Unique clinical presentation of pediatric shunt malfunction

cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologie s of hydrocephalus in the pediatric population. Malfunction of the shunt ma y present with unique symptoms and signs. This retrospective review investi gates the presenting signs and symptoms of pediatric patients with shunt ma lfunction. Clinical Material and Method: One-hundred-and-thirty CSF diversi on procedures were performed at two affiliated pediatric hospitals over a P -year period. Seventy consecutive cases of CSF shunt revision were reviewed . These 70 operations were performed on 65 patients. Their medical records and radiographic studies were reviewed, and supplemented with a telephone i nterview to obtain a minimum of 3 months follow-up. Results: The 65 patient s' age ranged from 3 months to 16 years. The original etiology of the hydro cephalus was Chiari II malformation in 17, idiopathic in 15 and intraventri cular hemorrhage in 10, neoplasm in 8 patients and meningitis in 5 patients . The most frequent presenting symptoms were headache (39 admissions), naus ea/vomiting (28) and drowsiness (21). Seven Chiari patients (41%) presented with neck pain, 2 (12%) presented with lower cranial nerve palsy, and 2 (1 2%) presented with symptomatic syrinx, complaints not reported by non-Chiar i patients (p < 0.01, chi(2) analysis). Four myelodysplastic patients prese nted with a new-onset or recurrent seizure episode, which was significantly more frequent than in nonmyelo-dysplastic patients (p < 0.05, chi(2) analy sis). On examination, increased head circumference was noted in 17 patients . Parinaud's syndrome was noted more prominently in patients with a history of intracranial neoplasm (4 of 8 cases) than in patients with nonneoplasti c diseases (2 of 62 cases; p < 0.05, chi(2) analysis). Other interesting pr esenting signs were pseudocyst (2), syringomyelia (2), hemiparesis (2) and Parkinson-like rigidity (2). Conclusion: Pediatric shunt malfunction genera lly presents with headache, nausea/vomiting, altered mental status, increas ed head circumference and bulging fontanelle. Other less frequent but uniqu e presenting signs and symptoms, such as neck pain, syringomyelia and lower cranial nerve palsy in the myelodysplastic population, and Parinaud's synd rome in patients with a history of intracranial neoplasm are frequently ass ociated with shunt mal function and should prompt a radiographic workup.