Unique Clinical Presentation of Pediatric Shunt Malfunction

Abstract

Introduction: 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 Material and Method: 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. Results: 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, χ² 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, χ² 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, χ² analysis). Other interesting presenting signs were pseudocyst (2), syringomyelia (2), hemiparesis (2) and Parkinson-like rigidity (2). Conclusion: 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.

Serial Neuropsychological Assessment and Evidence of Shunt Malfunction in Spina Bifida: A Longitudinal Case Study

“…Clinical features of acute obstructive hydrocephalus in children and adolescents tend to be neurological including headache, visual disturbances, nausea, vomiting, drowsiness/fatigue, papilloedema, bulging fontanel, failure of upward gaze, dyspraxic gait, general clumsiness, large head size, neck pain, and lower cranial nerve palsy (Arnell, Eriksson, & Olsen, 2003;Garton, Kestle, & Drake, 2001;Lee, Uribe, Ragheb, Morrison, & Jagid, 1999;Pople, 2002). Cognitive symptoms of acute hydrocephalic incidents are noted in the literature but tend to be described in terms of general mental status changes.…”

Ventriculoperitoneal shunt block: what are the best predictive clinical indicators?

“…4 These findings are in keeping with our published unit experience in which we noted a 28% incidence of shunt block over a 10 year period, and that 55% of patients experienced at least one episode of shunt malfunction during this time. 5 The presentation of shunt block may undoubtedly be with what might be termed "classical" symptoms, namely headache, vomiting, and drowsiness, 6 or may on occasion be more atypical and misleading. 7 Documented more atypical presentations include seizures, abdominal pseudocyst, syringomyelia, cranial nerve palsies, and hemiparesis.…”

Effects of ventriculoperitoneal shunt removal on cerebral oxygenation and brain compliance in chronic obstructive hydrocephalus

“…21,24 In spite of numerous studies on experimental hydrocephalus, the pathophysiological aspects of shunt malfunction have not been well examined. To undertake an experimental analysis of shunt malfunction, the hydrocephalus model should allow hydrocephalic animals to survive after induced shunt mal-function.…”