The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors’ own experience.
Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.
Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.
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The authors deeply acknowledge the contribution of Prof. SK Yachha, Prof. VK Kapoor, Dr. SS Sikora, Dr. Anu Behari, Dr. Ujjal Poddar, Dr. Anshu Srivastava, and Dr. Ankur Mandelia for their tremendous clinical and research contributions in the arena of pediatric EHPVO at their centre.
RL: Conception & design, drafting of manuscript; MSS: Acquisition & analysis of data, critical intellectual input; MKG: Assisted in manuscript drafting, data acquisition. RL will act as guarantor for the paper.
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