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A LONG TERM POST SCLEROTHERAPY FOLLOW UP OF EHPVO CHILDREN WITH UPPER GASTROINTESTINAL HAEMORRHAGE
VIRAL PATRAWALA#, HARIBHAKTI SEBA DAS#, PRABHA SAWANT***, PRAVIN RATHI**, KAUSHAL VYAS#, SHEETAL DHADPHALE#, NITIN BORSE*
*Resident; **Lecturer; ***Prof and Head; #Ex-Resident; Department of Gastroenterology, LT Mun. Medi. College and LT Mun. Gen. Hospital, Sion, Mumbai 400 022.
Background : Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in children in India. Variceal bleeding is the commonest mode of presentation and an important cause of morbidity and mortality in them. Though endoscopic sclerotherapy has been accepted as the treatment of choice for index bleeding, the treatment to prevent rebleeding is still the subject of controversy.
Aim and Objective : We retrospectively studied the efficacy of endoscopic sclerotherapy (EST) in 59 children with EHPVO who presented with oesophageal variceal bleed and their follow up from Feb 90 to Sept 99 to assess the risk of rebleeding, complication and prognosis.
Methods : Fifty-nine children of EHPVO (mean age 7.14 æ 3.35 years ranging from 7 months to 12 years) were studied retrospectively. EHPVO was diagnosed on the basis of portal cavernoma on ultrasonography in 55 patients and on splenoportogram in 4 patients. Endoscopic sclerotherapy was carried out at weekly interval for the first three to four weeks and at 3 weekly intervals thereafter till complete or near complete obliteration of varix was achieved. All patients were followed up with check endoscopy every 3-6 months (3 to 87 months) total or near total obliteration of oesophageal varices were achieved in 53 (89.8%) of 59 children, of the remaining 6, three underwent devascularization surgery. The mean number of sclerotherapy sessions required was 7.5 æ 2.2 (4-13). The mean number of blood transfusions required per bleeding episode was 3.8 æ 3.2. Of the 59 children 21 (35.6%) rebled, of which 17 (28.8%) bled during and 4 (6.8) after obliteration of varices. Seven (11.9%) children had more than one episode of bleeding. Fresh varices developed in 15 (26%) children out of 59 children on follow up after initial sclerotherapy schedule. Gastric varices were detected in 47 (60%) children. Bleeding from gastric varix occurred in 7 (9%) children. Ascites developed in 6 (10.6%) children. One child developed oesophageal stricture. There were 3 (5%) deaths.
Conclusion : We conclude that EHPVO is an important and common cause of upper gastrointestinal bleeding in children in Western India. EST is safe and useful in controlling oesophageal variceal bleeding in children and in preventing rebleeding.
INTRODUCTION
In India extrahepatic portal vein obstruction (EHPVO) is the commonest cause of bleeding oesophageal varices in children[1] and it affects 13-40% of patients with portal hypertension. [2,3] Bleeding is the major cause of death in EHPVO. For actively bleeding oesophageal varices, sclerotherapy is the treatment of choice. [4]
Due to high incidence of rebleeding, intervention is necessary to prevent recurrent variceal bleeding. The treatment to prevent the rebleeding in oesophageal variceal bleeding is still the matter of debate. Treatment options include shunt surgery or endoscopic strategies, each with its own advocates. Endoscopic sclerotherapy (EST) is now accepted as the first line therapy in these children. [5] EST has also been used successfully in the management of active variceal bleeding as well as to prevent recurrent variceal bleeding. [1,5] We report a retrospective study from Feb 90 to Sept 99 to assess the efficacy of EST for bleeding oesophagogastric varices secondary to EHPVO in 59 children. We assessed the risk of rebleeding, development of gastric varices and portal hypertensive gastropathy and of other complications along with the prognosis in the natural history of bleeding oesophageal varices in children of EHPVO treated with EST.
PATIENTS AND METHODS
Fifty-nine children of EHPVO presenting with oesophegal variceal bleeding between February 90 and September 99 were studied. The age at diagnosis ranged from 7 months to 12 years with mean age of 7.14 + 3.35 years. After taking thorough history, detail clinical examination was done in all patients. All of them underwent complete haemogram, liver function and renal function tests. Ultrasonography was done in all children. The standard criteria were used for the diagnosis of EHPVO on real time sonography. Splenoportogram was available in 4 patients. Liver biopsy was done in 4 patients as there was suspicion of chronic liver disease. OGDscopy was performed with either GIFXP-20 endoscope (Olympus Co. Japan). General anaesthesia was required in 48 children, those under age of 8 years and especially in initial 3-4 sessions of EST. General anaesthesia was given with thiopentone sodium (Rhone Poulenc, Mumbai, India; 5 mg/kg body weight) and endotracheal tube in situ. Varices were graded from I-IV according to standard classification of Paquet. [6] Gastric varices were classified according to the classification followed by Sarin et al. [7] During every endoscopic procedure specific effort was made to look for gastric and duodenal varices along with portal hypertensive gastropathy. We used 1% polidocanol as sclerosant for oesophageal varices. The injection was given paravariceally one cm. apart, starting from gastrooesophageal junction in a circumferential manner by freehand technique using Olympus injector. The amount of sclerosant injected was 0.5-1 ml/kg, usually not more than 10-12 ml / session. Sclerotherapy was carried out at weekly interval for first 3-4 weeks and at 3 week interval thereafter till complete or near complete obliteration of varices was achieved. Bleeding gastric varices were injected with cyanoacrylate glue. Endpoint of EST was considered when there was complete obliteration of varices or grade I-II white varices. All patients were followed up check endoscopy every 3 monthly for 1 year and 6 monthly there after following obliteration of varices, in order to diagnose development of fresh varices. Fresh varices were injected when the patient had re-bleeding. Patients in whom bleeding was not controlled with one EST, had undergone a second session within 24 hours and if failed, were subjected to emergency surgery. The cause of death, if any, was recorded during follow-up. P value was calculated by using chi-square test.
RESULTS
Fifty-nine children (36 males and 23 females) with oesophageal variceal bleeding were included in the study. These patients were followed up between 3-87 months with a mean follow up of 25.4 months, 37 patients had more than 12 months of follow up. On endoscopy gastric varices were detected in 47 (60%) children. In 39 children it was present at onset and in 8 children it developed following obliteration of varices (p- ns). In 28 (59.5%) patients it was of gastroesophageal varix (GOV-1), 9 (19%) patients had GOV-2 and 10 (21.2%) had isolated gastric varices in fundus {IGV-2}. [7] None were found to have duodenal varices. The frequency of portal hypertensive gastropathy (PHG) was found to be higher in post EST group of children 18 (30.8%) than pre EST 3 (5%) group. Bleeding from gastric varix occurred in 7 (9%) children and these children were injected with cyanoacrylate glue. Three (5%) children had bleeding due to PHG. Thirty five (59.3%) children required 6 or less sclerotherapy sessions to achieve the endpoint of sclerotherapy. Obliteration of varices was achieved in 53 of 59 patients (89.8%).Out of the remaining 6, 3 had undergone devascularization surgery procedure as they had recurrent bleeding which did not respond to sclerotherapy. They responded well to surgery. Other three had no bleeding episodes and are under regular follow up. The mean number of blood transfusions required to treat bleeding episodes was 3.8 + 2.2. Mean sclerotherapy sessions required were 7.5 + 2.2. Of the 59 children, 21 (35.6%) had rebled of which 17 (28.8%) bled during and 4 (6.8%) after obliteration of varices. These children were put again on sclerotherapy schedule. Of the children who had rebled 7 (11.9%) had more than one episode of bleeding. Fifteen (26%) out of 59 children developed fresh varices following the endpoint of initial sclerotherapy session. On follow-up, 4 had rebleeding. Ascites developed in 6 (10.16%) children. Out of them 4 had persistent ascites for more than a month and undergone liver biopsy which was found to be normal. One patient developed dysphagia due to oesophageal stricture following sclerotherapy, which responded well to dilatation. There were three deaths. Two patients died due to massive upper GI bleed while on sclerotherapy schedule before eradication of oesophageal varices. There were no deaths. In patients who bled after obliteration of varices. One patient died due to septicaemia and cerebral abscess.
DISCUSSION
The aetiopathogenesis, natural history, treatment modalities of EHPVO are still not well defined. The most common abnormality in EHPVO consists of a thrombus in the portal vein surrounded by a cavernous leash of collateral vessels. Children with EHPVO have preserved hepatic synthetic functions, thus they are not the candidates for liver transplantation. Bleeding is the major cause of morbidity and mortality. Endoscopic sclerotherapy is effective in controlling acute variceal haemorrhage. However it is imperative that some intervention is required for prevention of recurrent bleeding. The intervention may be surgical, radiographic or by endoscopic sclerotherapy each with its own advocates. The intervention is still the subject of controversy and must be tailored to the individual child on a case by case basis, largely dependent on the expertise and the experience of health care team. The problems with surgery are, it may not be possible if the splenoportal axis is blocked, which occurs in 25-30% of cases. [8] A good size splenic vein should be present before attempting the shunt surgery, may not be available in all children,. The shunt may get blocked and some may suffer from porto-systemic encephalopathy. A cumulative analysis of shunt surgery in EHPVO found the overall mortality and rebleeding rate to be 4.7% and 45.3% respectively. [9] Shunt surgery is preferred at few centres as it is a one time procedure. Moreover, endoscopic sclerotherapy may lead to ectopic varices in the long term which if bleeds torrentially may not be amenable to endoscopic sclerotherapy. Compared to this there was no mortality and rebleeding occurred in 26% in a mean follow up of 19 months after endoscopic sclerotherapy making endoscopy the preferred modality of treatment at many centres.10 Endoscopic band ligation is an attractive alternative to sclerotherapy. [11] The number of sessions required are less with a lower incidence of complications. However, the procedure requires expertise, the recurrence may be faster and the children may not co-operate for this procedure unlike the adults. Also the possibility of incidence of rebleed from ectopic varices may be higher after band ligation. Above all, the cost is the major deterrent for the developing country like India to routinely advocate band ligation. Patients with EHPVO who have not bled should be left alone as bleeding may never occurs and intervention may be counter productive. [12] In EHPVO prevention of bleeding is important. The most important preventive measure is avoidance of all nonsteroidal anti-inflammatory drugs especially aspirin. [13]
In an earlier study we have already reported that EHPVO is the commonest cause of portal hypertension and upper gastrointestinal bleeding in children in Western India, [14] in agreement with reports from North India. [1,2] EST has been an accepted mode of therapy at our centre. In our mean follow up period of 25.4 months the variceal eradication was achieved in 53 of 59 (89.8%) which is comparable to earlier report of 87-100%. [1 , 10] The acturial rate of rebleeding at 2 years was 35% which is in agreement with other reports. [1 , 31,32] There were 3 (5%) deaths, comparable to 5.77% reported by Chawla et al. [5] In agreement with an earlier study we also found the high frequency of PHF in children following obliteration of varies as compared with the pre-EST group (p < 0.05). Gastric varices were found in 47 (60%) children with EHPVO. In conformity with our earlier report the high frequency of ecotopic varices in EHPVO is known. [15]
We conclude EST is very effective in controlling oesophageal variceal bleeding in children with EHPVO. There is a high frequency of gastric varices and portal hypertensive gastropathy following sclerotherapy of varices. In the natural history of bleeding oesophagogastric varices secondary to EHPVO, rebleeding is uncommon while on EST and can be easily controlled with EST.
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