one unilateral
lambdoid synostosis patient was a 47 xxx female,
and one sagittal synostosis patient had the Goldenhar variant of the facio-auriculo-vertebral spectrum
(Fig 1).
It is striking that the Kleeblattscha¨del patients
were operated on at a much earlier age (median 25
days) than any other group. Because of the total
sutural involvement and high risk of resultant damage to the central nervous system from increased
intracranial pressure, these patients were treated
quite urgently. The sagittal synostosis group was the
next youngest group at the time of initial surgery
(median 101 days). Many of those patients were
treated by sagittal craniectomy using the technique
that has been described by McComb.9 Some of the
younger (less than 6 months) patients in the saggittal
synostosis group had a strip craniectomy. Some of
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Fig 1. A, B, and C, Three views of a 1-month-old male infant with multiple congenital anomalies including sagittal stenosis, bilateral cleft
lip and palate, and right lateral facial cleft. He carries the diagnosis of facio-auriculo-vertebral spectrum. D, The same patient at 10 months,
after sagittal craniectomy at 2 months through midline incision, and calvarial vault remodeling at 9 months. E, The same patient at 5 years.
TABLE 3.
Gender Distribution, Identifiable Syndromes, and Median Age at First Operation for 250 Consecutive Surgical Patients
With Craniosynostosis, by Sutural Involvement*
Sutural Involvement
Sagittal
No. Male
(% of group)
No. Female
(% of group)
No. Syndromic
(% of group)
Median Age At First
Operation (in days)
†82 (76.6)
†25 (23.4)
1 (0.9)
101
Unilateral Coronal
13 (46.4)
15 (53.6)
‡2 (7.1)
198
Bilateral Coronal
‡5 (23.8)
‡16 (76.2)
§12 (57.1)
136
Unilateral Lambdoid
19 (63.3)
11 (36.7)
1 (3.3)
196
6 (85.7)
1 (14.3)
0
203
1 (5)
195
Bilateral Lambdoid
Metopic
11 (55)
Multiple Suture
17 (56.7)
13 (43.3)
§4 (13.3)
Kleeblattscha¨del
4 (66.7)
2 (33.3)
\2 (33.3)
Acquired
Total
9 (45)
138
25
0
1 (100)
0
719
157 (62.8)
93 (37.2)
23 (9.2)
147
* P values compared to expected 50 –50 gender distribution and 0.7% rate of named syndromes, based on binomial (n # 20) or Poisson
(n . 20) distributions.
† P , .0001.
‡ P , .05.
§ P , .001.
\ P , .0001.
the older patients underwent the p or reverse p
procedure.10
Complications
Complications, deaths, and number of patients undergoing planned as well as unplanned reoperation
are shown in Table 4. The only two deaths, as well as
two nonfatal complications, occurred in the Kleeblattscha¨del group. One of the deaths was a male
infant with Antley-Bixler syndrome, who underwent
radical posterior craniectomy at 9 days, followed by
bilateral frontal-orbital advancement and remodeling at 18 days. He subsequently developed turricephaly, for which barrel stave osteotomies were
performed at 16 months. He died, shortly after that
surgery, of acute brain herniation. The second death
was a male infant with Pfeiffer syndrome who underwent posterior craniectomy at 25 days, which was
complicated by an intraoperative dural sinus hemorrhage requiring transfusion of 2 units of packed red
blood cells and 40 units of platelets. At 3 months,
bilateral frontal orbital advancement and remodeling
was performed, which was followed by postoperative hydrocephalus, requiring ventriculo-peritoneal
shunting 2 months later. On follow-up examination
at 14 months, there was found to be no evidence of
head growth since the shunt placement. The infant
was developmentally delayed. Posterior vault remodeling was performed and was complicated by
venous sinus hemorrhage. This was controlled intraoperatively, but the patient died of cerebral edema 3
days later.
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Fig 3. A, 1-year-old male child with severe plagiocephaly secondary to right coronal and right lambdoid synostosis. B, He underwent posterior craniectomy at 13 months and bilateral frontalorbital remodeling at 15 months. He is shown here at 3 years. He
still has lateral displacement of the cranial base on the right side,
but this has not been severe enough to recommend further surgery, making this a class 5 result.
Fig 2. A, 2-month-old female infant with metopic, sagittal, and
bilateral coronal synostosis. B, The same patient, on the operating
table at 3 months, demonstrating the severe deformity. C, Intraoperative view demonstrating sagittal synostectomy and bilateral
frontal-orbital advancement and remodeling. D, The appearance
at the completion of surgery. E, The appearance at 22 months.
Five of 21 bilateral coronal synostosis patients
(23.8%, P , .001) had complications, which included
tongue edema requiring reintubation, a dural and
cortical laceration resulting in a postoperative cerebrospinal fluid leak that resolved, femoral artery
thrombosis at a catheter site, a subdural fluid collection that required shunting, and a pyogenic granuloma at the surgical incision. Four of 30 multiple
sutures synostosis patients (13.3%, P , .05) had complications: postoperative apnea requiring reintubation, postoperative seizures, severe conjunctival
edema and herniation, and superficial stitch abscesses along the incision. Two of 30 unilateral lambdoid synostosis patients (6.7%, P , .05) had compli4 of 9
cations: a unilateral supranuclear facial palsy that
resolved 8 months after surgery, and Salmonella sepsis in a patient whose father had a diarrheal illness
shortly before surgery. Four of 107 sagittal synostosis
patients (3.7%, P , .001) had complications: pneumonia, postoperative hydrocephalus requiring
shunting, intraoperative metabolic alkalosis, and a
superficial wound infection.
Unplanned reoperation was required in 18 of the
250 patients (7.2%). Specifically, unplanned reoperation was needed in two Kleeblattscha¨del (33.3%),
four bilateral coronal (19.0%), five multiple suture
(16.7%), two unilateral coronal (7.1%), two unilateral
lambdoid (6.7%), one metopic (5%), and two sagittal
synostosis patients (1.9%).
There were 17 nonfatal complications and 2
deaths, a combined morbidity and mortality rate of
19 of 250 patients (7.6%) or 19 of 297 operations
(6.4%). For 250 primary operations, there were 10
complications and no deaths (a complication rate of
4.0%). For 47 secondary or tertiary operations, there
were two deaths and seven other complications, for a
complication rate of 14.9% (P , .01 compared to
primary operations by x2 analysis), and a combined
morbidity and mortality rate of 19.1% (P , .001).
Analyzing morbidity and mortality by the presence of an associated syndrome, it is striking that
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TABLE 4.
Complications, Planned and Unplanned Reoperations, and Deaths Among 250 Craniosynstosis Patients, by Sutural
Involvement*
Sutural
Involvement
Complications (%)
Patients Undergoing
Planned Reoperation (%)
†4 (3.7)
0
Bilateral Coronal
Unilateral Lambdoid
Sagittal
Unilateral Coronal
Patients Requiring
Unplanned Reoperation (%)
Deaths
0
2 (1.9)
0
0
‡2 (7.1)
0
†5 (23.8)
5 (23.8)
†4 (19.0)
0
‡2 (6.7)
0
‡2 (6.7)
0
Bilateral Lambdoid
0
0
0
0
Metopic
0
0
Multiple suture
‡4 (13.3)
13 (43.3)
†5 (16.7)
0
Kleeblattscha¨del
‡2 (33.3)
5 (83.3)
§2 (33.3)
2 (33.3)
0
0
0
0
17 (6.8)
23 (9.2)
18 (7.2)
2 (0.8)
Acquired
Total
1 (5)
0
* P values compared to zero, for complications and for unplanned reoperations, based on binomial (n # 20) or Poisson (n . 20)
distributions.
† P , .001.
‡ P , .05.
§ P , .01.
both deaths and 9 of 17 nonfatal complications occurred in syndromic patients. Because our entire patient group included 23 patients with syndrome diagnoses, the mortality rate was 8.7% for syndromic
patients and 0% for nonsyndromic patients. The nonfatal complication rate was 39% for syndromic patients as opposed to 3.5% for patients who did not
carry a syndrome diagnosis (P , .0001).
Multiple Suture Synostosis
The multiple suture synostosis group is a particularly interesting and challenging group of patients
that has not been well described elsewhere (Fig 2). In
this group, we include all patients with synostosis of
two or more cranial sutures other than isolated bilateral lambdoid synostosis, bilateral coronal synostosis, or the classic Kleeblattscha¨del (clover leaf skull)
deformity, which three groups are classified separately. In our series, this group was surprisingly
large, consisting of 30 patients, or 12% of the entire
population. Such a large number of unusual suture
combinations may reflect either increased awareness
and better ability to diagnose multiple suture involvement with modern imaging techniques, or a
selection bias based on referral to a tertiary care
center. The specific sutures involved for those 30
patients are listed in Table 5. The most common
combination was 10 patients with unilateral coronal
and unilateral lambdoid synostosis. It is striking that
the involvement of the two sutures was ipsilateral in
all 10 patients; the right side for 7 patients and the
left for 3 patients. The resulting deformity was usually quite severe (Fig 3). We have never seen a patient with unilateral coronal and unilateral lambdoid
synostosis on opposite sides.
Acquired Craniosynostosis
One female infant in our series had acquired craniosynostosis with congenital hydrocephalus who
had undergone ventriculo-peritoneal shunting and
developed fusion of multiple cranial sutures. This is
a known, but uncommon, sequela of shunting that
has been previously described.12 Our patient was
treated with extensive calvarial remodeling with
multiple bone flaps at 23 months. She did well after
surgery, but died 6 months later of unrelated sepsis
secondary to necrotizing enterocolitis and bowel perforation.
Outcome
Outcome after completion of planned surgery,
whether one or two operations, was analyzed according to the classification system listed in Table 1
(Fig 4). Sagittal synostosis patients were not included
in this analysis, because they will be analyzed separately and reported elsewhere. All patients followed
for a minimum of 6 months after completion of
planned surgery were included in this analysis. The
actual numbers are shown in Table 6, which includes
115 of 143 possible patients. To allow statistical analysis we assigned a numerical value to each class, as
follows: Class 1, 0 points; class 2, 0.5 point; class 3, 1
point; class 4, 2 points; class 5, 3 points; class 6, 4
points; and class 7, 4 points.
Although admittedly arbitrary, we attempted to
assign numbers proportionate to the amount by
which a result varied from class 1. Classes 6 and 7
have the same numerical value because they were
TABLE 5.
Distribution of Involved Sutures for 30 Patients
With Multiple Suture Synostosis
Sutural Involvement
No. % of Group
Unilateral coronal, unilateral lambdoid
10
Metopic, sagittal
5
Sagittal, unilateral coronal
2
Sagittal, bilateral coronal
2
Sagittal, unilateral lambdoid
2
Unilateral coronal, bilateral lambdoid
2
Metopic, unilateral coronal
1
Metopic, unilateral lambdoid
1
Metopic, sagittal, bilateral coronal
1
Sagittal, unilateral coronal, bilateral lambdoid 1
Sagittal, bilateral lambdoid
1
Bilateral coronal, unilateral lambdoid
1
Bilateral coronal, bilateral lambdoid
1
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33.3
16.7
6.7
6.7
6.7
6.7
3.3
3.3
3.3
3.3
3.3
3.3
3.3
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Fig 4. A and B, 3-month-old female infant with left unilateral coronal synostosis. C and D, The same patient at 16 months, after bilateral
frontal-orbital remodeling at 5 months. She had three palpable stainless steel wires, above the hairline, which have not needed to be
removed. Therefore, this was a class 2 result.
Fig 5. A, 14-month-old male child with untreated metopic synostosis. B, The same patient on the operating table, before correction, at 17
months. C, At the completion of surgery. D and E, At 2 years, 10 months.
believed to represent the same outcome, differing
only as to whether or not the family elected to have
further surgery. The mean and SE of the outcome
scores are listed in Table 7. The best outcome (lowest
score) was found in the metopic synostosis group
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(Fig 5). Comparing the seven groups by the Tukey
test, looking for pair differences, significantly worse
outcomes were found for Kleeblattscha¨del, bilateral
coronal, and multiple suture synostosis patients
compared with metopic synostosis patients. No
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TABLE 6.
Surgical Result After Reconstruction for Craniosynstosis in Patients Followed for a Minimum of 6 Months, Analyzed by
Sutural Involvement*
Class
Unilateral
Coronal
Bilateral
Coronal
Unilateral
Lambdoid
Bilateral
Lambdoid
Metopic
Multiple
Suture
Kleeblattscha¨del
1
2
3
4
5
6
7
8 (30.8)
3 (11.5)
2 (7.7)
1 (3.8)
11 (42.3)
1 (3.8)
0
6 (31.6)
1 (5.3)
3 (15.8)
0
5 (26.3)
4 (21.1)
0
7 (36.8)
3 (15.8)
6 (31.6)
0
1 (5.3)
2 (10.5)
0
3 (60)
0
1 (20)
0
1 (20)
0
0
8 (50)
6 (37.5)
1 (6.2)
1 (6.2)
0
0
0
2 (7.7)
7 (26.9)
5 (19.2)
2 (7.7)
6 (23.1)
3 (11.5)
1 (3.8)
1 (25)
0
0
0
0
2 (50)
1 (25)
* Percentage of each group shown in parentheses; see Table 1 for definitions of classes 1 to 7.
TABLE 7.
Numerical Outcome Scores for Patients After Surgical Treatment of Craniosynostosis, Ranked by Sutural Involvement, Minimum Follow-up 6 Months
Sutural Involvement
No.
Mean
SE
Metopic
Bilateral lambdoid
Unilateral lambdoid
Unilateral coronal
Multiple suture
Bilateral coronal
Kleeblattscha¨del
15
5
19
26
26
19
4
0.38
0.80
0.97
1.63
*1.79
*1.82
*3.00
0.13
0.58
0.30
0.28
0.28
0.38
1.00
* Significant pair difference, compared to metopic synostosis patients, by Tukey test.
other statistically significant pair differences were
found.
DISCUSSION
We found a striking male preponderance among
the sagittal synostosis patients; 82 males and 25 females. That finding is consistent with the hypothesis
of Graham et al,13 that fetal head constraint may
contribute to sagittal synostosis. The argument is
that the increased occurrence of sagittal synostosis in
males is related to larger fetal head size during the
third trimester of pregnancy, resulting in a higher
degree of physical constraint of the head in the maternal pelvis. Overall, our surgical results with sagittal synostosis were excellent, with only four complications (3.7%) and only two unplanned
reoperations (1.9%). The median age at surgery for
this group (a little more than 3 months) was lower
than for all other groups except the Kleeblattscha¨del
patients.
The unilateral coronal synostosis patients had no
perioperative complications, but a high proportion,
46%, had a compromised aesthetic result because of
residual postoperative asymmetry. In only one patient was the asymmetry severe enough for reoperation to be recommended. However, that is a very
subjective decision. In this series, the median age at
first operation for unilateral coronal synostosis was
almost 7 months. We are presently operating on
these patients at a younger age (4 to 6 months) hoping to achieve better symmetry because the bone is
more pliable and the deformity is less severe at a
younger age.
The bilateral coronal synostosis group is striking
for the preponderance of females (76.2%) and the
frequency of named syndromes (57.1%), the highest
of any group in this study. Median age at first oper-
ation was 4.5 months. Five of 21 patients underwent
planned second operation, and 4 of 21 required an
unplanned reoperation (P , .001). There was a high
complication rate, 23.8% (P , .001), and all five
complications occurred in syndromic patients. Outcome was significantly worse than for our best
group, the metopic synostosis patients. Bilateral
coronal synostosis patients are a challenging group,
at least partly due to the frequent presence of named
syndromes with other associated problems and abnormalities in these patients. Many, such as the Apert and Crouzon patients, also have severe mid-face
deformities, making it difficult to achieve or even
judge the proper amount of frontal and superior
orbital advancement, whether done as the initial operation or as a combined monobloc advancement.14
There is still not a consensus as to the optimal timing
and approach for these patients.8,15–19
The unilateral and bilateral lambdoid synostosis
patients did well overall, although the unilateral
lambdoid synostosis group did have a 6.7% complication rate, and two patients required unplanned
reoperation. As with the unilateral coronal patients,
the major problem encountered with unilateral lambdoid synostosis has been asymmetry. In fact, one of
our patients had such severe secondary forehead
asymmetry that she eventually underwent frontalorbital remodeling after two occipital procedures.
Although unilateral lambdoid and bilateral lambdoid synostosis patients comprised 12.0% and 2.8%
of the patients in this series, respectively, that has
changed as of 1992. We are now operating on a far
smaller number of these patients. At present, when
we have an opportunity to see patients before 1 year,
we treat them with a molding headband20 (Dynamic
Orthotic Cranioplasty, Southwest Orthotic-Prosthetic Laboratory, Phoenix, AZ). Initial results are
very encouraging and have been reported separately.21 A recent report22 described a dramatic increase
as of 1992 in the number of infants seen at a single
center for plagiocephaly without synostosis. These
infants characteristically present with unilateral occipital flattening that could be mistaken for unilateral
lambdoid synostosis. We do not believe that any of
our unilateral lambdoid synostosis patients actually
had this entity, because radiographic and actual surgical findings were, in all our cases, consistent with
true lambdoid synostosis. It has been hypothesized
that plagiocephaly without synostosis has been seen
with increased frequency as of 1992 because that is
the year that the American Academy of Pediatrics
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formally launched a campaign to educate the public
about the association of the prone sleeping position
with sudden infant death syndrome.23 An infant positioned supine for sleep, it is suggested, may favor
one side and thus expose that side to gentle but
constant pressure of enough magnitude to affect
head shape. Because that series ended in 1992, it
seems unlikely that such factors played a role in
many, if any, of these cases.
Our best surgical results were obtained in the
metopic synostosis patients (Table 7). We had no
complications, performed no planned reoperations,
and only one patient (5%) required unplanned reoperation in the metopic synostosis group. A recent
study, with 1-year follow-up CT measurements in 10
metopic synostosis patients, demonstrated improved
but persistent anterior orbital hypotelorism.24 Although our assessment was that these patients had
excellent outcome overall, a number of these patients
do have residual mild hypotelorism even after surgical correction.
The multiple suture synostosis patients were a
surprisingly large and heterogeneous group. Four of
the 30 patients had identified syndromes. There were
high rates of complications (13.3%), planned twostage correction (43.3%), as well as unplanned reoperations (16.7%). It is difficult to generalize about
such a diverse group, except to emphasize the need
to individualize the treatment plan based on the
involved sutures and the resulting deformity. If there
is significant lambdoid and/or coronal involvement,
posterior release (craniectomy or cranioplasty) at a
young age, perhaps 1 to 3 months, can relieve intracranial hypertension and allow waiting until 4 to 6
months for the definitive frontal-orbital reconstruction. By 4 to 6 months, we find the bone easier to
work with to accomplish and stabilize frontal-orbital
advancement and remodeling. The high incidence of
multiple suture synostosis in our series, 12%, is striking, particularly because bilateral coronal, bilateral
lambdoid, and Kleeblattscha¨del patients are grouped
separately and make up an additional 13.6% in our
series. Little has been written specifically about multiple suture synostosis. Hoffman and Reddy25,26 reported the experience from the Hospital for Sick
Children in Toronto, Canada, but what they described was a somewhat different entity. They found
11 patients (1.7%) in a retrospective review of 665
craniosynostosis patients over a 58-year period.
However, their reports emphasized delayed and progressive multiple suture synostosis, their patients
were older at presentation (24 to 117 months), most
of their cases were holocalvarial, and four of their
cases followed previous surgery and might better be
considered acquired craniosynostosis. Interestingly,
Shillito and Matson,1 in their 1968 work based on 619
surgical patients in a 40-year period at Children’s
Hospital Medical Center in Boston, reported 76 of the
619 patients (14.6%) as having multiple suture synostosis. Of those, 10 had involvement of two unpaired sutures, 36 had involvement of three sutures,
and 30 had involvement of four or more sutures.
Because that last group probably included patients
that we have separately listed as Kleeblattscha¨del
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deformity, it is striking how close their incidence,
14.6%, is to the combined incidence of our multiple
suture patients and Kleeblattscha¨del patients, 14.4%.
Also striking is that 38% of their multiple suture
patients were operated on more than once, in comparison with 43.3% of our multiple suture patients
who underwent planned reoperation and 16.7% who
required unplanned reoperation. Our rates of reoperation for the six Kleeblattscha¨del patients were
even higher.
The Kleeblattscha¨del deformity is characterized by
a trilocular or cloverleaf cranial configuration, associated facial malformations, hydrocephalus, and (in
some cases) micromyelia and skeletal anomalies.27
This has been a particularly difficult problem in our
experience, as well as that of others. Despite planning a two stage approach in five of our six patients,
two of those patients still required additional unplanned operations. The only two deaths in our series were those two patients.
Our one patient with acquired craniosynostosis
developed the problem after ventriculo-peritoneal
shunting for hydrocephalus. The presumed mechanism is rapid decompression of the cranial vault,
after shunting, resulting in overlapping of the bones
across what had been widely spaced sutures.12 If
brain growth does not soon spread the bones again,
the sutures can become synostosed.
We found a striking association of mortality and
morbidity with the presence of a named syndrome.
There are two possible explanations, both of which
may play a role. First, 16 of the 23 syndrome patients
had bilateral coronal synostosis, multiple suture synostosis, or Kleeblattscha¨del deformity, three groups
that are particularly challenging surgically and
would have required longer, more extensive, and
more complicated surgical approaches. Second,
many of the associated findings in the syndromic
patients, such as a retruded midface with airway
narrowing, would put those patients at increased
risk for complications.
In conclusion, we have reviewed a 6-year experience with surgical management of craniosynostosis
at a single center. This is the largest such series to be
analyzed and reported in almost 30 years. This report
provides outcome data for craniosynostosis right up
to the beginning of the recent controversies regarding the possible association of supine sleep positioning and plagiocephaly without synostosis, an entity
that should be managed without surgery.22 Our results demonstrate that true craniosynostosis can be
successfully corrected, using modern surgical techniques, with relatively low morbidity (6.8%) and
mortality (0.8%). Many unanswered questions remain, but we hope that this report of a large, recently
treated series of craniosynostosis patients will contribute to our evolving understanding and treatment
of these problems.
ACKNOWLEDGMENTS
This study was supported in part by RO1 DE10426 from the
Craniofacial Development and Disorders Program, National Institute of Dental Research, National Institutes of Health.
The authors thank all individuals who rendered invaluable
CRANIOSYNOSTOSIS SURGERY: OUTCOME IN 250 PATIENTS
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assistance in this study. Linda S. Chan, PhD and Carla Rother, MA
performed the statistical analysis. Susan E. Downey, MD, Larry S.
Nichter, MD, and John F. Reinisch, MD participated in the surgery. Ari Blumoff, MD and Cathleen M. Salata, RN assisted with
data collection.
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Surgical Treatment of Craniosynostosis: Outcome Analysis of 250 Consecutive
Patients
Gerald M. Sloan, Karin C. Wells, Corey Raffel and J. Gordon McComb
Pediatrics 1997;100;e2
DOI: 10.1542/peds.100.1.e2
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