Rathke Cleft Cyst Presenting With Hyponatremia
Rathke Cleft Cyst Presenting With Hyponatremia
The authors report a case of Rathke cleft cyst presenting with severe hyponatremia. A 33-year-old man suffered sudden severe headaches, visual changes, dizziness, nausea, vomiting, and a metallic taste in his mouth. Initial laboratory values demonstrated severe hyponatremia. Magnetic resonance imaging revealed a cystic lesion with questionable intracystic hemorrhage, concerning for pituitary apoplexy. Transsphenoidal decompression and drainage of the cyst confirmed the diagnosis of Rathke cleft cyst and resolved the symptoms. Postoperative follow-up studies at 6 months demonstrated normal endocrine function and no evidence of a cyst.
Rathke cleft cysts are benign cystic remnants of the craniopharyngeal duct. They are commonly located in the sellar and suprasellar region. First described by Luschka in 1860 as "an epithelial area in the capsule of the human hypophysis resembling oral mucosa," these remnants of the Rathke pouch have been estimated, based on autopsy examinations, to be present in 22% of the population. Despite this high prevalence, they remain largely incidental findings, with an increase in recent diagnoses likely resulting from improvements in imaging of the sellar and suprasellar region. Occasionally, however, they may become large enough to cause symptoms, and these cysts now comprise 5%–10% of all resected sellar lesions. Presenting symptoms typically include headache, visual disturbances, and pituitary dysfunction. Endocrinopathies associated with pituitary dysfunction include growth hormone deficiency, hypogonadism, hypothyroidism, and hypocortisolemia.
Rarely, RCCs can present in apoplexy, with acuteonset headache, vision changes or loss, nausea, vomiting, meningismus, and even hypothalamic dysfunction. The variation in clinical and imaging appearance and the fact that RCCs can present with nonhemorrhagic apoplexy make diagnosis of this presentation difficult. We report a case of an RCC in which the patient had profound hyponatremia and for whom we had preoperative concern for pituitary apoplexy, an unusual presentation.
Abstract and Introduction
Abstract
The authors report a case of Rathke cleft cyst presenting with severe hyponatremia. A 33-year-old man suffered sudden severe headaches, visual changes, dizziness, nausea, vomiting, and a metallic taste in his mouth. Initial laboratory values demonstrated severe hyponatremia. Magnetic resonance imaging revealed a cystic lesion with questionable intracystic hemorrhage, concerning for pituitary apoplexy. Transsphenoidal decompression and drainage of the cyst confirmed the diagnosis of Rathke cleft cyst and resolved the symptoms. Postoperative follow-up studies at 6 months demonstrated normal endocrine function and no evidence of a cyst.
Introduction
Rathke cleft cysts are benign cystic remnants of the craniopharyngeal duct. They are commonly located in the sellar and suprasellar region. First described by Luschka in 1860 as "an epithelial area in the capsule of the human hypophysis resembling oral mucosa," these remnants of the Rathke pouch have been estimated, based on autopsy examinations, to be present in 22% of the population. Despite this high prevalence, they remain largely incidental findings, with an increase in recent diagnoses likely resulting from improvements in imaging of the sellar and suprasellar region. Occasionally, however, they may become large enough to cause symptoms, and these cysts now comprise 5%–10% of all resected sellar lesions. Presenting symptoms typically include headache, visual disturbances, and pituitary dysfunction. Endocrinopathies associated with pituitary dysfunction include growth hormone deficiency, hypogonadism, hypothyroidism, and hypocortisolemia.
Rarely, RCCs can present in apoplexy, with acuteonset headache, vision changes or loss, nausea, vomiting, meningismus, and even hypothalamic dysfunction. The variation in clinical and imaging appearance and the fact that RCCs can present with nonhemorrhagic apoplexy make diagnosis of this presentation difficult. We report a case of an RCC in which the patient had profound hyponatremia and for whom we had preoperative concern for pituitary apoplexy, an unusual presentation.
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