Br. J. Surg. Vol. 63 (1976) 831-835 Chagas’ d i s e a s e of t h e colon G E R A L D 0 MILTON DA SILVEIRA* SUMMARY One hundred cases of’ chagasic megacolon operated upon in Bahia, Brazil, haue been analysed. The principal theories of the pathological physiology of the disease are discussed. The pre- and postoperative radiographic features of the colon are presented, and the surgical treatment as well as the incidence of complications and late results are reported. CHAGAS’disease or Brazilian trypanosomiasis is an endemic disease in certain rural areas of Brazil. The aetiological agent is Trypanosoma cruzi. The Machado Guerero complement fixation test is specific for this disease and is positive in 93 per cent of the patients with megacolon (Fonseca, 1968), which is often one of the presenting symptoms. The terminal colon and rectum become markedly dilated. The histopathological and clinical pictures of chagasic megacolon have been reproduced experimentally by the inoculation of the trypanosomes into mice (Okumura and Correia Neto, 1961). Chagas’ disease, however, produces histopathological changes in several organs, most frequently damaging the heart, the terminal oesophagus, the sigmoid and rectum. There are three theories which try to explain the pathogenesis of Chagas’ disease. These are known as the ‘focal’, the ‘toxic’ and the ‘allergic’ theories. Some believe that the megacolon is a result of lesions of the muscle fibres themselves (Andrade and Andrade, 1965, 1966; Vasconcelos, 1966), whilst others have proposed that it is due to a decrease in the number of autonomic nerve cells in the intestinal plexus (Koberle, 1960; Alencar, 1962; Gama, 1966; Rassi, 1967). In the acute phase of the disease the lesions in the intestinal tract are due to parasites and are composed of mononuclear interstitial infiltration and degenerative changes of the muscle fibres and of the neurons in Meissner’s and Auerbach’s plexuses. In the chronic phase parasites are rarely found (Fig. l), and there are foci of chronic myositis and myocarditis with a decreased number or absence of ganglion cells and a necrotizing arteritis. The number of ganglion cells found at autopsy is presented in Table I (Costa, 1968). In cases of chagasic megacolon the usual findings are elongation and dilatation of the distal colon, thickening of the intestinal wall due to muscular hypertrophy and mucosal ulcers. Electromanometric studies (Haddad et al., 1965) on patients with chagasic megacolon have shown changes in motor activity of the distal portion of the large bowel, characterized by an increase in frequency, amplitude and duration of waves. Similar alterations were found 3 years postoperatively on patients subjected to rectosigmoidectomy, although the clinical symptoms had disappeared. There was n o dilatation of the colon. The response to metacholin stimulation was similar before and after surgery. Following the work of Hiatt (1951) and Swenson et al. (1951, 1954) on the surgical treatment of congenital megacolon, patients with chagasic megacolon have been treated by rectosigmoidectomy (Cutait, Fig. 1. Amastigote forms of T . crrrzi within smooth muscle cell cytoplasm of the colon in a case of megacolon. HE. ( x 310.) Table I : NUMBER OF GANGLION CELLS FOUND IN STUDIES OF AUTOPSY MATERIAL OF NORMAL INDIVIDUALS AND CHAGASIC PATIENTS No. of ganglion cells Grouo Caecum Transverse colon Sigmoid Rectum Normal Chagasic 4163 189 4941 253 5785 381 4036 163 1953; Luz, 1954). This is now the surgical procedure most commonly used to treat chagasic megacolon (Cutait, 1953; Luz, 1954; Pinto, 1960; Raia, 1960; Haddad et al., 1965 ; Simonsen, 1966; Vasconcelos, 1966; Rassi, 1967; Reis Neto, 1968). The aganglionic segment of the bowel, which is the part functionally most affected, is resected. Although other segments of the colon also show changes, they do not appear to be sufficient to cause recurrent symptoms (Gama, 1966). Patients and methods This report is based on a review of 100 cases of chagasic megacolon. Ninety-three patients were * Federal-University of Bahia, Brazil. 831 Gerald0 Milton da Silveira highest count was 23 per cent eosinophils and the average 12.8 per cent. All the patients in the series were submitted to abdominoperineal rectosigmoidectomy. In 3 1 cases Hiatt’s (1951) technique of direct anastomosis was used and in 69 cases Cutait’s (1960) technique of delayed anastomosis. In the 31 cases of direct anastomosis the colon was prepared with purgatives, glycerin enemas, sulpha drugs and antibiotics. Antibiotics and sulpha drugs were not used in the cases undergoing t h e delayed type of anastomosis. 45 40 - 35 . Y jn .-5 3 0 Y a 2s Age tyr) Fig. 2. Age distribution of 100 patients with chagasic megacolon. Table 11: COMPLICATIONS FOLLOWING COLONIC SURGERY ON 100 PATIENTS WITH CHAGASIC MEGACOLON D e 1ayed Direct anastomosis anastomosis (69 cases) (31 cases) No. of No. of Complication cases ”/, cases ><; Infection of presacral 6 19.3 6 8.6 space 12.9 6 86 Partial breakdown of anastomosis -. 6.4 -Total breakdown of anastomosis 6.4 2 2-8 Stercoraceous fistula .3.2 Vesicorectal fistula 3.2 Peritonitis 3 4.3 Necrosis with retraction of pull-through 4 12.9 3 4.3 Stenosis of anastomosis 3.2 I Haemorrhage 10 14.4 Non-stenotic narrowing 3 9.6 I I.4 Postoperative mortality operated upon in the Hospital Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Brazil. Seven patients were operated upon in other hospitals. There were 59 males and 41 females. The age distribution is shown in Fig. 2. The youngest patient was 22 years old and the oldest 76. The main complaint was progressive constipation. The length of time between the initial symptoms and surgery varied from 1 to 25 years. Periods without bowel movements varied from 5 days to 7 months. The incidence of faecaloma in the series was 26 per cent. There was associated mega-oesophagus in 36 per cent of the cases. Chagasic myocarditis with abnormal electrocardiogram findings was seen in 52 per cent of the cases. Routine laboratory studies showed no specificalterations related to the disease, except eosinophilia occurring in 40 per cent of the patients. The 832 Results With the direct anastomosis (Hiatt technique) there was a higher incidence of complications. Temporary transverse colostomy was performed in 61.2 per cent of the cases. Of these, 41.9 per cent were done simultaneously with rectosigmoidectomy and 19 per cent during the early postoperative period. Among the 69 patients in whom the surgical technique of delayed anastomosis was used (Cutait technique), only 7.2 per cent of the colostomies were done in the early postoperative period. The postoperative complications are shown in Table II. With direct anastomosis there was a higher percentage of partial or total breakdown of the anastomosis leading to infection in the presacral space, peritonitis or fistula formation. The incidence of stenosis of the anastomosis, secondary haemorrhage and postoperative mortality was also higher, whereas in delayed anastomosis there was more necrosis due to retraction of the pull-through which caused some narrowing. The causes of death in 3 cases (9.6 per cent) with direct anastomosis were peritonitis in one, haemorrhage in another and vesicorectal fistula in the remaining case. The single death (1.4 per cent) in the delayed anastomosis group resulted from necrosis and retraction of the pull-through. Urinary infection occurred in many cases as prolonged catheterization was needed. All the cases in which delayed anastomosis was used had temporary anal sphincteric incontinence. This was due to the eversion of the rectum and consequent dilatation of the anal orifice produced by the sigmoid loop which was pulled through the anal orifice and remained in situ for 10-12 days. The longest period of time for total recovery of sphincteric function was 35 days. ln the present series no sexual impotence was recorded. Based upon our experience we conclude that abdominoperineal rectosigmoidectomy is the most useful therapeutic method for the treatment of chagasic megacolon. This is in agreement with the opinion of most Brazilian surgeons (Cutait, 1953; Luz, 1954; Pinto, 1960; Raia, 1960; Haddad et al., 1965; Simonsen, 1966; Rassi, 1967; Reis Neto, 1968). In order to obtain satisfactory results we feel it is necessary to follow these principles : 1. The anastomosis should be performed 2-4 cm from the pectinate line. Chagas’ disease of the colon a b Fig. 3. n, Preoperative barium enema film showing marked dilatation and elongation o f the sigmoid colon. h, Appearance o f the left colon 3 years after abdominoperineal rectosigmoidectomy. a Fig. 4. Raritcm enema films. after surgery. IF, b Preoperative view of the rectosigmoid and sigiiioid Nith marked dilatation. /I, Seven years 833 Geraldo Milton da Silveira a b Fig. 5. a, Typical barium enema appearances in chagdsic megacolon. 6, Eighteen years after surgery there is no dilatation of the colon. 2. The diameter of the bowel at the proximal line of resection should be normal or near normal. 3. Mobilization of the left colon, including the splenic flexure, should be performed whenever necessary in order to obtain an intestinal anastomosis without tension. 4. The pelvic dissection and ligation of the middle haemorrhoidal vessels should be performed close to the rectal wall. Immediate results of the surgical treatment of chagasic megacolon were satisfactory and all the preoperative symptoms disappeared. Late follow-up studies could not be obtained in the majority of cases. After discharge from hospital most of the patients returned to their homes, which were usually many miles away. The socio-economic status of the patients as rural workers prevented them from returning to the hospital for periodic examinations. Thus, late follow-up results are available for only 12 patients, for periods varying from 3 to 18 years. At the time of the follow-up 1 1 had daily bowel movements needing no laxatives. One had spontaneous bowel movements every other day. No morphological alterations of the colon were demontrated by barium enemas. Our findings are similar to other published series of cases (Figs. 3-5). Conclusions Surgery is indicated in cases of chagasic megacolon, and abdominoperineal rectosigmoidectomy with delayed anastomosis (Cutait, 1960) is the technique of choice. The late postoperative results are satisfactory. 834 References (1962) 0 sistema nervoso aut6nomo do aparelho digestivo na infestaqiio experimental do camundongo al bin0 pelo Schizotrypanum cruzi. J. Bras. Ner. 13, 1. ANDRADE z. A. and ANDRADE s. G. (1965) A patologia da doenqa de Chagas (forma crdnica cardkaca). Bol. Fund. Congalo Muniz 6, I . ANDRADE s. G. and ANDRADE z. A. (1966) Doenqa de Chagas e alteraqces neoronais no plexo de Auerbach (estudo experimental em camundongos). Rev. Inst. Med. Trop. Scio Paul0 8, 219-224. COSTA R . B. (1968) Histopatologia do colon na doenqa de Chagas. Rev. Goiana Med. 14, 3-10. CUTAIT D. E. (1953) Tratamento do mega-sigma pela retossigmoidectomia. Tese. Fac. Med. Univ. Scio Paulo. CUTAIT D. E. (1960) Nova tecnica de retossigmoidectomia abdominoperineal sem colostomia. 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