A Single-Institution Review of Portosystemic Shunts in Children: An Ongoing Discussion

Abstract

Purpose. Review the safety and long-term success with portosystemic shunts in children at a single institution. Methods. An IRB-approved, retrospective chart review of all children ages 19 and undergoing surgical portosystemic shunt from January 1990–September 2008. Results. Ten patients were identified, 8 females and 2 males, with a mean age of 15 years (range 5–19 years). Primary diagnoses were congenital hepatic fibrosis (5), hepatic vein thrombosis (2), portal vein thrombosis (2), and cystic fibrosis (1). Primary indications were repeated variceal bleeding (6), symptomatic hypersplenism (2), and significant liver dysfunction (2). Procedures performed were distal splenorenal bypass (4), side-to-side portocaval shunt (3), proximal splenorenal shunt (2), and an interposition H-graft portocaval shunt (1). There was no perioperative mortality and only minor morbidity. Seventy percent of patients had improvement of their symptoms. Eighty percent of shunts remained patent. Two were occluded at a median follow-up of 50 months (range 0.5–13.16 years). Two patients underwent subsequent liver transplantation. Two patients died at 0.5 and 12.8 years postoperatively, one from multisystem failure with cystic fibrosis and one from post-operative transplant complications. Conclusions. The need for portosystemic shunts in children is rare. However, in the era of liver transplantation, portosystemic shunts in selected patients with well-preserved liver function remains important. We conclude that portosystemic shunts are safe and efficacious in the control of variceal hemorrhage and symptoms related to hypersplenism.

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

“…In the patient described by Hodgson et al (9) with iCHF, renal dysfunction was noted by low creatinine clearance. Lillegard (10) reported encephalopathy in their patients with iCHF. Their patients’ renal function and timing of encephalopathy (before or after portosystemic shunting) were unclear.…”

Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists

“…In symptomatic noncirrhotic portal hypertension with a patent portal system, surgical spleen-preserving side-to-side splenorenal (SSSR) shunts are the preferred type of therapy because of the good prognosis of the disease, low risk of encephalopathy and post-splenectomy sepsis, and durable decompression providing excellent quality of life, especially in pediatric patients [4]. Table 1 Types of surgical portosystemic shunts.…”

“…Common causes of noncirrhotic portal hypertension include Budd-Chiari syndrome, veno-occlusive disease, extrahepatic portal vein obstruction (EHPVO), schistosomiasis, noncirrhotic portal fibrosis, idiopathic portal hypertension and congenital hepatic fibrosis [3,4]. Most types of noncirrhotic portal hypertension have preserved liver function even in advanced stages of the disease, thus hepatic decompensation is rare and liver transplantation is seldom indicated [3,4]. Variceal bleeding, ascites, hepatorenal syndrome, spontaneous bacterial peritonitis, hepatic encephalopathy and sequelae of hypersplenism are known complications of portal hypertension [2,4].…”

“…Most types of noncirrhotic portal hypertension have preserved liver function even in advanced stages of the disease, thus hepatic decompensation is rare and liver transplantation is seldom indicated [3,4]. Variceal bleeding, ascites, hepatorenal syndrome, spontaneous bacterial peritonitis, hepatic encephalopathy and sequelae of hypersplenism are known complications of portal hypertension [2,4]. The available therapies for portal hypertension are diverse, and factors that influence the prognosis should be considered when stratifying risks and selecting a therapy.…”

“…The use of SPPS to lower portal hypertension and decompress bleeding gastro-oesophageal varices has declined during the past decade in favour of alternative therapies, such as medical and endoscopic management, TIPS and liver transplantation [4,5]. TIPS has largely replaced surgical shunts, therefore experience with the surgical procedure and interpretation of imaging studies is declining.…”