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Cranial Fossa Volume in Differing Subtypes of Apert Syndrome

Lu, Xiaona MD*; Forte, Antonio Jorge MD; Wilson, Alexander BS; Alperovich, Michael MD; Steinbacher, Derek M. MD, DMD; Alonso, Nivaldo MD, PhD§; Persing, John A. MD

doi: 10.1097/SCS.0000000000005785
Original Articles

Background: Based on an established classification system of Apert syndrome subtypes, detailed regional morphology and volume analysis may be useful to provide additional clarification to individual Apert cranial structure characteristics, and treatment planning.

Methods: Computed tomography scans of 32 unoperated Apert syndrome and 50 controls were included and subgrouped as: type I, bilateral coronal synostosis; type II, pansynostosis; type III, perpendicular combination synostosis. Three-dimensional analysis of craniometric points was used to define structural components using Materialise Mimics and 3-Matics software.

Results: Occipitofrontal circumference of all subtypes of Apert syndrome patients is normal. Intracranial volumes of types I and II were normal, but type III was 20% greater than controls. Middle cranial fossa volume was increased in all 3 types, with the greatest increase in type II (86%). Type II developed a 69% increase in anterior cranial fossa volume, whereas type III had 39% greater posterior cranial fossa volume. Increased cranial fossa depth contributed most to above increased volume. The anteroposterior lengths of middle and posterior cranial fossae were reduced in type I (15% and 17%, respectively). However, only the anterior cranial fossa was significantly shortened in type III.

Conclusions: Occipitofrontal circumference and overall intracranial volume is not always consistent in individual subunits of Apert syndrome. Detailed and segmental anterior, middle, and posterior cranial fossae volumes and morphology should be analyzed to see what impact this may have related to surgical planning.

*Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital, Beijing, China

Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida, Jacksonville, FL

Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT

§Department of Plastic Surgery, University of São Paulo, São Paulo, Brazil.

Address correspondence and reprint requests to John A. Persing, MD, Division of Plastic and Reconstructive Surgery—Yale School of Medicine, 330 Cedar Street, 3rd floor Boardman Building, New Haven, CT 06520; E-mail:

Received 1 March, 2019

Accepted 24 May, 2019

The authors report no conflicts of interest.

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© 2019 by Mutaz B. Habal, MD.