A retrospective search of our inpatient records of last ten years was performed to identify patients with HVOTO. A complete history, examination, basic hematological and liver function tests, prothrombotic work up, and results of radiological studies were extracted. Prothrombotic panel including anti-phospholipid antibody (APLA), protein C and S, antithrombin III, factor V leiden mutation, prothrombin gene mutation, methylene tetrahydrofolate reductase (MTHFR) gene mutation, serum homocysteine level, lupus anticoagulant, JAK 2 mutation and paroxysmal nocturnal hemoglobinuria (PNH) profile were done whenever indicated and feasible. Radiological features (morphology, level of block) were noted. Treatment, outcome and follow-up were also recorded.

Case records of 11 children (6 boys) with HVOTO during study period were extracted and reviewed. Mean age of presentation was 10.2 years (range 3-16 years). Duration of symptoms ranged from 8-36 months. Ascites and dilated abdominal veins were the most common findings at presentation in 7 (63.6%) patients followed by hepatosplenomegaly in 6 (54.5%) patients and icterus in 4 (36%). The mean (range) bilirubin, albumin, SGOT, SGPT and PT/INR were 2.63 (1,5.2), 3.4 (2.6,4.1), 49 (33,72), 29 (31,66) and 1.07, respectively.

USG abdomen with Doppler was diagnostic in 8 (89%) cases. CT angiography of liver was performed in all cases, and it was helpful in localizing the block in all the 11 children (Table I). Etiological work-up was suggestive of thrombophilia in 4 (36.4%) patients, among them two were positive for APLA and one each was positive for JAK2 mutation and PNH (Table I).

TABLE I Management, Outcome, Post-operative Complications and Follow-up of Eleven Children with HVOTO

Radiological intervention could be considered only in three patients. It was successful in two, and both had only ascites and hepatomegaly at presentation. It was unsuccessful in one child who had ascites along with other features of chronic liver disease. One patient with PNH, who had IVC block, was managed with low molecular weight heparin (LMWH) followed by warfarin. Percutaneous intervention was not attempted, as he had underlying hemolysis. He was readmitted for portal vein thrombosis (PVT) and again managed by heparin. He recovered over two weeks and was discharged on warfarin. Seven patients were offered and underwent living related liver transplantation for decompensated chronic liver disease (CLD), and they did not undergo angioplasty. One patient died after liver transplantation because of sepsis and related complications. One patient had intraventricular hemorrhage on postoperative day 2. Another patient developed thrombotic thrombocytopenic purpura on day 15 postoperatively and was managed conservatively.

Most common presentation of HVOTO in our study was abdominal distension followed by hepato-splenomegaly and jaundice, which was comparable to an earlier study by Sharma, et al. [2]. In another study, hepatomegaly was the most common mode of presentation (84.8%), followed by ascites (82.6%), spider angiomas and dilated veins over abdomen (69.9%) [3].

Etiologies of HVOTO may include thrombotic states, inflammatory conditions, or neoplastic processes of the liver [4]. Three (33.3%) patients in our series had prothrombotic state, which is comparable to the proportion observed by Alam, et al. [5], but lower than 75% reported in another pediatric series from India [3].

Varying success rates (20-86%) of anti-coagulation combined with angioplasty have been reported in different series [6-8]. Approximately one-third of patients have short-length stenosis and are candidates for angioplasty. In the remaining 10-20% of patients whom anti-coagulation and intervention radiology procedures fail, liver transplantation remains the only option [6]. Most of the patients in our series had clinical, radiological and biochemical evidence of advanced liver disease. Radiological intervention could successfully be performed in only two patients and both these patients did not have features of advanced liver disease. Reports on the long-term outcomes for orthotopic liver transplantation (OLT) for HVOTO in children are limited to a few sporadic case reports and series [9,10]. With proper case selection, long-term survival rates for pediatric OLT are in excess of 90%.

To conclude, a diagnosis of pediatric HVOTO is often made late. Early referral of such patients to an equipped center has the best possible outcome by giving the opportunity of re-establishing physiological hepatic venous outflow without the need for a surgery. Transplantation in children with advanced stages of liver failure due to HVOTO has helped in improving overall survival.

Contributors: IM: analyzed the data and drafted the manuscript; VB: conceptualized the study; KK: collected the data; AS: reviewed the manuscript for final submission; NG: contributed the data and reviewed the manuscript for final submission. All authors approved the final version of manuscript, and are accountable for all aspects related to the study.

Funding: None; Competing interest: None stated.

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