Diagnostic, IV.

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.

Therapeutic, IV.

Therapeutic, IV.

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.

Therapeutic, III.

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.

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. (Plast.