Cranial vault expansion in treatment of paediatric idiopathic intracranial hypertension

Introduction: Idiopathic intracranial hypertension (IIH) is a rare clinical entity in the paediatric population. Clinical presentation is mostly similar to adult counterpart and can include headaches, vomiting, papilloedema, deterioration in visual acuity or fields, and 6th cranial nerve palsy, leading to significant morbidity. Therapeutic lumbar puncture and medical treatment with acetazolamide are usually the first-line treatments. In a minority of refractory cases, particularly where visual function is threatened, CSF diversion has been traditionally used in clinical practice. CSF diversion in IIH patients can be associated with high rate of shunt malfunction and revision due to small ventricular size leading to repeated procedures. We describe our experience with cranial vault expansion as a method of treatment of paediatric IIH cases refractory to medical treatment as a CSF diversion sparing method.

Methods: Following review of IIH cases undergoing surgical treatment in our unit over 15 years (years 2009-2024), cases receiving cranial vault expansion as primary surgical treatment were selected and further analysed. Inclusion criteria were paediatric cases (age < 16) undergoing vault expansion as first surgical treatment for IIH refractory to pharmacological treatment for vision-threatening papilloedema. All cases were discussed within multidisciplinary meeting and selected following consideration of all management options.

Results: Over the studied period, we identified two cases of refractory IIH with severe papilloedema undergoing cranial vault expansion as primary shunt-sparing surgery. Both patients presented with symptoms of headaches, vomiting, and blurred vision with ophthalmological confirmation of severe papilloedema despite pharmacological therapy. Following multidisciplinary discussions, both underwent successful supratentorial vault expansion. Pharmacological treatment was discontinued in both cases following surgery. Evaluation at latest follow-up showed resolution of symptoms and papilloedema with no need for subsequent cerebrospinal fluid diversion at the latest follow-up.

Conclusion: Cranial vault expansion is a viable and safe alternative surgical option in paediatric IIH cases refractory to medical treatment obviating the need for repeated shunt procedures. In cases with extremely small ventricles or where parental choice precludes CSF diversion, cranial vault expansion can be considered.