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Rathke cleft cysts in pediatric patients: presentation, surgical management, and postoperative outcomes
Arman Jahangiri1, Annette M Molinaro, Phiroz E Tarapore
1University of Texas Southwestern Medical School, Dallas, Texas, USA.
Insights
Pediatric and adult Rathke cleft cysts (RCCs) share similar symptoms and outcomes, despite lower incidence in children. Early Rathke pouch remnants may explain pediatric RCC development, with similar cyst sizes but greater relative impact in younger patients.
Area of Science:
- Neuroscience
- Pediatric Endocrinology
- Neurosurgery
Background:
- Rathke cleft cysts (RCCs) are benign sellar lesions typically found in adults, but also occur in children.
- While often asymptomatic, RCCs can necessitate surgical intervention via a transsphenoidal approach.
Purpose of the Study:
- To compare the clinical presentation, imaging characteristics, and surgical outcomes of Rathke cleft cysts in adult versus pediatric patient populations.
Main Methods:
- Retrospective review of 147 adult and 14 pediatric patients who underwent surgery for RCCs.
- Analysis included presenting symptoms, MRI findings, laboratory results, and pathology.
- Data were collected from patients treated between 1996 and 2008.
Main Results:
- Headache was the most common symptom in both groups; growth delay was unique to pediatric patients.
- Preoperative hypopituitarism rates were similar (41% adults, 45% pediatric).
- No significant differences were observed in cyst size (median 1.2 cm), postoperative diabetes insipidus rates, or 2-year recurrence rates (8% for both groups).
Conclusions:
- Pediatric RCCs, though less common, exhibit similar clinical and imaging features to adult RCCs.
- Pediatric RCCs may originate from earlier Rathke pouch remnant development.
- Consider RCCs in pediatric differential diagnoses for visual impairment, headache, or hypopituitarism, noting their potentially larger relative size in children.
Object:
Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC.
Methods:
The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008.
Results:
In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5).
Conclusions:
The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.
