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After being diagnosed with a life-threatening brain cyst, this high school senior and her family turned to the experts at UPMC for treatment.
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A Rathke's cleft cyst is a rare type of cyst believed to originate from remnants of the Rathke pouch. During embryonic development, the Rathke pouch forms as a depression in the roof of the mouth, eventually losing its connection to the pharynx and giving rise to the anterior pituitary gland.
If instead of regressing the cleft persists and enlarges, it may form into a Rathke's cleft cyst. Most of these cysts are:
In some instances, continual accumulation of fluid may cause the cyst to grow. As it grows it places pressure on surrounding structures, including:
This pressure results in associated symptoms of Rathke's cleft cysts.
Rathke's cleft cysts are very similar in presentation and origin to craniopharyngiomas. The difference is that craniopharyngiomas grow by cell division and fluid accumulation, whereas Rathke's cleft cysts grow only by fluid accumulation.
At UPMC, the preferred surgical treatment for Rathke's cleft cyst is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. Benefits of EEA include:
To diagnose a Rathke's cleft cyst, your doctor will perform a physical exam and ask about your symptoms as they relate to a positive Rathke's cleft cyst diagnosis.
Many Rathke's cleft cysts are asymptomatic, which means they do not cause any symptoms.
As the cysts get larger, they may press on a surrounding structure. Symptoms associated with compression include:
Imaging studies such as MRI and CT scans can detect the presence of a mass and show its cystic character however, MRI and CT scans are insufficient for a positive Rathke's cleft cyst diagnosis.
Additional tests must be performed to help rule out other possible diagnoses, such as:
A correct diagnosis is particularly important for Rathke's cleft cysts because the treatment differs from that of other possible lesions.
The diagnosis is confirmed by a sample of the cystic capsule that is sent to pathological analysis during surgical drainage of the cyst.
For asymptomatic Rathke's cleft cysts, long-term observation by a doctor may be sufficient. Based on the specific nature of the patient's Rathke's cleft cyst, treatment may require surgical drainage and/or removal.
Rathke's cleft cysts may be approached directly using the Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the cyst through the natural corridor of the nose, without making an open incision. Surgeons then remove the cyst through the nose and nasal cavities.
EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.
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