Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience.

Background: This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome.

Methods: EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV.

Results: One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism ( = 132, 95%); ii) growth retardation (GR,  = 95, 68%); iii) isolated gastric (IGV) and ectopic varices ( = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) ( = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy ( = 104); side-to-side splenorenal shunt ( = 4); mesocaval shunt ( = 1); inferior mesenteric vein (IMV) to left renal vein shunt ( = 2); IMV to inferior vena cava shunt ( = 3); H-graft interposition splenorenal shunt ( = 1); spleno-adrenal shunt ( = 3); makeshift shunt ( = 1). Esophagogastric devascularization ( = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly.

Conclusions: Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.