Management of hydrocephalus in Paget's disease of bone: systematic review and illustrative case.

Paget's disease of bone (PDB) is a focal bone disorder characterized by excessive bone resorption and deposition of pathologic bone. It can involve the skull and cause neurologic dysfunction. Hydrocephalus occurring as a complication has also been reported, but due to its rarity, the optimal treatment remains to be determined.We report the case of a 60-year-old female diagnosed with PDB presenting with symptomatic hydrocephalus and 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 employed.A total of 28 cases of PDB complicated by hydrocephalus were reported in the literature, including the current case. The median age was 67 years (range: 48 - 85; IQR: 7 years), with a female sex predilection. The most common neurologic manifestations were cognitive impairment, gait unsteadiness, urinary incontinence, hearing loss, and headache. Surgery was performed in 73% of cases, with the most common operation being insertion of a ventriculoperitoneal shunt (63.2%). CSF diversion resulted in significant or complete neurologic recovery in 78.9% of cases. Posterior fossa decompression was performed in 15.8% of operative cases but did not significantly alter the patients' clinical course.Hydrocephalus occurring as a complication of PDB most commonly presents with cognitive impairment, gait unsteadiness, and urinary incontinence. Surgery plays an important role in treatment, with CSF diversion procedures resulting in significant neurologic recovery in most cases.