Portal biliopathy, also referred to as portal cavernoma cholangiopathy, is a biliary complication of extrahepatic portal vein obstruction caused by cavernomatous transformation of the portal vein and resulting biliary compression. Although biliary abnormalities are common in long-standing portal hypertension, only a minority of patients become symptomatic and require intervention. Herein, we present a 13-year-old patient with long-standing extrahepatic portal vein obstruction who developed pruritus, jaundice, and a distal common bile duct stricture with proximal biliary dilatation secondary to portal cavernoma compression. Despite multiple endoscopic interventions, including biliary stenting, the clinical course was complicated by persistent biliary obstruction and procedure-related bleeding. Proximal splenorenal shunt surgery achieved effective portal decompression and resolution of esophageal varices; however, biliary abnormalities persisted during follow-up. Definitive biliary reconstruction with Roux-en-Y hepaticojejunostomy subsequently resulted in complete clinical and biochemical remission without recurrence during long-term follow-up. This case highlights the importance of an individualized staged management strategy in pediatric portal biliopathy, in which portal decompression may facilitate safer biliary reconstruction, although definitive biliary-enteric reconstruction may still be required in selected patients.

Key words : Portal cavernoma cholangiopathy, Portal hypertension, Portal vein obstruction

Introduction
Extrahepatic portal vein obstruction (EHPVO) is a leading cause of portal hypertension in children.¹ Long-standing portal hypertension may result in cavernomatous transformation of the portal vein, characterized by the formation of collateral venous channels within the portal pedicle that extend in and around the biliary ducts and gallbladder, potentially obstructing bile flow and giving rise to biliary changes collectively referred to as portal biliopathy, also known as portal cavernoma cholangiopathy (PCC), primarily due to external compression by the portal cavernoma and associated biliary ischemia.^2-4 Portal biliopathy is a serious complication observed in nearly all patients with long-standing portal hypertension (92%); however, only 5–19% become symptomatic. Biliary strictures have been reported in approximately 4% of cases during long-term follow-up.^3,5,6 In symptomatic patients, therapeutic strategies target both the management of biliary obstruction and the underlying portal hypertension. Biliary abnormalities are frequently identified on imaging studies in patients with EHPVO; however, most pediatric patients remain clinically asymptomatic.¹ Symptomatic PCC may present with cholestasis, recurrent cholangitis, biliary stones, or biliary strictures.^2,7 Endoscopic retrograde cholangiopancreatography (ERCP) is considered the criterion standard for the diagnosis of PCC; however, magnetic resonance cholangiopancreatography (MRCP) is generally preferred as the initial diagnostic modality because it is noninvasive, provides comprehensive biliary mapping, and avoids ionizing radiation exposure in children.^2,7 The clinical management of symptomatic PCC remains challenging, and the optimal sequence of endoscopic and surgical interventions remains a topic of debate.^8-10 Endoscopic interventions may offer transient relief of biliary obstruction but are often limited by technical difficulties and an increased risk of hemorrhage from choledochal varices, especially in children.¹¹ Consequently, surgical portal decompression through portosystemic shunt procedures has been proposed as a primary therapeutic strategy, aiming to reduce portal pressure and relieve biliary compression.¹² Nevertheless, portal decompression alone may be insufficient, and a considerable proportion of patients ultimately require definitive biliary reconstruction with biliary-enteric anastomosis.^8,10 Herein, we report a child with symptomatic PCC secondary to EHPVO, managed successfully with a 2-step approach consisting of proximal splenorenal shunt surgery followed by Roux-en-Y hepaticojejunostomy.

Case Report
A 13-year-old male patient was diagnosed with EHPVO at the age of 3 following the incidental detection of splenomegaly. Prenatal and perinatal histories were unremarkable, and there was no history of umbilical catheterization, thrombosis, or familial thromboembolic disorders. Initial investigations demonstrated cavernomatous transformation of the portal vein, splenomegaly, and portal hypertension with grade 1 to grade 2 esophageal varices. Laboratory evaluation revealed mild thrombocytopenia, whereas liver enzymes and coagulation parameters were within reference limits. Abdominal ultrasonography showed a homogeneous liver parenchyma, cavernous transformation of the portal vein, splenomegaly, and gallbladder wall thickening. The patient was diagnosed with EHPVO and managed conservatively with propranolol and proton pump inhibitor therapy. Although he had no history of variceal bleeding, endoscopic variceal ligation was performed on 3 occasions due to high-risk endoscopic features, including red color sign-positive varices. At the age of 11 years, the patient developed pruritus and jaundice accompanied by a cholestatic pattern of liver enzyme elevation (total bilirubin, 2.3 mg/dL; direct bilirubin, 1.8 mg/dL; γ-glutamyl transferase [GGT], 139 U/L; and alkaline phosphatase, 539 U/L). A liver biopsy was performed in response to concomitant elevation of transaminases and GGT levels, which revealed no evidence of fibrosis, cirrhosis, or cholestasis. The MRCP demonstrated a distal common bile duct stricture with proximal dilatation and narrowing of the common hepatic duct caused by extrinsic compression by the portal cavernoma (Figure 1). Based on these findings, a diagnosis of symptomatic PCC was established. Following the diagnosis, multiple ERCP procedures were performed at the referring center, including balloon extraction of microlithiasis and biliary stenting. The clinical course was complicated by persistent biliary stricture, an episode of biliary stent fragmentation, and a single occurrence of significant intraprocedural bleeding due to choledochal varices located anterior to the common bile duct. Due to these complications, the patient was subsequently referred to our tertiary care center for further management. On admission, physical examination revealed marked splenomegaly, without hepatomegaly or jaundice. Laboratory investigations revealed pancytopenia, but liver enzyme levels remained within reference limits (Table 1). A proximal splenorenal shunt surgery was performed to decompress the portal venous system and resulted in complete resolution of esophageal varices. Following shunt surgery and successful decompression of the portal cavernoma, biliary stent removal was planned, and the patient was closely monitored through clinical, biochemical, and radiological follow-up. Regular assessment of cholestatic liver enzymes was undertaken, and magnetic resonance imaging was performed in response to abnormal findings. Subsequent evaluations revealed elevated levels of alanine aminotransferase and GGT. The MRCP procedure demonstrated dilatation of the common bile duct and intrahepatic bile ducts, a dominant distal common bile duct stricture, and residual intrahepatic biliary stones (Figure 2). Given persistent biliary abnormalities despite endoscopic management, surgical biliary reconstruction with Roux-en-Y hepaticojejunostomy was performed 13 months after shunt surgery. At 7-year follow-up after shunt surgery and 6 years following hepaticojejunostomy, the patient remained clinically stable with no evidence of recurrent cholangitis or cholestasis.

Discussion
The management of symptomatic PCC in pediatric patients with EHPVO remains challenging, particularly concerning the optimal sequence of endoscopic, portal decompressive, and biliary reconstructive interventions.^8,12,13 In this context, endoscopic therapy is often considered as an initial, minimally invasive treatment option, especially in patients presenting with acute cholangitis or in patients for whom a portosystemic shunt is not feasible.¹⁴ The ERCP method is commonly used as an initial therapeutic intervention in symptomatic PCC to relieve biliary obstruction and manage associated biliary stones or strictures.² However, repeated endoscopic procedures may be limited by persistent strictures and complications, including biliary stent fragmentation and hemorrhage from choledochal varices.¹¹ These limitations are particularly pronounced in pediatric patients, in whom altered biliary anatomy and portal hypertensive collateral vessels increase both technical difficulty and the risk of bleeding.^2,4 In the present case, a staged management strategy was adopted, and repeated ERCP sessions were performed to achieve biliary decompression. However, the clinical course was complicated, and durable resolution was not achieved, which highlights the limitations of endoscopic therapy in advanced PCC. Similarly, as in our case, surgical series in the literature have reported that initial endoscopic management provides only temporary relief, and definitive treatment ultimately requires portal decompression followed by biliary reconstruction in selected patients.^9,15 Portosystemic shunt surgery is commonly recommended for portal decompression to treat underlying portal hypertension and reduce cavernoma-related biliary compression.^7,12 Successful portal decompression may lead to partial or complete regression of biliary abnormalities, particularly when performed before irreversible fibrotic strictures develop.¹² In our present case, proximal splenorenal shunt surgery resulted in effective portal decompression and resolution of esophageal varices. While endoscopic interventions have well-established efficacy in the management of esophageal varices, they are insufficient to address portal biliopathy unless portal venous pressure is adequately reduced.^1,5 Definitive treatment therefore requires portal decompression through shunt surgery to achieve systemic diversion of portal flow.^7,12 Consistent with previously reported surgical series demonstrating that initial portosystemic shunt surgery leads to symptomatic and biochemical improvement in patients with portal biliopathy, portal hypertension was successfully controlled in our patient.⁹ However, biliary obstruction persisted in our case, similar to findings reported in patients with long-standing disease, suggesting that portal decompression alone may be insufficient once structural biliary damage has developed.^8,12 In the present case, although EHPVO was diagnosed at the age of 3 years, surgical decompression could not be performed until the age of 11. Earlier reduction of portal venous pressure through shunt surgery might have prevented progressive cavernomatous transformation and subsequent portal biliopathy, potentially avoiding irreversible bile duct injury. This underscores the importance of timely surgical decision-making, as prolonged reliance on endoscopic therapy alone may result in severe and potentially irreversible biliary complications. In the presence of persistent biliary obstruction, a staged management strategy was therefore completed with definitive biliary-enteric reconstruction to achieve durable biliary drainage. Roux-en-Y hepaticojejunostomy has been shown to provide favorable long-term outcomes when performed after portal decompression, as reduced portal pressure lowers intraoperative bleeding risk and facilitates surgical exposure.^9,13 This approach resulted in sustained clinical and biochemical remission during long-term follow-up in our patient, consistent with previously published surgical series of portal biliopathy in which 13 of 14 patients with persistent biliary obstruction after shunt surgery underwent second-stage biliary reconstruction, most commonly Roux-en-Y hepaticojejunostomy.⁹ This case highlights the importance of an individualized and proactive management strategy in pediatric patients with non-cirrhotic or early-stage cirrhotic portal hypertension. Although endoscopic therapy and portal decompression play critical roles in initial management, selected patients with long-standing disease may still require definitive biliary reconstruction with Roux-en-Y hepaticojejunostomy despite successful shunt surgery. Therefore, the sequence and timing of interventions should be individualized according to anatomic, hemodynamic, and clinical characteristics. Optimal outcomes in this complex patient population also require close multidisciplinary collaboration among pediatric gastroenterology, hematology, interventional radiology, and surgical teams.