Sugiura Procedure in the Surgical Treatment of Bleeding Varices in Children: Long-Term Results By G. Belloli,
P. Campobasso,
Esophageal
and L. Musi
Vicenza, Italy l Recurrent bleeding, the high occurrence of encephalopathy, and the impairment of hepatic function in the successful cases of portasystemic shunts have led to increasing dissatisfaction with these procedures in recent years. Between March 1974 and November 1990 we have operated on 15 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization). In two cases the entire procedure was performed via the thoracic approach. The spleen was left in place in five cases. We have had no mortality. Operative complications included bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Follow-up was from 4 years 3 months to 16 years 9 months with an average of 10 years 4 months. Long-term results have been gratifying in 12 patients (80%) with disappearance of the varices and no evidence of recurrent bleeding. Three children (20% of the patients) had recurrent bleeding 4% years, 4 years 2 months, and 2% years after the surgical procedure. In all cases there was no evidence of esophageal stenosis, gastroesophageal reflux, or hiatal hernia, nor signs of encephalopathy nor impairment of hepatic function. In our opinion the Sugiura procedure is a valid procedure in the surgical treatment of esophageal varices bleeding in the pediatric age with a high rate of success (80%) and without late complications. Copyright o 1992 by W.B. Saunders Company
INDEX WORDS: Portal hypertension, varices, bleeding; Sugiura procedure.
pediatric;
esophageal
B
LEEDING from esophageal varices is the most important emergency in portal hypertension among children. The seriousness of the bleeding, frequency, and sometimes the impossibility of control with conservative treatment provides an indication for surgery. It is known that the portasystemic diversions have a nonacceptable percentage of failures in children1-3 and complications both in the short and long-term,4-6 with very few exceptions.7J Sugiura and Futagawa in 1973 described a surgical procedure to control the bleeding from esophageal varices that consisted of esophageal transection with paraesophagogastric devascularization.9 The rational approach to the problem and the reported successful results
have made us select this type of surgical procedure for the treatment of our pediatric patients. MATERIALS
AND
METHODS
From March 1974 to November 1990 15 children aged from 2 to 12 years have been operated on in our Department using the Sugiura procedure. The age of the patients at operation is shown in Fig 1. The esophageal varices have been demonstrated both radiologically and endoscopically. In 5 cases the diagnosis was hepatic fibrosis and in the other 10 cases it was prehepatic portal hypertension. All the patients at the time of the operation were in good condition and with good or acceptable hepatic function. In 8 cases there were signs of hypersplenism; one patient had already had splenectomy. All the children had from four to seven episodes of very severe bleeding that required large amounts of blood. In the former patients we did not have any experience with sclerotherapy in children and in the others the indication for surgery was the endoscopic finding of excessive tension of varices which, in our opinion, indicated a high risk for sclerotherapy. None of the patients had previous operations to control the bleeding. The operations were not carried out as emergencies. In 11 cases the surgical treatment was that described by Sugiura and Futagawa without any modification.g In four cases we used a different approach; in two cases the entire procedure was performed through thoracic approach and in the other two cases the first operation was the abdominal one. There was antibiotic prophylaxis after splenectomy. In 5 young patients the spleen was not removed. The average time of the operation was 4% hours. There were no deaths during the hospitalization period nor in the following years. The postoperative period was generally uneventful with the exception of two children who had moderate bleeding in the early postoperative period and two others who had partial leakage from the esophageal suture soon after the operation. This complication required an immediate reoperation with resuture of the esophagus, a pleura patch-graft, draining of the pleural cavity, antibiotic therapy, and total parenteral nutrition for 2 weeks. The first controls, endoscopically and radiologically, were performed after 3 to 6 months, and later an endoscopic control was carried out every year; all the patients also had a computerized 24-hour esophageal pH monitoring. The follow-up has been from 4 years 3 months to 16 years 8 months (average, 10 years 4 months). RESULTS
From the Department of Pediattic Surgery Regional Hospital, Vicenza, Italy. Date accepted: June 18. 1991. Address reprint requests to G. Belloli, MD, Department of Pediattic Surgery Regional Hospital, 36100 Vicenza, Italy. Copyright o 1992 by WB. Saunders Company 0022-3468/9212711-0013$03.00l0 1422
The long-term results have been gratifying in 12 patients (80%) with disappearance of the varices and no evidence of recurrent bleeding (Figs 2 and 3). Three children had recurrent bleeding 41/2years, 4 years 2 months, and 2% years after the surgical procedure. In all these patients radiographic and endoscopic controls showed again the presence of JournalofPediatric Surgery, Vol 27. No 11 (November), 1992: pp 1422-1426
TREATMENT
,fj
OF BLEEDING ESOPHAGEAL
VARICES
H. Pts. i
‘2
3
q
5 Fig 1.
6
7
8
3
101112
Age of patients at operation.
esophageal varices. Two patients were successfully treated with injection sclerotherapy. This treatment was unsuccessful in the other boy who later had a successful mesocaval shunt. In our experience 10 patients had, for a period of 4 to 6 months after the operation, slight intermittent abdominal pain, meteorism, and diarrhea with 4 to 6 bowel movements daily, related to transient gastrointestinal motility disorder perhaps due to some trauma to the vagal nerves. They had a symptomatic treatment with gradual and complete disappearance of the symptoms. The endoscopic, radiologic controls and 24-hour esophageal pH monitoring did not show esophageal stenosis, gastroesophageal reflux, or hiatal hernia. There was no increase in incidence of infections in the splenectomized patients. In the long-term follow-up there was no evidence of neurological or psychological disorders, nor were there signs of encephalopathy. In all cases an impairment of hepatic function was not noted. DISCUSSION
Frequently portal hypertension in children occurs secondary to extrahepatic portal vein obstruction. The presence of esophageal varices is frequent in portal hypertension. Bleeding from esophageal varices is the most common cause of severe gastrointestinal bleeding in children. Current pediatric texts imply that children with extrahepatic portal hypertension can tolerate bleeding well and that this stops spontaneously in late childhood, due to natural collaterals becoming more efficient in decompressing the portal bed.10,” It is true that some children show a spontaneous tendency for the bleeding to diminish through childhood but in other cases the episodes become more severe and life-threatening.
1423
In fact bleeding from esophageal varices is associated with a considerable morbidity and a reported mortality of 12% to 21% even in absence of hepatic disease.10,12-15The choice of management of severe bleeding can vary from nonsurgical (injection sclerotherapy) to surgical treatment. Sclerotherapy has been demonstrated to be an effective method to control esophageal varices bleeding even in children. Complications of ulceration and stricture may be seen during treatment and usually can be resolved with conservative management; complication rate ranges from 10% to 50%.16-19Rebleeding rates have varied from 0% to 25% and mortality rates from bleeding and sclerotherapy from 0% to 11%.19 However, it seems that early and long-term results are good with obliteration of varices and an apparent dramatic reduction in bleeding rate or a definitive resolution in the extrahepatic group.‘“-19 For these reasons the indication for surgery are now more restricted than in the past. In our opinion there is an indication for surgery after unsuccessful sclerotherapy and when very severe bleeding occurs due to varices under excessive tension or in the presence of gastric varices. Most reports concerned with the surgical management focus attention on the choice, timing, and early and late complications of surgery and the variety of procedures proposed reflects the difficulties of the treatment and the uncertainties of short- and longterm results.l0~11,14~20,21 Portasystemic shunts in the early stages seemed to be a successful form of surgical treatment even in children. The long-term controls have shown, with very few exceptions,7J a high percentage of recurrent bleeding,1-3 above all in very young babies, the occurrence of encephalopathy in about 30% to 60% of pediatric patients after the successful operation; furthermore, the impairment of hepatic function is not infrequent.4-hJ5 These problems have led to increasing dissatisfaction in the last years with portasystemic shunt procedures I-4.1 120 The Sugiura procedure realizes a direct attack on gastroesophageal varices and, at the same time, a localized portacaval shunt through the paraesophageal venous collaterals .9,21-26 Although the operation is time consuming, the operative mortality is very low and, if correctly carried out, the incidence of complications is also very low. 9.21-23.25 In fact, it is our opinion that the two postoperative esophageal leakages were due to technical errors and precisely to an excessive esophageal stretching during the abdominal stage of the esophagogastric devascularization. Because of this we actually prefer to perform the abdominal
1424
EELLOLI, CAMPOBASSO,
AND MUSI
Fig 2. Preoperative (top) and postoperative (bottom) radiographic findings (6 years after Sugiura procedure).
operation first. At the same time, in order to avoid this serious complication, we believe that stitching of the varices without opening the esophageal mucosa is useful. In our opinion splenectomy is not indicated in
young children. In cases of hypersplenism we believe that a partial dearteralization of the spleen or a partial splenic embolization26 is preferible and can avoid the recognized hazards of splenectomy. In children under 3 years of age with serious
TREATMENT
OF BLEEDING ESOPHAGEAL
VARICES
1425
Fig 3. Preoperative (top) and postoperative (bottom) 81ndoecopic view (4 years after SUgiura procedure).
bleeding, we believe that the thoracic operation may be sufficient, postponing the abdominal operation until later or if recurrent bleeding occurs. Our follow-up is of sufficient time to be able to judge our results as definitively stable. The long term success rate of the Sugiura procedure is rather high (80%). In our experience we have not observed late esophageal complications and, above all, we have not
observed signs of encephalopathy or impairment of hepatic functioning. These are indeed very serious, late complications rather common in the wellfunctioning portasystemic shunts. Because of this, we believe that our long-term results have shown that the Sugiura procedure is a valid operation in the surgical treatment of esophageal varices bleeding and is preferable to shunting procedures in the pediatric age group.
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extrahepatic portal hypertension in children. Ann Surg 180:487491.1974 21. Belloli G, Campesato A, Campobasso P, et al: Portal hypertension and bleeding esophageal varices in children. Esophageal transection with paraesophagogastric devascularization versus shunting procedures. Med Surg Pediatr 16:263-268, 1981 22. Sugiura M, Futagawa S: Further evaluation of the Sugiura procedure in the treatment of esophageal varices. Arch Surg 112:1317-1321,1977 23. Belloli G, Musi L, Campobasso P, et al: Sugiura procedure in the surgical treatment of esophageal varices bleeding in children. Med Surg Pediatr 8:639-642,1986 24. Superina RA, Weber JL, Shandling B: A modified Sugiura operation for bleeding varices in children. J Pediatr Surg 18:794799,1983 25. Tocornal J, Cruz F: Portosystemic shunts for extrahepatic portal hypertension in children. Surg Gynecol Obstet 153:53-56, 1981 26. Grassi CJ, Boxt LM, Bettmann MA: Partial splenic embolization for painful splenomegaly. Cardiovasc Intervent Radio1 101291. 295,1987