Objective: A hallmark feature of failed ventriculoperitoneal shunts in pediatric patients with hydrocephalus is raised intracranial pressure, necessitating immediate shunt revision or temporization to prevent significant morbidity and mortality. Neuro-ophthalmological findings (NOFs) are essential objective indicators for diagnosing shunt failure, which include papilledema, vision loss, optic disc pallor, and disturbances in ocular motility and alignment. These NOFs are also invaluable for long-term follow-up and monitoring of the progression and treatment response in hydrocephalus. However, there are nuances in their sensitivity and specificity regarding shunt failure. This study presents a systematic review of the literature on the clinical correlation between NOFs and shunt failure and their responses following treatment for hydrocephalus.
Methods: A systematic review of the literature focused on the association between NOFs and shunt failure was conducted according to established guidelines. Data were extracted from studies that included patients < 21 years of age with confirmed shunt failure and reported NOFs. Shunt failure was only considered if surgery confirmed the diagnosis. Patients with idiopathic intracranial hypertension and those with ventriculoperitoneal shunt failure without documented prerevision assessment of NOFs were excluded. Descriptive statistics were reported, including percentages for categorical variables and means for continuous variables. The risk of bias was assessed for each study. A common-effects model was used to calculate pooled estimates and account for study variances, with heterogeneity assessed using the I2 statistic. Forest plots illustrated the prevalence of NOFs, overall rates, 95% confidence intervals, and contributing weights.
Results: Four studies with 82 patients had surgically confirmed shunt failures, of whom 51 (62%) exhibited NOFs, including 17 (21%) with papilledema, 11 (13%) with cranial nerve palsy, 9 (11%) with upgaze palsy, and 14 (17%) with vision loss. Concomitant neurological symptoms were noted in 20 (24%) of those patients, including headaches (15%), nausea/vomiting (10%), and lethargy or neurological symptoms (13%). Following shunt revision, improvement in papilledema was reported in 83% of affected patients, and 92% in those with cranial nerve or upgaze palsy, but no improvement was observed in visual acuity. The overall weighted prevalence of papilledema was 30% (95% CI 0.07-0.70; I2 = 67%, p = 0.03).
Conclusions: These findings advocate for the integration of neuro-ophthalmological evaluations in addition to radiographic and clinical assessments to enhance the diagnostic accuracy of shunt failures in pediatric patients with hydrocephalus. Adopting a multidisciplinary approach can augment diagnostic precision and potentially hasten the initiation of treatment for these complex and often nuanced pathologies.
Keywords: hydrocephalus; intracranial pressure; neuro-ophthalmological findings; ophthalmology; papilledema; shunt failure.