Dandy-walker malformation associated with a large growing occipital meningocele: A rare case report and literature review

Abstract

Dandy-Walker malformation (DWM) is a rare congenital anomaly of the posterior fossa. It is characterized by hypoplasia or agenesis of the cerebellar vermis, cystic dilatation of the fourth ventricle, and upward tentorial displacement. It is commonly associated with central nervous system (CNS) or systemic abnormalities. Occipital meningocele (OMC) is an extremely rare association with DWM, reported in only around 40 cases in the global literature. To our knowledge, this is the second reported case of a growing OMC associated with DWM in English literature, and the first documented in Saudi Arabia. By sharing our case report, we aim to contribute to the existing literature by adding to the available database for future research, which will help address such cases and establish a standardized management protocol to enhance patient care. We present a 4-month-and-7-day-old male infant diagnosed with DWM who developed progressive head enlargement and a growing occipital mass. The patient underwent surgical resection of the OMC sac with cysto-peritoneal shunt insertion and cranioplasty with titanium mesh to repair the bony defect. The occipital mass was large, firm, oval-shaped, and midline at the occiput, measuring 7 cm × 6 cm. Surgical intervention was performed successfully without perioperative complications. Postoperative recovery was uneventful. OMC in association with DWM is rare, and a progressively enlarging OMC is exceptionally uncommon. Management remains controversial due to the absence of standardized surgical guidelines. Our case underscores the importance of reporting such rare occurrences to expand the existing literature and guide future management strategies. This report contributes to the growing body of knowledge on the rare association of OMC with DWM. Collective case documentation is crucial to establishing an evidence base that supports the development of standardized protocols, ultimately enhancing patient safety, surgical outcomes, and long-term prognosis.