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 Table of Contents  
ORIGINAL ARTICLE
Year : 2015  |  Volume : 29  |  Issue : 2  |  Page : 101-105

Management of extrahepatic portal vein obstruction in children: Experience in a tertiary care center in Northeast India


1 Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam, India
2 Department of Physiology, Assam Medical College and Hospital, Dibrugarh, Assam, India

Date of Web Publication20-Aug-2015

Correspondence Address:
Hemonta Kr Dutta
Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh - 786 002, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-4958.163200

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  Abstract 

Background: Variceal bleeding in children is often a life-threatening condition for which timely and appropriate management is mandatory. It is a great challenge, especially in resource-constrained centers, to offer the best possible treatment by optimizing the available resources. Aim: To share the experience of management of extrahepatic portal vein obstruction (EHPVO) in children in a resource-constrained setup during the last one decade. Mode of presentation of the children, treatment options available, and outcomes were analyzed. Settings and Design: The study is a hospital-based prospective study conducted between August 2000 and December 2013. Materials and Methods: Seventy six children with EHPVO who bled at least once were included in the study. Common presentations were hematemesis and/or melena, splenomegaly, pain in abdomen, and ascites. In group A, 30 patients residing within the city area were managed conservatively with variceal banding [endoscopic variceal ligation (EVL)] alone. Group B consisted of 48 patients who hailed from outside the city area and had fundal varices and were offered shunt surgery. Fisher's exact test and Chi-square tests were employed for statistical analysis. Results: The average number of EVL sessions for group A was 3.2. Variceal obliteration was achieved in 18 children and rebleeding was noted in 6. One patients from group A died and one had mild dysphagia. Fifty two shunt procedures were performed in group B patients. Mean operating time, blood loss, and hospital stay were 4.23 h, 690 mL, and 12 days, respectively. There was no operative mortality. Other complications were intestinal obstruction, ascites, rebleeding, and blocked shunt. Spleen size regressed appreciably following surgery and ascites resolved spontaneously. Two patients needed a second shunt surgery for the treatment of blocked shunt. The rates of rebleeding differed significantly between the two groups. Patients were blinded and independently evaluated in the pediatric gastroenterology clinic. Growth and development of the patients in both the groups were within normal limits. Mean follow-ups were 52.5 months for group A and 48.2 months for group B. Conclusion: Children presenting with variceal bleeding can be effectively managed with either EVL or shunt surgery depending on the available resources. However, children from far-off places are better managed with onetime shunt surgery.

Keywords: Extrahepatic portal vein obstruction (EHPVO), esophageal varices, meso-Rex bypass, portal hypertension, portosystemic shunts, variceal banding


How to cite this article:
Dutta HK, Baruah M. Management of extrahepatic portal vein obstruction in children: Experience in a tertiary care center in Northeast India. J Med Soc 2015;29:101-5

How to cite this URL:
Dutta HK, Baruah M. Management of extrahepatic portal vein obstruction in children: Experience in a tertiary care center in Northeast India. J Med Soc [serial online] 2015 [cited 2020 Oct 20];29:101-5. Available from: https://www.jmedsoc.org/text.asp?2015/29/2/101/163200


  Introduction Top


Extrahepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension and upper gastrointestinal bleeding in children. Bleeding esophageal varices may be a life-threatening condition and effective and timely measures against portal hypertension are mandatory. Shunt surgery for the treatment of EHPVO is effective providing a cure of esophageal varices and abolition of the risk of hemorrhage. It is particularly suitable for patients coming from remote places who cannot reach hospital on time in case of a bleed. Judicious use of resources for prompt and effective management of such children is important especially in areas where resources are scanty and patient load is enormous.


  Materials and Methods Top


Seventy six patients (45 males, 31 females, mean age 97.8 months, range from 1.5 years to 21 years) presented between 2000 and 2013 with EHPVO were studied prospectively. Patients who bled at least once (hematemesis or melena) were included in the study. Clinical presentation included hematemesis (64), melena (70), splenomegaly (71), hypersplenism (22), and ascites (16). Three patients had hemoglobin E (HbE) thalassemia with evidence of hypersplenism and cholelithiasis. Patients residing within the city area, where round-the-clock ambulance services were available, were managed conservatively with variceal banding alone (group A). Patients who hailed from outside the city area, patients with fundal varices, and patients who had repeated major bleeds following initial banding were subjected to shunt surgery (group B). Group A had 30 patients (13 males, mean age 85.7 months, from 1.5 years to 16 years), 16 patients had grade IV and 12 had grade III varices. None of the patients in group A had a history of encephalopathy, biliopathy, or other complications. There were 48 patients (32 males, mean age 104.3 months, from 2.5 years to 21 years) in group B and all were treated with various shunt procedures by a single surgeon. Bleeding episodes at presentation were 2-5 (mean 2.6) in group A and 3-6 (mean 3.4) in group B. Varices were noted in all patients in group B: 12 had grade IV, 25 had grade III, and 11 patients had grade II varices. Twelve patients had fundal varices. Emergency banding was done in 12 patients. Liver function tests and Doppler scan of the portal, splenic, and superior mesenteric veins were done preoperatively in all patients. All follow-up patients were given code numbers and were blinded and evaluated by a pediatric gastroenterologist. Fisher's exact test and Chi-square tests were employed for statistical analysis.


  Results Top


Average number of endoscopic variceal ligation (EVL) sessions in group A was 3.2. Variceal obliteration was achieved in 18 patients. Among them six patients continued to have low-grade varices without progression and bleeding,. six children presented with recurrent major bleed after initial regression, two were subjected to shunt surgery, four responded to few sessions of EVL each. One patient died of variceal hemorrhage during transit to hospital. One patient developed mild dysphagia and who recovered after few sessions of antegrade dilatation. A total of 52 shunt procedures were performed in 48 patients. In the early part of the study, side-to-side lienorenal shunt (LRS-13) and distal splenorenal shunt (DSRS-4) were mostly done. Subsequently, as we gained more experience, mesocaval H-graft (29) and meso-Rex (3) shunts using the internal jugular vein were our procedures of choice. Splenectomy with proximal LRS and cholecystectomy was needed in three adolescent patients who had HbE thalassemia with hypersplenism and cholelithiasis. Mean operating time was 4.23 h (3-7.5 h) and mean blood loss was 690 mL (300-1,200 mL). Oral feeding was started in 2-3 days following the surgery and mean hospital stay was 12 days (8-22 days). There was no operative mortality. One patient needed surgery for intestinal obstruction 2 weeks after the shunt surgery. Patients were called for follow-up at 1 month, 3 months, and 6 months after the surgery, and then at yearly intervals. At follow-up, blood counts (leucocyte and platelet counts), spleen size, and physical growth of patients were noted and Doppler scan was done to check shunt patency and portal flow. Endoscopy was done when there was bleeding or evidence of shunt block. Twenty patients developed postoperative ascites, which regressed within 1 month. The spleen size regressed appreciably in all shunted patients except in splenectomized patients. Five patients in the operated group had recurrence of bleeding (2-5 episodes) from 26 months to 54 months following the surgery. Doppler scan revealed blocked shunt in two patients, who needed a second shunt surgery. One of them had earlier side-to-side LRS and the other had DSRS. Mesocaval shunt was done in both of them. Five patients had partial shunt block on Doppler and were followed up with EVL alone. Side-to-side and mesocaval shunts were done earlier in these three patients. There was significant difference of rebleeding rate between the two groups. Six out of 30 patients in group A and 5 out of 48 patients in group B had rebleeding. The degree of freedom on Chi-square test was found to be 1 with P value of 0.157 [Table 4]. Growth and development in both the groups were within the normal limits. Mean follow-up was 52.5 months in group A and 48.2 months in group B. Six patients in group A and 8 patients in group B lost to follow-up.


  Discussion Top


Portal vein thrombosis and cavernous formation are the commonest cause of portal hypertension in children. [1] These patients often present with life-threatening bleeding. [2] Gauthier et al. reported 59 patients with EHPVO, 22 of them were secondary to umbilical vein catheterization during neonatal period. [3] All our patients had portal vein thrombosis, and among them in 16 patients a cavernoma formation was also noted. History of umbilical vein catherization and sepsis was present in only six patients.

Although there are controversy regarding the best form of treatment in such patients, portosystemic shunt surgery has the advantage of relieving the portal pressure and risk of subsequent life-threatening bleeding. [4],[5],[6] On the other hand, there are many who favor the conservative technique of endoscopic sclerotherapy (EST) or banding. [7],[8] Direct operation on varices carries high mortality and high rebleed rates in children. [9],[10] Newer therapies using EVL, octreotide, β-blocker, and transjugular intrahepatic portosystemic shunts (TIPS) have been described. However, these procedures are also associated with high rebleed rate and may not provide satisfactory results. The natural history of childhood EHPVO shows a tendency to decrease incidence of bleeding after about 20 years of age. [11] In our series, 12 patients were over 15 years of age, the oldest being 21 years old and all the patients had major bleed from 4 to 6 times before the treatment.

EVL was originally described by Stiegmann et al., which involves ligation of distal esophageal varices using an elastic band ligature device attached to a flexible endoscope. [12] EVL is equally efficacious and has fewer complications than EST. [13],[14] Thirty patients in our series initially received EVL as a primary therapy [Table 1]. Sixteen patients presented with acute bleed and responded to EVL. For patients below 5 years, were administered intravenous anesthesia with propofol, while those above 5 years received intravenous sedation. A repeat endoscopy was done at 3 months follow-up or earlier if the patients bled in the interval. A total of 96 sessions was needed for 28 patients (mean 3.2 sessions); 2 patients from this group needed shunt surgery for repeated major bleed and hence were shifted to group B. In 18 patients (60%), complete variceal obliteration was achieved. Although low-grade varices were present in six patients, who remained symptom free at 1-5 years of follow-up. One patient who died of hemorrhage received one session of EVL 10 months earlier and did not report for follow-up. One patient had mild stricture of lower esophagus following EVL, but responded to few sessions of antegrade dilatations.

A great variety of surgical procedures, both shunt and nonshunt operations, have been described for treatment of portal hypertension in children. Mesocaval shunt was popularized in the USA by Clatworthy. [15] Experience with central splenorenal shunt was reported by Bismuth in 1980. [16] Mesocaval H-shunt using patient's own vein was first described by Reynolds and Southwick in 1951. [17] The effectiveness of the H-type interposition shunt using internal jugular vein was advocated by Hays et al. [18] Recent studies suggest that children with EHPVO develop progressive liver disease as adults as a consequence of portal biliopathy. [19] The availability of meso-Rex bypass has changed the management plan designed for these children. [20] Meso-Rex shunt, unlike other portosystemic shunts that helped in palliation of the symptoms of portal hypertension in the past, is a restorative procedure in which mesenteric flow of blood is redirected back into the liver.

In the present series, a total of 52 shunt operations were done on 48 patients, including the 2 patients from group A. Shunt surgery was considered for these patients because of the distance to the tertiary care center from their place of residence. As road communications were not always good and the district hospitals did not have proper facilities of critical care and blood transfusion, it was decided that this group of patients would be offered the benefit of onetime surgery in order to eradicate the varices. In the early part of the study side-to-side LRS and DSRS were our procedures of choice. As we gained experience, proximal LRS, mesocaval shunts [Figure 1],[Figure 2], and meso-Rex bypass were performed with equally good results. Meso-Rex bypass was done in three patients. A normal left branch of the portal vein was confirmed on preoperative Doppler study in these three patients, and patient's right internal jugular vein was used as graft [Figure 3],[Figure 4],[Figure 5] and [Figure 6]. All the three patients are doing well on follow-up from 3 to 16 months. Five patients in group B had postshunt bleeding, two of them needed a second shunt surgery and three recovered well with EVL. There was no mortality in group B. Various series report mortality rates of 0-22% and shunt thrombosis rates of 5.6-47% after shunt operations [Table 2]. Rebleeding rate varies from 2% to 47%. [9],[10] Shunt thrombosis particularly affected children under 10 years of age. [21] Shunt thrombosis and rebleeding rates in our series were 14.6% and 10.4%, respectively [Table 3]. The high postshunt complication rate reported from some series may be due to the complications resulting from splenectomy done in proximal splenorenal shunt. [22] In our series, 22 patients had evidence of hypersplenism. In 12 patients from group B, the spleen size decreased significantly and the blood counts improved after shunt surgery. In 4 patients from group A, blood transfusion was needed periodically for low hemoglobin level.
Figure 1 : This figure shows four columns of esophageal varices

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Figure 2 : Same patients 6 months after EVL

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Figure 3 : Side-to-side lienorenal shunt

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Figure 4 : Mesocaval shunt using patient's internal jugular vein

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Figure 5 : Meso-Rex bypass: Upper end of internal jugular vein (IJV) anastomosed to the superior mesenteric vein

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Figure 6 : Meso-Rex bypass: Lower end of IJV anastomosed to the left branch of the portal vein

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Table 1: Series showing results of endoscopic variceal ligation

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Table 2: Series showing results of portosystemic shunts

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Table 3: Results of various shunt procedures in the present series

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Table 4: Rebleeding rate in both the groups

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  Conclusion Top


Children with EHPVO often present with life-threatening bleeding. Both EVL and shunt surgery are effective method of treatments for the control of bleeding. Patients living in remote areas with poor infrastructure should receive the benefit of onetime abolition of varices and cure of portal hypertension by shunt surgery. Patients residing in the vicinity of a tertiary care hospital and with good road communications may be offered EVL, which is associated with minimum complications and can be applied repeatedly in case of recurrence of bleeding.

Acknowledgment

Dr. S. Saikia, Statistical Officer, Assam Medical College, Dibrugarh, was gratefully acknowledged for his technical support during the analysis of the results.

Financial support and sponsorship

No grant or financial support was received for this study.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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2.
Howard ER, Stamatakis JD, Mowat AP. Management of esophageal varices in children by injection sclerotherapy. J Pediatr Surg 1984;19; 2-5.  Back to cited text no. 2
    
3.
Gauthier F, De Dreuzy O, Valayer J, Montupet P. H-type shunt with an autologous venous graft for treatment of portal hypertension in children. J Pediatr Surg 1989;24:1041-3.  Back to cited text no. 3
    
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Bismuth H, Franco D, Alagille D. Portal diversion for portal hypertension in children. The first ninety patients. Ann Surg 1980;192: 18-24.  Back to cited text no. 4
    
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Price MR, Sartorelli KH, Karrer FM, Narkewicz MR, Sokol RJ, Lilly JR. Management of esophageal varices in children by endoscopic variceal ligation. J Pediatr Surg 1996;31:1056-9.  Back to cited text no. 14
    
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Clatworthy HW, Wall T, Watman R. New type of portal to systemic shunt for portal hypertension. Archives of Surgery 1955;71:588-92.  Back to cited text no. 15
    
16.
Bismuth H, Franco D, Alagille D. Portal diversion for portal hypertension in children. Annals of Surgery 1980;24:18-24.  Back to cited text no. 16
    
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Reynolds JT, Southwick JW. Portal hypertension: Use of venous grafts when side to side anastomosis is impossible. Archives of Surgery 1951;68:789-800.  Back to cited text no. 17
    
18.
Hay JM, Valayer J, Maillard JN. Le greffon interpose dans le traitment de hypertension portale de enfant. Chirurgie Pediatrique 1982;23: 211-3.  Back to cited text no. 18
    
19.
Superina R, Shneider B, Emres S, Sarin S, de Ville de Soyet. Surgical guidelines for the management of extra-hepatic portal vein obstruction. Pediatr Transplantation 2006;10:908-13.  Back to cited text no. 19
    
20.
de Viile de Goyet J, Alberti D, Clapuyt P, Falchetti D, Rigamonti V, Bax NM, et al. Direct bypassing of extrahepatic portal venous obstruction children: A new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension. J Pediatr Surg 1998;33:597-601.  Back to cited text no. 20
    
21.
Fonkalsrud EW, Myers NA, Robinson MJ. Management of extrahepatic portal hypertension in children. Annals Surgery 1974;180:487-93.  Back to cited text no. 21
    
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Ulshe MH. To shunt or not to shunt? Gastroenterology 1984;87:446-7.  Back to cited text no. 22
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

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