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Published on: November 30, 2010
Surgical management of Rathke cleft cysts in pediatric patients: a single institution experience
Michael G Brandel1, Christine Lin1, Robert C Rennert2
1Department of Neurosurgery, University of California, San Diego-Rady Children's Hospital, San Diego, CA, 92123, USA.
Insights
Pediatric Rathke cleft cysts (RCCs) can grow rapidly and cause symptoms. Surgical management offers good neurological outcomes but carries risks of hormonal deficits and high recurrence rates, necessitating long-term follow-up.
Area of Science:
- Pediatric Neurosurgery
- Endocrinology
- Sellar Region Pathology
Background:
- Rathke cleft cysts (RCCs) are rare, benign sellar lesions originating from craniopharyngeal duct remnants.
- Limited data exist on the natural history and management of growing or symptomatic pediatric RCCs.
- This study details institutional surgical experience with pediatric RCCs.
Purpose of the Study:
- To evaluate the surgical management outcomes of pediatric Rathke cleft cysts.
- To assess the efficacy and safety of surgical interventions for symptomatic or growing RCCs in children.
- To analyze recurrence rates and long-term follow-up data for pediatric RCCs.
Main Methods:
- Retrospective review of pediatric patients (≤18 years) surgically treated for RCCs between 2006 and 2022.
- Analysis of presenting symptoms, surgical approaches (transsphenoidal and orbitozygomatic), and postoperative outcomes.
- Assessment of complication rates, recurrence, and long-term neurological and endocrinological status.
Main Results:
- Thirty-one pediatric RCCs were surgically managed; common symptoms included headache (58%) and visual changes (32%).
- Transsphenoidal surgery (90%) led to symptom resolution in a majority of cases, with low complication rates.
- Postoperative pituitary deficits occurred in 9.7%, and recurrence requiring reoperation was observed in 32% of patients.
Conclusions:
- Surgical management of pediatric RCCs is safe and effective for relieving mass effect symptoms.
- Potential risks include endocrinologic injury and a significant rate of recurrence, emphasizing the need for long-term monitoring.
- Good neurological outcomes are achievable, but careful follow-up is crucial for managing recurrent disease.
Objective:
Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs.
Methods:
We performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022.
Results:
Overall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up.
Conclusion:
Due to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.
