Outcomes after Endoscope-Assisted Strip Craniectomy and Orthotic Therapy for Syndromic Craniosynostosis.

BACKGROUND

Endoscopic craniosynostosis repair has emerged as an effective alternative to open repair, but data are limited on treatment of the 15% to 24% of patients with syndromic diagnoses. In this study, the authors examine postoperative outcomes after endoscopic repair in syndromic craniosynostosis.

METHODS

Retrospective review was performed of all consecutive patients undergoing endoscopic repair and all syndromic patients undergoing open repair from 2006 to 2021. Demographics, complications, and reoperations were compared between groups. Patient-reported measures of stigma and cognitive function were recorded at age 5 years and older.

RESULTS

A total of 335 patients underwent endoscopic repair, of which 38 (11%) had syndromic craniosynostosis. Syndromic craniosynostosis was associated with bicoronal involvement ( P < 0.001) and female sex ( P = 0.003). Secondary procedures were significantly more common in the syndromic group (24% versus 2.4%; P < 0.001), as were transfusions (18% versus 6.4%; P = 0.018). Secondary procedures were performed at a mean 2.8 years of age (range, 10 months to 8 years), and most commonly consisted of fronto-orbital advancement (seven in the syndromic group, and three in the nonsyndromic group). The degree of patient-reported stigma was higher in patients with syndromes ( P = 0.002), but cognitive function did not differ significantly ( P = 0.065). The incidence of reoperations after open repair was 13%, but baseline differences precluded direct comparison with the endoscopic group.

CONCLUSIONS

Minimally invasive approaches in early infancy can alleviate the need for additional cranial procedures in the growing child. Syndromic craniosynostoses are a complex and heterogeneous group, and in more severe cases, endoscopic repair can be considered an adjunct technique to reduce the number of major craniofacial procedures.

CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.