Apert syndrome is frequently combined with respiratory insufficiency, due to the mid-facial deformity, which, in turn, is influenced by the malformation of the skull base. Respiratory impairment resulting from Apert syndrome is caused by multilevel limitations in airway space. Therefore, this study evaluated the segmented nasopharyngeal and laryngopharyngeal anatomy to clarify subcranial anatomy in children with Apert syndrome and its relevance to clinical management.
Twenty-seven patients (Apert, n=10; control, n=17) were included. All of the CT scans were obtained from the patients preoperatively, and no patient had confounding disease comorbidity. CT scans were analyzed using Surgicase CMF. Craniometric data relating to the midface, airway, and sub-cranial structures were collected. Statistical significance was determined using t-test analysis.
Although all the nasal measurements were consistent with those of the controls, the distance between nasion-PNS, sphenethmoid-PNS, sella-PNS, and basion-PNS were decreased 20% (p<0.001), 23% (p=0.001), 29% (p<0.001), and 22% (p<0.001), respectively. The distance between bilateral gonions and condylions was decreased 17% (p=0.017) and 18% (p=0.004) respectively. The pharyngeal airway volume was reduced by 40% (p=0.01).
The airway compromise seen in patients with Apert syndrome is more attributable to the pharyngeal region than the nasal cavity, with a gradually worsening trend from the anterior to the posterior airway, resulting in a significantly reduced volume in the hypopharynx.
1. Division of Plastic Surgery, Mayo Clinic, Jacksonville, Florida, USA
2. Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital, Beijing, China
3. Section of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, Connecticut, USA
4. Department of Plastic Surgery, University of São Paulo, São Paulo, Brazil
Financial Disclosure Statement: None
Corresponding Author: John A. Persing, MD, Irving & Silik Polayes Professor of Plastic Surgery, Section of Plastic and Reconstructive Surgery, Yale School of Medicine, 330 Cedar Street, 3rd floor Boardman Building New Haven, CT 06520 USA. firstname.lastname@example.org