Unique clinical presentation of pediatric shunt malfunction

A cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologies of hydrocephalus in the pediatric population. Malfunction of the shunt may present with unique symptoms and signs. This retrospective review investigates the presenting signs and symptoms of pediatric patients with shunt malfunction. Clinical

One-hundred-and-thirty CSF diversion procedures were performed at two affiliated pediatric hospitals over a 2-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 interview to obtain a minimum of 3 months follow-up.

The 65 patients' age ranged from 3 months to 16 years. The original etiology of the hydrocephalus was Chiari II malformation in 17, idiopathic in 15 and intraventricular hemorrhage in 10, neoplasm in 8 patients and meningitis in 5 patients. The most frequent presenting symptoms were headache (39 admissions), nausea/vomiting (28) and drowsiness (21). Seven Chiari patients (41%) presented with neck pain, 2 (12%) presented with lower cranial nerve palsy, and 2 (12%) presented with symptomatic syrinx, complaints not reported by non-Chiari patients (p < 0.01, chi2 analysis). Four myelodysplastic patients presented with a new-onset or recurrent seizure episode, which was significantly more frequent than in nonmyelodysplastic patients (p < 0.05, chi2 analysis). 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 nonneoplastic diseases (2 of 62 cases; p < 0.05, chi2 analysis). Other interesting presenting signs were pseudocyst (2), syringomyelia (2), hemiparesis (2) and Parkinson-like rigidity (2).

Pediatric shunt malfunction generally presents with headache, nausea/vomiting, altered mental status, increased head circumference and bulging fontanelle. Other less frequent but unique presenting signs and symptoms, such as neck pain, syringomyelia and lower cranial nerve palsy in the myelodysplastic population, and Parinaud's syndrome in patients with a history of intracranial neoplasm are frequently associated with shunt malfunction and should prompt a radiographic workup.