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

Kaynakça

1. Wu KL, Changchien CS, Kuo CM, et al. Caroli's disease-a report of two siblings. Eur J Gastroenterol Hepatol 2002; 14:1397-1399.
2. Madjov R, Chervenkov P, Madjova V, Balev B. Caroli's disease. Report of 5 cases and review of literature. Hepatogastroenterol 2005; 52:606-609.
3. Caroli J. Diseases of the intrahepatic biliary tree. Clin Gastroen- terol 1973; 2:147-161.
4. Benhamou JP. Congenital hepatic fibrosis and Caroli’s syndrome. In: Schiff L, Schiff ER, eds. Diseases of the liver. 7th ed. Phila- delphia, Pa: Lippincott, 1993; 1204-1209.
5. Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s syn- drome. World J Gastroenterol 2007; 13: 1934-1937.
6. Hussain SZ, Bloom DA, Tolia V. Caroli’s disease diagnosed in a child by MRCP. Clin Imaging 2002; 24: 289-291.
7. De Kerckhove L, De Meyer M, Verbaandert C, et al. The place of liver transplantation in Caroli's disease and syndrome. Transpl Int 2006; 19:381-388.
8. Suchy FJ. Cystic diseases of the biliary tract and liver. In: Behr- man RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 17th ed. Philadelphia: WB Saunders, 2004;1343-1344.

9. De Tommaso AM, Santos DS, Hessel G. Caroli’s disease : 6 case studies. Acta Gastroenterol Latinoam 2003; 33:47-51.
10. Mall JC, Chahremani GG, Boyer JL, Caroli’s disease associated with congenital hepatic fibrosis and renal tubular ectasia. Gastro- enterol 1974; 66:1029-1053.
11. Bernstein J, Slovis TI. Polycystic disease of the kidney. In Elder- man CM Jr eds Paediatric Kidney Disease. Vol. 2, I Boston; Lit- tle Brown, 1992:1139-1153.
12. Mrowka C, Adam G, Sieberth HG, Matern S. Caroli's syndrome associated with medullary sponge kidney and nephrocalcinosis. Nephrol Dial Transpl 1996; 11:1142-1145.
13. Abdullah AM, Nazer H, Atiyeh M, Ali MA. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr 1991; 37:240-243.
14. Shun A, Delaney DP, Martin HC, Henry GM, Stephen M. Porto- systemic shunting for pediatric portal hypertension. J Pediatr Surg 1997; 32: 489-493.
15. Dayton MT, Longmire WP, Tompkins RK. Caroli’s disease: a premalignant condition?. Am J Surg 1983; 145:41-47.
16. Totkas S, Hohenberger P. Cholangiocellular carcinoma associ- ated with segmental Caroli’s disease. Eur J Surg Oncol 2000; 26: 520-521.
17. Kabul Tarihi: 07.11.2008