Symptoms of Rathke's Cleft Cyst
Most Rathke's cleft cysts are asymptomatic and discovered incidentally, but when symptomatic, they present with headache (most common), endocrine dysfunction, and visual disturbances due to mass effect on surrounding structures. 1, 2
Common Clinical Presentations
Headache
- Headache is the most frequent symptom, occurring in approximately 80% of symptomatic patients requiring surgical intervention 1
- The headache results from cyst expansion causing pressure on the sella turcica and surrounding dura 2
Endocrine Dysfunction
- Hypopituitarism affects 37-85% of patients with sellar masses, though the frequency varies with cyst size 3
- Endocrine insufficiency occurs in approximately 60% of symptomatic pediatric patients requiring surgery 1
- Growth hormone axis is most commonly affected (61-100%), followed by gonadal (36-96%), adrenal (17-62%), and thyroid axes (8-81%) 3
- Diabetes insipidus is uncommon, occurring in only 7% of patients at presentation 3
- Patients may present with short stature, delayed puberty, or panhypopituitarism, particularly in adolescents 4
Visual Disturbances
- Visual field defects occur when cysts extend suprasellarly and compress the optic chiasm 2
- Visual impairment affects approximately 5.7% of patients with cysts ≥10 mm in diameter 5
- Visual acuity impairment can occur in addition to field defects 5
Asymptomatic Presentation
- The majority of Rathke's cleft cysts are asymptomatic and discovered incidentally on brain imaging 6, 2
- Cysts found in 13-33% of the general population remain clinically silent 6
- Cysts <10 mm in diameter rarely cause symptoms, with only 2.9% having pituitary hormone deficiencies and 2% having visual field impairments 5
Rare Presentations
- Meningitis followed by visual loss has been reported in isolated cases 1
- Signs of increased intracranial pressure, including papilledema, may occur but warrant immediate assessment for other causes 3
Natural History Considerations
- In 57% of radiographically diagnosed cysts, no detectable size change occurs over time 6
- Cysts may increase in size (28%) or decrease in size (15%) during follow-up 6
- Cysts ≥10 mm that are not operated tend to decrease in size over time 5
Important Clinical Pitfalls
- Do not dismiss empty sella findings without hormonal screening, though urgent workup is unnecessary in asymptomatic patients 3
- Do not attribute headache directly to Rathke's cleft cysts without considering more common primary headache disorders 3
- Comprehensive pituitary hormone assessment should be performed in all patients with suspected cysts, regardless of size 7
- Formal visual field testing should be obtained in all patients, particularly those with suprasellar extension 7