Cranial reconstruction for treatment of intracranial hypertension from sclerosteosis: case-based update. World Neurosurg 2014 Feb;81(2):442.e1-5
Date
11/28/2012Pubmed ID
23182730DOI
10.1016/j.wneu.2012.11.061Scopus ID
2-s2.0-84896393761 (requires institutional sign-in at Scopus site) 5 CitationsAbstract
BACKGROUND: Sclerosteosis (OMIM 269500) is a progressive, autosomal recessive, sclerosing bone disorder with a well defined phenotype. This phenotype is correlated with a deficiency in the protein product sclerostin, leading to bony overgrowth from the loss of inhibition of osteocyte function. Calvarial overgrowth can lead to cranial nerve palsies, visual impairment, and compression of the medulla at the foramen magnum. There is a presumption that calvarial thickening may lead to elevated intracranial pressure in these patients, although pressure measurements have not been published.
CASE DESCRIPTION: The authors report the case of a 28-year-old Saudi Arabian man with sclerostosis, progressive headaches, and a cervical spinal cord syrinx. A cranial reconstruction was performed by aggressively thinning the thickened cortical bone, thereby expanding the intracranial space. The measured intracranial pressure was 25-40 mm HG under anesthesia.
CONCLUSIONS: After surgery, the patient had resolution of the headaches and radiographic near-resolution of the syrinx. The authors review their experience and the relevant literature with this rare case.
Author List
Tholpady S, Dodd ZH, Havlik RJ, Fulkerson DHAuthor
Robert Havlik MD Chair, Professor in the Plastic Surgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultHumans
Hyperostosis
Intracranial Hypertension
Magnetic Resonance Imaging
Male
Skull
Syndactyly
Treatment Outcome
