Symptomatic Rathke’s Cleft Cyst in a Pediatric Patient: A Case Report

Background: A Rathke’s cleft cyst (RCC) is an epithelium-lined intrasellar benign growth believed to originate from remnants of the Rathke’s pouch. It is commonly believed that the cleft regresses during embryologic development, and the persistence and enlargement of the cleft lead to the formation of RCC. However, other theories have been proposed to explain their exact origins, such as derivation from the neuroepithelium or endoderm, or metaplastic anterior pituitary cells.1 Based on autopsies, asymptomatic RCC is relatively common, being detected in 2-26% of individuals. Symptomatic cysts, on the other hand, are rare, with approximately 150 cases reported.1 Symptoms of RCC arise from cyst enlargement and compression of the optic chiasm, the pituitary gland/stalk, and/or the hypothalamus. According to the Eguchi study, visual symptoms were present in 47% of patients.2 Transsphenoidal surgery is the most common approach to treat RCC, in which the cyst is partially excised and drained. This method preserves the pituitary function and has been effective in reducing symptoms.1 This case report details a child with symptomatic RCC exacerbated by traumatic brain injury (TBI) and discusses the radiographic findings. Case Information: A 14-year-old female with a past medical history of one prior concussion several years ago and lifelong occasional headaches, presented two weeks following a head injury during a basketball game. The patient fell while playing basketball and hit her head. She denied any loss of consciousness. The patient has had headaches in the past, but since the injury, the headaches have increased in frequency. They now occur multiple times a day with each episode lasting about 15 minutes, and the pain is localized to the retroorbital and bilateral temporal regions. The patient has lifelong vision issues which have also worsened since the injury. She also reports photophobia, fatigue, anxiety, trouble breathing, and difficulty focusing during school. The photophobia is so intense that she must wear sunglasses all the time, even during class. A pertinent physical exam revealed flattening of the right nasolabial fold and protruding tongue deviation to the right, suggesting possible facial (CN VII) and hypoglossal (CN XII) cranial nerve involvement. Magnetic Resonance Imaging (MRI) revealed an RCC and brain capillary telangiectasia versus hemosiderin deposition in the setting of traumatic brain injury. The patient was urgently referred to neurology and neurosurgery following imaging results, as well as optometry and ophthalmology. Despite brain and physical rest for a week, and a new prescription for glasses, her headaches and visual symptoms continued to worsen. Due to severe photophobia, persistent daily headaches, and inability to complete classwork, the patient was removed from in-person school and all sports. Neurology is currently monitoring the patient for further progression of symptoms, and she is awaiting neurosurgery and ophthalmology evaluations. Conclusion: Given the common occurrence of RCC, symptomatic RCC should always be considered when assessing patients with persistent headaches and visual field defects. Furthermore, it is important to promptly order a brain MRI and refer patients to neurology and neurosurgery to assess the need for transsphenoidal surgery.