Left Ophthalmic Segment Internal Carotid Artery Aneurysm Treated with Flow Diversion in a Child with Apert Syndrome: Technical Note.

INTRODUCTION: Prevalence of intracranial aneurysms in children with Apert syndrome has not been described, and development of an aneurysm as a complication secondary to craniofacial surgery has never been reported. CASE PRESENTATION: We report the rare case of a 10-year-old boy with Apert syndrome who underwent craniofacial reconstruction surgery consisting of subcranial Le Fort III osteotomies, bilateral lateral canthopexies, and nasal nares dilations for midfacial hypoplasia and resultant obstructive sleep apnea, and on routine follow-up magnetic resonance imaging (MRI) 1 year later, he was found to have a large left ophthalmic internal carotid artery (ICA) aneurysm that was not seen on MRI obtained 2 years prior. Immediately after the craniofacial surgery, the patient experienced a severe headache behind his left eye and extraocular movement abnormalities that subsided over the next days to months. Given the new and rapid growth of the aneurysm on follow-up MRI, the patient underwent a diagnostic cerebral angiogram followed by successful flow diversion treatment of the aneurysm with the pipeline embolization device (Medtronic, Dublin, Ireland). CONCLUSION: Post-procedurally, over the next year, the patient developed word-finding difficulty and stuttering speech. He was found to have in-stent ICA stenosis and middle cerebral artery (MCA) stenosis at the first follow-up and underwent an initial angioplasty. After several weeks, ICA, MCA, and anterior cerebral artery stenoses were identified, and the patient underwent angioplasties for the ICA and MCA stenoses. On follow-up examination after the second procedure, the patient had tremendous improvement in his speech difficulties and was doing well clinically.

Perioperative Outcomes Following Pediatric Cranial Vault Remodeling: Are Improvements Possible?

PURPOSE: The Pediatric Craniofacial Collaborative Group recently reported pooled perioperative data from 31 North American centers performing open cranial vault remodeling procedures. The authors sought to determine if outcomes were different at a single higher-volume center and if identified, ascertain reasons for any differences and propose strategies for improvement. METHODS: A retrospective review was performed of all open pediatric cranial vault procedures performed at our center during the identical 3.25-year period reported by the Collaborative group, including demographic, perioperative management and outcome data, to permit multiple comparative analyses. RESULTS: The 310 procedures were performed by our center during this time period, compared to 1223 by the combined 31 institutions (median: 29.5 cases/center; interquartile range: 12-54.5). Multiple outcome differences were found: our higher-volume center had a significantly lower overall red blood cell transfusion rate (≤2 years: 7.5 percent vs 91 percent, P <0.001), those requiring transfusions were transfused considerably smaller volumes (≤2 years: 3.8mL/kg vs 45.3 mL/kg, P <0.001), and exposure to ≥3 blood donors was significantly less (none vs 20 percent, P <0.001). There were no mortalities in either group, but almost all matched adverse events were less common at our center. Both the intensive care unit and hospital lengths of stay were significantly shorter at our center (1 vs 2 days, 2 vs 4 days, both P <0.001). CONCLUSIONS: Perioperative outcomes following pediatric craniosynostosis corrections performed at a single higher-volume center compare favorably to median national data. Multiple potential strategies to reduce blood utilization, minimize perioperative complications, and shorten hospitalizations are proposed.

Craniosynostosis and Guanine Nucleotide-binding Protein Alpha Stimulating Mutation: Risk of Bleeding Diathesis and Circulatory Collapse in Patients Undergoing Cranial Vault Reconstruction.

Reconstruction of the craniosynostosis deformity is a relatively safe operation with low overall complication risks. Despite expected risk of significant blood loss, life-threatening bleeding is relatively rare, and there is a low incidence of reported deaths in the literature. Several modalities have been described for perioperative mitigation of blood loss and transfusion requirements. Due to the low overall risk of life-threatening bleeding and circulatory collapse, it is judicious that any potential causes of such unusual but potentially significant fatal bleeding complication be evaluated and reported to increase awareness for craniofacial surgeons treating these conditions. In this report and literature review, the authors present a highly unusual patient with significant bone bleeding and circulatory collapse in a metopic craniosynostosis patient with guanine nucleotide-binding protein alpha stimulating (GNAS) mutation; perform a literature review regarding bleeding diathesis in craniosynostosis patients with GNAS mutations; and suggest guidelines to potentially prevent mortality in such patients.

Long-Term Evaluation of Mandibular Growth in Children With FGFR2 Mutations.

BACKGROUND: Understanding mandibular growth in children with fibroblast growth factor receptor 2 (FGFR2) mutations is important for planning the degree of midface advancement, and for determining the best treatment for obstructive sleep apnea. Yet, relatively little is known about growth of the unoperated mandible in affected children. The purpose of this study was to evaluate mandibular growth through skeletal maturity in Apert, Crouzon, and Pfeiffer syndromes. METHODS: A retrospective chart review was performed. Long-term, unoperated mandibular growth was assessed using multiple anthropologic measurements including: mandibular width, height, depth, and the cranial base width (approximating bicondylar width). Measurements were compared over 3 age intervals: 6 to 7 years, 10 years, and at skeletal maturity (15 years+). RESULTS: Out of 327 treated patients with FGFR2 mutations, 21 were found to have complete mandibular measurements through skeletal maturity (11 Apert, 7 Crouzon, and 3 Pfeiffer). Initial measurements revealed that mandibular height and bigonial breadth were slightly larger than normal, but sagittal depth and cranial base width were deficient. Early growth was slightly accelerated along the vertical and sagittal axes, stable across the bigonial distance, and marked deficient at the cranial base. At skeletal maturity, vertical height and bigonial width remained above average, but mandibular depth (forward sagittal growth) and cranial base width, remained deficient. CONCLUSIONS: Mandibular growth in children with FGFR2 mutations is not normal with impairments found in forward sagittal growth and skull base widening. Knowledge of these deficiencies has significant implications for both planning the degree of midfacial advancements, as well as treating obstructive sleep apnea.

A Randomized Controlled Trial of Oral Versus Intravenous Administration of a Nonnarcotic Analgesia Protocol Following Pediatric Craniosynostosis Corrections on Nausea and Vomiting Rates.

BACKGROUND: The authors' center uses a nonnarcotic postoperative regimen following craniosynostosis corrections. Despite opioid avoidance, the authors noted that some children still experienced nausea and vomiting following the oral administration of either acetaminophen or ibuprofen. This study sought to evaluate whether intravenous administration of these medications might reduce nausea and vomiting rates. METHODS: A total of 50 children undergoing craniosynostosis corrections were prospectively randomized to a control group given only oral ibuprofen (10 mg/kg) and acetaminophen (15 mg/kg), or a treatment group given only intravenous ketorolac (0.5 mg/kg) and acetaminophen (15 mg/kg). All patients were assessed for postoperative nausea and vomiting by a blinded research nurse. RESULTS: Twenty-eight patients randomized to the oral control group, and 22 to the intravenous treatment group. No statistically significant differences were identified between groups, including: age, weight, sex, before history of severe postoperative nausea and vomiting, or procedure. With similar anesthesia times there was significantly more vomiting episodes in the oral group (71% versus 41%). Using a multivariate logistic regression, controlling for age, weight and procedure, the odds ratio for vomiting in the oral control versus intravenous experimental groups was 3.61 (95% CI 1.11-1.76; P = 0.033), and for postoperative nausea was 14.0 (95% CI 1.40-71.69, P = 0.010). CONCLUSIONS: The authors found a significant reduction in nausea and vomiting among children randomized to receive intravenous medications. In addition, the intravenous delivery of medications has the theoretical advantage of insuring an effective full dose delivery. Based on these findings, our standard process is to preferentially manage all children following craniosynostosis corrections with intravenous nonnarcotics.

Classification and Management of Metopic Craniosynostosis.

BACKGROUND: In the craniofacial surgery literature, there is a wide disparity of opinions regarding the management of nonsyndromic metopic synostosis. With the lack of level I evidence to support a particular regimen, we aimed to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. METHODS: A survey was sent to 102 craniofacial surgeons. The survey featured 2 parts: clinical scenarios and questions regarding the following: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated metopic synostosis. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. RESULTS: The total response rate was 72% (73/102) for the clinical scenarios and 63% (64/102) for the complete survey. There was a large discrepancy when classifying and managing mild metopic synostosis, with between 16% and 35% of surgeons electing to operate on a mild case. All surgeons agreed to operate on moderate and severe cases. For 95% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management was most commonly performed at 6 months (29%) of age. Open frontal orbital advancement was the most commonly performed procedure in mild (27%), moderate (77%), and severe (89%) cases. Endoscopic approaches were more likely to be used in milder cases by 19% of surgeons. CONCLUSION: Our survey demonstrates that there is a wide disparity of opinion among craniofacial surgeons regarding the diagnosis and management of mild nonsyndromic metopic synostosis. LEVEL OF EVIDENCE: Diagnostic, level 5.

A comparative study of anterior cranial vault distraction versus remodeling.

INTRODUCTION: The use of distraction for treating craniosynostosis offers the potential to achieve greater calvarial enlargements than with single-staged procedures. However, distraction does require 2 operative procedures and a prolonged treatment course. We sought to compare our experience with these 2 techniques to determine relative effectiveness and to refine indications. METHODS: A retrospective case-controlled review of age-matched children with syndromic synostosis undergoing anterior cranial vault enlargement utilizing either distraction or conventional remodeling was performed. Complication and reoperative rates were tracked, and results were assessed using serial anthropometric measurements. RESULTS: Over a 19-month period, 10 children with Apert syndrome underwent calvarial enlargement, 5 by distraction (mean age 28 mo) and 5 by standard remodeling (mean age 31 mo). The average distraction distance was 26 mm. Distraction patients initially underwent 2.25 operations versus 1 operation for the remodeling group. Some aesthetically undesired skull widening was noted in 2/5 patients undergoing distraction (the youngest patients). Anthropometry (length, height, circumference, volume) revealed no significant differences between the 2 treatment groups. With a mean follow-up of 9.7 years, 2/5 distraction patients underwent secondary procedures versus 1/5 of the remodeling group. CONCLUSIONS: These analyses revealed less skull enlargement was actually achieved utilizing distraction than anticipated. We were unable to demonstrate any significant advantages of this distraction technique over our remodeling procedures with respect to either anthropometric skull measurements or the avoidance of future enlargement procedures. Given the burdens of distraction, further comparative clinical trials are needed to better refine indications for this emerging technique.

Thirty-year Experience Treating Syndromic Craniosynostosis: Long-term Outcomes Following Cranial Expansions.

INTRODUCTION: Children with syndromic craniosynostosis require multiple cranial expansion procedures. The purpose of this study was to determine how many expansions are typically performed through maturity, to assess complication rates, and to identify trends that might reduce the burden of care. METHODS: A retrospective chart review was performed of all consecutive patients undergoing cranial vault enlargement procedures for syndromic craniosynostosis by a single surgeon. RESULTS: Of 444 patients treated with Apert or Crouzon/Pfeiffer syndromes, 348 had complete long-term records. The mean age at last follow up was 16.9 years. Initial cranial expansions were performed at a significantly younger age for those beginning care at outside centers: 7.7-months versus 25.7-months in Dallas. 26.4% had ventriculoperitoneal shunts (syndrome-specific incidences: Apert 16%, Crouzon 27%, Pfeiffer 54%). Of 498 procedures, 80% were anterior, 18% posterior, and 2% were mid-vaults. The overall complication rate was 2%, with no differences found between anterior and posterior procedures. Those treated solely in Dallas underwent a mean of 1.9 expansions. Children with Pfeiffer syndrome underwent more expansions (2.7) than those with Apert or Crouzon syndromes. Factors correlationing with more expansions included: earlier surgical interventions, use of distraction, venticuloperitoneal shunts, and treatment begun at outside centers (3.1 procedures). CONCLUSIONS: Children with syndromic craniosynostosis treated at a single center underwent an average of 2 skull expansion procedures with a 2% complication rate. Calvarial distraction, ventriculoperitoneal shunts, and earlier surgical interventions, were all associated with higher numbers of expansion procedures. Further research is needed to determine if these correlations are causal.

Isolated Fibrous Dysplasia of the Bilateral Nasal Bones: Complex Management of the Bony Vault.

Fibrous dysplasia is a benign fibro-osseous process affecting the skeletal system, with resulting cystic and fibrous tissue expansion. Craniofacial fibrous dysplasia represents a small subset of monostotic disease, accounting for approximately 10%-25% of all such cases. Involvement of the frontal, temporal, and sphenoid bones has most commonly been described, with a limited number of reported cases citing disease isolated to the nasal bones. The case reported here is differentiated by the degree of expansion of the bilateral nasal bones and the required clinical management of the bony vault in the setting of gross nasal asymmetry.

Screw implantation in the globe: the risk of delayed hardware migration from craniofacial repair.

Plating system modification has enabled the use of rigid fixation in younger patients having maxillofacial surgery. One of the common reported complications of the use of plates and screws in children is screw migration due to skeletal maturation. Ophthalmic complications due to maxillofacial surgery reported to date include oculomotor and abducens palsies, lacrimal damage and vision loss due to infection, retrobulbar hemorrhage, and compartment syndrome. We describe a complication unique to screw migration resulting in orbital fixation and near-globe rupture in a patient with Treacher Collins syndrome. We hope to alert our colleagues to the potential risk of screw and hardware migration and breakage, particularly in the setting of craniofacial surgery performed on a child before maturation of craniofacial osseous structures.

Secondary Corrections for Single-Suture Craniosynostosis: Perioperative Outcomes and Predisposing Factors.

BACKGROUND: Many single-suture craniosynostosis corrections end up needing secondary remodeling procedures. The authors sought to determine whether these more complicated operations carry higher complication rates, and to explore potential predisposing factors. METHODS: The authors performed a retrospective chart review of all patients undergoing primary and secondary remodeling corrections at a single center, between 2010 and 2020. RESULTS: Of 491 sequential single-suture corrections, 380 were primary procedures and 111 were secondary (89.2% initially treated elsewhere); 10.3% of primary procedures received allogeneic blood versus only 1.8% of secondary corrections ( p =0.005). Median hospital lengths of stays were similar between groups [primary, 2.0 days (interquartile range [IQR], 2 to 2); secondary, 2.0 days (IQR, 2 to 2)], as were surgical infection rates (primary, 0%; secondary, 0.9%). With respect to predisposing factors, the affected suture and presence of a genetic mutation did not appear predictive; however, the median age at primary correction was significantly younger for those needing second procedures [6.0 months (IQR, 4 to 9 months) versus 12.0 months (IQR, 11 to 16 months)]. An odds ratio estimate suggests that for every month increase in age, the odds of undergoing a reoperation drops 40%. With respect to surgical indications, concerns for raised intracranial pressure and skull defects were more commonly cited following strip craniectomies than remodeling procedures. CONCLUSIONS: This single-center review was unable to identify a higher risk profile for reoperations. In addition, analyses suggest that performing primary corrections at a younger age and, possibly, strip craniectomies were associated with a higher likelihood of a subsequent secondary correction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Optic Nerve Atrophy in Syndromic Craniosynostosis.

BACKGROUND: Numerous children born with syndromic craniosynostosis will develop visual impairments. Based on the hypothesis that elevations in intracranial pressure might have greater impacts on vision than development, this review sought to ascertain the prevalence of optic nerve atrophy in syndromic craniosynostosis and to look for potential predictive factors. METHODS: The authors conducted a retrospective chart review of all children with syndromic craniosynostosis treated at a single center. RESULTS: Of 442 patients with syndromic craniosynostosis, complete ophthalmologic records were available for 253. Although no instances of optic nerve atrophy were noted among those with Saethre-Chotzen or Muenke syndromes, an overall 14.7 percent prevalence was noted among those with Apert (7.8 percent), Crouzon (27.9 percent), and Pfeiffer syndromes (23.1 percent), with initial diagnoses occurring at a mean age of 10 years. The presence of a Chiari malformation was found to significantly correlate with the subsequent diagnosis of optic nerve atrophy (OR, 3.544; p = 0.002); however, the timing of the first cranial vault procedure, presence of a ventriculoperitoneal shunt, degree of brachycephaly, number of vault expansions, and diagnosis of sleep apnea, did not show significant associations. CONCLUSIONS: A substantial percentage of children with Apert, Crouzon, and Pfeiffer syndromes were found to develop optic nerve atrophy, with a prevalence likely to trend higher with longer follow-up. Chiari malformations were the only significant potential predictor for optic nerve atrophy. With the goal of preventing visual losses, more frequent monitoring for raised intracranial pressure with ophthalmologic evaluations and magnetic resonance imaging measurements of optic nerve sheath diameters should be considered. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Long-Term Growth following Trigonocephaly Repairs: Are Overcorrections Necessary?

BACKGROUND: Does bifrontal width change with growth following trigonocephaly corrections? Postoperative long-term growth was assessed, along with the impacts of phenotypic severity, surgical timing, and operative technique, to determine how wide to surgically set bifrontal width. METHODS: A retrospective review of all trigonocephaly repairs was performed. Exclusion criteria included syndromic conditions, incomplete records, and follow-up under 1 year. Anthropometric measurements taken through completion of growth were evaluated and compared to sex- and age-matched normal standards for Z score conversion. RESULTS: Of 370 consecutive patients undergoing repairs, 95 had sufficient anthropometric data. The mean surgical age was 10.8 months (range, 2 months to 7 years) and mean follow-up was 54.3 months (range, 12 months to 17.8 years). Sequential measurements revealed progressive increases in bifrontal width. However, following conversion to Z scores, the initial overcorrection (mean overcorrection, 8.7 mm; mean Z score, +2.3) steadily diminished to an undercorrection (mean measurement, -5.5 mm; mean Z score, -1.1). Compared to treatment at an older age (10 to 12 months), repairs performed in those younger than 8 months showed worse growth (p = 0.004). Those more severely affected (lowest bifrontal Z scores) had growth similar to that of those more mildly impacted. Only two patients (2.1 percent) underwent secondary procedures for recurrences. No correlation was found between anthropologic measurements and observers' severity assessments. CONCLUSIONS: Subnormal bifrontal growth occurs following trigonocephaly corrections, especially with earlier corrections. Repairs performed at approximately 11 months of age had to be overcorrected by approximately 1.5 cm to produce a normal bifrontal width at maturity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Beyond the bandeau: 4 variations on fronto-orbital advancements.

The ubiquitous tongue and groove osteotomy remains the standard for treating the anterior craniosynostoses, despite the fact that significant variations in three-dimensional fronto-orbital dystopia can occur with various sutural fusions. Four variations on bandeau design are described that allow surgeons to go beyond a simple forward advancements toward a precise three-dimensional positioning (forward advancement, vertical repositioning, and medial narrowing), or allowing the avoidance of sinus crossing osteotomies that can potentially reduce infection risk. These osteotomy designs permit more accurate positioning of the supraorbits; they are also structurally and inherently stable, offering surgeons greater latitude in fixation techniques.

Abandoning the Supraorbital Bandeau in Anterior Craniosynostosis Repairs, for a Single-Segment Reconstruction.

BACKGROUND: Following correction of anterior sutural fusions, long-term forehead irregularities may arise. Based on the premise that frontal reconstructions using a seamless construct might produce better long-term aesthetic results, the supraorbital bandeau was abandoned for a single-piece frontal reconstruction. The purpose of this review was to compare outcomes and complications between children who underwent reconstruction with the traditional supraorbital bandeau and those who underwent reconstruction with a single bony segment. METHODS: A retrospective sequential chart review was performed of all children undergoing anterior sutural fusion repairs to compare supraorbital bandeau to single-segment reconstructions. Length of surgery, blood loss, hospital stay, and complications were assessed. Aesthetic outcomes and reoperation rates were also examined. RESULTS: Over 10 years, 199 patients with anterior sutural fusions underwent correction: 124 with a single-piece frontal reconstruction and 75 with traditional supraorbital bandeaus. Outcome analyses revealed no significant differences between techniques with respect to blood loss, surgical times, complication rates, and hospital length of stay. A preliminary analysis suggested lower reoperation rates (0 percent versus 2.7 percent) and more favorable aesthetic rankings for those treated with a single segment, although the follow-up for this group was significantly shorter compared with those treated with a supraorbital bandeau (16 months versus 43 months). CONCLUSIONS: Abandoning the traditional supraorbital bandeau for a simplified single-segment frontal reconstruction has the potential to result in improved forehead aesthetics, although longer term evaluations are needed. This review suggests that this technical variation does not appear to be associated with longer surgical times, increased blood loss, or higher complication rates. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Autogenous Bone Reconstruction of Large Secondary Skull Defects.

BACKGROUND: The authors sought to ascertain the upper limits of secondary skull defect size amenable to autogenous reconstructions and to examine outcomes of a surgical series. Published data for autogenous and alloplastic skull reconstructions were also examined to explore associations that might guide treatment. METHODS: A retrospective review of autogenously reconstructed secondary skull defects was undertaken. A structured literature review was also performed to assess potential differences in reported outcomes between autogenous bone and synthetic alloplastic skull reconstructions. Weighted risks were calculated for statistical testing. RESULTS: Ninety-six patients underwent autogenous skull reconstruction for an average defect size of 93 cm (range, 4 to 506 cm) at a mean age of 12.9 years. The mean operative time was 3.4 hours, 2 percent required allogeneic blood transfusions, and the average length of stay was less than 3 days. The mean length of follow-up was 28 months. There were no postoperative infections requiring surgery, but one patient underwent secondary grafting for partial bone resorption. An analysis of 34 studies revealed that complications, infections, and reoperations were more commonly reported with alloplastic than with autogenous reconstructions (relative risk, 1.57, 4.8, and 1.48, respectively). CONCLUSIONS: Autogenous reconstructions are feasible, with minimal associated morbidity, for patients with skull defect sizes as large as 500 cm. A structured literature review suggests that autogenous bone reconstructions are associated with lower reported infection, complication, and reoperation rates compared with synthetic alloplasts. Based on these findings, surgeons might consider using autogenous reconstructions even for larger skull defects. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

An Appraisal of the Cephalic Index in Sagittal Craniosynostosis, and the Unseen Third Dimension.

BACKGROUND: The cephalic index is often used to evaluate sagittal craniosynostosis corrections; however, validation of this measure remains untested. METHODS: A three-part study was designed to (1) determine the normal distribution of cephalic indices in untreated sagittal craniosynostosis; (2) examine index values in treated children, subsequently determined to require secondary surgery; and (3) explore the correlation between a photography-based assessment of scaphocephaly severity and the cephalic index. RESULTS: Of 392 preoperatively measured patients (mean cephalic index, 70.7; 95 percent CI, 57.4 to 89.5), 343 (87.5 percent) had Z scores falling within 2 SD of the mean; only 49 (12.5 percent) fell more than 2 SD below the mean, whereas 13 percent exceeded the mean. For 10 patients requiring secondary surgery (mean cephalic index, 74.8; 95 percent CI, 68.1 to 83.4), the mean Z score was -0.5 (range, -2.5 to 1.6). The polled results of 10 observers revealed no significant correlation between subjectively ranked severity scores and cephalic indices. CONCLUSIONS: Although the average preoperative cephalic index in children with sagittal craniosynostosis was below normal, the majority of measurements fell within a statistically normal distribution. Furthermore, many children requiring secondary corrections were found to have relatively normal indices, suggesting that normal values are not necessarily predictive of satisfactory outcomes. We also found no correlation between this index and a subjective assessment of severity. These findings suggest that the cephalic index is not a reliable outcome measure, perhaps because of the inability for this ratio to capture the amount of correction of the reduced posterior skull height associated with sagittal craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.