The prevalence of strabismus in unilateral coronal synostosis.

BACKGROUND: While there is a clear correlation between unilateral coronal synostosis (UCS) and ocular motility abnormalities, the literature provides little information as to the true epidemiology of strabismus, or the underlying etiology of these paralleled pathologies. The purpose of this study is to investigate the rate of oculomotor abnormalities associated with UCS and its management. METHODS: A retrospective review of all patients identified to have single-suture, nonsyndromic UCS treated by fronto-orbital advancement at a tertiary craniofacial referral center from 1977 to 2013 was performed. Inclusion criteria mandated complete medical, surgical, and ophthalmological records. Patients were evaluated for strabismus both preoperatively and postoperatively, and as to whether eye muscle surgery was performed. RESULTS: A total of 181 patients underwent treatment for UCS at our institution during the study period, of which 79 met the inclusion criteria. Twenty-nine patients had strabismus prior to any craniofacial surgical intervention. Following fronto-orbital advancement, 23 patients (46 %) developed a new onset strabismus. Fifty-five patients had no change in their preoperative ocular examination, and one patient had resolution of preoperative strabismus. Of the 51 patients who had postoperative strabismus, 30 went on to have eye muscle surgery. There were no statistically significant differences in gender (p=0.477), race (p=0.395), sidedness of suture involvement (p=0.552), or age at intervention (p=0.66) in comparing the group with new postoperative strabismus and those without. CONCLUSIONS: This study sheds new light on the prevalence of strabismus in UCS, and more importantly, the risk of developing strabismus in the setting of conventional fronto-orbital advancement. This data will allow more accurate preoperative counseling and reinforces the important role of ophthalmologists as members of the multidisciplinary craniofacial team.

Perioperative complications associated with intracranial procedures in patients with nonsyndromic single-suture craniosynostosis.

Within the diagnosis "craniosynostosis," there is a subset of patients who present with isolated, nonsyndromic, single-suture involvement. This study evaluates perioperative complications in this specific subset of patients over 4 decades at a single institution. To do so, we performed a retrospective review on consecutive patients undergoing correction of single-suture synostosis from May 1977 to January 2013 at a tertiary pediatric craniofacial center. Demographic information, operative details, and perioperative course were collected. Complications were categorized as either major or minor. A χ(2) test and Fisher exact test were used to compare all categorical variables. Continuous variables were analyzed using Wilcoxon rank-sum and Kruskal-Wallis tests.Seven hundred forty-six patients underwent surgical correction of nonsyndromic craniosynostosis. Of these, there were 307 (41.2%) sagittal, 201 (26.9%) metopic, and 238 (31.9%) unicoronal. Thirty-four patients had complications (4.6%). Eight were considered major (1.1%), including one postoperative mortality in a patient with hypoplastic left-sided heart syndrome. Minor complications occurred in 26 patients (3.5%) and included subgaleal hematoma (n = 3), seroma (n = 4), and superficial wound infection (n = 5). Metopic and sagittal suture involvement was significantly associated with a higher complication rate (P = 0.04). A child with isolated single suture synostosis and any comorbidity had a significantly greater risk of any complication (P < 0.001; odds ratio, 3.8) and specifically an increased risk of major complication (P = 0.031; odds ratio, 6.0). Subclassification of patients by time period yielded no statistically significant changes in perioperative morbidity. To conclude, these data allow us to counsel families more accurately with regard to morbidity and mortality and may potentially serve as a benchmark for future quality improvement work.

The impact of a parent liaison at a major craniofacial center.

The psychosocial impact of craniofacial disfigurement affects both the developing child and his/her family. The Facial Reconstruction Center at the Children's Hospital of Philadelphia has employed a Parent Liaison (PL) to provide psychosocial support to families and has been an invaluable resource in this regard. We hypothesize that a PL impacts the overall outcome of the surgery by building trust between the parents and medical institution, and increasing satisfaction. An anonymous satisfaction survey was sent to families of craniofacial patients treated between January 1976 and June 2012. All patients who had undergone at least 1 craniofacial procedure had addresses on file and were included in this study. Statistical analyses were performed using the Mann-Whitney U test.During the study, 441 surveys were mailed to families meeting the inclusion criteria. A total of 151 families returned completed surveys (34.2%), and 121 surveys were included for analysis (27.4%). In rating overall satisfaction, families who met with the PL had statistically higher scores than those who had not (P = 0.0011). Parents who met with the PL preoperatively reported greater satisfaction in time spent answering questions (P = 0.0029) and the perception that questions were adequately answered (P = 0.0039). No statistical difference was observed in postoperative preparedness between families that did and did not meet the PL. The results demonstrate that the PL is beneficial in the education, experience, and satisfaction of families treated at a large Craniofacial Center. The PL complements the surgeon's treatment of the physical by adding psychosocial support.

Does the incidence of traumatic brain injury in children increase after craniofrontal surgery?

BACKGROUND: Craniofrontal reshaping (CR) for the treatment of craniosynostosis results in a temporary cranial defect due to the advancement and/or expansion of the supraorbital and frontal bones. The goal of the present study was to determine whether the incidence traumatic brain injury (TBI) was increased in patients after CR as a result of this breach in skull integrity. METHODS: We performed a retrospective chart review of all patients with a diagnosis of craniosynostosis who underwent CR between 1997 and 2007 at our institution. The incidence of TBI (defined as a computed tomography-documented brain or skull injury or loss of consciousness) was determined during a 2-year postoperative follow-up period. Patients older than 1 year at the time of surgery, or those with less than 2 years of follow-up after surgery, were excluded. RESULTS: A total of 396 patients met the inclusion criteria. The incidence of TBI was zero (95% confidence interval, 0-760/100,000). CONCLUSIONS: The estimated incidence of TBI after CR surgery is between 0 and 760 per 100,000 patients in our clinic population. These data indicate that although CR may be associated with a slightly increased risk of TBI while the skull is healing, the absolute incidence of such injury remains extremely rare.

Metopic craniosynostosis due to mutations in GLI3: A novel association.

We report on the novel association of trigonocephaly and polysyndactyly in two unrelated patients due to mutations within the last third (exon 14) and first third (exon 6) of the GLI3 gene, respectively. GLI3 acts as a downstream mediator of the Sonic hedgehog signal-transduction pathway which is essential for early development; and plays a role in cell growth, specialization, and patterning of structures such as the brain and limbs. GLI3 mutations have been identified in patients with Pallister-Hall, Grieg cephalopolysyndactyly syndrome (GCPS), postaxial polydactyly type A1, preaxial polydactyly type IV, and in one patient with acrocallosal syndrome (ACLS). Furthermore, deletions including the GLI3 gene have been reported in patients with features of GCPS and ACLS. To date, trigonocephaly has not been associated with abnormalities of GLI3 and craniosynostosis is not a feature of GCPS. However, Hootnick and Holmes reported on a father with polysyndactyly and son with trigonocephaly, polysyndactyly, and agenesis of the corpus callosum, considered GCPS thereafter. Guzzetta et al. subsequently described a patient with trigonocephaly, polysyndactyly, and agenesis of the corpus callosum postulating a diagnosis of GCPS, later considered ACLS. In retrospect, these two patients, evaluated prior to mutational analysis, and our patients, with confirmed mutations, likely fall within the GLI3 morphopathy spectrum and may provide a bridge to better understanding those patients with overlapping features of GCPS and ACLS. Based on this observation, we suggest GLI3 studies in patients presenting with this constellation of findings, specifically metopic craniosynostosis with polysyndactyly, in order to provide appropriate medical management and genetic counseling.

The Whitaker Classification of Craniosynostosis Outcomes: An Assessment of Interrater Reliability.

BACKGROUND: The Whitaker classification is a simple and widely used system for describing aesthetic outcomes after craniosynostosis surgery. The purpose of this study is to evaluate its interrater reliability for patients who have undergone fronto-orbital surgery. METHODS: A retrospective review of patients with craniosynostosis who underwent surgical intervention at a tertiary referral center was conducted. Inclusion criteria were as follows: single-suture craniosynostosis, surgical intervention before age 2 years, and photographs taken before revisions between 5 and 20 years of age. Thirteen craniofacial surgeons independently reviewed the subjects' photographs and assigned Whitaker classifications. Interrater reliability was assessed with the Cohen kappa statistic. RESULTS: Twenty-nine subjects were included. Average ages at surgery and at the time of postoperative photography were 0.8 year and 12.8 years, respectively. The κ value for all 13 raters was 0.1567 (p < 0.0001), indicating "slight agreement." Pairwise comparisons demonstrated κ values ranging from 0.0384 to 0.5492. The average rating for the set of 29 photographs differed significantly across the 13 raters (p = 0.0020) and ranged from 1.79 ± 0.68 to 2.79 ± 0.77. Finally, we found that average Whitaker classification did not differ significantly between subjects who subsequently underwent cranioplasty and/or fronto-orbital advancement and those who did not (subsequent procedures, 2.45 ± 0.55; no subsequent procedures, 1.88 ± 0.78; p = 0.1087). CONCLUSIONS: The Whitaker classification exhibits low interrater reliability and does not predict future treatment. It may benefit craniofacial surgeons to create new evaluation tools with greater precision, to improve the quality of patient care and craniofacial outcomes research.

Assessing the midface in Muenke syndrome: A cephalometric analysis and review of the literature.

BACKGROUND: Max Muenke included midface hypoplasia as part of the clinical syndrome caused by the Pro250Arg FGFR3 mutation that now bears his name. Murine models have demonstrated midface hypoplasia in homozygous recessive mice only, with heterozygotes having normal midfaces; as the majority of humans with the syndrome are heterozygotes, we investigated the incidence of midface hypoplasia in our institution's clinical cohort. METHODS: We retrospectively reviewed all patients with a genetic and clinical diagnosis of Muenke syndrome from 1990 to 2014. Review of clinical records and photographs included skeletal Angle Class, dental occlusion, and incidence of orthognathic intervention. Cephalometric evaluation of our patients was compared to the Eastman Standard Values. RESULTS: 18 patients met inclusion criteria - 7 females and 11 males, with average follow-up of 11.2 years (1.0-23.1). Cephalometric analysis revealed an average sella-nasion-A point angle (SNA) of 82.5 (67.8-88.8) and an average sella-nasion-B point angle (SNB) of 77.9 (59.6-84.1). The SNA of our cohort was found to be significantly different from the Eastman Standards (p = 0.017); subgroup analysis revealed that this was due to the mixed dentition group which had a higher than average SNA. 12 patients were noted to be in Class I occlusion, 4 in Class II malocclusion, and 2 in Class III malocclusion. Only one patient (6%) underwent orthognathic surgery for Class III malocclusion. CONCLUSIONS: While a part of the original description of Muenke syndrome, clinically significant midface hypoplasia is not a common feature. This data is important, as it allows more accurate counseling of patients and families. LEVEL OF EVIDENCE: III.

The art and science of anti-aging therapies.

A meeting entitled "The Art and Science of Anti-Aging Therapies: Convergence of Theory and Practice" took place on 18 and 19 March 2005 at the University of Pennsylvania School of Medicine. The symposium was held for health care professionals, researchers, and consumers to provide them with information about current trends in anti-aging therapies. The program, which combined medical, surgical, and nonpharmacological approaches to healthy successful aging, gave attendees the opportunity to make sense of the issues at hand and to sort out safe treatments from perilous quick fixes.

A critical evaluation of long-term aesthetic outcomes of fronto-orbital advancement and cranial vault remodeling in nonsyndromic unicoronal craniosynostosis.

BACKGROUND: This study reports long-term aesthetic outcomes with fronto-orbital advancement and cranial vault remodeling in treating unicoronal synostosis over a 35-year period. METHODS: Retrospective review was performed on patients with isolated unicoronal synostosis from 1977 to 2012. Demographic, preoperative phenotypic, and long-term aesthetic outcomes data were analyzed with chi-squared and Fisher's exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis rank for continuous data. RESULTS: A total of 238 patients were treated; 207 met inclusion criteria. None underwent secondary intervention for intracranial pressure. At definitive intervention, there 96 (55 percent) Whitaker class I patients, 11 (6 percent) class II, 62 (35 percent) class III, and six (3 percent) class IV. Nasal root deviation and occipital bossing each conferred an increased risk of Whitaker class III/IV [OR, 4.4 (1.4 to 13.9), p = 0.011; OR, 2.6 (1.0 to 6.8), p = 0.049]. Patients who underwent bilateral cranial vault remodeling with extended unilateral bandeau were less likely Whitaker class III/IV at latest follow-up compared with those undergoing strictly unilateral procedures [OR, 0.2 (0.1 to 0.7), p = 0.011]. Overcorrection resulted in decreased risk of temporal hollowing [OR, 0.3 (0.1 to 1.0), p = 0.05]. Patients with 5 years or more of follow-up were more likely to develop supraorbital retrusion [OR, 7.2 (2.2 to 23.4), p = 0.001] and temporal hollowing [OR, 3.7 (1.5 to 9.6), p = 0.006] and have Whitaker class III/IV outcomes [OR, 4.9 (1.8 to 12.8), p = 0.001]. CONCLUSION: Traditional fronto-orbital advancement and cranial vault remodeling appears to mitigate risk of intracranial pressure but may lead to aesthetic shortcomings as patients mature, namely fronto-orbital retrusion and temporal hollowing. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Congenital nasal anomalies: a classification scheme.

The purpose of this work was to develop a simple yet comprehensive classification scheme dedicated to congenital nasal anomalies. To date, no such classification system has been proposed and widely used. A 22-year retrospective review was performed. Two hundred sixty-one patients with congenital nasal anomalies were identified. From this extensive database, a systematic morphogenic classification system was devised. Congenital nasal deformities were classified into four categories. Type I, hypoplasia and atrophy, represents paucity, atrophy, or underdevelopments of skin, subcutaneous tissue, muscle, cartilage, and/or bone. Type II, hyperplasia and duplications, representing anomalies of excess tissue, ranging from duplications of parts to complete multiples, are categorized here. In the type III category, clefts, the comprehensive and widely utilized Tessier classification of craniofacial clefts is applied. Type IV deformities consist of neoplasms and vascular anomalies. Both benign and malignant neoplasms are found in this category.

Immediate versus delayed midface distraction in a primate model using a new intraoral internal device.

The theoretic advantage of distraction osteogenesis of the craniofacial skeleton, especially in cases of severe midface retrusion and in the presence of maxillary scarring, is prevention of relapse following significant advancements. The purpose of this study is to demonstrate the utility of a new low-profile, intraoral, internal device for midface distraction at the conventional or high Le Fort I level. In addition, the present study compares the efficacy of immediate versus delayed distraction on subsequent maxillary relapse. Four adult rhesus Macaca mulatta monkeys were divided into two groups. Group 1 underwent immediate midface distraction; group 2 underwent delayed distraction. All four monkeys underwent a modified Le Fort I osteotomy through an upper buccal sulcus incision and bilateral application of the intraoral midface distraction devices. No other osteotomies or incisions were necessary. Immediate distraction, performed in group 1, entailed intraoperative activation of the devices and distraction of 10 mm followed by a 5-day lag period before postoperative activation and distraction of an additional 10 mm at the rate of 1 mm/day. Delayed distraction, performed in group 2, entailed a 5-day postoperative lag period before device activation and distraction of 20 mm at the rate of 1 mm/day. Both groups thus underwent 20 mm of midface distraction. All devices were removed 6 weeks after completion of distraction. All monkeys tolerated the devices and daily distraction uneventfully. On the basis of serial cephalograms and dental models obtained throughout the experimental period, there was no evidence of relapse in either the immediate or delayed groups 6 months after distraction. In addition, on the basis of histologic, ultrastructural, and dry skull analysis, no significant differences were observed in the quality of regenerate bone obtained when comparing the immediate and delayed distraction groups. Significant midface advancement is thus feasible using this new internal, intraoral distraction device, which presents several advantages over other internal devices that require coronal incisions and additional osteotomies to achieve midface advancement. In addition, immediate distraction may abbreviate the distraction period without adverse sequelae.

Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity.

An algorithm for the management of sagittal synostosis in older children who underwent delayed cranial vault reconstruction is presented. This algorithm tailors the surgical approach to the specific craniofacial deformity present in each case. The scaphocephalic deformity characteristic of sagittal synostosis varies significantly when presentation is delayed beyond the first year of life, the time during which reconstruction is usually performed. Sixteen patients with sagittal synostosis who presented after 12 months of age, and were a mean of 3.2 years of age at the time of cranial vault reconstruction, were reviewed. Four patients demonstrated preoperative symptoms and objective findings indicative of increased intracranial pressure, including frequent headaches and emesis, papilledema, or digital markings on computed tomographic scan. Each of the 16 patients underwent either (1) single-stage total vault reconstruction with or without concomitant fronto-orbital expansion; (2) two-stage total vault reconstruction with anterior two-thirds vault expansion followed by transverse occipital expansion and recession a mean of 8.7 months later; or (3) anterior two-thirds vault reconstruction with or without fronto-orbital expansion. In each case, the extent of the scaphocephalic deformity determined the procedure used. The presence of severe frontal bossing associated with transverse restriction of the orbitotemporal region was an indication for fronto-orbital expansion in addition to vault reconstruction, whereas significant occipital protrusion was an indication for transverse posterior vault expansion and recession in addition to anterior two-thirds vault reconstruction. Excellent aesthetic results were obtained in all cases regardless of the type of reconstruction performed. However, it is essential that the extent of the deformity be carefully evaluated preoperatively to permit selection of the appropriate technique for reconstruction.

Body image concerns of breast augmentation patients.

This study investigated the body image concerns of women who sought cosmetic breast augmentation. Thirty breast augmentation candidates completed several measures of body image before their initial surgical consultation. Thirty physically similar women who were not interested in breast augmentation were recruited from the medical center and university community and also completed the measures. Breast augmentation candidates, as compared with women not seeking augmentation, reported greater dissatisfaction with their breasts. Augmentation candidates rated their ideal breast size, as well as the breast size preferred by women, as significantly larger than did controls. In addition, women interested in breast augmentation reported greater investment in their appearance, greater distress about their appearance in a variety of situations, and more frequent teasing about their appearance. Finally, breast augmentation candidates also reported more frequent use of psychotherapy in the year before the operation as compared with women not seeking augmentation. These results replicate and extend previous studies of body image in cosmetic surgery patients.

Metopic synostosis: Defining the temporal sequence of normal suture fusion and differentiating it from synostosis on the basis of computed tomography images.

Only the metopic suture normally fuses during early childhood; all other cranial sutures normally fuse much later in life. Despite this, metopic synostosis is one of the least common forms of craniosynostosis. The temporal sequence of normal physiologic metopic suture fusion remains undefined and controversial. Therefore, diagnosis of metopic synostosis on the basis of computed tomography images alone can prove misleading. The present study sought to determine the normal sequence of metopic suture fusion and characterize both endocranial and ectocranial suture morphology. An analysis of computed tomography scans of 76 trauma patients, ranging in age from 10 days to 18 months, provided normative craniofacial data that could be compared to similar data obtained from the preoperative computed tomography scans of 30 patients who had undergone surgical treatment for metopic synostosis. Metopic suture fusion was complete by 6 to 8 months in all nonsynostotic patients, with initiation of suture fusion evident as early as 3 months of age. Fusion was found to commence at the nasion, proceed superiorly in progressive fashion, and conclude at the anterior fontanelle. Although an endocranial ridge was not commonly seen in synostotic patients, an endocranial metopic notch was virtually diagnostic of premature suture fusion and was seen in 93 percent of synostotic patients. A metopic notch was not seen in any nonsynostotic patient. The morphologic and normative craniofacial data presented permit diagnosis of metopic synostosis based on computed tomography images obtained beyond the normal fusion period.

Craniosynostosis and altered patterns of fetal TGF-beta expression induced by intrauterine constraint.

Recent work has demonstrated that fusion of the calvarial sutures is mediated by locally elaborated soluble growth factors, including the transforming growth factor-betas (TGF-betas), leading some to speculate that external biomechanical forces play little role in suture development. Clinical evidence has long suggested, however, that fetal head constraint may play a critical role in the pathogenesis of many cases of nonsyndromic craniosynostosis. The purpose of these experiments was to test the hypothesis that intrauterine constraint leads to an alteration in normal patterns of TGF-beta expression and that these alterations are associated with craniosynostosis. Fetal constraint was induced by allowing C57Bl/6 murine fetuses to grow for 2.5 days beyond the normal 20-day gestation by performing uterine cerclage on the eighteenth day. Cranial suture morphology was examined in hematoxylin and eosin-stained sections and in cleared whole-mount specimens, double stained with alizarin red S and Alcian blue. Expression patterns of TGF-beta1 and TGF-beta3 were examined by immunohistochemical techniques. Gross and microscopic examination of the cranial sutures of 17 constrained fetuses revealed changes that ranged from narrowing to complete osseous obliteration of the coronal and squamosal sutures. All sutures of 14 nonconstrained control pups remained patent. Fetal head constraint was associated with increased TGF-beta1 immunoreactivity within the new bone and the underlying dura when compared with nonconstrained age-matched controls. TGF-beta3 immunoreactivity was associated with the dura underlying patent, nonconstrained sutures, whereas constraint-induced synostosis was characterized by down-regulation of dural TGF-beta3 expression. These experiments confirm the ability of intrauterine constraint to induce premature fusion of the cranial sutures and provide evidence that intrauterine head constraint induces the expression of osteogenic growth factors in fetal calvarial bone and the underlying dura.

An evaluation of complications, revisions, and long-term aesthetic outcomes in nonsyndromic metopic craniosynostosis.

BACKGROUND: The authors evaluated the complications, revisions, and long-term aesthetic outcomes of patients with isolated metopic synostosis. METHODS: A retrospective chart review was performed on consecutive metopic craniosynostosis patients treated from June of 1987 to June of 2012 at The Children's Hospital of Philadelphia. Patient demographics, operative details, and postoperative data were collected. Outcomes were reported as Whitaker classification and postoperative clinical characteristics assessed before additional interventions. Reoperation in patients with greater than 5 years of follow-up was noted. Appropriate statistical analyses were applied. RESULTS: From 1987 to 2012, 178 patients underwent surgical correction of isolated metopic craniosynostosis, and 147 met inclusion criteria. Average age at surgery was 0.83 year (range, 0.3 to 4.7 years); average follow-up was 5.8 years (range, 1.0 to 17.8 years). There were 13 surgical complications (8.8 percent), three major (2.0 percent), and 10 minor (6.8 percent). At follow-up, 67 patients (56.8 percent) were classified as Whitaker class I, six (5.1 percent) as class II, 43 (36.4 percent) as class III, and two (1.7 percent) as class IV. Patients with greater than 5 years' follow-up (n = 57) were more likely to have temporal hollowing (OR, 2.9; 95 percent CI, 1.2 to 7.3; p = 0.021), lateral orbital retrusion (OR, 4.9; 95 percent CI, 1.9 to 12.7; p = 0.001), and Whitaker class III or IV classification (OR, 4.0; 95 percent CI, 1.5 to 10.6; p = 0.006) compared with those with less than 5 years' follow-up. CONCLUSION: This study reports low complication and reoperation rates in the treatment of isolated metopic craniosynostosis, but demonstrates a clear trend toward worsening aesthetic outcomes over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Soft-tissue refinements in the treatment of unicoronal craniosynostosis: the rotational forehead flap.

Unicoronal craniosynostosis is characterized by ipsilateral superior and posterior displacement of the supraorbital rim and frontal bone, ipsilateral widening of the palpebral fissure, ipsilateral superior displacement of the brow, and contralateral brow depression. In the literature, surgical treatment has focused on bony anatomy, with little written about soft-tissue correction. Over the last 25 years, the senior author (L.A.W.) has incorporated soft-tissue refinements, including a rotational flap of the forehead, elevating the contralateral brow, depressing the ipsilateral brow, and equilibrating the supratarsal sulci in unicoronal craniosynostosis. This rotational forehead repositioning is a simple innovation that has provided for improved orbital and brow symmetry on long-term follow-up.

Normocephalic pancraniosynostosis resulting in late presentation of elevated intracranial pressures.

BACKGROUND: Pancraniosynostosis presents with premature fusion of three or more major cranial sutures. The four presentations of primary pancraniosynostosis are kleeblattschädel, complex, progressive, and normocephalic. This study compares the clinical presentation and treatment course of patients with normocephalic presentation against the other pancraniosynostosis groups. METHODS: The authors conducted a retrospective chart review of patients who presented to the craniofacial clinic at The Children's Hospital of Philadelphia with primary pancraniosynostosis born between January 1, 2000, and February 1, 2009. Patients with secondary causes of pancraniosynostosis were excluded. Data extracted included presenting symptoms, head shape, cranial sutures involved, and presence of clinical and radiologic evidence of elevated intracranial pressure. Surgical intervention(s) and the subsequent clinical course were noted. The between-group difference in the initial age of presentation and age at first cranial vault surgery were compared using the Mann-Whitney U test. RESULTS: There were 17 patients with primary pancraniosynostosis: four kleeblattschädel, eight complex, one progressive, and four normocephalic. The average age at presentation and length of follow-up were 1.1 years and 3.8 years, respectively. Patients with normocephalic pancraniosynostosis presented at an average age of 3.5 years compared with 2 months in the other three groups (p = 0.01). The age at first cranial vault remodeling surgery for the normocephalic pancraniosynostosis group was 4 years 8 months compared with 11 months in the other three groups (p = 0.01). CONCLUSIONS: Patients with normocephalic pancraniosynostosis have an insidious clinical course. Because of their indistinct cranial morphology, they present late with significant symptoms of elevated intracranial pressure requiring urgent surgical decompression.