Severe cranial deformity following cerebrospinal fluid diversion in an adolescent with osteogenesis imperfecta

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  • 1 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland; and
  • 2 Department of Neurosurgery, University of Virginia, Charlottesville, Virginia
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Osteogenesis imperfecta (OI) is an inherited connective tissue disorder that causes bone fragility and deformity. Neurological manifestations, including macrocephaly and hydrocephalus, have been reported. Increased vascular fragility or bleeding diathesis also predisposes OI patients to intracranial hemorrhage. The development of chronic subdural fluid collections or hydrocephalus may require CSF diversion. The authors report a previously unrecognized complication of CSF diversion in a patient with OI, that is, a delayed severe cranial deformity, presumably due to over-shunting. In addition to the cosmetic concern, the deformity caused severe headaches and tenderness. The patient underwent craniectomy and titanium mesh cranioplasty, which resulted in the complete resolution of symptoms. This report raises the possibility that over-shunting in patients with OI could predispose to the formation of cranial deformity requiring surgical intervention.

ABBREVIATIONS OI = osteogenesis imperfecta; SPS = subdural peritoneal shunt; VPS = ventriculoperitoneal shunt.

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Contributor Notes

Correspondence John A. Jane Jr.: University of Virginia Health System, Charlottesville, VA. johnjanejr@virginia.edu.

INCLUDE WHEN CITING Published online July 6, 2018; DOI: 10.3171/2018.4.PEDS18109.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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