Crouzon Syndrome with Hydrocephalus

Background: Hydrocephalous in the presence of primary craniosynostosis is a unique condition with respect to pathogenesis, clinical significance, and morphological appearance. It is rarely observed in nonsyndromic craniosynostosis, and in these cases usually attributable to coincidental disorders. C...

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Main Authors: Saad Javed, Eesha Yaqoob, Ashraf Mahmood, Bipin Chaurasia
Format: Article
Language:English
Published: Al-Azhar University, Faculty of Medicine (Damietta) 2023-03-01
Series:International Journal of Medical Arts
Subjects:
Online Access:https://ijma.journals.ekb.eg/article_297835_c7f9b287e4e62b9d97561ca0ae6dcf7d.pdf
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author Saad Javed
Eesha Yaqoob
Ashraf Mahmood
Bipin Chaurasia
author_facet Saad Javed
Eesha Yaqoob
Ashraf Mahmood
Bipin Chaurasia
author_sort Saad Javed
collection DOAJ
description Background: Hydrocephalous in the presence of primary craniosynostosis is a unique condition with respect to pathogenesis, clinical significance, and morphological appearance. It is rarely observed in nonsyndromic craniosynostosis, and in these cases usually attributable to coincidental disorders. Conversely, it is a common feature of syndromic craniosynostosis. The management of CS requires a multidisciplinary approach, and early diagnosis is vital in order to avoid complications such as blindness and raised intracranial pressure.Case presentation: A 7-years-old boy presented to the Neurosurgery OPD complaining of facial disfigurement, headache, vomiting and one episode of fit. X-Ray Skull films showed typical features of impressions of sulci and gyri demonstrating raised ICP due to fused sutures. Computed tomography scan showed hydrocephalus and prominent markings on the skull’s inner table with shallow orbits. Based on all these findings the patient was diagnosed to have the Crouzon syndrome.Conclusion: In true hydrocephalus ventriculo-peritoneal shunting is currently the single promising mode of treatment in case of craniosynostosis case.
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spelling doaj.art-09d505477bb445f78ef7046af2de6dcc2024-01-05T19:35:53ZengAl-Azhar University, Faculty of Medicine (Damietta)International Journal of Medical Arts2636-41742682-37802023-03-01533133313610.21608/ijma.2023.173207.1547297835Crouzon Syndrome with HydrocephalusSaad Javed0Eesha Yaqoob1Ashraf Mahmood2Bipin Chaurasia3Department of Neurosurgery, Rawalpindi Medical University, Rawalpindi, PakistanDepartment of Sociology, PMAS Arid Agriculture University, Rawalpindi, PakistanDepartment of Neurosurgery, Rawalpindi Medical University, Rawalpindi, PakistanDepartment of Neurosurgery, Neurosurgery Clinic , Birgunj, nepalBackground: Hydrocephalous in the presence of primary craniosynostosis is a unique condition with respect to pathogenesis, clinical significance, and morphological appearance. It is rarely observed in nonsyndromic craniosynostosis, and in these cases usually attributable to coincidental disorders. Conversely, it is a common feature of syndromic craniosynostosis. The management of CS requires a multidisciplinary approach, and early diagnosis is vital in order to avoid complications such as blindness and raised intracranial pressure.Case presentation: A 7-years-old boy presented to the Neurosurgery OPD complaining of facial disfigurement, headache, vomiting and one episode of fit. X-Ray Skull films showed typical features of impressions of sulci and gyri demonstrating raised ICP due to fused sutures. Computed tomography scan showed hydrocephalus and prominent markings on the skull’s inner table with shallow orbits. Based on all these findings the patient was diagnosed to have the Crouzon syndrome.Conclusion: In true hydrocephalus ventriculo-peritoneal shunting is currently the single promising mode of treatment in case of craniosynostosis case.https://ijma.journals.ekb.eg/article_297835_c7f9b287e4e62b9d97561ca0ae6dcf7d.pdfcrouzon syndromehydrocephalouscraniosynostosis
spellingShingle Saad Javed
Eesha Yaqoob
Ashraf Mahmood
Bipin Chaurasia
Crouzon Syndrome with Hydrocephalus
International Journal of Medical Arts
crouzon syndrome
hydrocephalous
craniosynostosis
title Crouzon Syndrome with Hydrocephalus
title_full Crouzon Syndrome with Hydrocephalus
title_fullStr Crouzon Syndrome with Hydrocephalus
title_full_unstemmed Crouzon Syndrome with Hydrocephalus
title_short Crouzon Syndrome with Hydrocephalus
title_sort crouzon syndrome with hydrocephalus
topic crouzon syndrome
hydrocephalous
craniosynostosis
url https://ijma.journals.ekb.eg/article_297835_c7f9b287e4e62b9d97561ca0ae6dcf7d.pdf
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AT bipinchaurasia crouzonsyndromewithhydrocephalus