Surgical treatment of idiopathic megarectum in constipated children.

BACKGROUND: Chronic constipation in children is associated with primary megarectum. This study investigated outcomes of surgical treatment of idiopathic megarectum in children. METHODS: This retrospective comparative study included 52 children with idiopathic megarectum (mean age, 9.4 ±â€¯1.7 years) treated from 2007 to 2016. Patients were divided into 2 groups. Group 1 included 23 patients who underwent a Soave pull-through operation. Group 2 included 29 children who underwent laparoscopic low anterior resection with endorectal stapled anastomosis using laparoscopic ultrasound guidance. All patients had clinical and laboratory evaluations with anorectal manometry, colonoscopy and contrast enema studies. RESULTS: Six patients (26.1%) in Group 1 and one (3.5%) in Group 2 experienced anastomosis leakage requiring colostomy (χ2 = 3.867, P = 0.049). In long-term follow-up, 3 children (13.1%) in Group 1 and 2 (6.9%) in Group 2 had ongoing constipation; this difference was not significant. Frequent loose stools with soiling were significantly more common in Group 1 (14 patients; 60.9%) than in Group 2 (4 patients; 13.8%) (χ2 = 10.566, P = 0.001). CONCLUSIONS: Our experience shows that laparoscopic video-assisted low anterior resection of the colon with endorectal stapled anastomosis under laparoscopic ultrasound guidance to determine the level of colon resection is the better operation then Soave for children with idiopathic megarectum and chronic constipation. This approach provides good functional results and reduces complications. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

[Preliminary exploration on accurately preoperative evaluation of colonic lesions in slow transit constipation complicated with adult megacolon].

OBJECTIVE: To investigate the application value of colonic transit test (CTT) combined with anorectal manometry (ARM), barium enema (BE) and defecography (DFG) in accurately evaluating colonic lesions of slow transit constipation complicated with adult megacolon. METHODS: Clinical data of 47 above patients admitted between October 2007 and February 2015 in the People's Hospital of Hunan Province were analyzed retrospectively. All the patients were examined with≥2 times of CTT combined with ARM and BE, and 42 cases received additional DFG at the same time, to evaluate colonic lesions before operation. Operative biopsy pathology was used as the standard. The sensitivity, specificity, positive predictive value(PPV) and negative predictive value(NPV) of positioning in the ascending colon and caecum, transverse colon and descending colon were calculated, and the consistency was represented by Kappa test(Kappa value≥0.75 indicates good consistency, meanwhile higher Kappa value indicates better consistency). The Heikkinen score was used to evaluate defecation function at postoperative 6 months. RESULTS: The age of 47 patients was from 18 to 56 years old. Compared with intraoperative findings and biopsy pathology, the diagnostic coincidence rate was 89.4% by CTT combined with BE and DFG positioning, which suggested pathology-changed colonic segment locating in the ascending colon and cecum (n=12), transverse colon (n=26) and descending colon (n=9), while intraoperative findings and biopsy pathology suggested pathology-changed colonic segment locating in the ascending colon and cecum (n=11), transverse colon (n=23) and descending colon (n=13). The sensitivity was 88.3%, specificity 93.5%, PPV 92.1%, NPV 94.9% and Kappa value was 0.827(P<0.001). Procedures performed included segmental colectomy (n=8), subtotal colectomy (n=29), total colectomy (n=10). There was no serious complication during and after operation. Defecatory function was excellent in 24 cases (60.0%), good in 10 (25.0%), and moderate in 6 (15.0%) evaluated by Heikkinen score at postoperative 6 months. A total of 40 patients were followed up from 1 to 7 years (median 3 years) and there was no long-term diarrhea and recurrence of constipation or giant colon after operation. CONCLUSION: Preoperative detection of CTT combined with ARM, BE and DFG in patients with slow transit constipation complicated with adult megacolon can make a more precise assessment of the extent of colonic lesions in advance, which has a good clinical predictive value.

Dolicomegacolon chagásico / Chagasic dolicho-megacolon

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[Diagnosis and treatment of slow transit constipation complicated with adult megacolon].

OBJECTIVE: To summarize the experience in the management of slow transit constipation complicated with adult megacolon. METHODS: The clinical data of 32 above patients admitted between October 2007 and June 2011 were retrospectively studied. RESULTS: Thirty-two patients were diagnosed as slow transit constipation according to the Roman III criteria. There were 15 males and 17 females aging from 18 to 56 years old. Sitz marker study showed prolonged colon transit time. Barium enema and defecography suggested bowel stricture locating in the transverse colon (n=3), descending colon (n=4), rectum (n=20), and concurrent transverse colon or descending colon and rectum (n=5). Anal manometry showed that anorectal inhibitory reflex was absent in 23 patients, while the other 9 patients were normal. Procedures performed included segmental colectomy and side-to-side anastomosis (n=1), subtotal colectomy and modified Duhamel anastomosis (n=16), total colectomy and ileal J-pouch Duhamel anastomosis (n=9). There were no postoperative complications. During the follow-up ranging from 3 to 47 months, defacatory function was excellent in 18, good in 9, and moderate in 5 patients. CONCLUSIONS: Adult megacolon should be considered differential diagnosis of slow transit constipation. Detailed history taking and thorough evaluation of testing is the key to obviate misdiagnosis. Extent of resection should include the diseased dilated colon and slow transit colon.

[Effect of combined drug treatment on megacolon with severe constipation].

OBJECTIVE: To assess the effectiveness of combined drug treatment on megacolon complicated by severe constipation. METHODS: Ten patients with megacolon confirmed by barium enema examination, 4 males and 6 females, aged 38 (15 - 66), with a mean course of 10 years (2 weeks - 23 years), all complicated by severe constipation and 5 cases with colonic obstruction confirmed by X-ray examination, 1 being diagnosed as with Hirschsprung' disease, 3 secondary to chronic constipation, 1 with diabetes mellitus, 1 with a history of anorectal malformation, 4 with colonic pseudo-obstruction, and 4 with colonic pseudo-obstruction, were treated with combined conservative therapy including tegaserod (6 mg 2/d), polyethylene glycol (PEG) 4000 (20 - 40 g/d), and liuweianxiao (traditional Chinese medicine, 5 # 3/d). Colon enema was used in the first week if necessary. Follow-up was conducted for 1 - 7 months. The major clinical data included bowel symptoms, complications and adverse effects. RESULTS: After 1 - 2 weeks of treatment, properties of feces, defecation times, defecation difficulty, and abdominal symptoms, and X-ray findings were all notably improved. No relapse of colonic obstruction was found. The 5 patients with colonic obstruction all showed release. Regarding adverse effect, mild diarrhea was found in 2 cases and was relieved when the dosage was decreased. CONCLUSION: Combined drug treatment including tegaserod, PEG 4000 and traditional Chinese medicine is effective in treating megacolon with severe constipation and may help avoid surgical treatment.

La colectomía subtotal y cecorrecto anastomosis en el tratamiento del megacolon adquirido del adulto: una alternativa válida / The subtotal colectomy and cecum rectum anastomosis in the acquired adult megacolon treatment: a valid alternative

Antecedentes: la constipación crónica secundaria a megacolon adquirido puede conducir a una incapacidad socio-laboral. El tratamiento a efectuar en pacientes que no responden a la terapia médica genera un desafío para el cirujano. Objetivo: Mostrar la experiencia desarrollada en el manejo de pacientes portadores de megacolon adquirido y la vigencia del empleo de la colectomía subtotal y cecorrecto anastomosis. Lugar de aplicación: Hospital Público Polivalente. Diseño: Retrospectivo. Población: 27 pacientes portadores de megacolon adquirido seleccionados para este procedimiento, desde enero de 1989 a enero de 2005. Método: Se incluyeron los portadores de megacolon adquirido recidivado o con compromiso colónico proximal al sigmoide, dentro del período consignado. Se analizó: edad, sexo, procedencia de zona endémica, patología chagásica concomitante, sintomatología, métodos complementarios, preparación colónica, cirugía practicada, complicaciones y resultados funcionales postoperatorios. Resultados: 15 pacientes fueron varones (55,55 por ciento) con un promedio de 48,25 años (16/76). Veinticuatro provenían de zonas endémicas para enfermedad de Chagas (88,9 por ciento); veinte de ellos tenían serología positiva (83,33 por ciento) y el 80 por ciento cardiomiopatía chagásica. Todos fueron estudiados mediante colon por enema y fibrocolonoscopía, practicándoseles una colectomía subtotal y cecorrecto anastomosis. En tres oportunidades se confeccionó una ileostomía lateral. Las complicaciones observadas alcanzaron a 11 pacientes. No hubo mortalidad en la serie. Los controles postoperatorios se efectuaron en 22 casos (81,5 por ciento), constatándose buen funcionamiento evacuatorio, sin dilatación, con buena continencia y sin trastornos urogenitales. Conclusiones: a la fecha, la colectomía subtotal con cecorrecto anastomosis resulta una alternativa válida para el tratamiento del megacolon del adulto, especialmente en los recidivados o extendidos, exhibiendo algunas venta...

Transverse to descending colon volvulus and megacolon with mesenterium commune: report of a case.

We report a case of volvulus of the transverse-descending (T-D) colon and megacolon associated with mesenterium commune. A 70-year-old man was referred to our hospital for investigation of severe constipation and abdominal fullness. On physical examination, his abdomen was remarkably distended with generalized tenderness, and weak bowel sounds. Abdominal X-ray showed megacolon at the splenic flexure and a contrast medium enema study showed tapering of the upper rectum. Accordingly, under a diagnosis of T-D colon volvulus, we performed an emergency operation to release the colon volvulus. The intraoperative findings showed a volvulus of the T-D colon with mesenterium commune. The patient recovered uneventfully and his symptoms resolved; however, a postoperative barium enema showed residual megacolon at the splenic flexure.

Tratamiento quirúrgico del megacolon del adulto / Surgical treatment of the adult megacolon

En un período de 6 años el autor ha manejado quirúrgicamente a 43 pacientes portadores de megacolon del adulto. El motivo de consulta en 21 fue el vólvulo, en 12 el fecaloma, en 9 la constipación pertinaz y en uno el megacolon tóxico. En los 43 pacientes se practicaron dos reacciones serológicas (test de Elisa y reacción de inmunofluorescencia indirecta), certificando la enfermedad de Chagas en 30, con dos falsos negativos (94 por ciento de positividad). El enema baritado practicado en 30, mostró al dolicomegasigmoides como megaformación predominante (53 por ciento). En 32 (74 por ciento) de ellos se demostró una etiología chagásica, en 8 megacolon idiopático, en dos megacolon congénito y en uno secundario al uso crónico de fármacos anticolinérgicos. Se practicaron 22 operaciones de urgencia, en 21 por vólvulo y en uno por megacolon tóxico. En 10 pacientes se practicó destorsión y pexia, en 7 casos operación de Hartmann, en 4 resección anterior como procedimiento definitivo. Treinta y nueve casos fueron operados en forma electiva, 23 casos con técnica de Duhamel, 8 casos con resección anterior baja, 4 con resección anterior y 4 con colectomía total. No hubo morbilidad ni mortalidad en los casos tratados de urgencia. La morbilidad en cirugía electiva está representada por un caso de oclusión intestinal por brida, un caso de absceso presacro y un fecaloma del muñón rectal. Fallecen dos pacientes. El seguimiento es completo en los 43 casos entre 6 y 72 meses. No hubo recidiva

Operación de Duhamel en el tratamiento quirúrgico del megacolon / Duhamel surgery for the treatment of megacolon

Se presenta la experiencia personal en el tratamiento quirúrgico del megacolon del adulto con la operación de Duhamel. Entre abril del 93 y enro del 98 se practicaron 20 operaciones de Duhamel, 12 con la modificación de Haddad y 8 con anastomosis primaria manual más apertura del tabique colorrectal con sutura mecánica lineal cortante. Se trata de 11 mujeres y 9 hombres, con edades extremas de 25 y 83 años. El motivo de consulta en 9 fue el fecaloma, en 7 el vólvulo y en 4 la constipación pertinaz. Ocho pacientes tenían antecedentes de una intervención previa. El enema baritado se practicó en 16, mostrando el dolicomegasigmoides como megaformación predominante (62,5 por ciento). En 17 (85 por ciento) de ellos se demostró una etiología chagásica, en 2 megacolon idiopático y en 1 megacolon congénito. No hubo mortalidad. La morbilidad específica está representada por 1 caso de abceso presacro que requirió de una colostomía para su manejo y 1 fecaloma del muñón rectal. Es seguimiento es completo en los 20 casos entre 6 y 62 meses. El hábito defecatorio es normal en todos, sin alteraciones de la continencia. No hubo recidiva

Idiopathic megacolon associated with oesophageal achalasia.

We report a rare case of achalasia coexistent with megacolon. The patient, a 25-year-old woman, presented at our hospital with a history of abdominal pain with distension, and was finally operated on for a megacolon. Five months later she presented symptoms of progressive dysphagia and heartburn. Oesophageal manometry of the upper and lower oesophageal sphincter and X-ray studies showed images compatible with achalasia. Oesophagomyotomy of the oesophagogastric junction (Heller procedure with Dor haemifundoplication technique) was performed. In the specimens taken for biopsy, neither pathology of the myenteric plexuses, nor atrophy of the muscle fibres was evident. Chagas' disease serological diagnosis for Trypanosoma cruzii, neurological disease, diabetes and all the pathological events related with neuromuscular disorders of the gastrointestinal tract proved negative. We believe that the pathological findings are related to a dysfunction of the physiological mediators of the upper and lower digestive tract motility. The present case is extraordinary and, to our knowledge, extremely rare. The association of the two pathological diseases is questionable, and the literature is reviewed.

[Myotonic dystrophy with marked megacolon: report of a case].

A 45-year-old woman was incidentally suspected to have megacolon. Chest X-rays showed elevated left diaphragm due to colonic gas, and the heart was deviated to the midline. Barium enema revealed marked dilation of the sigmoid colon, confirming the diagnosis of megacolon. Maximal diameter of the sigmoid colon was 23 cm, but she had no gastrointestinal symptoms. During the work up for megacolon, the presence of myotonic dystrophy was suspected. She had hatchet face, but was not bald. Muscles of the neck and extremities were slightly atrophic. There was percussion myotonia of the tongue and both hands, and grip myotonia of the hands. Laboratory examinations showed impaired glucose tolerance and low level of serum IgG. EMG showed myotonic discharges and myopathic units in the limbs. Brain CT imaging revealed a thick skull. Cases of myotonic dystrophy associated with marked megacolon are rare in Japan. Megacolon presents a high risk for ileus, volvulus, and rupture, and myotonic dystrophy is associated with a high operative and anesthesic risk. Megacolon, therefore, is an important complication to look for in the management of myotonic dystrophy.

Chagasic megacolon associated with colon cancer.

Two cases of chagasic megacolon associated with colon cancer are reported. This is the first communication with complete clinical details of this association. Our two cases presented tumors (adenocarcinomas) in a nondilated segment of the transverse colon. The associated tumor lesions were diagnosed by a barium enema. Both patients had a typical clinical epidemiology and history of chagasic megacolon.

Chagasic megacolon associated to colan cancer: report of a case fram an endemic region of chagas'disease

É relatado um raro caso de associaçäo de megacolo chagásico e câncer do cólon em uma paciente de regiåo endêmica de doença de Chagas - Estado de Goiás, Brasil. Paciente do sexo feminino, 57 anos, com obstipaçåo crônica há 10 anos passanddo até 15 dias sem evacuar, enema opaco mostrava um megassigmóide com uma lesåo estenosante de cólon transverso; o ECG era compatível com cardiopatia chagásica (BRD 3.ºgrau e HBAE); a paciente foi submetida á cirurgia de Duhamel-Haddad e evoluiu posteriormente com metástases pulmonares e hepáticas. Såo discutidos os aspectos patológicos, epidemiológicos e clínicos da forma digestiva da doença dde Chagas e a associaçåo com câncer colorretal

A rare case of achalasia coexistent with sigmoid megacolon and associated with epilepsy.

A case of achalasia coexistent with sigmoid megacolon in a 38-year-old man with known epilepsy is described. The patient was referred to the Ryukyu University Hospital with a 4-year history of dysphagia and heartburn and a 1-year history of abnormal bowel movement. On admission, upper gastrointestinal (GI) series demonstrated a dilated, tortuous thoracic esophagus with a flask-type configuration. Barium enema studies showed a dilated sigmoid colon from the rectosigmoid junction to the descending colon. Myotomy (modified Jekler-Lhotka's procedure) for achalasia and simple sigmoidectomy for sigmoid megacolon were carried out. The biopsied wall of the narrowed esophageal segment at operation showed decreased numbers of ganglion cells in Auerbach's plexus and atrophy of the muscle fibers. The resected dilated sigmoid colon revealed degeneration and markedly decreased numbers of ganglion cells in Auerbach's and Meissner's plexuses. The patient's postoperative course was uneventful and he has been doing well since surgery. The present case is very interesting and to our knowledge, such a case is rare in the literature. We believe that the abnormalities of the ganglion cells may be due to the same etiologic factor as the sigmoid megacolon. The association of the two pathologic processes is discussed, together with a brief review of the literature.

Biliary tract complications in patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome.

The biliary tract system was studied in two patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome (CIIPS) to evaluate manifestations of these diseases, especially as for underlying motility disorder. In a 3-year-old boy with hypoganglionosis, cholelithiasis was diagnosed and the gallbladder specimen showed a markedly hypoplastic neural plexus on histopathology as was found in his total alimentary tract. In a 2-year-old girl with CIIPS, echo-guided gallbladder wall motility testing demonstrated an impaired response to a ceruletide diethylamine stimulation. These clinical experiences in two patients suggest that these pathological entities may be frequently associated with biliary tract complications not only due to a consequence of total parenteral nutrition, but also due to a possible intrinsic involvement of biliary tract dysmotility.

Incidência de doença diverticular do intestino grosso em indivíduos näo chagásicos e em chagásicos com e sem megacólon / Incidence of diverticular disease of the large bowel in non-chagasic and chagasic individuals with and without megacolon

Com a finalidade de estudar a freqüência de associaçäo entre duas colopatias comuns entre nós, o megacólon chagásico e a doença diverticular, foram revistos enemas opacos de 243 pacientes com idade superior a 35 anos. Foi observada a presença de divertículos em 22 (21,6%) dos 102 indivíduos näo chagásicos, em dez (35,7%) dos 28 chagásicos sem megacólon e em 14 (12,4%) dos 113 chagásicos com megacólon. Houve diferença estatisticamente significante entre os dois grupos de chagásicos, mas näo entre cada um deles e o grupo de näo chagásicos. A incidência de divertículos no sigmóide do grupo de näo chagásicos foi significativamente maior do que no sigmóide dos outros dois grupos estudados. Nos pacientes com a associaçäo de megacólon e doença diverticular, os divertículos situaram-se, sem exceçäo, nas porçöes näo dilatadas do intestino grosso. Sugere-se que no cólon dilatado de chagásicos existem condiçöes totalmente desfavoráveis à gênese e/ou manutençäo de divertículos

[Incidence of diverticular disease of the large bowel in non-chagasic and chagasic individuals with and without megacolon]. / Incidência de doença diverticular do intestino grosso em indivíduos não chagásicos e em chagásicos com e sem megacólon.

To study the frequency of association between two common colonic disease in our midst, chagasic megacolon and diverticular disease, we reviewed the barium enemas of 243 patients aged more than 35 years. Diverticula were detected in 22 (21.6%) of the 102 non-chagasic individuals, in 10 (35.7%) of 20 chagasics without megacolon, and in 14 (12.4%) of the 113 chagasics with megacolon. There was a statistically significant difference between the two chagasic groups, but not between each one of them and the non-chagasic group. The incidence of diverticula in the sigmoid colon of the non-chagasic group was significantly higher than in the sigmoid colon of the other two groups studied. Among the patients with association of megacolon and diverticular disease, the diverticula were always located in the nondilated portions of the large bowel. It is suggested that totally unfavorable conditions for the genesis and/or maintenance of diverticula exist in the dilated colon of chagasic patients.