Neurosurgical management of leukoencephalopathy, cerebral calcifications, and cysts: A case report and review of literature

BACKGROUND: Leukoencephalopathy, Calcification, and Cyst (LCC) is a syndrome describing the rare concurrence of these three unusual radiographic findings. Here, we describe the neurosurgical management in a patient afflicted with LCC and review the existing literature on surgical indications and outcomes. CASE DESCRIPTION: A 24-year-old man presented with symptoms of progressive headache, gait imbalance and horizontal diplopia. Magnetic resonance imaging (MRI) showed radiographic findings typically associated with LCC, including a large pontine cyst with significant mass effect. The patient's symptoms resolved after open surgical cyst drainage. However, he suffered cyst re-accumulation 3 months after the initial procedure and ultimately underwent placement of a ventriculo-cysto-peritoneal shunt. At the 3-year follow-up, the patient remained symptom free with continued cyst decompression. CONCLUSION: Our case report suggests that ventriculo-cysto-peritoneal shunting appeared an effective strategy in LCC patients in whom the cyst fenestration failed. We present this case report in the context of the first systematic review of literature on neurosurgical management strategies for patients afflicted with LCC.