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

Introduction

Rathke's cleft cysts (RCCs) represent benign cystic formations originating from embryonic Rathke's pouch remnants, typically occupying sellar/suprasellar regions. While often asymptomatic and discovered incidentally, symptomatic RCCs can present with headaches, visual disturbances, and endocrine dysfunction due to mass effect on surrounding structures.[1] These cysts are typically small and confined to the pituitary region.[2] Pediatric RCCs are rare, constituting only a small fraction of surgically treated cases, and they pose distinct clinical challenges due to their suprasellar extension, overlap with neoplastic lesions, and potential for severe endocrine or neurological complications.[3]

Radiologically, typical RCCs contain homogeneous cystic contents with thin walls. The presence of a solid or calcified component or irregular enhancement is unusual and raises suspicion for alternative diagnoses.[4]

While rare extensive RCCs arising primarily in the posterior fossa or cerebellopontine angle have been reported,[5] [6] extension of a sellar RCC into the posterior fossa is exceedingly uncommon, and to our knowledge, no pediatric case with concomitant intraventricular and cerebellopontine angle extension has been previously described.

Case Description

Initial Presentation

A 12-year-old male presented with a 4-month history of progressive gait imbalance and mild headaches. The headaches were described as occipital, occurring predominantly in the early morning and occasionally waking up the patient from sleep. There was no associated nausea, vomiting, weakness, or numbness. Initial evaluation at an outside facility revealed papilledema, prompting brain imaging. CT of the brain demonstrated a large cystic lesion with suprasellar, intraventricular, and left cerebellopontine angle components ([Fig. 1]). The differential diagnosis included craniopharyngioma and pilocytic astrocytoma.

Fig. 1 Preoperative CT scan of the brain showing a large suprasellar cystic lesion with left cerebellopontine (CP) angle extension.

Preoperative Evaluation

Brain magnetic resonance imaging (MRI) revealed a large, predominantly cystic suprasellar mass measuring 7.0 × 6.6 × 4.3 cm (anteroposterior × craniocaudal × transverse) with calcified/solid components exhibiting rim enhancement and diffusion restriction. The lesion was displacing the third ventricle and septum pellucidum to the right, resulting in obstructive hydrocephalus with a cystic extension into the left posterior fossa, compressing the brainstem ([Fig. 2]).

Fig. 2 Preoperative MRI of the brain showing the lesion.

Ophthalmological assessment demonstrated visual acuity (20/130), bilateral optic disc swelling, decreased left eye, and enlarged blind spots. Endocrine evaluation revealed a prepubertal hormonal profile, low IGF-1, and mild hyperprolactinemia.

Operative Management

Given the symptomatic nature of the lesion and its significant mass effect, the patient was taken to the operating room for a left frontal craniotomy. A left transcortical transventricular approach was utilized to access the lesion. Intraoperatively, the cyst was identified in the left lateral ventricle, and cystic fluid was aspirated. The cyst wall was dissected safely and grossly totally resected, with residual solid component adherent to the hypothalamus left in situ to avoid injury. An external ventricular drain (EVD) was placed to manage hydrocephalus.

A left transcortical transventricular approach was selected rather than an endoscopic endonasal route, not only because of the intraventricular component but also due to the overall configuration of the lesion. In this patient, the cyst formed a large intraventricular dome with superior displacement of the third ventricle and a posterior extension into the left cerebellopontine angle, causing obstructive hydrocephalus. A purely endonasal approach would have provided limited access to the intraventricular and posterior fossa components and would not have allowed safe circumferential dissection of the cyst wall.

Histopathology

Histopathological examination of the resected specimen confirmed the diagnosis of a ruptured RCC with xanthogranulomatous changes.

Postoperative Course

The patient was monitored in the pediatric intensive care unit (PICU). The EVD was converted to a ventriculoperitoneal shunt due to persistent hydrocephalus. The patient was discharged on postoperative day 8 in stable condition.

Long-Term Follow-up

Over the next 3 years, the patient developed multiple endocrine deficiencies, including central hypothyroidism, growth hormone deficiency, and delayed puberty. He also developed morbid obesity (body mass index: 39 kg/m²) and mild hyperprolactinemia.

Serial MRI scans over 4 years demonstrated stable residual without progression ([Fig. 3]).

Fig. 3 Follow-up MRI of the brain demonstrating stable residual suprasellar soft tissue.

Endocrine Management

The patient was started on levothyroxine for central hypothyroidism and somatropin for growth hormone deficiency. Despite these interventions, he remained prepubertal at the age of 15 years, with no secondary sexual characteristics. Testosterone therapy was initiated to address delayed puberty. Additionally, cabergoline was prescribed to manage hyperprolactinemia.

Discussion

The presented case highlights the diagnostic and therapeutic challenges associated with atypical RCCs in pediatric populations. This case demonstrates the potential for extensive growth and multisystem involvement in children, necessitating collaborative management strategies.

Presentation and Diagnostic Challenges

The patient's symptoms—imbalance, morning headaches, and papilledema—reflect mass effect on surrounding structures, consistent with reports that headaches (80%) and endocrine dysfunction (60%) are the most common presentations of symptomatic pediatric RCCs.[3] However, the cyst's extension into the cerebellopontine angle and third ventricle is highly atypical, as RCCs are classically confined to the sellar region.[2] The initial diagnostic dilemma—differentiating this lesion from craniopharyngioma or pilocytic astrocytoma—mirrors the literature.

Importantly, imaging also revealed a solid nodule intimately adherent to the hypothalamus. Such a solid or calcified component is unusual for RCC and broadened the differential to include cystic craniopharyngioma and degenerating pituitary adenoma.[7] Only a handful of primary RCCs arising in the cerebellopontine angle have been reported,[5] [6] and, to our knowledge, extension of a sellar RCC into this region has not previously been described. However, the absence of calcification and the homogeneous cyst fluid signal on MRI were more in keeping with RCC rather than craniopharyngioma.[3] [8]

Surgical Considerations and Outcomes

The decision to pursue a transcortical transventricular approach was necessitated by the lesion's intraventricular extension, uncommon for RCCs. This contrasts with the transsphenoidal approach, which is preferred for sellar/suprasellar RCCs due to its efficacy (complete drainage in 90% of cases) and lower morbidity.[3] In our case, subtotal resection with avoidance of hypothalamic adhesions reflects the balance between decompression and minimizing morbidity, a principle emphasized in pediatric RCC series. The patient's postoperative complications highlight risks associated with large or complex RCCs, consistent with reported morbidity in pediatric series.[3]

Intraoperatively, the cyst wall was thin and friable, but a firm nodule was densely adherent to the hypothalamus and optic chiasm. Attempts at complete excision risked hypothalamic injury; therefore, this solid component was left in situ to preserve neurologic function. While some reports advocate for more extensive cyst wall removal to reduce recurrence, studies have not demonstrated a clear advantage and suggest a possible increase in postoperative morbidity. Endoscopic endonasal approaches remain the preferred option for sellar or suprasellar cysts, whereas lesions with lateral or intraventricular extension—such as in our case—may still necessitate a transcranial approach.[9]

Recurrence and Imaging Surveillance

The stability of the residual suprasellar tissue on serial MRI over 4 years is reassuring, as recurrence after surgery for RCC is relatively uncommon. Aho et al reported an 18% recurrence rate within 5 years of surgery, with most recurrences occurring even after initially complete radiographic resection often within 5 years.[10] However, the literature cautions that recurrences may occur beyond typical follow-up periods (e.g., 6 years postoperatively), justifying long-term follow up.[3]

Conclusion

This case illustrates the extensive nature of pediatric RCCs with rare, significant intracranial involvement causing severe neurological and endocrine sequelae. The patient's atypical anatomical involvement—resulting in panhypopituitarism, hydrocephalus, and long-term deficits—reflects the well-documented challenges of managing complex RCCs. This report highlights the critical role of multidisciplinary collaboration in achieving optimal outcomes and reinforces the necessity of long-term surveillance for recurrence and endocrine dysfunction. Importantly, it also underscores a key surgical teaching point: solid components that are densely adherent to the hypothalamus should not be aggressively resected, and preservation of hypothalamic function must take precedence over radiographic completeness of resection.

Conflict of Interest

None declared.

Acknowledgments

The authors thank the multidisciplinary team involved in the care of this patient, including radiology, pathology, pediatric endocrinology, and ophthalmology.

Patients' Consent

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

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Publication History

Article published online:
03 February 2026