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.

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.

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.

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.

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.

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.