Ultra-early synostectomy and cranial remodeling orthoses in the management of craniosynostoses

Abstract

OBJECTIVE The advent of endoscopic synostectomy has enabled early surgery for infants with craniosynostosis. Even though diagnosis is often made at birth, endoscopic synostectomy has traditionally been delayed until the infant is 3 months of age. There have been very few published reports of this procedure being performed in the early neonatal period. The authors discuss their experience with ultra-early endoscopic synostectomy, defined as an operation for infants aged 8 weeks or younger. METHODS A retrospective analysis of infants who underwent operations at or before 8 weeks of age between 2011 and 2020 was done. RESULTS Twenty-five infants underwent operations: 11 were 2 weeks of age or younger, 8 were between 3 and 4 weeks of age, and 6 were between 5 and 8 weeks of age. The infants weighed between 2.25 and 4.8 kg. Eighteen had single-suture synostosis, and 7 had multiple sutures involved. Of these 7, 4 had syndromic craniosynostosis. The average operative time was 35 minutes, and it was less than 40 minutes in 19 cases. The estimated operative blood loss was 25 ml or less in 19 cases; 5 infants required an intraoperative blood transfusion. In 1 child with syndromic multisuture craniosynostosis, the surgery was staged due to blood loss. Two children experienced complications related to the procedure: one had an incidental durotomy with skin infection, and the other had postoperative kernicterus. All infants were fitted for cranial remodeling orthoses following surgery. Three of the 25 infants required reoperations, with 2 patients with syndromic craniosynostosis needing repeat surgery for cranial volume expansion and cosmetic appearance. Another child with syndromic craniosynostosis is awaiting cranial expansion surgery. Follow-up varied between 6 months and 8 years. CONCLUSIONS The data show that ultra-early synostectomy is safe and not associated with increased complications compared with surgery performed between 3 and 6 months of age. Infants with multisuture synostosis had increased operative time, required blood transfusion, and were more likely to require a second operation.