An Integrated Approach to the Management of Meningoencephalocoeles

Abstract

Meningoencephalocoeles are congenital herniations of meningeal and cerebral tissues through a cranial defect. They occur most commonly in South-East Asia, and are relatively rare amongst European ancestry populations, with an estimated prevalence of 1/40,000 live births. The treatment of congenital meningoencephalocoeles is primarily surgical and are best managed by dedicated multi-disciplinary craniofacial teams. The authors performed a retrospective case review of all primary meningoencephalocoeles managed in the Oxford University Hospitals NHS Foundation Trust between 1986 and 2012. Twenty-nine cases (13 frontal, 9 occipital, 2 parietal, and 5 basal) were included in this study. The median age at presentation was 11 months (range 0-60 years). Twenty-five cases presented with an external mass; 3 with recurrent meningitis and 1 with otorrhoea. Twenty-six cases underwent surgery, and 17 of these were managed by an integrated approach between 2 or more surgical specialties. Twenty out of 26 operations were performed via a transcranial approach. The authors describe a particularly complex case in order to highlight the challenges associated with management of meningoencephalocoeles, the surgical technique employed, and the importance of a multidisciplinary surgical approach. This is the largest reported case series of meningoencephalocoeles managed in a single hospital in the United Kingdom. Designated craniofacial units with access to multidisciplinary surgical specialties provide a safe and optimal setting for the management of meningoencephalocoeles.