Surgical management of intracranial hypertension in rickets-related craniosynostosis

Hypophosphatemic rickets is occasionally associated with craniosynostosis. Delayed diagnosis can result in intracranial hypertension, potentially leading to serious neurological complications. We report the case of a 9-year-old boy with X-linked hypophosphatemic rickets who presented with headache,...

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Main Authors: Alessio Iacoangeli, Sergio Capelli, Andrea Held, Emidio Procaccini, Davide Luglietto, Matteo Barba, Alessandro De Benedictis, Carlo Gandolfo, Carlo Efisio Marras
Format: Article
Language:English
Published: Elsevier 2025-03-01
Series:Interdisciplinary Neurosurgery
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Online Access:http://www.sciencedirect.com/science/article/pii/S2214751925000106
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author Alessio Iacoangeli
Sergio Capelli
Andrea Held
Emidio Procaccini
Davide Luglietto
Matteo Barba
Alessandro De Benedictis
Carlo Gandolfo
Carlo Efisio Marras
author_facet Alessio Iacoangeli
Sergio Capelli
Andrea Held
Emidio Procaccini
Davide Luglietto
Matteo Barba
Alessandro De Benedictis
Carlo Gandolfo
Carlo Efisio Marras
author_sort Alessio Iacoangeli
collection DOAJ
description Hypophosphatemic rickets is occasionally associated with craniosynostosis. Delayed diagnosis can result in intracranial hypertension, potentially leading to serious neurological complications. We report the case of a 9-year-old boy with X-linked hypophosphatemic rickets who presented with headache, fundus oculi edema, and double sinus pericranii. A CT scan revealed fusion of the cranial sutures, and continuous intracranial pressure monitoring confirmed intracranial hypertension. The patient successfully underwent bilateral cranial expansion. Secondary craniosynostosis is a rare condition, with hypophosphatemic rickets being the most common metabolic cause. Sinus pericranii is a rare radiological manifestation of intracranial hypertension. Craniosynostosis presenting after infancy or early childhood should prompt consideration of secondary or metabolic causes. Regular monitoring for head shape abnormalities and signs of intracranial hypertension is essential in patients with hypophosphatemic rickets. Skull expansion is an effective therapeutic option.
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institution Kabale University
issn 2214-7519
language English
publishDate 2025-03-01
publisher Elsevier
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series Interdisciplinary Neurosurgery
spelling doaj-art-7f0077b2c37a4390b6509e36f792e1482025-02-12T05:31:09ZengElsevierInterdisciplinary Neurosurgery2214-75192025-03-0139101998Surgical management of intracranial hypertension in rickets-related craniosynostosisAlessio Iacoangeli0Sergio Capelli1Andrea Held2Emidio Procaccini3Davide Luglietto4Matteo Barba5Alessandro De Benedictis6Carlo Gandolfo7Carlo Efisio Marras8Department of Neurosurgery Università Politecnica Delle Marche 10,Via Tronto Ancona ItalyDivision of Neurosurgery Department of Medical and Surgical Specialties Radiological Sciences and Public Health University of Brescia 11 Viale Europa Brescia Italy; Division of Neurosurgery, Department of Biotechnology and Life Sciences, University of Insubria, Via J.H. Dunant 3 21100 Varese ItalyDepartment of Medicine and Surgery, University of Milano Bicocca, 1, Piazza Dell’Ateneo Nuovo, Milan, Italy; Neurosurgery Department, Ospedale San Gerardo, Monza, 33, Via G.B. Pergolesi, Monza MB, ItalyNeurosurgery Unit, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, ItalyNeurosurgery Unit, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, Italy; Department of Electrical Engineering and Information Technology (DIETI), University “Federico II”, 21, Via Claudio, Naples, Italy; Corresponding author.Neurosurgery Unit, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, ItalyNeurosurgery Unit, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, ItalyFunctional and Interventional Neuro-Imaging Department, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, ItalyNeurosurgery Unit, Bambino Gesù Children’s Hospital, IRCCS, 4, Piazza S. Onofrio, Rome, ItalyHypophosphatemic rickets is occasionally associated with craniosynostosis. Delayed diagnosis can result in intracranial hypertension, potentially leading to serious neurological complications. We report the case of a 9-year-old boy with X-linked hypophosphatemic rickets who presented with headache, fundus oculi edema, and double sinus pericranii. A CT scan revealed fusion of the cranial sutures, and continuous intracranial pressure monitoring confirmed intracranial hypertension. The patient successfully underwent bilateral cranial expansion. Secondary craniosynostosis is a rare condition, with hypophosphatemic rickets being the most common metabolic cause. Sinus pericranii is a rare radiological manifestation of intracranial hypertension. Craniosynostosis presenting after infancy or early childhood should prompt consideration of secondary or metabolic causes. Regular monitoring for head shape abnormalities and signs of intracranial hypertension is essential in patients with hypophosphatemic rickets. Skull expansion is an effective therapeutic option.http://www.sciencedirect.com/science/article/pii/S2214751925000106Intracranial hypertensionHypophosphatemic ricketsCraniosynostosisCase reportBiparietal craniectomySinus pericranii
spellingShingle Alessio Iacoangeli
Sergio Capelli
Andrea Held
Emidio Procaccini
Davide Luglietto
Matteo Barba
Alessandro De Benedictis
Carlo Gandolfo
Carlo Efisio Marras
Surgical management of intracranial hypertension in rickets-related craniosynostosis
Interdisciplinary Neurosurgery
Intracranial hypertension
Hypophosphatemic rickets
Craniosynostosis
Case report
Biparietal craniectomy
Sinus pericranii
title Surgical management of intracranial hypertension in rickets-related craniosynostosis
title_full Surgical management of intracranial hypertension in rickets-related craniosynostosis
title_fullStr Surgical management of intracranial hypertension in rickets-related craniosynostosis
title_full_unstemmed Surgical management of intracranial hypertension in rickets-related craniosynostosis
title_short Surgical management of intracranial hypertension in rickets-related craniosynostosis
title_sort surgical management of intracranial hypertension in rickets related craniosynostosis
topic Intracranial hypertension
Hypophosphatemic rickets
Craniosynostosis
Case report
Biparietal craniectomy
Sinus pericranii
url http://www.sciencedirect.com/science/article/pii/S2214751925000106
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