Management of Hydrocephalus in Paget's Disease of Bone: Systematic Review and Illustrative Case

OBJECTIVE: Paget's disease of the bone (PDB) is a focal bone disorder characterized by excessive resorption and deposition of pathologic bone. It can involve the skull and cause neurologic dysfunction. Hydrocephalus occurring as a complication has also been reported. However, owing to its rarity, the optimal treatment has not yet been determined. METHODS: We report the case of a 60-year-old woman with a diagnosis of PDB who had presented with symptomatic hydrocephalus and was successfully treated with ventriculoperitoneal shunt insertion. We also performed a systematic review of the Scopus and PubMed databases for case reports and series documenting patients with PDB complicated by hydrocephalus and discussed the clinical features and therapeutic strategies used. RESULTS: We found 28 cases of PDB complicated by hydrocephalus in reported studies, including the present case. The median age was 67 years (range, 48-85 years; interquartile range, 7 years), with a female sex predilection. The most common neurologic manifestations were cognitive impairment, gait unsteadiness, urinary incontinence, hearing loss, and headache. Surgery had been performed in 73% of the 28 cases. The most common operation was insertion of a ventriculoperitoneal shunt (63.2%). Cerebrospinal fluid diversion resulted in significant or complete neurologic recovery in 78.9% of the 28 patients. Posterior fossa decompression was performed in 15.8% of operative cases but did not significantly alter the clinical course. CONCLUSION: Patients with hydrocephalus occurring as a complication of PDB will most commonly present with cognitive impairment, gait unsteadiness, and urinary incontinence. Surgery plays an important role in the treatment, with CSF diversion procedures resulting in significant neurologic recovery in most cases.