EXTRA

                       

                       

HEPATIC M

            

                       

PORTAL VMESENTER

      

VEIN OBSTRIC VENOU

TRUCTIONUS THROM

  

(EHPVO) BOSIS IN A

WITH EXTA CHILD 

TENSIVE

Case Report

INTRODUCTION

Extra hepatic portal venous obstruction (EHPVO) is thecommonest cause of portal hypertension in the developingworld accounting for 70% of pediatric patients with portalhypertension and is second only to cirrhosis in the West. It isalso the most common cause of upper gastrointestinalbleeding in children [1]. Classical presentation in thesechildren is with painless hematmesis with splenomegalywithout hepatic decompensation [2]. The predisposingfactors are thought to be – direct injury to vessels, rarecongenital portal vein anomalies, sepsis, dehydration,multiple exchange transfusion and hypercoagulable state [3].

The management of these patients usually involvesvariceal banding or sclerotherapy. Surgical options includedifferent types of porto-systemic shunts. In smallpercentage of patients with extensive mesenteric venousthrombosis with SMV and SV thrombosis only surgicaloption is to do a gastro oesophageal devascularisation andoesophageal transection to control active bleeding or toprevent recurrent hematmesis.

CASE REPORT

A 3-years old male child presented with history of threeepisodes of hematmesis in the past. All three episodesrequired hospital admission but were treated conservatively

EXTRA HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) WITH EXTENSIVEMESENTERIC VENOUS THROMBOSIS IN A CHILD

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,Bannerghatta Road, Apollo Hospital, Bangalore 560 076, India.

Correspondence to: Dr Suresh Singhvi, Senior Consultant (Surgery), Institute of Liver & Pancreatic Disorder andSolid 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 uncommoncondition causing extensive varices formation in the oesophagus, stomach and in other parts ofgastrointestinal tract including rectal varix as well as splenomegaly and associated hypersplenism. Mostcommonly the child presents with hematmesis and due to extensive varices it is difficult to obliterate thevarices using endoscopic therapy. Due to thrombosed SMV and SV shunt surgery is not possible. Wedescribe here a case of EHPVO with SMV and SV thrombosis with bleeding gastric varix that underwentgastro- oesophageal devascularisation with splenectomy and oesophageal transection to preventrecurrent bleed from gastric varices.

Key words: Extra hepatic portal vein obstruction, Mesenteric venous thrombosis, Portal hypertension,Gastro-oesophageal devascularisation.

in different hospitals. Parents also provided history ofumbilical sepsis at the age of 6 months.

The child underwent upper gastrointestinal (UGI)endoscopy which showed grade III oesophageal varicesextending upto the middle third of oesophagus as well ashad extensive gastric varices involving cardia and fundus(Fig 1). The patient underwent ultrasound (US) doppler as

Fig 1 Grade III oesophageal varices with extensive gastricvarices involving cardia and fundus.

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well as computerized tomography (CT) which showedpresence of thrombosis in right and left portal vein branchas well as thrombosis of main portal vein (MPV), superiormesenteric vein (SMV) and splenic vein (SV) andsplenomegaly. Liver was normal on US and CT. Blood testsof the patient showed normal liver function. Patientunderwent detailed thrombophilia study which revealedprotein C and protein S deficiency.

To prevent recurrent bleeding from gastric andoesophageal varices different options were considered.Sclerotherapy or banding was not possible in this case ashad extensive cardiac and fundal varices. In view ofthrombosed SMV and SV shunt surgery was not possible.Hence, the patient underwent elective gastro-oesophagealdevasculari-sation through an abdominal incision includinglower oesophageal transection and splenectomy. Duringsurgery, a liver biopsy was also carried out. The patientmade an uncomplicated recovery and was dischargedhome on 8th post operative day. The histology of liver wasnormal.

DISCUSSION

Classical presentation of children with EHPVO is withsplenomegaly and repeated episodes of painless, massivehematemesis. The etiology of EHPVO may not be obviousin many cases but a detailed history to rule out causes likesevere dehydration and omphalitis must be taken. Athrombophilia profile is also mandatory to rule outhereditary or acquired thrombophilia. Our patient hadProtein C and protein S deficiency. Other Indian andWestern studies have shown that protein C deficiency is thesecond most common cause of inherited thrombophilia inpatients with portal vein thrombosis (PVT) [4].

Amarapurkar, et al showed that protein C deficiencywas the commonest hereditary risk factor (26%) in a studyon 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 venousthrombosis reported by Harward, et al [6].

Children with EHPVO presenting with hematmesis areusually treated with variceal banding and sclerotherapy.Patients who fail endoscopic therapy are considered forsurgical intervention. Shunt surgery including newer shuntprocedures like Rex shunt ( mesentrico – left portal shunt)can result in resolution of symptom in majority of patients[7]. Gastro-oesophageal devascularisation is usuallyreserved for patients in whom emergency surgery isrequired to control the bleeding. However 33 - 50% ofpatients may have extensive thrombosis of portal andsplenic veins making them unsuitable for shunt surgery [8].

The unshuntable portal hypertension is a challenge totreat. These patients require frequent hospital admissionsfor gastro intestinal bleed and require massive bloodtransfusion. Endoscopic therapy is usually not effective asmajority of patients bleed from large fundic varices[9].The patient also had large fundal varices. Cyanoacrylateglue has also been used to control fundic varices but in caseof extensive fundic varices it may not be effective andrecurrence rate of bleeding is high [10].

These patients are suitable for oesophageal-gastricdevasularisation. The Sugiura’s procedure has achievedgreat success in the treatment of EHPVO in Japan as well asoutside [8]. But this involves thoracotomy causingsignificant morbidity. The modification of Sugiura’sprocedure has been described which involves abdominalincision and oesophageal stapling through a gstrostomy[11]. In our case we carried out a similar procedure ofgastro-oesophageal devascularisation with splenectomyand oesophageal transaction through abdominal incision.

In recent years quality of life (QOL) has become anestablished endpoint of medical care in patients withEHPVO [12]. It has been observed that splenomegaly andgrowth retardation are independent contributing factorsthat adversely affect the QOL in children with EHPVO[12]. As gastro – oesophageal devascularisation withsplenectomy corrects problem with splenomegaly it mightimprove the QOL in long term follow up.

CONCLUSION

EHPVO with SV and SMV is a challenging problem totreat. These patients need detailed investigation to rule outthrombophilia. Shunt surgeries are not possible in thisgroup of patients and bleeding from fundic varices isdifficult to manage with endoscopic therapy. To preventrecurrent bleed from gastro-oesophageal varices electivegastro – oesophageal devascularisation should beconsidered.

REFERENCES

1. Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, HamidS, Hashizume M, et al. Consensus on extra-hepaticportal vein obstruction. Liver Int. 2006; 26:512-519.

2. Peter L, Dadhich SK, Yachha SK. Clinical and laboratorydifferentiation of cirrhosis and extra hepatic portalvenous obstruction in children. J Gastroenterol Hepatol.2003: 18(2); 185-189.

3. Bellomo-Brandao MA, Morcillo AM, Hessel G, et al. Growthassessment in children with extra-hepatic portal veinobstruction and portal hypertension. Arq Gastroenterol.2005; 40: 247-250.

4. Bajaj JS, Bhattacharjee J, Sarin SK. Coagulation profileand platelet function in patients with extra hepatic portal

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vein obstruction and noncirrhotic portal fibrosis. JGastroenterol Hepatol. 2001; 16: 641-646.

5. Amarapurkar DN, Patel ND, Jatania J. Primarymesenteric venous thrombosis: a study from westernIndia. 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 ofextra hepatic portal vein thrombosis by the mesenteric toleft portal vein bypass. Ann Surg. 2006; 243: 515-521.

8. Orozco H, Takahashi T, Mercado M, et al. Surgicalmanagement of extra hepatic portal hypertension andvariceal bleeding. World Journal of Surgery. 1994; 18:246-250.

9. Orloff MJ, Orloff MS, Daily PO, et al. Long term results ofradical esophagogastrectomy for bleeding varices dueto unshuntable extra hepatic portal hypertension.American Journal of Surgery. 1994; 167: 96-103.

10. Oho K, Iwao T, Sumino M, et al. Ethanolamine oleateversus butyl cyanoacrylate for bleeding gastric varices: anon randomized study. Endoscopy. 1995; 27: 349-354.

11. Shah SR, Nagral SS, Mathur SK. Results of a modifiedsugiura’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 oflife in children managed for extrahepatic portalvenous obstruction. Pediatr Gastroenterol Nutr. 2010;50:531-536.

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