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Child's Nervous System

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01-05-2018 | Case Report

Endoscopic third ventriculostomy for hydrocephalus in osteopetrosis: a case report and review of the literature

Authors: Nobuyuki Akutsu, Junji Koyama, Atsufumi Kawamura, Tatsuya Nagashima, Masaaki Taniguchi, Eiji Kohmura

Published in: Child's Nervous System | Issue 5/2018

Abstract

Introduction

There are very few reports in the literature associating in hydrocephalus in osteopetrosis. As a complication of shunt procedure, there are two reports on shunt malfunction due to osseous overgrowth at the burr hole in patients with osteopetrosis. We herein report a case of osteopetrosis with hydrocephalus that was successfully treated with endoscopic third ventriculostomy (ETV).

Case report

At 5 months of age, a male patient presented with developmental delay. Head computed tomography (CT) demonstrated triventricular hydrocephalus with a cerebellar tonsillar herniation. At 7 months of age, he underwent suboccipital decompression with decompression of the foramen magnum. The hydrocephalus did not improve postoperatively, and the patient was transferred to our hospital. At 12 months of age, the hydrocephalus was successfully treated with ETV. The postoperative period was uneventful. Postoperative CT demonstrated an improvement in the ventricle size.

Conclusions

The etiology of hydrocephalus in osteopetrosis is not completely understood; however, there have been several reports in which ETV was effective. ETV should be considered the treatment of choice for hydrocephalus in osteopetrosis, as it avoids the characteristic shunt complications that can occur in patients with osteopetrosis.

Al-Mefty O, Fox JL, Al-Rodhan N, Dew JH (1988) Optic nerve decompression in osteopetrosis. J Neurosurg 68(1):80–84. https://doi.org/10.3171/jns.1988.68.1.0080 CrossRefPubMed

Al-Tamimi YZ, Tyagi AK, Chumas PD, Crimmins DW (2008) Patients with autosomal-recessive osteopetrosis presenting with hydrocephalus and hindbrain posterior fossa crowding. J Neurosurg Pediatr 1(1):103–106. https://doi.org/10.3171/PED-08/01/103 CrossRefPubMed

Amacher AL (1977) Neurological complications of osteopetrosis. Childs Brain 3(5):257–264PubMed

Baird PA, Robinson GC, Hardwick DF, Sovereign AE (1968) Congenital osteopetrosis: an unusual cause of hydrocephalus. Can Med Assoc J 98(7):362–365PubMedPubMedCentral

Dhamija B, Pettorini BL, Solanki G (2011) Endoscopic third ventriculostomy for the treatment of osteopetrosis-related hydrocephalus: a case-based update. Childs Nerv Syst 27(11):1861–1865. https://doi.org/10.1007/s00381-011-1474-1 CrossRefPubMed

Etus V, Ceylan S (2005) The role of endoscopic third ventriculostomy in the treatment of triventricular hydrocephalus seen in children with achondroplasia. Report of 2 cases. J Neurosurg 103(3 Suppl):260–265. https://doi.org/10.3171/ped.2005.103.3.0260 PubMed

Grossman R, Feldman Z (2004) Bone growth causing ventriculoperitoneal shunt malfunction in a patient with osteopetrosis. Case report. J Neurosurg 100(5 Suppl Pediatrics):530–531. https://doi.org/10.3171/ped.2004.100.5.0530 PubMed

Kehler U, Regelsberger J, Gliemroth J, Westphal M (2006) Outcome prediction of third ventriculostomy: a proposed hydrocephalus grading system. Minim Invasive Neurosurg 49(4):238–243. https://doi.org/10.1055/s-2006-950382 CrossRefPubMed

King JA, Vachhrajani S, Drake JM, Rutka JT (2009) Neurosurgical implications of achondroplasia. A review. J Neurosurg Pediatr 4(4):297–306. https://doi.org/10.3171/2009.3.PEDS08344 CrossRefPubMed

10.

Lehman RA, Reeves JD, Wilson WB, Wesenberg RL (1977) Neurological complications of infantile osteopetrosis. Ann Neurol 2(5):378–384. https://doi.org/10.1002/ana.410020506 CrossRefPubMed

11.

Mahmoud Adel AH, Abdullah AA, Eissa F (2013) Infantile osteopetrosis, craniosynostosis, and Chiari malformation type I with novel OSTEM1 mutation. J Pediatr Neurosci 8(1):34–37. https://doi.org/10.4103/1817-1745.111420 CrossRefPubMedPubMedCentral

12.

Rossini Z, Castellani C, Borsa S, Carrabba G, Locatelli M, Di Cristofori A (2016) Atypical Association of Ethmoidal Encephalocele and Hydrocephalus in an adult patient with Autosomal-Dominant Osteopetrosis Type I (ADO-I): a case report. World Neurosurg 89:731.e13–731.e17CrossRef

13.

Scott WW, Weprin BE, Swift DM (2014) A unifying theory for the multifactorial origin of cerebellar tonsillar herniation and hydrocephalus in osteopetrosis. J Neurosurg Pediatr 14(6):665–673. https://doi.org/10.3171/2014.8.PEDS14190 CrossRefPubMed

14.

Steward CG (2003) Neurological aspects of osteopetrosis. Neuropathol Appl Neurobiol 29(2):87–97. https://doi.org/10.1046/j.1365-2990.2003.00474.x CrossRefPubMed

15.

Swift DM, Nagy L, Robertson B (2012) Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia. Report of 3 cases. J Neurosurg Pediatr 9(1):73–81. https://doi.org/10.3171/2011.10.PEDS1169 CrossRefPubMed

16.

Tolar J, Teitelbaum SL, Orchard PJ (2004) Osteopetrosis. N Engl J Med 351(27):2839–2849. https://doi.org/10.1056/NEJMra040952 CrossRefPubMed

17.

Turgut M, Aral YZ, Ozsunar Y (2010) Autosomal recessive osteopetrosis as an unusual cause of hydrocephalus, extensive calcification of tentorium cerebelli, and calvarial hyperostosis. Case report. J Neurosurg Pediatr 5(4):419–421. https://doi.org/10.3171/2009.11.PEDS09370 CrossRefPubMed

Title: Endoscopic third ventriculostomy for hydrocephalus in osteopetrosis: a case report and review of the literature
Authors: Nobuyuki Akutsu
Junji Koyama
Atsufumi Kawamura
Tatsuya Nagashima
Masaaki Taniguchi
Eiji Kohmura
Publication date: 01-05-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 5/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3719-8

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Endoscopic third ventriculostomy for hydrocephalus in osteopetrosis: a case report and review of the literature

Abstract

Introduction

Case report

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Introduction

Case report

Conclusions

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