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Background A patient's health-related quality of life can be significantly impacted by facial scarring and disfigurement. Facial soft-tissue reconstruction should aim to improve this, with outcomes measured from the patient's perspective using patient-reported outcome measures. This systematic review identifies patient-reported outcome measures for soft-tissue facial reconstruction and appraises their methodologic and psychometric properties using up-to-date methods. Methods A systematic search of the MEDLINE, Embase, PsychINFO, and Cochrane databases was performed. Identified patient-reported outcome measures were assessed using the updated Consensus-Based Standards for the Selection of Health Measurement Instruments checklist. Psychometric properties were also assessed and a modified Grading of Recommendation Assessment, Development and Evaluation analysis was performed to aid in recommendations for future questionnaire use. Results Thirty-four studies covering nine patient-reported outcome measures were included. Methodologic quality and psychometric evidence were variable. FACE-Q, Skin Cancer Index, Patient Outcome of Surgery-Head/Neck, and the Derriford Appearance Scale 59/24 all demonstrated high enough evidence to be recommended as having potential for inclusion in future studies. Conclusions This is the first systematic review to identify and critically appraise patient-reported outcome measures for soft-tissue facial reconstruction using internationally accepted criteria. Four questionnaires were deemed to have adequate levels of methodologic and psychometric evidence, although further studies should be conducted before they are used routinely in patients undergoing facial reconstruction. Through the use of psychometrically well-validated questionnaires, it is hoped that patients' concerns can be truly appreciated, the level of care improved, and the quality of reconstructive options offered advanced.

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.

Background: Effective postoperative monitoring of the vascular pedicle to a free flap can potentiate rapid return to the operating room in the setting of compromise, allowing for the potential to salvage the flap. The only ubiquitous method for postoperative monitoring of free flaps is clinical bedside monitoring, but although the use of clinical monitoring may be inferred in large reported series of free flaps, there has been little discussed in the literature of specific clinical outcome measures. Methods: The authors present their experience with 1140 consecutive cases of free tissue transfer and the use of clinical monitoring as a sole method of monitoring, and subgroup analysis of different recipient sites. Results: There were 94 take-backs, four of which had no pedicle compromise (false-positives) and there were four false-negatives. The overall flap salvage rate was 62.8 percent and the false-positive rate was 0.4 percent. Subgroup analyses demonstrated statistically significant differences between recipient sites for the false-positive rates: fewer false-positives with breast reconstruction cases (p < 0.05) and significantly more false-positives in the extremity group (p < 0.05). There was an improved flap salvage rate in cases of venous compromise compared with arterial compromise (69 percent versus 51 percent, p = 0.015). Conclusions: This largest reported series to date provides an outcome-based analysis of postoperative monitoring for free flaps, providing a benchmark standard against which adjunctive monitoring techniques can be compared. Future studies need to be assessed in the context of individual recipient sites, with significant differences in monitoring outcomes between sites.

Background: Complete loss of eyelid pair is associated with chronic discomfort, corneal ulceration, and visual impairment. Contemporary reconstructive techniques rarely provide functionally acceptable results. Composite tissue allotransplantation may provide a viable alternative. This study reports on neurovascular anatomy and technical details of harvesting an isolated periorbital unit and discusses its functional potential. Methods: Twenty-four hemifaces (12 fresh cadavers) were dissected to study surgically relevant neurovascular structures and to develop an efficient harvest method. Angiographic analysis was performed in seven hemifaces following harvest. Results: The superficial temporal and facial vessels demonstrated consistent location and diameters. Anatomic variability was characterized by the absence of the frontal branch of the superficial temporal artery or facial-to-angular artery continuation, but never of both vessels in the same hemiface. Angiographic analysis demonstrated filling of the eyelid arcades, provided the anastomoses between the internal and external carotid branches were preserved. The facial nerve exhibited consistent planar arrangement and diameters in the intraparotid and proximal extraparotid regions, but less so in the distal nerve course. The inferior zygomatic and buccal branches frequently coinnervated the orbicularis oculi and lower facial muscles with an unpredictable intennuscular course. Based on the foregoing, an effective surgical harvest of the periorbital composite was developed. Conclusions: Surgical harvest of a functional periorbital allotransplant is technically feasible. Revascularization of the isolated periorbital unit is influenced by variations in regional anatomy and cannot be guaranteed by a single vascular pedicle. The orbicularis oculi muscle and its innervation can be preserved, and recovery, albeit without the certainty of reflexive blinking, is expected.

Background: Patients with Saethre-Chotzen syndrome have a heterogeneous phenotype. The purpose of this study was to use the genotypic diagnosis of the authors' series of patients with TTWST1-confirmed Saethre-Chotzen syndrome to describe their natural history and long-term surgical outcomes. Methods: A retrospective chart review was performed on patients treated at The Children's Hospital of Philadelphia with TWIST1-confirmed Saethre-Chotzen syndrome (n = 22) over 23 years (1985 to 2008). Their phenotype, need for primary cranial vault remodeling surgery, and subsequent need for reoperation were recorded. Genetic records were reviewed to identify each patient's TWIST1 mutation. Results: There were nine female patients and 13 male patients. Ten had bi-coronal (45 percent), six had unicoronal (27 percent), and four had multisuture (18 percent) craniosynostosis. One had metopic and another had sagittal craniosynostosis. Average follow-up was 7.6 years (range, birth to 19.6 years). Seventeen (77 percent) underwent initial cranial vault remodeling and 10 (59 percent) required repeat intracranial vault remodeling (Whitaker class IV). One patient required major reoperation with bone grafting (Whitaker class III). Three patients needed minor revision procedures (Whitaker class II). Three patients needed no further intervention (Whitaker class I) . The locations of the TWIST1 gene mutations in this study did not correlate to a specific surgical outcome. Conclusions: TWIST1-confirmed Saethre-Chotzen syndrome is heterogeneous and manifests as either mild or severe craniofacial deformities. Our patients with TWIST1-confirmed Saethre-Chotzen syndrome had a reoperation rate of 65 percent for Whitaker class III and IV surgical outcome, and 59 percent required a secondary intracranial procedure for recurrent supraorbital retrusion.

Background The ability of the immature skull to spontaneously heal large bony defects created after craniofacial procedures was examined over a 25-year period of craniofacial surgery at the Children's Hospital of Philadelphia. Methods Only patients who underwent frontal orbital advancement and reconstruction, had at least 1 year of documented follow-up, and had the presence or absence of a bony defect documented on clinical examination were included. The sex, age at operation, diagnosis, history of a prior craniectomy, and presence or absence of a postoperative infection were determined for each patient. A variety of statistics were applied to the data. Results Eighty-one patients met the inclusion criteria. A statistically significant association between age at operation and closure of bony defect was demonstrated. Children who closed a bony defect after frontal orbital advancement and reconstruction were significantly younger than those children who had a persistent bony defect. Iterative regression analyses demonstrated that a transition point between closure and the inability to close bony defects occurred between 9 and 11 months of age. Closure of bony defects was not statistically associated with sex, prior craniectomy, an FGFR mutation, or a postoperative infection in the regression analysis. Conclusions Healing of bony defects after frontal orbital advancement and reconstruction is significantly related to age at initial operation, with a mean age for closure of less than 12 months. Between 9 and 11 months of age, a change occurs that results in an increasingly lower probability of bony defect closure; thus, all other considerations being equal, initial frontal orbital advancement and reconstruction would ideally take place before this occurs.

This retrospective chart review study examined the mental health histories and psychiatric medication usage of persons who sought cosmetic surgery. The medical records of 232 patients who had undergone a consultation for a cosmetic procedure were reviewed. The rates of self-reported psychiatric disorders and of psychiatric medication usage of these patients were compared with those of 200 patients who underwent noncosmetic procedures. Nineteen percent of cosmetic surgery patients reported a mental health history, significantly greater than the 4 percent of the noncosmetic patients who reported a psychiatric history. Eighteen percent of the cosmetic patients reported using a psychiatric medication at the time of their consultation, also significantly greater than the 5 percent of the noncosmetic patients who reported psychiatric medication usage. These results underscore the importance of collecting a psychiatric history from prospective cosmetic surgery patients. Recommendations for assessing patients' psychiatric history and status are provided.

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.

Clinical teaching dictates that isolated unicoronal synostosis is sporadic in occurrence and is possibly related to intrauterine constraint. Despite this, isolated reports document a familial occurrence. It has previously been recognized that there may be a familial pattern of inheritance. Recently, mutations in fibroblast growth factor receptors (FGFRs) have been implicated in several syndromic craniosynostoses. At the authors' institution, mutations in FGFR3, located at chromosome 4p16, have been found to cause coronal synostosis. Two cases of unicoronal synostosis were found to have the same Pro250Arg missense mutation in FGFR3. This finding suggested that all patients with a diagnosis of unicoronal synostosis be screened for the FGFR3 mutation. Between January and December of 1996, patients with a diagnosis of plagiocephaly at the Children's Hospital of Philadelphia were evaluated for the FGFR3 mutation. Thirty-seven patients with unicoronal synostosis had mutational studies. Two additional patients were known to have the FGFR3 mutation at the onset of the study. Of the 37 patients screened, four were found to have the FGFR3 mutation, for a total of six patients with both unicoronal synostosis and the FGFR3 mutation. All patients with unicoronal synostosis were evaluated for facial dysmorphology and operative outcome. The six patients with the FGFR3 mutation had more severe cranial dysmorphology and were more likely to need surgical revision than those without the FGFR3 mutation. The occurrence of the FGFR3 mutation among patients with unicoronal synostosis provides evidence for a genetic basis of certain forms of plagiocephaly. The clinical, radiologic, and molecular findings will be an important addition to the surgical management and counseling of patients with unicoronal synostosis.

This study represents an initial investigation into the adult psychological functioning of individuals born with craniofacial disfigurement. A total of 24 men and women born with a craniofacial anomaly completed paper and pencil measures of body image dissatisfaction, self-esteem, quality of life, and experiences of discrimination. An age- and gender-matched control group of 24 non-facially disfigured adults also completed the measures. As expected, craniofacially disfigured adults reported greater dissatisfaction with their facial appearance than did the control group. Craniofacially disfigured adults also reported significantly lower levels of self-esteem and quality of life. Dissatisfaction with facial appearance, self-esteem, and quality of life were related to self-ratings of physical attractiveness. More than one-third of craniofacially disfigured adults (38 percent) reported experiences of discrimination in employment or social settings. Among disfigured adults, psychological functioning was not related to number of surgeries, although the degree of residual facial deformity was related to increased dissatisfaction with facial appearance and greater experiences of discrimination. Results suggest that adults who were born with craniofacial disfigurement, as compared with non-facially disfigured adults, experience greater dissatisfaction with facial appearance and lower self-esteem and quality of life; however, these experiences do not seem to be universal.

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.

Issues of radiolucency and biocompatibility of currently available mammary implants have prompted the search for alternatives. Several new filler materials have been suggested recently but have involved the use of materials foreign to the body. We have studied the use of a naturally found polysaccharide molecule, hyaluronic acid, as an alternative filler material to silicone gel. We tested hyaluronic acid-filled implants using standard mammographic techniques, applanation tonometry, and in an in vivo animal model (n = 24) up to 1 year after implantation. The present study demonstrates that hyaluronic acid-filled implants have softness comparable with that of silicone gel and saline implants and are more radiolucent, allowing better visualization of breast structures around the implant. Furthermore, in vivo studies fail to demonstrate any adverse reactions to the material over a period of 1 year. Hyaluronic acid has unique properties in modulating the process of wound healing, and these properties may be applied to the tissues surrounding the implants as a result of leaching of hyaluronic acid through the covering shell. Although further studies using larger volumes of filler, characterization of the hyaluronic acid within the implant, quantification of the exact amounts of hyaluronic acid leached into surrounding tissues, and a more appropriate primate model need to be undertaken, this pilot study points out that there may be more biologically compatible materials for the use in breast implants that warrant further investigation.

To determine the most desirable ear level (vertical position), a set of four drawings was assessed by 40 observers. Two levels were almost equally- preferred when viewed in profile, one with the upper edge of the ear at the level of the most lateral point of the eyebrow and the other with the upper edge at the height of the upper eyelid. Various factors affecting the visual impression of auricular level and their clinical significance are discussed

Certain procedures for facial advancement may carry greater risk than others. While many believe that separating the cranial base by monobloc advancement leads to a higher complication rate, no comparative series between the Le Fort III and monobloc advancements has ever been reported. We reviewed our series of these different techniques. Over a 15-year period, 29 patients underwent 30 surgical procedures, with either a midfacial or frontofacial advancement. The average age of patients at the time of surgery was 12 years, with a range from 3 to 26 years. There were 20 Le Fort III and 10 monobloc advancements. Follow-up averaged 4 years, with a range from 10 weeks to 13 years. There were no deaths in this series. The infectious complications differed significantly between the two groups, with all major infections occurring in the monobloc group. The noninfectious complications (2 major and 20 minor) were proportionately distributed between the Le Fort III and monobloc groups. Aesthetic results of the midface, judged by the percentage of revisions necessary, were found to be the same between the two procedures. Aesthetic results were noted to correlate strongly with age at the time of surgery, with the older patients being judged as having a better aesthetic result and most of the younger patients requiring a repeat of the facial advancement. We conclude that while we were unable to determine any definitive aesthetic advantage of one procedure over the other in our series, there was a significantly higher infection rate with the monobloc advancement. On the basis of these results, we recommend a staging of the forehead and midfacial advancements.

Massive teratomas involving the cranial base are extremely rare, and to our knowledge, there are no reports of this condition in surviving children. We describe the management of four cases of massive intracranial teratomas, reflecting a combined experience from two major referral centers. We found that when corrected at an early age, the brain parenchyma demonstrated a remarkable ability to reexpand in those children who had appeared to have sustained parenchymal deficits secondary to the mass effect from the tumor. Follow-up thus far suggests that regional skull growth potential may be compromised in some of these patients. Often, the exact pathologic diagnosis of these entities is somewhat different, since they may fall somewhere within the hamartoma-heterotopia-teratoma continuum. We believe that the best care of these children is offered through a combined simultaneous craniofacial and neurosurgical approach.

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.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.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.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.

Background: Craniofacial microsomia is one of the most common and well-characterized craniofacial anomalies. Tongue dysmorphism, however, has been neither thoroughly investigated nor reported in the context of this disease. This review focuses on the true prevalence of tongue dysmorphology in craniofacial microsomia and its relation to the deformities seen in this condition. Methods: A 20-year retrospective study was performed to determine the number of patients who had a documented tongue anomaly and any relation to the development of abnormal speech. In recognition of the limitations of this approach, a 1-year prospective study was also performed to see the true prevalence of tongue dysmorphology in these patients. Results: Eight of 167 patients (4.8 percent) in the retrospective study were found to have tongue dysmorphologies, as opposed to 24 of 55 (43.6 percent) in the prospective study. The majority of tongue anomalies were mild. Of the eight retrospective patients, seven currently have intelligible speech with a combination of intensive speech therapy and/or surgical correction. The eighth patient is without intelligible speech. Tongue dysmorphology was positively correlated with the degree of hard- and soft-tissue deformity. Conclusions: Tongue dysmorphologies in craniofacial microsomia, although usually mild, are frequently overlooked. The correlation of the tongue, soft tissue, and mandible anomalies may point to a common error early in gestation or an interdependence of adjacent growth centers.

Background: Posterior vault reconstruction involves exposure, craniotomy, and reconstruction of the occipital cranium. Although it is commonly performed, the safety and complications associated with posterior vault reconstruction have not been specifically or thoroughly examined. Methods: A retrospective chart review was performed for all patients undergoing posterior vault reconstruction at the Children's Hospital of Philadelphia between 1996 and 2006. Diagnosis, operative indication, age and weight at time of surgery, procedure type, intraoperative and postoperative complications, estimated blood loss, blood transfusion volume, and length of surgery and admission were recorded. Results: Fifty posterior vault reconstruction cases were included in the study (17 syndromic and 33 nonsyndromic). Mean estimated blood loss was 53.6 percent (range, 15.9 to 142.9 percent) of total blood volume and was statistically greater in syndromic patients (60.6 percent versus 50.0 percent; p = 0.038). Transfusion volume averaged 66.1 percent of total blood volume but was not statistically significant. Length of surgery and number of intraoperative complications were significantly elevated in the syndromic cohort (p = 0.007 and p = 0.037, respectively), whereas length of admission and number of postoperative complications were not. Complications were intraoperative in seven patients (14 percent) and postoperative in seven (14 percent), for an overall complication rate of 28 percent. Conclusions: This is the largest series to date addressing the safety and complications of posterior vault reconstruction. In the authors' experience, posterior vault reconstruction carries a higher risk of intraoperative complications and blood loss compared with that reported in the literature for other types of cranial vault reconstruction. In addition, syndromic patients were more likely than nonsyndromic patients to encounter intraoperative problems.

Rigid fixation of the craniofacial skeleton has proven of great value in adult orthognathic and traumatic reconstructive surgical procedures. This technique has gained increased acceptance in the surgical treatment of infants and young children with congenital malformations, despite the fact that its effects on subsequent craniofacial growth are unknown. To examine this question, an experimental model using 25 young kittens was developed to compare rigid fixation with conventional wire fixation, with and without osteotomy. Our findings demonstrate a regional restriction of growth in the developing craniofacial skeleton when both wire and plate and screw fixation are utilized in concert with osteotomy. Further, a compensatory growth was observed in individual animals when plate fixation was utilized that was not seen in the wire-treated group. This suggests that there is a dynamic growth interaction between restriction and compensation in this setting.

The posterior mandible begins just behind the mental nerve and second bicuspid bilaterally, extends to the posterior edge of the ramus, and then runs superiorly to the zygomatic arch. Augmentation of the posterior mandible is possible by use of a synthetic implant that is tailored individually to each patient's specific needs. Implant plant thickness varies from 4 to 8 mm, with an average thickness of 6 mm. Careful preoperative planning is done based on an aesthetic assessment of the amount of highlighting desired, thickness of the soft tissues, and the use of life-size photographs and cephalometric and Panorex x-rays. A pattern is cut, and the implant is carved to fit the patient. Insertion of the material after careful tailoring to the individual patient's own mandibular size and configuration requires a generous posterior lower buccal sulcus incision. Antibiotic irrigation and systemic antibiotics are essential, and careful closure in two layers completes the procedure. One implant in the series extruded in a patient who had had radiation therapy, and one patient required repositioning of the implant. Otherwise, in 22 patients there were no infections or permanent morbidity. The procedure seems to be a realistic and safe one for both the youthful and aging face, as demonstrated in patients in this series, with ages varying from 16 to 40 years.

We present what we believe is the first case of a brain abscess resulting from orthognathic surgery reported in the literature. Although recent advances have significantly improved the treatment of brain abscesses, delays in diagnosis lead to persistent high rates of mortality. Often, the initial symptoms are vague and nonspecific. Commonly, the neurologic status of the patient deteriorates significantly before suspicion is raised and a CT scan is obtained. This case report is intended to benefit all practitioners who participate in the perioperative care of orthognathic surgery patients. Although the occurrence of a brain abscess is rare, it is hoped that this case will heighten the awareness of clinicians to this potential complication.

Background Slit ventricle syndrome and postshunt craniosynostosis are uncommon complications after shunting procedures for congenital hydrocephalus. Slit ventricle syndrome occurs as a complication in 1 to 5 percent of patients after shunting procedures for hydrocephalus during infancy. These patients usually have had a shunt in place for years, with overdrainage of cerebrospinal fluid resulting in very small ventricles. Excessive intracranial decompression may result in ventricular collapse with the formation of slit-like ventricles and cranial vault collapse with secondary craniosynostosis. Methods A retrospective review of 12 patients who had undergone cranial vault expansion for management of postshunt craniosynostosis and slit ventricle syndrome refractory to other treatment modalities was performed. All patients had initially been shunted for congenital hydrocephalus and subsequently underwent a mean of 4.9 shunt revisions before cranial vault expansion. Results All 12 patients demonstrated decreased ventricular volume, with noncompliant slit-like ventricles in nine of these patients. Five patients demonstrated pancraniosynostosis, three patients demonstrated multiple sutural fusion, and isolated sagittal synostosis occurred in two patients. Two patients appeared to have "functional" synostoses with narrowed, overlapping sutures that were not actually fused. Eleven patients underwent bilateral fronto-orbital advancement with frontotemporoparietal expansion; one patient underwent only posterior vault expansion. Seven of the 12 patients required a mean of 2.3 shunt revisions after cranial vault expansion; five patients did not require subsequent revisions. Improvement in cranial vault shape was achieved in all 12 patients, as was improvement of neurologic symptoms in nine of the 12 patients. Conclusions Cranial vault expansion is a useful approach in the management of the restricted cranium associated with slit ventricle syndrome and postshunt craniosynostosis. This approach resulted in a decreased need for shunt revisions and improvement of neurologic symptoms and cranial vault shape.

Purpose Classic literature indicates an infrequency of metopic craniosynostosis (3 to 10 percent) compared to other single-suture craniosynostosis. Recent observation challenges these conceptions, warranting long-term demographic analysis. Methods Syndromic craniofacial dysostoses and multiple suture involvement were exclusion criteria, leaving only single suture synostoses treated between 1975 and 2004. A chart review was performed and patient information recorded. Chi square analysis and Fisher's exact were used to determine differences in patient characteristics. A Moran's I statistic was used to determine differences in spatial means and whether changes in incidence of single-suture synostoses over time are a function of regional phenomena. Results Over 800 patients presented to The Children's Hospital of Philadelphia with a diagnosis of single-suture synostosis. There was an outpacing of sagittal suture involvement over other sutures. Data demonstrate a decrease in unicoronal synostosis and an increase in metopic synostosis (p = 0.011). Geostatistical analysis reveals increasing separation between populations over the study period: 9.8 miles in the first 5 years to 20.8 miles in the last 5 years. Metopic maternal age increased between 1975-1989 and 1990-2004 (p = 0.002, 0.0002), while unicoronal maternal age did not. The proportion of male patients increased considerably in the metopic group compared to unicoronals from 1990-2004 (p = 0.0001), as did the proportion of syndromic metopic patients (p = 0.02). Plausible etiologies for these epidemiological shifts are discussed. Conclusions Metopic synostosis is on the rise. Changing demographic bases and increasing proportions of syndromic patients may be clues to the etiology of this epidemiologic event.

Background Fronto-orbital reconstruction is the standard of care for repair of unilateral coronal synostosis. This study examined the relationship between age at initial surgery and need for secondary surgery, to determine the optimal age for reconstruction. Methods The series comprised 81 patients with unicoronal synostosis who underwent reconstruction over a 30-year period by a single surgeon. Measured outcomes included reoperation rate, relapse/defect rate, relapse interval, and problem rate. Results Average age at surgery was 11.3 months (range, 1.5 to 71 months). The problem rate was 27.2 percent. Mean follow-up was 69 months (range, 6 to 234 months). The postoperative defect rate was 73 percent. Sixteen patients (20 percent) underwent reoperation to correct secondary deformities. Whitaker category I or II results were achieved in 90.1 percent. Problem rates for patients aged 0 to 6 months, 6 to 12 months, and older than 12 months were 13.3, 28, and 35, respectively. Secondary surgery was performed in 40 percent of patients whose first surgery was at 0 to 6 months, 7 percent aged 6 to 12 months, and 30.4 percent older than 12 months. After surgery, 87 percent of patients aged 0 to 6 months, 95 percent aged 6 to 12 months, and 87 percent older than 12 months had Whitaker category I or II results. Conclusions Fronto-orbital advancement at younger than 6 months had the highest incidence of readvancement. Patients treated at 6 to 12 months had the least need for any secondary surgery; however, patients older than 12 months had the lowest incidence of readvancement. These results indicate that fronto-orbital advancement should be delayed until at least age 6 months to avoid relapse.

Both the safety and efficacy of the treatment of isolated craniofacial dysostosis (plagiocephaly) in infancy have been demonstrated. Opinions remain divided, however, as to the optimal type of procedure to be undertaken. In an attempt to answer this question, we have retrospectively evaluated a study population of 48 children operated on in infancy by either a unilateral or bilateral approach. Results of treatment at a minimal follow-up of 3 years were assessed based on preoperative and postoperative photographs and direct patient examination. Based on this retrospective comparison of the unilateral and bilateral approaches to the treatment of isolated craniofacial dysostosis, we conclude that (1) either approach as specifically outlined will give excellent results in the majority of patients, (2) there is no statistically significant difference in the results obtained by using either procedure, (3) in the majority of instances, less than ideal correction was manifested by contour irregularities evident in the temporal and/or lateral forehead region, and strict attention should be given to these areas in an attempt to further improve results, and (4) in those cases where significant protrusion is observed on the "normal side," a bilateral approach is preferable.