Surgery for craniosynostosis has evolved rapidly over the
past two decades, with increased emphasis on early, extensive
operations.1 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.
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Table 1.
Classification of Surgical Result After Reconstruction for
Craniosynostosis
[View Table]
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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 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, Kleeblattschädel, and acquired. The Kleeblattschädel 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 Kleeblattschädel patients.
To be able to analyze surgical results, we developed a seven-category
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 sagittal 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%).
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Table 2.
Suture Involvement for 250 Consecutive Surgical Patients With
Craniosynostosis
[View Table]
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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.
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Table 3.
Gender Distribution, Identifiable Syndromes, and Median Age at First
Operation for 250 Consecutive Surgical Patients With Craniosynostosis,
by Sutural Involvement*
[View Table]
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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 Kleeblattschädel 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).
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.
[View Larger Version of this Image (89K GIF file)]
It is striking that the Kleeblattschädel 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 the older patients
underwent the 
or reverse 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 Klee-blattschädel 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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Table 4.
Complications, Planned and Unplanned Reoperations, and Deaths Among 250 Craniosynstosis Patients, by Sutural Involvement*
[View Table]
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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 complications: 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 Kleeblattschädel (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
2 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 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 Kleeblattschädel (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.
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.
[View Larger Version of this Image (99K GIF file)]
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Table 5.
Distribution of Involved Sutures for 30 Patients With Multiple Suture
Synostosis
[View Table]
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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 frontal-orbital 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.
[View Larger Version of this Image (140K GIF file)]
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.
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.
[View Larger Version of this Image (133K GIF file)]
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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*
[View Table]
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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
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 (Fig
5). Comparing the seven groups by the Tukey test,
looking for pair differences, significantly worse outcomes were found for Kleeblattschädel, bilateral coronal, and multiple suture synostosis patients compared with metopic synostosis patients. No other
statistically significant pair differences were found.
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Table 7.
Numerical Outcome Scores for Patients After Surgical Treatment of
Craniosynostosis, Ranked by Sutural Involvement, Minimum Follow-up 6 Months
[View Table]
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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.
[View Larger Version of this Image (94K GIF file)]
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 Kleeblattschädel 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
operation 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
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 frontal-orbital 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
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 two-stage
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
Kleeblattschädel 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
Kleeblattschädel deformity, it is striking how close their
incidence, 14.6%, is to the combined incidence of our multiple suture
patients and Kleeblattschädel 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
Kleeblattschädel patients were even higher.
The Kleeblattschädel 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
Kleeblattschädel 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.
Presented in part at the 73rd annual meeting of the American
Association of Plastic Surgeons, St Louis, MO, May 1-4, 1994.
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.
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 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.
,
ABSTRACT
results and unsolved problems.
Plast Reconstr
Surg.
1995;
96:272-283[Medline]
