Single suture craniosynostosis: Identification of rare variants in genes associated with syndromic forms.

We report RNA-Sequencing results on a cohort of patients with single suture craniosynostosis and demonstrate significant enrichment of heterozygous, rare, and damaging variants among key craniosynostosis-related genes. Genetic burden analysis identified a significant increase in damaging variants in ATR, EFNA4, ERF, MEGF8, SCARF2, and TGFBR2. Of 391 participants, 15% were found to have damaging and potentially causal variants in 29 genes. We observed transmission in 96% of the affected individuals, and thus penetrance, epigenetics, and oligogenic factors need to be considered when recommending genetic testing in patients with nonsyndromic craniosynostosis.

Defining and Correcting Asymmetry in Isolated Unilateral Frontosphenoidal Synostosis: Differences in Orbital Shape, Facial Scoliosis, and Skullbase Twist Compared to Unilateral Coronal Synostosis.

INTRODUCTION: Isolated frontosphenoidal synostosis (FS) is a rare cause of fronto-orbital plagiocephaly that can be challenging to distinguish from isolated unicoronal synostosis (UC). The purpose of this paper is to analyze differences in fronto-orbital dysmorphology between the 2 conditions, to describe approaches for surgical correction, and to report surgical outcomes between FS and UC patients in a casecontrol fashion. METHODS: Patients treated for craniosynostosis over a 12-year period at our institution were retrospectively evaluated under institutional review board approval. Frontosphenoidal synostosis patients who underwent bilateral fronto-orbital correction of anterior plagiocephaly with minimum 2-year follow-up, adequate pre-, and minimum 2-year postoperative computed tomography scans were included in the case-control portion of the study. These patients were randomly age-matched to UC patients meeting the same inclusion criteria. Preoperative and postoperative orbital shape and volumetric analysis was performed using Mimics software. RESULTS: Twelve FS patients were treated during the study period. Seven of these patients met casecontrol inclusion criteria with average follow-up of 47.5 months. The characteristic FS orbit was a relatively wide, short, and shallow trapezoid, while the characteristic UC orbit was a relatively narrow, tall, and deep parallelogram. Frontosphenoidal synostosis orbits were significantly wider, shorter, shallower, and smaller than UC orbits. Surgical correction tailored to the differential dysmorphologies resulted in statistical equalization of these differences between affected and contralateral control orbits at follow-up, with the exception of UC orbital width, which remained significantly narrower than unaffected contralateral control. One patient in each group required cranioplasty for skull defects at follow-up, while no patient underwent surgical readvancement. CONCLUSIONS: Frontosphenoidal synostosis and UC orbital shape differ significantly, and can be normalized using fronto-orbital advancement tailored to the distinct orbital dysmorphologies of these 2 groups.

Safety of Open Cranial Vault Surgery for Single-Suture Craniosynostosis: A Case for the Multidisciplinary Team.

INTRODUCTION: Single Suture Craniosynostosis (SSC) occurs in 1 in 2,500 live births and is the most common type of craniosynostosis treated in most centers. Surgical treatment has evolved over the past century and open techniques are tailored to the specific suture type. Additionally, the concept of multi-disciplinary team care has proliferated and is becoming the standard of care for SSC. The combination of these evolutions, we believe, has improved the safety of cranial vault surgery for SSC. METHODS: A retrospective review of patients participating in the Infant Learning Project at Seattle Children's Hospital who underwent cranial vault surgery for treatment of SSC between 2002 and 2006 was performed. Pre-operative assessment, surgical techniques, anesthetic and intraoperative events and both intra-operative and post-operative adverse events were analyzed. RESULTS: Eighty eight patients fulfilled the inclusion criteria (42 sagittal, 23 metopic, 19 unicoronal, 4 lambdoid). Length of procedure varied (FOA 5.2 hrs, modified pi 2.5 hrs, total vault 4.9 hrs and switch cranioplasty 4.6 hrs), as did transfusion amount (FOA 385 mL, modified pi 216 mL, total vault 600 mL, switch cranioplasty 207 mL) although 99% of patients received a transfusion of some sort. There were no deaths and no major intraoperative complications. Minor events include; ET tube malposition (1), desaturation (1), acidosis (1), hypothermia (9), coagulopathy (2), Hct < 25 (55). Average hospital stay was 3.4 days with no major post-operative complications. One patient was readmitted to the ICU and 1 had a scalp hematoma, but no patients returned to the operating room within 6 months after surgery. DISCUSSION: The surgical treatment of SSC has evolved from lengthy, risky procedures to become almost routine at most craniofacial centers. Additionally, the care for patients with SSC has evolved from a single provider to a multidisciplinary team concept based around protocols for workup, delivery of anesthesia, streamlined surgical procedures and post-operative care and assessment. This evolution has given open cranial vault surgery for SSC an acceptable safety profile.

Oculoauriculofrontonasal syndrome: case series revealing new bony nasal anomalies in an old syndrome.

Frontonasal Dysplasia (FND) and Oculo-auriculo-vertebral spectrum (OAVS) are two well-recognized clinical entities. With features of both FND and OAVS, the term oculoauriculofrontonasal syndrome (OAFNS) was coined in 1981. The OAFNS phenotype combines elements of abnormal morphogenesis of the frontonasal and maxillary process (derived from forebrain neural crest) with abnormal development of the first and second branchial arches (derived from hindbrain neural crest). We present a case series of 33 children with OAFNS ascertained from a comprehensive review of the literature and report an additional retrospective series of eight patients displaying features consistent with OAFNS. Notably, in a subset of our cases, we have observed abnormalities in nasal ossification and bony structures of the maxilla that have not previously described in OAFNS and are not seen in either FND or OAVS. We present the phenotype and novel naso-maxillary findings and explore potential etiologic and developmental pathways for OAFNS. We highlight the differences in phenotypic characteristics of OAFNS compared to OAVS and FND. These observations support the classification of OAFNS as a discrete syndrome. Further phenotypic refinements of OAFNS are needed to understand pathogenesis of this syndrome and the newly described nasal malformation may help identify the etiology.

Making the diagnosis: metopic ridge versus metopic craniosynostosis.

INTRODUCTION: The metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with metopic craniosynostosis. Metopic ridging (MR) is treated nonsurgically while metopic craniosynostosis (MCS) is treated surgically. Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. The goal of this study is to describe the physical examination and CT scan characteristics which may help to differentiate between physiological closure of the metopic suture with ridging (MR) and MCS. METHODS: A retrospective chart review of all patients seen at Seattle Children's Hospital between 2004 and 2009 with the diagnosis of either MCS or MR (n = 282) was performed. Physical examination characteristics described by diagnosing practitioners were analyzed. Clinical photos were assessed by 3 expert raters to determine the importance of these characteristics. CT scan findings were abstracted and compared between the two diagnoses. RESULTS: The "classic" triad of narrow forehead, biparietal widening, and hypotelorism was present in only 14% of patients with MCS. Ninety-eight percent of patients in both groups had a palpable metopic ridge. The photographic finding of narrow forehead and pterional constriction was present in all patients with MCS, but only in 11.2% and 2.8% of patients with MR. On CT scan, the presence of 3 or more MCS findings was diagnostic of MCS in 96% of patients. Patients with MCS were more likely to present before 6 months of age (66% vs. 32%). CONCLUSIONS: Patients with MCS tend to present earlier than those with MR. Upon physical examination, the relationship between the lateral frontal bone and the lateral orbit is important in distinguishing between the two diagnoses. A CT scan can be helpful in making the diagnosis not to confirm a closed suture but to identify 3 or more MCS characteristics.

Nasal lining mobilization for primary and secondary palatoplasty.

BACKGROUND: Repair of wide primary cleft palates and secondary cases are challenging. Much literature is dedicated to technique modifications and useful pearls for approaching these patients. Nasal lining is not often highlighted or addressed as a solution to these challenging cleft palate surgeries. The goal of this article was to describe and demonstrate through case examples the simple dissection and significant mobilization of nasal lining as a tool for cleft palate repair. METHODS: Retrospective case examples were selected to highlight the technique. RESULTS AND DISCUSSION: The surgical technique is standardly used by the senior author and has not been previously published. This technique for tension-free nasal lining closure is detailed, and case examples are provided. This technique for nasal lining mobilization is easily reproducible and is versatile. It can be used as an adjunct to any palatoplasty technique and is particularly useful in the treatment of wide clefts and revision cases.

Unilateral Cleft Lip Nasal Deformity: Foundation-Based Approach to Primary Rhinoplasty.

BACKGROUND: Cleft lip results in disruption of the nasal foundation and collapse of tip structures. Most approaches to primary rhinoplasty focus on correction of lower lateral cartilages; however, recurrent deformity is common, and secondary revision is frequently required. The authors describe an alternate approach that focuses on the foundation to "upright the nose," without any nasal tip dissection. This study assessed changes with surgery and with growth. Secondary goals were to compare methods of sidewall reconstruction and septoplasty and to identify predictors of relapse. METHODS: Consecutive patients undergoing repair (n = 102) were assessed. Images were captured preoperatively, postoperatively, and at 5 years of age (when available) using three-dimensional stereophotogrammetry. Standard anthropometric and contemporary shape-based analysis (volume ratio, dorsal deviation, and alar-cheek definition) was performed to assess longitudinal changes. Images of age-matched normal control subjects were used for comparison. RESULTS: Significant changes in anthropometric and morphometric measurements occurred following surgery. Postoperative form was similar to controls immediately after surgery and at 5 years. Nasal corrections were satisfactory, and only two patients have elected to undergo revision. When subjects were grouped according to cleft type, we found the same trends. When comparing different methods of nasal sidewall reconstruction or septoplasty, we found no differences. Alveolar cleft width was a significant predictor of worse preoperative and postoperative form. CONCLUSIONS: Significant nasal correction can be achieved by means of reconstruction of nasal foundation, without nasal tip dissection. Preservation of tissue planes may allow for easier secondary revision, if necessary. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Evolution of Bandeau Shape, Orbital Morphology, and Craniofacial Twist after Fronto-Orbital Advancement for Isolated Unilateral Coronal Synostosis: A Case-Control Study of 2-Year Outcomes.

BACKGROUND: The authors' purpose was to quantify the change in unicoronal synostosis symmetry between presentation (time 0), after fronto-orbital advancement (time 1), and 2 years later (time 2). METHODS: Bandeau/orbital symmetry ratios and skull base/midface twists were measured on computed tomographic scans of consecutive isolated unicoronal synostosis patients. Comparisons were made across three time points and against normal controls. RESULTS: Forty-three unicoronal synostosis patients and 36 controls were included. The mean bandeau ratio (symmetry = 1) changed from 0.76 (time 0), to 1.13 (time 1), and then to 1.01 (time 2). The median bandeau ratio change from time 1 to time 2 was -9.1 percent and was impacted by the degree of time 1 asymmetry. The odds of a desired symmetric or overcorrected result (bandeau ratio > 1.0) at time 2 were increased in patients with less severe preoperative asymmetry (OR, 4.2; p = 0.04) and in those who obtained symmetry or overcorrection at surgery (OR, 4.9; p = 0.02). Craniofacial twist did not significantly change after surgery but decreased at time 2. Orbital height ratios were 1.08, 1.00, and then 1.02 at time 2, respectively. The orbital width ratio was not significantly impacted by surgery, remaining at 0.89 at time 2. CONCLUSIONS: Overcorrection of the unicoronal synostosis bandeau resulted in these patients being five times more likely to have a desired result at time 2. Patients with a more severe brow presentation were four times more likely to be asymmetric at time 2. Orbital height was improved, but attention should be directed at addressing orbital width. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Evidence-Based Medicine: Evaluation and Treatment of Zygoma Fractures.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Diagnose zygomaticomaxillary complex fractures from physical examination and radiographic findings. 2. Plan the necessary surgical approaches for operative treatment of zygomaticomaxillary complex fractures depending on severity. 3. Understand the three-dimensional anatomy of the orbit and zygomaticomaxillary complex and the importance of the zygomaticosphenoid suture along the lateral orbital sidewall. 4. Be aware of pitfalls and associated fractures that can complicate anatomical reduction of zygomaticomaxillary complex fractures. SUMMARY: Fractures of the zygoma are some of the most commonly treated facial fractures, yet reconstruction of the three-dimensional structure of the zygomaticomaxillary complex can be challenging, and malunions are common. This article presents an evidence-based, systemic approach to the assessment and treatment of zygoma fractures from the simple to the complex. Anatomy, approaches, techniques, and pitfalls are described in an effort to improve the treatment of these common facial fractures.

Crowdsourcing as a Novel Method to Evaluate Aesthetic Outcomes of Treatment for Unilateral Cleft Lip.

BACKGROUND: Lack of convenient and reliable methods to grade aesthetic outcomes limits the ability to study results and optimize treatment of unilateral cleft lip. Crowdsourcing methods solicit contributions from a large group to achieve a greater task. The authors hypothesized that crowdsourcing could be used to reliably grade aesthetic outcomes of unilateral cleft lip. METHODS: Fifty deidentified photographs of 8- to 10-year-old subjects (46 with unilateral cleft lip and four controls) were assembled. Outcomes were assessed using multiple pairwise comparisons that produced a rank order (Elo rank) of nasal appearance and, on a separate survey, by Asher-McDade ratings. Both surveys were repeated to assess reliability. A group of expert surgeons repeated the same tasks on a smaller subset of photographs. RESULTS: The authors obtained 2500 and 1900 anonymous, layperson evaluations by means of crowdsourcing on each Elo rank and Asher-McDade survey, respectively. Elo rank and Asher-McDade scores were highly reproducible (correlation coefficients, 0.87 and 0.98), and crowd evaluations agreed with those by expert surgeons (0.980 and 0.96 for Elo rank and Asher-McDade score, respectively). Crowdsourcing surveys were completed within 9 hours, whereas the expert surgeons required 3 months. On further analysis of their cleft subject sample set, the authors found that greater initial cleft severity was associated with worse aesthetic outcome. CONCLUSIONS: Outcomes assessed by crowds were reliable and correlated well with expert assessments. Crowdsourcing allows acquisition of massive numbers of layperson assessments on an unprecedented scale, and is a convenient, rapid, and reliable means of assessing aesthetic outcome of treatment for unilateral cleft lip. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.

Diagnostic and surgical challenges in the pediatric skull base.

Advances in management of adult skull base pathologies are increasingly being applied in children. Pediatric patients present special challenges because of their smaller anatomy, but potential gains in reduced morbidity make improvements in skull base approaches well worth pursuing.

Neurofibromatosis of the head and neck: classification and surgical management.

BACKGROUND: Neurofibromatosis is common and presents with variable penetrance and manifestations in one in 2500 to one in 3000 live births. The management of these patients is often multidisciplinary because of the complexity of the disease. Plastic surgeons are frequently involved in the surgical management of patients with head and neck involvement. METHODS: A 20-year retrospective review of patients treated surgically for head and neck neurofibroma was performed. Patients were identified according to International Classification of Diseases, Ninth Revision codes for neurofibromatosis and from the senior author's database. RESULTS: A total of 59 patients with head and neck neurofibroma were identified. These patients were categorized into five distinct, but not exclusive, categories to assist with diagnosis and surgical management. These categories included plexiform, cranioorbital, facial, neck, and parotid/auricular neurofibromatosis. CONCLUSIONS: A surgical classification system and clinical characteristics of head and neck neurofibromatosis is presented to assist practitioners with diagnosis and surgical management of this complex disease. The surgical management of the cranioorbital type is discussed in detail in 24 patients. The importance and safety of facial nerve dissection and preservation using intraoperative nerve monitoring were validated in 16 dissections in 15 patients. Massive involvement of the neck extending from the skull base to the mediastinum, frequently considered inoperable, has been safely resected by the use of access osteotomies of the clavicle and sternum, muscle takedown, and brachial plexus dissection and preservation using intraoperative nerve monitoring. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Trapdoor fracture of the orbit in a pediatric population.

Orbital-floor blowout fractures of the trapdoor variety, first described by Soll and Poley, have also appeared in the French and Japanese literature. The trapdoor fracture is described as a pure orbital-floor fracture, linear in form and hinged medially, which allows herniation of orbital contents through the fracture and then entraps these herniated contents. A review of the orbital-floor fracture literature reveals a high incidence of persistent diplopia associated with ocular-muscle entrapment, which may later necessitate corrective surgery of extraocular muscles. Recent publications in the ophthalmologic literature have stressed the importance of early surgical intervention. This article reports a retrospective series of 19 pediatric patients (age range, 5 to 16 years) who presented to two institutions. All patients had radiographic confirmation of a trapdoor fracture. Physical examination demonstrated a high association between these fractures and restricted ocular motility (17 of the 19 patients). In the cases with trapdoor fracture and restricted ocular movement, early intervention was associated with better postoperative function. It is thus recommended that the symptomatic trapdoor orbit fracture be considered an urgent indication for surgical intervention. Practitioners therefore must have a high index of suspicion for these injuries. Prompt diagnosis is critical to maximize clinical outcome.

Adopted children with cleft lip and/or palate: a unique and growing population.

BACKGROUND: Standard clinical pathways are well established for children with cleft lip and/or palate. Treatment of internationally adopted children differs because of the late age at presentation, a newly evolving child-family relationship, and variable extent and quality of previous treatment. METHODS: The authors characterized the presentation and treatment patterns of all internationally adopted children with clefts at their institution between 1997 and 2011. RESULTS: Among 1841 children with clefts, 216 (12 percent) were internationally adopted: 78 percent had cleft lip and palate, 18 percent had cleft lip, and 4 percent had cleft palate. Patients originated predominantly from China (80 percent). Median age at presentation was 31 months, and the rate of new presentations increased five-fold during the study period. Eighty-two percent presented with prior cheiloplasty, and revision was recommended for 64 percent of them. Thirty-seven percent of patients had prior palatoplasty, of which 34 percent presented with a palatal fistula. Secondary palatoplasty/pharyngoplasty was performed more frequently for patients who underwent primary palatoplasty before adoption than after adoption (95 percent CI, 0.20 to 0.77). Overall, adoptees required secondary surgery more often than nonadoptees (49 percent versus 28 percent) regardless of where their primary surgery was performed. Changes in adoptee growth indices suggested improvements in systemic health following adoption. CONCLUSIONS: Internationally adopted children with clefts have unique treatment challenges. Children with unrepaired clefts undergo surgery late, and children with prior repairs frequently undergo revision. Compared with nonadoptees, adoptees require more revisions and have a higher fistula rate. Further detailed study is important to optimize care.

Contemporary concepts for the bilateral cleft lip and nasal repair.

The bilateral cleft lip and nasal deformity presents a complex challenge for repair. Surgical techniques continue to evolve and are focused on primary anatomic realignment of the tissues. This can be accomplished in a single-stage or two-stage repair early in infancy to provide a foundation for future growth of the lip and nasal tissue. Most cleft surgeons currently perform a single-stage repair for simplifying patient care. Certain institutions utilize presurgical orthopedics for alignment of the maxillary segments and nasal shaping. Methods for the bilateral cleft lip repair are combined with various open and closed rhinoplasty techniques to achieve improved correction of the primary nasal deformity. There is recent focus on shaping the nose for columellar and tip support, as well as alar contour and alar base position. The authors will present a new technique for closure of the nasal floor to prevent the alveolar cleft fistula. Although the alveolar fistula is closed, alveolar bone grafting is still required at the usual time in dental development to fuse the maxilla. It is paramount to try and minimize the stigmata of secondary deformities that historically have been characteristic of the repaired bilateral cleft lip. A properly planned and executed repair reduces the number of revisions and can spare a child from living with secondary deformities.

Zygomaticomaxillary complex fractures and their association with naso-orbito-ethmoid fractures: a 5-year review.

BACKGROUND: Zygomaticomaxillary complex fractures associated with ipsilateral naso-orbito-ethmoidal fractures are more complex injuries than isolated zygomaticomaxillary complex fractures. This injury pattern can have significant long-term morbidity if not recognized and treated appropriately during the initial operation. The purpose of this study is to compare mechanisms of injury, treatment, and outcome between patients with zygomaticomaxillary complex fractures and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures. METHODS: A 5-year retrospective review of all patients treated with zygomaticomaxillary complex fractures at a level I trauma center was performed. Computed tomographic scans were reviewed to divide patients into those with zygomaticomaxillary complex fractures alone and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures. Demographics, treatment protocols, outcomes, complications, reoperations, and length of follow-up were identified for both groups and compared to determine differences between these populations. RESULTS: A total of 245 patients were identified by the Current Procedural Terminology codes for zygomaticomaxillary complex fractures. One hundred eighty-five patients had zygomaticomaxillary complex fractures and 60 patients had zygomaticomaxillary complex/naso-orbito-ethmoidal injuries. The demographics for both populations were similar. There are differences between the groups with regard to mechanism of injury, operative findings, and techniques. The patients with zygomaticomaxillary complex/naso-orbito-ethmoidal fractures had higher rates of postoperative complications and deformities. CONCLUSIONS: Patients who sustain a zygomaticomaxillary complex fracture associated with an ipsilateral naso-orbito-ethmoidal fracture have a higher incidence of postoperative complications and deformities. It is important to recognize this fracture pattern early to help minimize postoperative morbidity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Changes in frontal morphology after single-stage open posterior-middle vault expansion for sagittal craniosynostosis.

BACKGROUND: There is controversy regarding whether the frontal bossing associated with sagittal synostosis requires direct surgical correction or spontaneously remodels after isolated posterior cranial expansion. The authors retrospectively measured changes in frontal bone morphology in patients with isolated sagittal synostosis 2 years after open posterior and midvault cranial expansion and compared these changes with those occurring in age-comparable healthy control groups. METHODS: Forty-three patients age 1 year or younger (mean, 6 months) with sagittal synostosis underwent computed tomography scan digital analysis immediately after and 2 years after posterior-middle cranial vault expansion. Quantitative angular and linear measures were taken along the midsagittal and axial planes to capture both aspects of frontal bossing. The change in values over the 2 years were compared with healthy controls with normal computed tomography scans taken to rule out head trauma. RESULTS: All measures indicative of frontal bossing decreased significantly from the time of posterior-middle vault expansion to 2 years postoperatively. Whereas the majority of patients at time of the operation had frontal bossing measures greater than two standard deviations outside the age-comparable control mean, almost all patients were within two standard deviations of the norm 2 years later. Lateral forehead bossing and anterior cranial growth was greater the older the patient was at the time of the operation, suggesting that the more time that passed before the operation, the more compensatory anterior fossa growth occurred. Central forehead position relative to the anterior cranial base was greatest in the younger patients at the time of operation, suggesting that a central forehead bulge was an early compensatory response to premature sagittal fusion. CONCLUSIONS: As a group, patients with sagittal synostosis start to normalize their forehead morphology within 2 years if an isolated posterior operation is performed at 1 year of age or younger, and this occurs by a combination of restriction of growth and reduction relative to patients without synostosis. This protocol decreases the risks of intraoperative positioning, forehead contour deformities, and two-stage operations. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Bupivacaine administration and postoperative pain following anterior iliac crest bone graft for alveolar cleft repair.

OBJECTIVE: To determine whether placement of a bupivacaine-soaked absorbable sponge (BAS) in addition to bupivacaine infiltration at the anterior iliac crest (AIC) donor site alters postoperative pain for children undergoing alveolar bone grafting (ABG) for cleft lip with or without cleft palate (CL+/-P). The comparison group received only bupivacaine infiltration (NO BAS) at the AIC. DESIGN: Retrospective cohort. Medical records were abstracted by one investigator, blinded to BAS versus NO BAS use. SETTING AND PATIENTS: Consecutive patients with CL+/-P who underwent ABG between 2000 and 2006 at one large U.S. craniofacial center. INTERVENTION: BAS was used in 118 procedures and NO BAS in 89. OUTCOME MEASURES: Postoperative pain score, total and opioid pain medication requirement, length of hospital stay (LOS), and time to initial ambulation. RESULTS: One hundred eighty-two patients underwent 207 ABG procedures. Mean pain scores were significantly lower when BAS was used compared with NO BAS (1.3 versus 1.8; p = .01). Patients who received BAS required significantly less pain medication than NO BAS patients: opioids (0.14 versus 0.20 mg/kg; p = .01) and total (0.60 versus 0.71 mg/kg; p = .02). Relative to the NO BAS group, those who received BAS had a shorter LOS (30.9 versus 42.4 hours; p < .0001) and less time to initial ambulation following surgery (14.4 versus 20.6 hours; p < .0001). CONCLUSION: Use of BAS at the AIC donor site significantly reduced postoperative pain score, pain medication requirement, LOS, and time to ambulation relative to children who did not receive BAS following ABG.

Symptom outcomes following cranial vault expansion for craniosynostosis in children older than 2 years.

BACKGROUND: Most craniosynostosis cases are treated by cranial expansion before 1 year of age. Occasionally, patients present at a later age with nonspecific symptoms of increased intracranial pressure. The purpose of this study was to review the symptoms of patients undergoing late cranial vault expansion. In all cases, the indication for surgery was treatment of the cranial dysmorphology. METHODS: The authors performed a retrospective review of all patients (n = 17) undergoing cranial vault expansion from 2000 to 2007 aged older than 2 years (median, 7.4 years) for abnormal head shape who also reported preoperative symptoms consistent with increased intracranial pressure, including headaches, nausea or vomiting, or vision changes. Median follow-up was 30 months; 11 patients had nonsyndromic and six had syndromic craniosynostosis. RESULTS: Fourteen patients had headaches, nine had nausea and vomiting, and 11 had vision changes. Ten patients had signs of intracranial pressure elevation, such as a computed tomographic scan findings, papilledema, or both. Twelve of 14 patients had complete resolution of headaches following surgery and four had late mild recurrence. All patients with nausea and vomiting and nine of 11 patients with vision changes had resolution of symptoms. CONCLUSIONS: The diagnosis of increased intracranial pressure in older children with craniosynostosis is difficult in the absence of papilledema or computed tomographic findings. Clinical symptoms frequently associated with increased intracranial pressure are improved with late cranial vault expansion and demonstrate a possible benefit beyond improvement in head shape.