Santral sinir sistemi tutulumlu langerhans hücreli histiositozis ve rathke kleft kisti: Bir olgu

Çocukluklarda kalınlaşmış hipofiz sapı ile ilişkili santral diabetes insipidusa neden olan durumların tanısı zordur. Langerhans hücreli histiyositozis bunun nadir nedenidir. Onbeş yaşındaki erkek çocuk, 6 aydır süren çok su içme ve sık idrara çıkma yakınmalarıyla kliniğimize başvurdu ve hipofiz sapında kalınlaşma bulundu. ølk başta langerhans hücreli histiyositozis tanısı için ek bulgular yoktu. Dokuz ay sonra kafa grafisinde litik lezyon görüldü ve langerhans hücreli histiyositozis tanısı kondu. Burada olgumuz sebebiyle, langerhans hücreli histiyo- sitozis tanısında seri takip ve manyetik rezonans görüntülemenin önemi ve santral diyabetes insipidusa eşlik eden hipofiz sap kalınlaşması bulunan olgularda klinik izlemin önemi vurgulanmaktadır. Rathke kleft kistleri hipofizin nadir rastlanan lezyonlarıdır. Bu kistler genellikle iyi huylu olup, hormonal ve görme bozukluk- ların önemli bir nedenidir. Klinik olarak üç temel özellik gösterirler: Baş ağrısı, endokrin bozukluk, görmede kayıp. En sık görülen hormonal bozukluk hiperprolak- tinemi, gonadotropin yetersizliği, panhipopitüitarizm, hipotiroidi ve hipokortizolizmdir. Bu olguda rathke kleft kisti, langerhans hücreli histiyositozise neden olan hipo- fiz sap kalınlaşması ile birlikte görülmüş olup, daha önce bildirilmemesi nedeniyle ilgili literatürlerle tartışılmıştır.

(Langerhans cell histiocytosis is associated with central nervous system disease and Rathke cleft cyst: A case)

Central diabetes insipidus associated with a thickened pituitary stalk is a diagnostic challenge in the pediatric population. Langerhans cell histiocytosis is a rare cause of this entity. 15-year-old male child presented with central diabetes insipidus of 6-month duration, associated with a thickened pituitary stalk. The etiology for the same remained elusive as the patient had no other manifestation to suggest langerhans cell histiocytosis. Nine month later, lytic lesion was detected in X-ray head. The present case highlights the need for serial follow-up and MR imaging that led to a diagnosis of langerhans cell histiocytosis. Rathke cleft cyst are benign but they are important causes of endocrine and visual symptoms. They are small and asymptomatic but they may also be symptomatic. Clinically they have three major features: headache, endocrine abnormality and visual loss. The most frequent abnormalities are hyperprolactinemia, gonadotropin insufficiency, pan- hypopituitarism, hypothyroidism and hypocortisolism. In this article we discuss a case of langerhans cell histiocytosis accompanied by rathke cleft cyst.

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