Cleft Palate in Apert Syndrome: A Descriptive Study of Incidence and Surgical Outcome

Abstract

Objective

Apert syndrome (AS) is a rare congenital craniofacial disorder that requires a multidisciplinary approach to treatment and multiple surgeries. Given that cleft palate (CP) is presented in some of these cases, this poses an additional risk of aggravating obstructed airways after closure. The timing and outcome of CP repair in these patients remains disputed and requires additional attention.

Design

This retrospective analysis included patients diagnosed with CP and AS, born between 1950 and 2020, and treated at our institution. Data were collected from medical records and evaluated using descriptive statistics.

Setting

Data analyses were conducted at Sahlgrenska University Hospital in Gothenburg, Sweden.

Patients/Participants

A registry of 83 patients with AS resulted in a cohort of 26 patients also presenting with CP.

Main Outcome Measures

Postoperative complications, requirement for intensive care, and reoperations following CP repair.

Results

CP incidence among all registered patients was 31%. Patients undergoing CP repair at low age (mean: 22.5 months) tended to experience more frequent postoperative complications and requirements for intensive care. Among the evaluated cohort with medical records describing CP repair (n = 14), 29% experienced postoperative complications, all of which involved aggravation of obstructed airways.

Conclusions

This study highlights the importance of airway assessment before and after CP repair in AS. The findings suggest that surgical outcomes might benefit from postponing CP repair, avoiding combined surgeries, and operating in two stages when indicated. However, additional and larger studies are required.

Keywords

apert syndrome cleft palate surgical complications airway obstruction craniofacial surgery palatoplasty syndrome upper airway obstruction

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