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Extra Hepatic Portal Vein Obstruction (EHPVO) with Extensive Mesenteric Venous Thrombosis in a Child

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Extra hepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension in children. EHPVO along with thrombosis of splenic vein (SV) and superior mesenteric vein (SMV) is an uncommon condition causing extensive varices formation in the oesophagus, stomach and in other parts of gastrointestinal tract including rectal varix as well as splenomegaly and associated hypersplenism. Most commonly the child presents with hematmesis and due to extensive varices it is difficult to obliterate the varices using endoscopic therapy. Due to thrombosed SMV and SV shunt surgery is not possible. We describe here a case of EHPVO with SMV and SV thrombosis with bleeding gastric varix that underwent gastro- oesophageal devascularisation with splenectomy and oesophageal transection to prevent recurrent bleed from gastric varices.

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Extra Hepatic Portal Vein Obstruction (EHPVO) with Extensive Mesenteric Venous Thrombosis in a Child

  1. 1. EXTRA HEPATIC PORTAL V MESENTER M VEIN OBST RIC VENOU TRUCTION US THROM (EHPVO) BOSIS IN A WITH EXT CHILD TENSIVE
  2. 2. Case Report EXTRA HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) WITH EXTENSIVE Rakesh Rai*, ST Gopal*,Suresh Singhvi*, Radhakrishna Hedge# and Anand Alladi** *Senior Consultant Surgeon Institute of Liver & Pancreatic Disorder and Solid Organ Transplantation, #Senior Consultant, Department of Pediatric Medicine,**Senior Consultant, Department of Pediatric Surgery, Correspondence to: Dr Suresh Singhvi, Senior Consultant (Surgery), Institute of Liver & Pancreatic Disorder and Solid Organ Transplantation, Apollo Hospital, Bannerghatta Road, Bangalore 560 076, India. Extra hepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension in children. EHPVO along with thrombosis of splenic vein (SV) and superior mesenteric vein (SMV) is an uncommon condition causing extensive varices formation in the oesophagus, stomach and in other parts of gastrointestinal tract including rectal varix as well as splenomegaly and associated hypersplenism. Most commonly the child presents with hematmesis and due to extensive varices it is difficult to obliterate the varices using endoscopic therapy. Due to thrombosed SMV and SV shunt surgery is not possible. We describe here a case of EHPVO with SMV and SV thrombosis with bleeding gastric varix that underwent gastro- oesophageal devascularisation with splenectomy and oesophageal transection to prevent recurrent bleed from gastric varices. Key words: Extra hepatic portal vein obstruction, Mesenteric venous thrombosis, Portal hypertension, INTRODUCTION MESENTERIC VENOUS THROMBOSIS IN A CHILD Bannerghatta Road, Apollo Hospital, Bangalore 560 076, India. Gastro-oesophageal devascularisation. Extra hepatic portal venous obstruction (EHPVO) is the commonest cause of portal hypertension in the developing world accounting for 70% of pediatric patients with portal hypertension and is second only to cirrhosis in the West. It is also the most common cause of upper gastrointestinal bleeding in children [1]. Classical presentation in these children is with painless hematmesis with splenomegaly without hepatic decompensation [2]. The predisposing factors are thought to be – direct injury to vessels, rare congenital portal vein anomalies, sepsis, dehydration, multiple exchange transfusion and hypercoagulable state [3]. The management of these patients usually involves variceal banding or sclerotherapy. Surgical options include different types of porto-systemic shunts. In small percentage of patients with extensive mesenteric venous thrombosis with SMV and SV thrombosis only surgical option is to do a gastro oesophageal devascularisation and oesophageal transection to control active bleeding or to prevent recurrent hematmesis. CASE REPORT A 3-years old male child presented with history of three episodes of hematmesis in the past. All three episodes required hospital admission but were treated conservatively in different hospitals. Parents also provided history of umbilical sepsis at the age of 6 months. The child underwent upper gastrointestinal (UGI) endoscopy which showed grade III oesophageal varices extending upto the middle third of oesophagus as well as had extensive gastric varices involving cardia and fundus (Fig 1). The patient underwent ultrasound (US) doppler as Fig 1 Grade III oesophageal varices with extensive gastric varices involving cardia and fundus. Apollo Medicine, Vol. 7, No. 4, December 2010 310
  3. 3. Case Report well as computerized tomography (CT) which showed presence of thrombosis in right and left portal vein branch as well as thrombosis of main portal vein (MPV), superior mesenteric vein (SMV) and splenic vein (SV) and splenomegaly. Liver was normal on US and CT. Blood tests of the patient showed normal liver function. Patient underwent detailed thrombophilia study which revealed protein C and protein S deficiency. To prevent recurrent bleeding from gastric and oesophageal varices different options were considered. Sclerotherapy or banding was not possible in this case as had extensive cardiac and fundal varices. In view of thrombosed SMV and SV shunt surgery was not possible. Hence, the patient underwent elective gastro-oesophageal devasculari-sation through an abdominal incision including lower oesophageal transection and splenectomy. During surgery, a liver biopsy was also carried out. The patient made an uncomplicated recovery and was discharged home on 8th post operative day. The histology of liver was normal. DISCUSSION Classical presentation of children with EHPVO is with splenomegaly and repeated episodes of painless, massive hematemesis. The etiology of EHPVO may not be obvious in many cases but a detailed history to rule out causes like severe dehydration and omphalitis must be taken. A thrombophilia profile is also mandatory to rule out hereditary or acquired thrombophilia. Our patient had Protein C and protein S deficiency. Other Indian and Western studies have shown that protein C deficiency is the second most common cause of inherited thrombophilia in patients with portal vein thrombosis (PVT) [4]. Amarapurkar, et al showed that protein C deficiency was the commonest hereditary risk factor (26%) in a study on 28 patients with mesenteric venous thrombosis [5]. Protein C was also the commonest risk factor (38% patients) in a series of 16 patients with mesenteric venous thrombosis reported by Harward, et al [6]. Children with EHPVO presenting with hematmesis are usually treated with variceal banding and sclerotherapy. Patients who fail endoscopic therapy are considered for surgical intervention. Shunt surgery including newer shunt procedures like Rex shunt ( mesentrico – left portal shunt) can result in resolution of symptom in majority of patients [7]. Gastro-oesophageal devascularisation is usually reserved for patients in whom emergency surgery is required to control the bleeding. However 33 - 50% of patients may have extensive thrombosis of portal and splenic veins making them unsuitable for shunt surgery [8]. The unshuntable portal hypertension is a challenge to treat. These patients require frequent hospital admissions for gastro intestinal bleed and require massive blood transfusion. Endoscopic therapy is usually not effective as majority of patients bleed from large fundic varices [9].The patient also had large fundal varices. Cyanoacrylate glue has also been used to control fundic varices but in case of extensive fundic varices it may not be effective and recurrence rate of bleeding is high [10]. These patients are suitable for oesophageal-gastric devasularisation. The Sugiura’s procedure has achieved great success in the treatment of EHPVO in Japan as well as outside [8]. But this involves thoracotomy causing significant morbidity. The modification of Sugiura’s procedure has been described which involves abdominal incision and oesophageal stapling through a gstrostomy [11]. In our case we carried out a similar procedure of gastro-oesophageal devascularisation with splenectomy and oesophageal transaction through abdominal incision. In recent years quality of life (QOL) has become an established endpoint of medical care in patients with EHPVO [12]. It has been observed that splenomegaly and growth retardation are independent contributing factors that adversely affect the QOL in children with EHPVO [12]. As gastro – oesophageal devascularisation with splenectomy corrects problem with splenomegaly it might improve the QOL in long term follow up. CONCLUSION EHPVO with SV and SMV is a challenging problem to treat. These patients need detailed investigation to rule out thrombophilia. Shunt surgeries are not possible in this group of patients and bleeding from fundic varices is difficult to manage with endoscopic therapy. To prevent recurrent bleed from gastro-oesophageal varices elective gastro – oesophageal devascularisation should be considered. REFERENCES 1. Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, et al. Consensus on extra-hepatic portal vein obstruction. Liver Int. 2006; 26:512-519. 2. Peter L, Dadhich SK, Yachha SK. Clinical and laboratory differentiation of cirrhosis and extra hepatic portal venous obstruction in children. J Gastroenterol Hepatol. 2003: 18(2); 185-189. 3. Bellomo-Brandao MA, Morcillo AM, Hessel G, et al. Growth assessment in children with extra-hepatic portal vein obstruction and portal hypertension. Arq Gastroenterol. 2005; 40: 247-250. 4. Bajaj JS, Bhattacharjee J, Sarin SK. Coagulation profile and platelet function in patients with extra hepatic portal 311 Apollo Medicine, Vol. 7, No. 4, December 2010
  4. 4. Case Report vein obstruction and noncirrhotic portal fibrosis. J Gastroenterol Hepatol. 2001; 16: 641-646. 5. Amarapurkar DN, Patel ND, Jatania J. Primary mesenteric venous thrombosis: a study from western India. Indian J Gastroenterol. 2007; 26: 113-117. 6. Harward TR, Green D, Bergan JJ, Rizzo RJ, Yao JS. Mesenteric venous thrombosis. J Vasc Surg. 1989; 9: 328-333. 7. Superina R, Bambini DA, Lokar J, et al. Correction of extra hepatic portal vein thrombosis by the mesenteric to left portal vein bypass. Ann Surg. 2006; 243: 515-521. 8. Orozco H, Takahashi T, Mercado M, et al. Surgical management of extra hepatic portal hypertension and variceal bleeding. World Journal of Surgery. 1994; 18: 246-250. Apollo Medicine, Vol. 7, No. 4, December 2010 312 9. Orloff MJ, Orloff MS, Daily PO, et al. Long term results of radical esophagogastrectomy for bleeding varices due to unshuntable extra hepatic portal hypertension. American Journal of Surgery. 1994; 167: 96-103. 10. Oho K, Iwao T, Sumino M, et al. Ethanolamine oleate versus butyl cyanoacrylate for bleeding gastric varices: a non randomized study. Endoscopy. 1995; 27: 349-354. 11. Shah SR, Nagral SS, Mathur SK. Results of a modified sugiura’s devascularisation in the management of “unshuntable” portal hypertension. HPB Surg. 1999; 11: 235-239. 12. Krishna YR, Yachha SK, Srivastava A, et al. Quality of life in children managed for extrahepatic portal venous obstruction. Pediatr Gastroenterol Nutr. 2010; 50:531-536.
  5. 5. Apollo hospitals: http://www.apollohospitals.com/ Twitter: https://twitter.com/HospitalsApollo Youtube: http://www.youtube.com/apollohospitalsindia Facebook: http://www.facebook.com/TheApolloHospitals Slideshare: http://www.slideshare.net/Apollo_Hospitals Linkedin: http://www.linkedin.com/company/apollo-hospitals BBlloogg:: http://www.letstalkhealth.in/

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