Endoscopic strip craniectomy with orthotic helmeting for safe improvement of head growth in children with Apert syndrome

Abstract

OBJECTIVE Bilateral coronal craniosynostosis in Apert syndrome is traditionally managed with open cranial vault remodeling procedures like fronto-orbital advancement (FOA). However, as minimally invasive procedures gain popularity, limited data exist to determine their efficacy in this syndromic population. This study examines whether endoscopic strip craniectomy (ESC) is inferior to FOA in correcting head growth in patients with Apert syndrome. METHODS The authors conducted a retrospective review of children with Apert syndrome over a 23-year period. Postoperative head circumferences until 24 months of age were compared for patients treated with ESC versus FOA by using normative growth curves. Intraoperative and postoperative morbidity was compared between groups. RESULTS The median postoperative follow-up for the FOA (n = 14) and ESC (n = 16) groups was 40 and 28.5 months, the median age at operation was 12.8 and 2.7 months, and the median operative time was 285 and 65 minutes, respectively (p < 0.001). The FOA group had significantly higher rates of blood transfusion, ICU admission, and longer hospital length of stay (p < 0.01). There were no statistically significant differences in premature reossification rates, complications, need for further procedures, or complaints of asymmetry. Compared to normative growth curves, all patients in both groups had head circumferences comparable to or above the 85th percentile at last follow-up. CONCLUSIONS Children with Apert syndrome and bilateral coronal craniosynostosis treated with ESC experience early normalization of head growth and cephalic index that is not inferior to those treated with FOA. Longer-term assessments are needed to determine long-term aesthetic results and the correlation between head growth and neurocognitive development in this population.

The Need for Additional Surgery after Passive versus Active Approaches to Syndromic Craniosynostosis: A Meta-analysis

“…This approach presents with separate confounders, however, since the decision to undergo EACS versus open procedures is usually nonrandom. Furthermore, only four articles 23,27,42,44 (three of which were excluded due to potential patient overlap or inadequate follow-up) reported outcomes from both approaches, and so such an analysis is not possible with the literature currently available. Finally, Whitaker outcome and need for reoperation is often subjective, 45 and in most of the EACS patients in this study, the reason for reoperation was not explicitly stated beyond that the attending surgeon deemed it necessary.…”

“…The syndrome breakdown in Riordan 2020 7 was not available, so articles previously excluded due to overlapping patient populations [21][22][23][24] were included in the subgroup table. Patients in the Riesel 2022 study 23 undergoing FOA were still excluded due to potential overlap with an included study. 28 Meta-analysis of UOR by syndrome was not conducted due to small patient samples in the passive category.…”

“…20 All articles included patients from a single institution. Four studies [21][22][23][24] included Fig. 1.…”

“…Of the 27 articles meeting inclusion criteria for undesirable outcome analysis, 16 articles (three passive, 13 active) included outcome data on individual syndromes, the results of which are included in Table 2. The syndrome breakdown in Riordan 2020 7 was not available, so articles previously excluded due to overlapping patient populations [21][22][23][24] were included in the subgroup table. Patients in the Riesel 2022 study 23 undergoing FOA were still excluded due to potential overlap with an included study.…”

“…In the four EACS articles 21,23,32,33 which reported precise reoperation times, reoperation occurred on average 13.7 (95% CI, 7.4-20) months postsurgery (n = 10 patients), whereas active surgery required reoperation 37.1 (95% CI, 24.5-49.7) months postsurgery on average (20 patients, five articles 20,[34][35][36][37] ) (P = 0.003) (Table 3).…”