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

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  • 1 Division of Plastic and Reconstructive Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada;
  • | 2 Department of Neurosurgery, Boston Children’s Hospital, Boston, Massachusetts;
  • | 3 University of Massachusetts Medical School, Worcester, Massachusetts;
  • | 4 Division of Plastic and Craniofacial Surgery, Connecticut Children’s, Hartford, Connecticut;
  • | 5 Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital; and
  • | 6 Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, Massachusetts
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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.

ABBREVIATIONS

CI = cephalic index; ESC = endoscopic strip craniectomy; FOA = fronto-orbital advancement; GEE = generalized estimating equation; ICP = intracranial pressure; IQR = interquartile range; LOS = length of hospital stay.

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