Clinical outcome of cerebrospinal fluid shunting for communicating hydrocephalus in mucopolysaccharidoses I, II, and III: a retrospective analysis of 13 patients.
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2010-12
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Abstract
Background
Intracranial pathology is a well-documented feature of mucopolysaccharidoses (MPSs), including communicating hydrocephalus (CH). Neither the success nor the complications of cerebrospinal fluid shunting in MPS patients have been well documented.Objective
To retrospectively analyze 13 children with communicating hydrocephalus and MPS at our institution between 1998 and 2006.Methods
Thirteen patients diagnosed with MPS I, II, or III presenting for stem cell transplantation were retrospectively analyzed. Patients underwent a rigorous pretransplantation workup, including magnetic resonance imaging of the brain. If imaging revealed ventriculomegaly, a lumbar puncture was performed. If intracranial pressure was >20 cm H20 or the patient demonstrated clinical signs of hydrocephalus or evidence of clinical decline with increasing ventricular size on imaging, a ventriculoperitoneal shunt (VPS) was placed. Clinical outcomes were analyzed after dividing the patients into 2 groups: patients who underwent VPS before (group A) and after (Group B) stem cell transplantation.Results
There were 8 patients in group A and 5 in group B. Group B patients developed more severe complications, including 2 patients who required VPS early after transplantation, one who died secondary to intracerebral hemorrhage and another who developed a subdural empyema. Of the 8 patients in group A, 5 had complications, including 2 shunt infections, a punctate intracerebral hematoma, shunt tube migration, and 3 shunt failures.Conclusion
This is the largest review of MPS patients with communicating hydrocephalus. It demonstrates that VPS is an effective treatment. MPS patients need to be evaluated for hydrocephalus before stem cell transplantation because pretransplantation shunting appears to have the most favorable risk/benefit ratio.Type
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Aliabadi, Hamidreza, Renee Reynolds, Ciaran J Powers, Gerald Grant, Herbert Fuchs and Joanne Kurtzberg (2010). Clinical outcome of cerebrospinal fluid shunting for communicating hydrocephalus in mucopolysaccharidoses I, II, and III: a retrospective analysis of 13 patients. Neurosurgery, 67(6). pp. 1476–1481. 10.1227/neu.0b013e3181f8c11d Retrieved from https://hdl.handle.net/10161/24643.
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Scholars@Duke
Herbert Edgar Fuchs
Clinical neuro-oncology research including collaborations studying molecular genetics of childhood brain tumors.
Potential role of the free electron laser in surgery of pediatric brain tumors. Current work includes animal models with human brain tumor xenografts in preclinical studies.
Collaboration with the neurooncology laboratory of Dr. Darell Bigner in preclinical studies of new therapeutic agents.
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