Cephalocranial Disproportionate Fossa Volume and Normal Skull Base Angle in Pfeiffer Syndrome

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art_LU_Cephalocranial_Disproportionate_Fossa_Volume_and_Normal_Skull_Base_2021.PDF (892.87 KB)
Citações na Scopus
6
Tipo de produção
article
Data de publicação
2021
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
LIPPINCOTT WILLIAMS & WILKINS
Autores
LU, Xiaona
FORTE, Antonio Jorge
ALLAM, Omar
PARK, Kitae Eric
ALPEROVICH, Michael
STEINBACHER, Derek M.
TONELLO, Cristiano
PERSING, John A.
Citação
JOURNAL OF CRANIOFACIAL SURGERY, v.32, n.2, p.581-586, 2021
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Background: Pfeiffer syndrome is a rare syndromic craniosynostosis disorder, with a wide range of clinical manifestations. This study aims to investigate the structural abnormalities of cranial fossa and skull base development in Pfeiffer patients, to provide an anatomic basis for surgical interventions. Method: Thirty preoperative CT scans of Pfeiffer syndrome patients were compared to 35 normal controls. Subgroup comparisons, related to differing suture synostosis, were performed. Results: Overall, the volume of anterior and middle cranial fossae in Pfeiffer patients were increased by 31% (P < 0.001) and 19% (P = 0.004), versus controls. Volume of the posterior fossa in Pfeiffer patients was reduced by 14% (P = 0.026). When only associated with bicoronal synostosis, Pfeiffer syndrome patients developed enlarged anterior (68%, P = 0.001) and middle (40%, P = 0.031) fossae. However, sagittal synostosis cases only developed an enlarged anterior fossa (47%, P < 0.001). The patients with solely bilateral squamosal synostosis, developed simultaneous reduced anterior, middle and posterior cranial fossae volume (all P <= 0.002). The overall skull base angulation, measured on both intracranial and subcranial surfaces, grew normally. Conclusion: Enlarged anterior cranial fossae in Pfeiffer syndrome children is evident, except for the squamosal synostosis cases which developed reduced volume in all fossae. Volume of the middle cranial fossa is influenced by associated cranial vault suture synostosis, specifically, sagittal synostosis cases develop normal middle fossa volume, while the bicoronal cases develop increased middle fossa volume. Posterior cranial fossa development is restricted by shortened posterior cranial base length. Surgical intervention in Pfeiffer syndrome patients optimally should be indexed to different suture synostosis.
Palavras-chave
Cranial fossa, craniosynostosis, Pfeiffer syndrome, skull base, squamosal Suture
URI
https://observatorio.fm.usp.br/handle/OPI/41242
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MCG
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/04