Caroli'S Syndrome Associated with Medullary Sponge Kidney: A Case Report and Review of The Literature

Abstract

Herein, we presented a case of Caroli's syndrome associated with medullary sponge kidney and reviewed the literature. A 21-year-old male was admitted to our internal medicine department with the complaints of fatique, anorexia and recurrent right upper abdominal pain. Abdominal ultrasonography showed hepatomegaly and multiple dilated intrahepatic bile ducts. Computed tomography of the abdomen indicated cystic images distributed in both lobes of the liver and multiple medullary cysts within the kidneys. The magnetic resonance cholangiopancreatography (MRCP) pointed out multiple cystic dilatations of intrahepatic bile ducts. The main bile duct and extrahepatic bile ducts were appeared to be normal. The patient had a brother who had been diagnosed of Caroli's syndrome 12 years ago. Radiologic investigation of the other family members revealed no abnormality.

Keywords

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Medullar Sünger Böbrek İle İlişkili Karoli Sendromu: Olgu Sunumu ve Literatürün Gözden Geçirilmesi

Öz

Biz burada medullar sünger böbreği ile beraber Caroli sendromu olan bir olguyu sunup literatürü gözden geçirdik. 21 yaşındaki bir erkek iç hastalıkları kliniğimize yorgunluk, iştahsızlık ve tekrarlayan sağ üst kadran ağrısı ile başvurdu. Batın ultrasonografisinde hepatomegali ve intrahepatik safra kanallarında birçok dilatasyon görüldü. Batının bilgisayarlı tomografisi ile incelenmesinde karaciğerin her iki lobunda kistik oluşumlar, ayrıca böbrekte medullar kistler görüldü. Manyetik rezonans kolanjiopankreatografi tetkikinde intrahepatik safra kanallarında birçok kistik dilatasyon tespit edildi. Ana safra kanalı ve ekstrahepatik safra kanalları normaldi. Hastanın 12 yıl önce Caroli sendromu tanısı konulan bir erkek kardeşi vardı, ancak ailenin diğer üyelerinin radyolojik araştırmalarında anormallik görülmedi.

Anahtar Kelimeler

• Karoli sendromu, medullar sünger böbrek

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17. Kabul Tarihi: 07.11.2008