"Acta chir belg Cavernous Transformation of the Portal Vein"
Acta chir belg, 2004, 104, 457-458 Cavernous Transformation of the Portal Vein Causing Jaundice, Presenting in the Form of Wilson’s Disease V. Ruszinkó, M. Kovács, L. Szönyi*, T. Verebély*, P. Willner# Dept. of Pediatrics, Petz Aladár County Teaching Hospital, Györ ; * 1st Dept. of Pediatrics, Semmelweis Medical University, Budapest ; # Dept. of Surgery, Telki Private Hospital, Budapest, Hungary. Key words. portal vein cavernoma, cholestasis, Wilson’s disease. Abstract. The following is a case review of portal vein cavernous malformation presenting with intermittent cholesta- sis and jaundice in a 4 year old child. Correct assessment was supported by radiology, later laparoscopy, yet hindered by histopathology representative Wilson’s disease and elevated urinary copper excretion. During surgical procedure the stenosis of the common bile duct secondary to extremely dilated portal vein reticulation was solved by Roux-en-Y choledochojejunostomy. After a one-year follow up the child remains asymptomatic. Introduction tered, but the child’s condition fluctuated with intermit- tent jaundice elevated cholestatic enzymes (AST : 100 Cavernous transformation of the portal vein is a rare U/l, ALT : 186 U/l, _GT : 286 U/l, AP : 983 U/l). The condition which can cause complete or incomplete enzyme values temporarily returned to normal following mechanic jaundice due to compression of the common Ursofalk therapy. This was the fact why our attention bile duct. It is caused by partial or complete recanaliza- was transferred to a possible anatomical reason. tion of the portal vein, deriving from thrombotic com- After a repeated ultrasound evaluation suggested a plication of umbilical vein inflammation. Pylethrom- presence of dilated intrahepatic bile ducts, MRCP bosis is often caused by abdominal infection, tumor or (fig. 1), followed by ERCP were done (fig. 2). Both of polycythemia in adults. We found a single case of portal them confirmed dilated extra- and intrahepatic bile ducts vein malformation causing cholestasis in the pediatric with stricture of the common bile duct. In addition, CT literature (1). scan examination also raised a possibility of vascular Case report Sz. E., 4 year old girl was admitted with a three-week history of pruritus and jaundice. The family and person- al history were negative, physical examination was nor- mal apart from hepatosplenomegaly and jaundice. Serum transaminase (AST : 83 U/l), serum bilirubine (95/47 umol/l), serum copper (44,9 umol/l ; norm : 12,5- 20,4 umol/l) and coeruloplasmine (79,5 mg/dl ; norm : 20-40 mg/dl) values were all elevated and urinary cop- per excretion increased 90 fold following administration of penicillamin. Abdominal ultrasound remained un- changed. The jaundice eventually resolved spontaneous- ly following hospital admission. Due to the continuous elevation of hepatic enzymes liver biopsy and Shicata Orcein staining were per- formed. This demonstrated a presence of a large quanti- ty of copper-associated protein. Wilson’s disease, chron- ic hepatitis and portal fibrosis were diagnosed. At the same time, genetic examination (His 1069 Gln gene Fig. 1 mutation PCR) for Wilson’s disease turned out negative. The dilated (1 cm in diameter) common bile duct is nicely Accordingly Byanodine (penicillamin) was adminis- demonstrated on MRCP. 458 V. Ruszinkó et al. Fig. 3 The cavernous malformation is suspected based on the vascu- lar structure around the common bile duct on CT scan. Fig. 2 Dilated extra- and intrahepatic bile ducts and significant stric- hepatic balloon dilatation of the common bile duct (5). ture of the common bile duct was confirmed by ERCP. Definitive management, especially in children, can only be achieved by surgical repair. Roux-en-Y choledocho- compression (fig. 3). The ERCP procedure was compli- jejunostomy is the most physiological reconstruction cated by significant bleeding from the Vater’s papilla and thus is the operation of choice. Further hypothetical and required blood transfusion. advantage of this procedure is the fact that scar forma- Because radiology showed common bile duct com- tion at the operation site can prevent recurrence of intra- pression laparoscopy was performed, which confirmed mural varices (6). the cavernous malformation of the portal vein. Roux-en- Our case highlights the fact that ruling out anatomical Y choledochojejunostomy was accomplished and the malformations in cholestasis is of primary importance gallbladder was removed. not only in the adult but also in the pediatric population. The patient has remained asymptomatic during a one The non invasive methods employed (US, MRCP ) year follow up. Laboratory results have all normalized proved to be sufficient in every aspect. as well. References Discussion 1. TAKEHARA H., KOMI N., OKADA A. et al. A case of biliary stricture caused by cavernous transformatio.n of the portal vein. Acta Following occlusion of the portal vein collateral reticula Paediatr Jpn, 1993, 35 : 158-61 2. MEREDITH H. C., VUJIC I., SCHABEL S. I., O’BRIEN P. H. Obstructive can form cavernous malformations in the hepatoduode- jaundice caused by cavernous transformation of the portal vein. Br nal ligament, leading to compression of the common J Radiol, 1978, 51 : 1011-2. bile duct. The entity was first described by MEREDITH et 3. THERVET L., FAULQUES B., PISSAS A. et al. Endoscopic management of obstructive jaundice due to portal cavernoma. Endoscopy, al. in 1978 (2). 1993, 25 : 423-5. The correct assessment of such clinical syndrome is 4. YATTO G. N., KHUROO M. S. Endoscopic management of obstructive best achieved by ERCP and MRCP. Cavernous transfor- jaundice due to portal cavernoma. Endoscopy, 1995, 27 : 462-3. 5. PEREGO P., COZZI G., BERTOLINI A. Portal biliopathy. Surg Endosc, mation can also be readily diagnosed by CT scan. In our 2003, 17 : 351-2. case histology of the liver biopsy resembling Wilson’s 6. HENNE-BRUNS D., KREMER B., SOEHENDRA N. Cavernöse disease with cholestasis and elevated urine copper ini- Transformation der Pfortader. Chirurg, 1989, 60 : 704-6. tially led to a diagnostic dilemma. In the majority of cases completion of the bilio-diges- tive anastomosis is impossible because of the extensive Ruszinkó Viktória MD cavernous reticulation. In these cases the only option Department of Pediatrics Petz Aladár Teaching Hospital is a stent implantation by endoscopy (3, 4). This was 9002. Györ, POBox 92. (Hungary) also the treatment option in the single reported case in Tel. : +36 96 507 936 childhood (1). Another option is the percutaneous trans- Fax : +36 96 317 621