Surgery for Portal Hypertension in Children

Abstract

Management of children with portal hypertension has evolved considerably over the past decades. Development of physiologic shunts (meso-Rex bypass) and successful liver transplant has changed the paradigm of portal hypertension surgery. Children with pre-hepatic portal hypertension are investigated and, if suitable, candidates are offered the mesenteric-to-left portal vein bypass (meso-Rex) preemptively, before development of symptoms of portal hypertension. Aggressive medical management, endoscopic ligation of bleeding varices, and radiologically placed intrahepatic stents have greatly reduced the need for emergent surgical procedures. A larger number of surgical options offer a permanent solution for children with portal hypertension in the setting of well-compensated liver function. Portal hypertension in the setting of decompensated liver disease is managed medically (via endoscopy) or radiologically (via transjugular intrahepatic portosystemic shunt) with the aim to offer liver transplant as a permanent solution.

Surgical management of portal hypertension in children

“…[18][19][20][21] However, children with a thrombosed left portal vein within the Rex recessus who cannot benefit from MRB should be continued on a conventional endoscopic program and conservative management, as this also provides satisfactory long-term results by prevention of bleeding. [22][23][24][25][26] Such children might become candidates for portosystemic shunt surgery when complications cannot be managed medically or endoscopically, and our preference is for a "selective" shunt, such as a Warren shunt, to preserve some hepatopetal flow through the cavernoma preserving the hepatotrophic effect of mesenteric blood. Other teams use nonselective H-type shunts (eg, mesocaval and lateral splenorenal) in this scenario and obtain excellent clinical results, possibly because H-type shunts may not cause full diversion of the portal flow.…”

“…These may include those with well-compensated disease (Pediatric End-stage Liver Disease [PELD] Ͻ10), such as those with PHT group 2a causes (ie, congenital hepatic fibrosis) and, less commonly, groups 2c and 3, with wellpreserved liver function but symptomatic variceal bleeding. 3,5,23,24,44 Nonsurgical interventional options such as TIPSS may be more appropriate and should be discussed with the transplant team. This alternative preserves the intact abdomen, and stents can be removed at the time of transplantation.…”

“…27 Portosystemic shunts may be either "selective" or "nonselective." 23,24 Selective shunts divert selectively a region of the abdomen, for example, the stomach and spleen, to decompress esophagogastric varices. Blood is predominantly shunted into the low-pressure systemic venous circulation, and the varices then flow at a lower pressure with no residual risk of bleeding.…”

Pediatric Portal Hypertension

“…[27] When the cause of portal hypertension is liver cirrhosis in the modern era the suitable option is a liver transplant. [38] There are other surgical options for portal hypertension which are considered palliative rather than therapeutic: (1) Selective shunt: the technique is known as distal splenorenal shunt (Warren shunt). The principle of this technique is diverting part of the portal circulation to the systemic circulation by dividing the splenic vein and anastomosing the distal end to the left renal vein.…”

Novel Application and Serial Evaluation of Tissue-Engineered Portal Vein Grafts in a Murine Model

“…Since its description by de Ville de Goyet in 1992 [3], mesenterico-left portal vein (meso-Rex) bypass has been widely adopted as an efficacious intervention for EHPVO. Connection of the superior mesenteric vein to the left portal vein alleviates the risk of severe gastrointestinal bleeding and other sequelae of sustained portal hypertension while restoring physiologic blood supply to the liver [2,4,5].…”