Basilar invagination in osteogenesis imperfecta—Case report

Osteogenesis imperfecta (OI) is a genetic disorder characterized by defective type I collagen synthesis, leading to fragile bones and skeletal deformities. One of the serious complications of OI involves abnormalities of the craniovertebral junction, including basilar invagination, basilar impressio...

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Main Authors: Aviyani Aviyani, Raisa Mahmudah
Format: Article
Language:English
Published: Elsevier 2025-10-01
Series:Radiology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S1930043325005977
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author Aviyani Aviyani
Raisa Mahmudah
author_facet Aviyani Aviyani
Raisa Mahmudah
author_sort Aviyani Aviyani
collection DOAJ
description Osteogenesis imperfecta (OI) is a genetic disorder characterized by defective type I collagen synthesis, leading to fragile bones and skeletal deformities. One of the serious complications of OI involves abnormalities of the craniovertebral junction, including basilar invagination, basilar impression, and platybasia, which can cause skull base deformities and brainstem compression. We report a case of a 19-year-old patient with OI presenting with headaches, neurological deficits, and visual impairment, whose contrast-enhanced head CT scan revealed basilar invagination, fusion of midbrain colliculi, tonsillar herniation into the foramen magnum, and communicating hydrocephalus. These cranial abnormalities arise from weakened bone structures inherent in OI and may disrupt cerebrospinal fluid flow, leading to significant neurological symptoms. Early detection and ongoing monitoring of craniovertebral anomalies in OI patients are essential to prevent severe complications and improve clinical outcomes.
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spelling doaj-art-50a26b1e50b24427bbfcf48779d5d1962025-08-20T03:50:22ZengElsevierRadiology Case Reports1930-04332025-10-0120104913491610.1016/j.radcr.2025.06.048Basilar invagination in osteogenesis imperfecta—Case reportAviyani Aviyani0Raisa Mahmudah1Corresponding author.; Department of Radiology, Faculty of Medicine, Universitas Padjadjaran, West Java, IndonesiaDepartment of Radiology, Faculty of Medicine, Universitas Padjadjaran, West Java, IndonesiaOsteogenesis imperfecta (OI) is a genetic disorder characterized by defective type I collagen synthesis, leading to fragile bones and skeletal deformities. One of the serious complications of OI involves abnormalities of the craniovertebral junction, including basilar invagination, basilar impression, and platybasia, which can cause skull base deformities and brainstem compression. We report a case of a 19-year-old patient with OI presenting with headaches, neurological deficits, and visual impairment, whose contrast-enhanced head CT scan revealed basilar invagination, fusion of midbrain colliculi, tonsillar herniation into the foramen magnum, and communicating hydrocephalus. These cranial abnormalities arise from weakened bone structures inherent in OI and may disrupt cerebrospinal fluid flow, leading to significant neurological symptoms. Early detection and ongoing monitoring of craniovertebral anomalies in OI patients are essential to prevent severe complications and improve clinical outcomes.http://www.sciencedirect.com/science/article/pii/S1930043325005977Osteogenesis imperfectaBasilar invagination
spellingShingle Aviyani Aviyani
Raisa Mahmudah
Basilar invagination in osteogenesis imperfecta—Case report
Radiology Case Reports
Osteogenesis imperfecta
Basilar invagination
title Basilar invagination in osteogenesis imperfecta—Case report
title_full Basilar invagination in osteogenesis imperfecta—Case report
title_fullStr Basilar invagination in osteogenesis imperfecta—Case report
title_full_unstemmed Basilar invagination in osteogenesis imperfecta—Case report
title_short Basilar invagination in osteogenesis imperfecta—Case report
title_sort basilar invagination in osteogenesis imperfecta case report
topic Osteogenesis imperfecta
Basilar invagination
url http://www.sciencedirect.com/science/article/pii/S1930043325005977
work_keys_str_mv AT aviyaniaviyani basilarinvaginationinosteogenesisimperfectacasereport
AT raisamahmudah basilarinvaginationinosteogenesisimperfectacasereport