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

Abstract

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.

Keywords

Rathke's cleft cyst - suprasellar lesion - pediatric neurosurgery - endocrine dysfunction - hydrocephalus

Patients' Consent

Informed consent was obtained from all the participants of the study.

Publikationsverlauf

Artikel online veröffentlicht:
03. Februar 2026

• References

• 1 Larkin S, Karavitaki N, Ansorge O. Rathke's cleft cyst. Handb Clin Neurol 2014; 124: 255-269
• 2 Montaser AS, Catalino MP, Laws ER. Professor Rathke's gift to neurosurgery: the cyst, its diagnosis, surgical management, and outcomes. Pituitary 2021; 24 (05) 787-796
• 3 Zada G, Ditty B, McNatt SA, McComb JG, Krieger MD. Surgical treatment of Rathke cleft cysts in children. Neurosurgery 2009; 64 (06) 1132-1137 , author reply 1037–1038
• 4 Cuellar-Hernández JJ, Ortega-Ruiz OR, Rodriguez-Armendariz AG. et al. Accurate preoperative diagnosis of a Rathke cleft cyst with the aid of a novel classification for sellar cystic lesions and a diagnostic algorithm decision: Tools for differentiating cystic sellar lesions with a representative case. Surg Neurol Int 2024; 15: 120
• 5 Brors D, Appelt A, Bockmeyer B, Preuss A. Primary Rathke's cleft cyst in the cerebellopontine angle associated with apoplexy: case report and review of the literature. J Neurol Surg B Skull Base 2013; 74 (Suppl. 01) e10-e14
• 6 Fan J, Qi S, Peng Y, Zhang XA, Qiu B, Pan J. An isolated primary Rathke's cleft cyst in the cerebellopontine angle. J Neurosurg 2014; 121 (04) 846-850
• 7 Brou C, Tatar IG. Different faces of Rathke's cleft cyst. J Belg Soc Radiol 2023; 107 (01) 15
• 8 Choi SH, Kwon BJ, Na DG, Kim JH, Han MH, Chang KH. Pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions: differentiation using MRI. Clin Radiol 2007; 62 (05) 453-462
• 9 Lu VM, Ravindran K, Perry A. et al. Recurrence of Rathke's cleft cysts based on gross total resection of cyst wall: a meta-analysis. Neurosurg Rev 2020; 43 (03) 957-966
• 10 Aho CJ, Liu C, Zelman V, Couldwell WT, Weiss MH. Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 2005; 102 (02) 189-193