Variations in Paranasal Sinus Anatomy in Children With Apert Syndrome: A Radiological Analysis.

Abstract

Apert syndrome is a genetic disorder characterized by craniofacial abnormalities and premature closure of the coronal sutures. The restriction of cranial development may have a subsequent effect on paranasal anatomy development. The aim of the study was to gain an understanding of paranasal sinus anatomical variations seen in children with Apert syndrome. This was a retrospective review of computed tomography and magnetic resonance images of children with Apert syndrome from 2000 to 2020. Images were reviewed to identify anatomical variations in paranasal sinus anatomy. Twenty-one patients were included in the study. The most commonly seen variation was septal deviation in 86% of cases, with 60% of patients having a septal defect. The presence of protrusion or dehiscence of the infraorbital nerve, carotid canal and Vidian nerve, and presence of a concha bullosa were not observed in any patients. Keros type I was the most commonly observed olfactory fossa depth in 79% of patients, and type I Kuhn cells were observed in 83% of patients. To our knowledge, this is the first study which describes the prevalence of variations in paranasal sinus anatomy found in children with Apert syndrome. Septal deviation, type I Kuhn cells and Keros type I olfactory fossa depth were observed in a higher prevalence in our cohort than in the general population. As such, assessment for the presence of chronic rhinosinusitis and nasal obstruction should be evaluated as part of the multidisciplinary assessment.