Cranial Fossa Volume and Morphology Development in Apert Syndrome.


Journal

Plastic and reconstructive surgery
ISSN: 1529-4242
Titre abrégé: Plast Reconstr Surg
Pays: United States
ID NLM: 1306050

Informations de publication

Date de publication:
04 2020
Historique:
entrez: 30 3 2020
pubmed: 30 3 2020
medline: 9 7 2020
Statut: ppublish

Résumé

Apert syndrome causes normal or enlarged intracranial volume overall as patients grow. This study aimed to trace the segmental anterior, middle, and posterior cranial fossae volume and structural morphology in these patients, to help discern a more focused and individualized surgical treatment plan for patients with Apert syndrome. This study included 82 preoperative computed tomographic scans (Apert, n = 32; control, n = 50) divided into five age-related subgroups. The scans were measured using image processing and three-dimensional modeling software. The middle cranial fossa volume was increased and was the earliest change noted. It was increased by 45 percent (p = 0.023) compared with controls before 6 months of age and remained increased into adulthood (161 percent, p = 0.016), with gradually increasing severity. The anterior and posterior cranial fossae volumes also increased, by 35 percent (p = 0.032) and 39 percent (p = 0.007), respectively. Increased depth of cranial fossae contributed most to the increase in volumes of patients with Apert syndrome, with correlation coefficients of 0.799, 0.908, and 0.888 for anterior, middle, and posterior cranial fossa, respectively. The intracranial volume was increased 12 percent (p = 0.098) across the entire test age range (0 to 26 years old), but only had statistical significance during the age range of 6 to 18 years (22 percent, p = 0.001). Malformation of the middle cranial fossa is an early, perhaps the initial, pivotal cranial morphologic change in Apert syndrome. Increased cranial fossae depth is an inherent characteristic of the maldevelopment. Normalization of cranial volume and circumference overall may not achieve a normal skull structure, as it does not correct regional craniocerebral disproportion.

Sections du résumé

BACKGROUND
Apert syndrome causes normal or enlarged intracranial volume overall as patients grow. This study aimed to trace the segmental anterior, middle, and posterior cranial fossae volume and structural morphology in these patients, to help discern a more focused and individualized surgical treatment plan for patients with Apert syndrome.
METHODS
This study included 82 preoperative computed tomographic scans (Apert, n = 32; control, n = 50) divided into five age-related subgroups. The scans were measured using image processing and three-dimensional modeling software.
RESULTS
The middle cranial fossa volume was increased and was the earliest change noted. It was increased by 45 percent (p = 0.023) compared with controls before 6 months of age and remained increased into adulthood (161 percent, p = 0.016), with gradually increasing severity. The anterior and posterior cranial fossae volumes also increased, by 35 percent (p = 0.032) and 39 percent (p = 0.007), respectively. Increased depth of cranial fossae contributed most to the increase in volumes of patients with Apert syndrome, with correlation coefficients of 0.799, 0.908, and 0.888 for anterior, middle, and posterior cranial fossa, respectively. The intracranial volume was increased 12 percent (p = 0.098) across the entire test age range (0 to 26 years old), but only had statistical significance during the age range of 6 to 18 years (22 percent, p = 0.001).
CONCLUSIONS
Malformation of the middle cranial fossa is an early, perhaps the initial, pivotal cranial morphologic change in Apert syndrome. Increased cranial fossae depth is an inherent characteristic of the maldevelopment. Normalization of cranial volume and circumference overall may not achieve a normal skull structure, as it does not correct regional craniocerebral disproportion.

Identifiants

pubmed: 32221226
doi: 10.1097/PRS.0000000000006679
pii: 00006534-202004000-00036
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

790e-802e

Références

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Auteurs

Xiaona Lu (X)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Antonio Jorge Forte (AJ)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Alexander Wilson (A)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Derek M Steinbacher (DM)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Michael Alperovich (M)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Nivaldo Alonso (N)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

John A Persing (JA)

From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

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