Aggressive cranial vault decompression for cranial hyperostosis: Technical case report of two cases

J. Mocco, Ricardo J. Komotar, Brad Zacharia, Neil A. Feldstein, Jeffrey N. Bruce, Charles J. Hodge, Jack P. Rock, Joel D. MacDonald

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

OBJECTIVE AND IMPORTANCE: Camurati-Engelmann's disease, also known as progressive diaphysial dysplasia, is a disorder of the bone metabolism. Neurological manifestations of progressive diaphysial dysplasia include cranial nerve dysfunction, generalized weakness, cerebellar herniation, and increased intracranial pressure. In the past, surgical intervention has been of limited and temporary benefit. We present two patients with cranial hyperostosis secondary to Camurati-Engelmann's disease who were treated successfully with a single surgery involving a combination of multiple craniotomies for cranial vault decompression. CLINICAL PRESENTATION: Two patients presented with signs and symptoms of increased intracranial pressure secondary to Camurati-Engelmann's syndrome. Radiological workup revealed marked cranial hyperostosis. INTERVENTION: The patients underwent aggressive cranial vault decompression. Multiple craniotomies were performed, and the inner table was then drilled down until the bone was 1 cm thick. CONCLUSION: Effective surgical options are needed for clinically significant cranial hyperostosis. In an effort to further define operative management in these patients, we describe a single, aggressive surgical procedure that may be used for successful cranial decompression.

Original languageEnglish (US)
JournalNeurosurgery
Volume57
Issue number1 SUPPL.
DOIs
StatePublished - Jul 1 2005

Fingerprint

Hyperostosis
Camurati-Engelmann Syndrome
Decompression
Craniotomy
Intracranial Pressure
Encephalocele
Bone and Bones
Cranial Nerves
Neurologic Manifestations
Signs and Symptoms

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Mocco, J., Komotar, R. J., Zacharia, B., Feldstein, N. A., Bruce, J. N., Hodge, C. J., ... MacDonald, J. D. (2005). Aggressive cranial vault decompression for cranial hyperostosis: Technical case report of two cases. Neurosurgery, 57(1 SUPPL.). https://doi.org/10.1227/01.NEU.0000163686.75095.B8
Mocco, J. ; Komotar, Ricardo J. ; Zacharia, Brad ; Feldstein, Neil A. ; Bruce, Jeffrey N. ; Hodge, Charles J. ; Rock, Jack P. ; MacDonald, Joel D. / Aggressive cranial vault decompression for cranial hyperostosis : Technical case report of two cases. In: Neurosurgery. 2005 ; Vol. 57, No. 1 SUPPL.
@article{932fbbf08ec94ba9b5750b4db499fe13,
title = "Aggressive cranial vault decompression for cranial hyperostosis: Technical case report of two cases",
abstract = "OBJECTIVE AND IMPORTANCE: Camurati-Engelmann's disease, also known as progressive diaphysial dysplasia, is a disorder of the bone metabolism. Neurological manifestations of progressive diaphysial dysplasia include cranial nerve dysfunction, generalized weakness, cerebellar herniation, and increased intracranial pressure. In the past, surgical intervention has been of limited and temporary benefit. We present two patients with cranial hyperostosis secondary to Camurati-Engelmann's disease who were treated successfully with a single surgery involving a combination of multiple craniotomies for cranial vault decompression. CLINICAL PRESENTATION: Two patients presented with signs and symptoms of increased intracranial pressure secondary to Camurati-Engelmann's syndrome. Radiological workup revealed marked cranial hyperostosis. INTERVENTION: The patients underwent aggressive cranial vault decompression. Multiple craniotomies were performed, and the inner table was then drilled down until the bone was 1 cm thick. CONCLUSION: Effective surgical options are needed for clinically significant cranial hyperostosis. In an effort to further define operative management in these patients, we describe a single, aggressive surgical procedure that may be used for successful cranial decompression.",
author = "J. Mocco and Komotar, {Ricardo J.} and Brad Zacharia and Feldstein, {Neil A.} and Bruce, {Jeffrey N.} and Hodge, {Charles J.} and Rock, {Jack P.} and MacDonald, {Joel D.}",
year = "2005",
month = "7",
day = "1",
doi = "10.1227/01.NEU.0000163686.75095.B8",
language = "English (US)",
volume = "57",
journal = "Neurosurgery",
issn = "0148-396X",
publisher = "Lippincott Williams and Wilkins",
number = "1 SUPPL.",

}

Mocco, J, Komotar, RJ, Zacharia, B, Feldstein, NA, Bruce, JN, Hodge, CJ, Rock, JP & MacDonald, JD 2005, 'Aggressive cranial vault decompression for cranial hyperostosis: Technical case report of two cases', Neurosurgery, vol. 57, no. 1 SUPPL.. https://doi.org/10.1227/01.NEU.0000163686.75095.B8

Aggressive cranial vault decompression for cranial hyperostosis : Technical case report of two cases. / Mocco, J.; Komotar, Ricardo J.; Zacharia, Brad; Feldstein, Neil A.; Bruce, Jeffrey N.; Hodge, Charles J.; Rock, Jack P.; MacDonald, Joel D.

In: Neurosurgery, Vol. 57, No. 1 SUPPL., 01.07.2005.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Aggressive cranial vault decompression for cranial hyperostosis

T2 - Technical case report of two cases

AU - Mocco, J.

AU - Komotar, Ricardo J.

AU - Zacharia, Brad

AU - Feldstein, Neil A.

AU - Bruce, Jeffrey N.

AU - Hodge, Charles J.

AU - Rock, Jack P.

AU - MacDonald, Joel D.

PY - 2005/7/1

Y1 - 2005/7/1

N2 - OBJECTIVE AND IMPORTANCE: Camurati-Engelmann's disease, also known as progressive diaphysial dysplasia, is a disorder of the bone metabolism. Neurological manifestations of progressive diaphysial dysplasia include cranial nerve dysfunction, generalized weakness, cerebellar herniation, and increased intracranial pressure. In the past, surgical intervention has been of limited and temporary benefit. We present two patients with cranial hyperostosis secondary to Camurati-Engelmann's disease who were treated successfully with a single surgery involving a combination of multiple craniotomies for cranial vault decompression. CLINICAL PRESENTATION: Two patients presented with signs and symptoms of increased intracranial pressure secondary to Camurati-Engelmann's syndrome. Radiological workup revealed marked cranial hyperostosis. INTERVENTION: The patients underwent aggressive cranial vault decompression. Multiple craniotomies were performed, and the inner table was then drilled down until the bone was 1 cm thick. CONCLUSION: Effective surgical options are needed for clinically significant cranial hyperostosis. In an effort to further define operative management in these patients, we describe a single, aggressive surgical procedure that may be used for successful cranial decompression.

AB - OBJECTIVE AND IMPORTANCE: Camurati-Engelmann's disease, also known as progressive diaphysial dysplasia, is a disorder of the bone metabolism. Neurological manifestations of progressive diaphysial dysplasia include cranial nerve dysfunction, generalized weakness, cerebellar herniation, and increased intracranial pressure. In the past, surgical intervention has been of limited and temporary benefit. We present two patients with cranial hyperostosis secondary to Camurati-Engelmann's disease who were treated successfully with a single surgery involving a combination of multiple craniotomies for cranial vault decompression. CLINICAL PRESENTATION: Two patients presented with signs and symptoms of increased intracranial pressure secondary to Camurati-Engelmann's syndrome. Radiological workup revealed marked cranial hyperostosis. INTERVENTION: The patients underwent aggressive cranial vault decompression. Multiple craniotomies were performed, and the inner table was then drilled down until the bone was 1 cm thick. CONCLUSION: Effective surgical options are needed for clinically significant cranial hyperostosis. In an effort to further define operative management in these patients, we describe a single, aggressive surgical procedure that may be used for successful cranial decompression.

UR - http://www.scopus.com/inward/record.url?scp=21744447338&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=21744447338&partnerID=8YFLogxK

U2 - 10.1227/01.NEU.0000163686.75095.B8

DO - 10.1227/01.NEU.0000163686.75095.B8

M3 - Article

C2 - 15987595

AN - SCOPUS:21744447338

VL - 57

JO - Neurosurgery

JF - Neurosurgery

SN - 0148-396X

IS - 1 SUPPL.

ER -