Extensive Pediatric Rathke's Cleft Cyst with Suprasellar, Intraventricular, and Cerebellopontine Angle Invasion: A Case Report with Long-Term Outcome.

Rathke's cleft cysts (RCCs) are benign, sellar, or suprasellar lesions that originate from remnants of Rathke's pouch. While often asymptomatic and detected incidentally, symptomatic RCCs may cause headaches, visual disturbances, and endocrine dysfunction due to mass effect. Pediatric RCCs are rare and often present distinct challenges. A 12-year-old male presented with a 4-month history of imbalance and morning headaches. Imaging revealed a large cystic lesion with suprasellar, intraventricular, and cerebellopontine angle extension, along with obstructive hydrocephalus. The patient underwent a left transcortical transventricular surgical approach for decompression, during which an adherent solid component was deliberately left in situ to avoid hypothalamic injury. Histopathological analysis confirmed a ruptured RCC with xanthogranulomatous changes. Postoperatively, the patient required a ventriculoperitoneal shunt. During long-term follow-up, he developed multiple endocrine deficiencies. Serial imaging over 4 years showed stable residual. This case demonstrates extensive pediatric RCC with rare intracranial spread, causing panhypopituitarism, hydrocephalus, and persistent deficits. It highlights multidisciplinary management, the need for long-term surveillance for recurrence and endocrine dysfunction, and emphasizes that hypothalamic-adherent solid components should be left unresected to prioritize hypothalamic functional preservation over a long-term follow-up period.