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Surgical treatment of rathke cleft cysts in children
Gabriel Zada1, Benjamin Ditty, Sean A McNatt
1Division of Neurosurgery, Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, Los Angeles, California 90089, USA. gzada@usc.edu
Insights
Surgery for Rathke cleft cysts (RCCs) in children effectively resolves symptoms like headaches. The transsphenoidal approach is recommended for cyst drainage, though hormonal deficits may persist post-operation.
Area of Science:
- Pediatric Neurosurgery
- Endocrinology
- Sellar/Suprasellar Lesions
Background:
- Rathke cleft cysts (RCCs) are epithelial lesions in the sellar/suprasellar regions, often found incidentally.
- Symptomatic or enlarging RCCs may necessitate surgical intervention for fenestration and drainage.
Purpose of the Study:
- To review the surgical outcomes of pediatric patients with Rathke cleft cysts.
- To evaluate the efficacy of surgical approaches for RCCs in children.
Main Methods:
- Retrospective review of pediatric patients surgically treated for RCCs (1999-2007).
- Analysis of clinical notes, operative reports, radiological, and pathology data.
- Median follow-up of 34 months.
Main Results:
- Ten pediatric patients underwent surgery for RCCs; 90% achieved complete cyst drainage via transsphenoidal surgery.
- Headaches improved in 87.5% of patients; however, hormonal disturbances persisted or new deficits emerged in some.
- One patient experienced recurrence 6 years post-surgery.
Conclusions:
- Surgical fenestration and drainage, particularly via the transsphenoidal approach, are effective for symptomatic pediatric RCCs.
- While symptoms like headaches often resolve, hormonal axis deficits typically persist.
- Careful patient selection is crucial for optimizing surgical outcomes in pediatric RCC cases.
Objective:
Rathke cleft cysts (RCCs) are cystic epithelial lesions in the sellar and suprasellar regions that are often discovered incidentally. They require surgical fenestration and drainage in a small proportion of patients who develop symptoms or demonstrate progressive enlargement. Our aim was to review our experience with pediatric patients treated surgically for RCCs.
Methods:
A retrospective review was conducted of all patients treated surgically for RCCs at Childrens Hospital Los Angeles between 1999 and 2007 after approval by the institutional review board. Clinical notes, operative reports, radiological studies, and pathology reports were reviewed. The median follow-up period was 34 months.
Results:
Ten patients undergoing surgical treatment of an RCC were identified, making up 20% of the 51 patients with RCCs followed clinically over the same time period. The mean age was 13 years (age range, 2-17 years). There were 6 females and 4 males. Patients requiring surgery presented with the following clinical symptoms: headache (8 patients, 80%), endocrine insufficiency (6 patients, 60%), meningitis followed by visual loss (1 patient, 10%), and incidental finding (1 patient, 10%). The mean cyst diameter was 13.6 mm (range, 8-18 mm). Four patients had strictly sellar lesions, 4 patients had suprasellar extension of an RCC, and 2 patients had primarily suprasellar RCCs. Nine of 10 patients underwent transsphenoidal surgery, and 1 patient underwent a pterional craniotomy. Complete cyst drainage on radiography was noted in 9 of 10 patients (90%), all of whom underwent transsphenoidal surgery. One patient experienced a symptomatic recurrence 6 years after complete surgical drainage. Headaches improved in 7 of 8 patients after surgery. Two patients had complete resolution of a hormonal axis deficit, whereas 3 patients developed new anterior pituitary axis deficits. Two patients developed persistent diabetes insipidus after surgery.
Conclusion:
RCCs are an infrequent cause of symptoms in pediatric patients. The transsphenoidal approach offers an effective means of achieving complete cyst drainage for lesions requiring surgery. Fenestration and aspiration of the cyst are usually sufficient to achieve total resolution of symptoms and signs caused by RCCs. Clinical symptoms such as headaches improved in the majority of patients, whereas hormonal disturbances typically persisted. Patient selection remains of paramount importance when considering surgery for pediatric patients with RCCs.
