Laparoscopic-Assisted Total Resection and Endorectal Pull-Through Technique for Congenital Megarectum with Anorectal Malformation.

BACKGROUND/PURPOSE: Congenital megarectum (CMR) is sometimes associated with anorectal malformations (ARM), although there is no established therapeutic strategy. This study aims to clarify the clinical features of ARM with CMR, and to demonstrate the effectiveness of a surgical treatment, namely laparoscopic-assisted total resection and endorectal pull-through technique. METHODS: We conducted a review of the clinical records of the patients with ARM with CMR treated at our institution between January 2003 and December 2020. RESULTS: Seven of 33 cases of ARM (21.2%) were diagnosed with CMR, four males and three females. The types of ARM were 'intermediate' in four, and 'low' in three patients. Five of the seven patients (71.4%) required resection of megarectum for intractable constipation and underwent laparoscopic-assisted total resection and endorectal pull-through technique. Bowel function was improved after resection in all five cases. All five specimens showed hypertrophy of the circular fibers, and three of them showed abnormal location of ganglion cells within the circular muscle fibers. CONCLUSIONS: CMR often causes intractable constipation and requires resection of the dilated rectum. Laparoscopic-assisted total resection and endorectal pull-through technique for ARM with CMR considered to be an effective, minimally invasive treatment for intractable constipation. LEVEL OF EVIDENCE FOR CLINICAL RESEARCH PAPERS: Level Ⅳ. TYPE OF STUDY: Treatment study.

Clinical features and outcomes of adult idiopathic megarectum.

OBJECTIVE: Idiopathic megarectum is characterized by abnormal, pronounced rectal dilatation in the absence of identifiable organic pathology. Idiopathic megarectum is uncommon and under-recognized. This study aims to describe the clinical features and management of idiopathic megarectum. METHODS: A retrospective review was undertaken on patients diagnosed with idiopathic megarectum with or without idiopathic megacolon over a 14-year period until 2021. Patients were identified from the hospital's International Classification of Diseases codes, and pre-existing clinic patient databases. Patient demographics, disease characteristics, healthcare utilization and treatment history data were collected. RESULTS: Eight patients with idiopathic megarectum were identified; half of the patients were female, with the median age of symptom onset being 14 years (interquartile range [IQR] 9-24). The median rectal diameter measured was 11.5 cm (IQR 9.4-12.1). The most common presenting symptom was constipation, bloating and faecal incontinence. All patients required prior sustained periods of regular phosphate enemas and 88% were using ongoing oral aperients. Concomitant anxiety and or depression were found in 63% of patients and 25% were diagnosed with an intellectual disability. Healthcare utilization was high with a median of three emergency department presentations or ward admissions related to idiopathic megarectum per patient over the follow-up period; 38% of patients required surgical intervention during the period of follow-up. CONCLUSION: Idiopathic megarectum is uncommon and associated with significant physical and psychiatric morbidity and high healthcare utilization.

Per rectal endoscopic myotomy for Hirschsprung's disease and megacolon.

Hirschsprung's disease (HD) is a congenital disorder characterized by absence of intrinsic ganglion cells of the hindgut. It commonly presents in infancy with refractory constipation and failure to thrive. Short segment HD affecting the rectosigmoid region is the commonest variant. Although surgical or laparoscopic single or multi-stage pull-through procedures have been the gold standard for more than six decades, these procedures are associated with significant morbidity, recurrence, and often multi-stage procedures. Per-rectal endoscopic myotomy (PREM) is a recently described novel minimally invasive procedure based on the principles of third space endoscopy. It is based on the principle to open spastic aganglionic bowel segments by performing a myotomy through a submucosal tunnel. This review describes the patient selection and preparation and technique of PREM and discusses the status of PREM for treatment of HD.

[Etiological analysis and surgical method selection of adult megacolon].

Adult megacolon is a rare disease with heterogeneneous etiology. The treatment schemes of megacolon caused by different causes are also different, but surgery is the final and the most effective method. Due to the lack of early understanding of the disease, many patients have not been clearly diagnosed as adult megacolon and have not been properly treated. This article classifies adult megacolon according to the etiology and summarizes its surgical options. For adult Hirschsprung's disease, modified Duhamel, the Jinling procedure, low anterior resection, or pull-through low anterior resection can be used. For patients with idiopathic megacolon, one-stage subtotal colorectal resection can be selected with adequate preoperative preparations. Some patients admitted to the hospital with emergency intestinal obstruction can be treated with conservative treatment or decompression under colonoscopy followed by selective surgery. For patients with aganglionosis, the procedure is subtotal colorectal resection, the same as that of idiopathic megacolon. The procedure is to remove both the dilated proximal intestine and the stenotic distal intestine, then an ileorectal anastomosis or ascending colon rectal anastomosis is performed. For toxic megacolon, colostomy can be done for mild cases, and for severe infections, subtotal colorectal resection is required. Latrogenic megacolon is mostly caused by segmental stenosis or lack of peristalsis, resulting in chronic dilatation of the proximal end and the formation of megacolon. It is necessary to choose a reasonable surgical procedure according to the specific conditions of the patient. The first choice for the treatment of acute colonic pseudo-obstruction syndrome is decompression under colonoscopy. For those with the secondary changes in the intestine, ostomy is still the most effective surgical procedure, but should be performed with caution.

Evaluation of outcomes following subtotal colectomy for the treatment of idiopathic megacolon in cats.

OBJECTIVE: To evaluate outcomes in cats undergoing subtotal colectomy for the treatment of idiopathic megacolon and to determine whether removal versus nonremoval of the ileocecocolic junction (ICJ) was associated with differences in outcome. ANIMALS: 166 client-owned cats. PROCEDURES: For this retrospective cohort study, medical records databases of 18 participating veterinary hospitals were searched to identify records of cats with idiopathic megacolon treated by subtotal colectomy from January 2000 to December 2018. Data collection included perioperative and surgical variables, complications, outcome, and owner perception of the procedure. Data were analyzed for associations with outcomes of interest, and Kaplan-Meier survival time analysis was performed. RESULTS: Major perioperative complications occurred in 9.9% (15/151) of cats, and 14% (12/87) of cats died as a direct result of treatment or complications of megacolon. The median survival time was not reached. Cats with (vs without) a body condition score < 4/9 (hazard ratio [HR], 5.97), preexisting heart disease (HR, 3.21), major perioperative complications (HR, 27.8), or long-term postoperative liquid feces (HR, 10.4) had greater hazard of shorter survival time. Constipation recurrence occurred in 32% (24/74) of cats at a median time of 344 days and was not associated with retention versus removal of the ICJ; however, ICJ removal was associated with long-term liquid feces (OR, 3.45), and a fair or poor outcome on owner assessment (OR, 3.6). CONCLUSIONS AND CLINICAL RELEVANCE: Results indicated that subtotal colectomy was associated with long survival times and a high rate of owner satisfaction. Removal of the ICJ was associated with less favorable outcomes in cats of the present study.

Idiopathic megacolon and megarectum in an adult treated with laparoscopic modified Duhamel procedure.

Idiopathic megacolon (IMC) and idiopathic megarectum (IMR) describe an abnormality of the colon or rectum, characterised by a permanent dilatation of the bowel diameter in the absence of an identifiable cause. We present a 23-year-old woman with chronic constipation and excessive straining during defecation who presented at the emergency department in partial gut obstruction with a palpable fecaloma. Manual faecal disimpaction and a sigmoid loop colostomy was initially done. A full thickness rectal biopsy was positive for ganglion cells. Further workup led to the diagnosis of chronic IMC and IMR. The patient underwent laparoscopic modified Duhamel procedure, with an uneventful postoperative course.

The "misfortune" of being a COVID-19 negative patient during the coronavirus pandemic.

COVID-19 pandemic has obviously affected patients' behavior towards seeking medical help as well as physicians' decision in the management of emergencies. Our recent experience as surgeons at a COVID-19 referral hospital revealed cases which share an alerting characteristic: the delay in appropriate management. Unfortunately for COVID-19 negative patients a "coronacentric" health system has been adopted. In view of measures applied to avoid spread of the disease, a significant delay in patients' presentation as well as in their in-hospital management is observed. We present cases where delay in appropriate management affected the patients' outcome and underline the fact that balancing between COVID-19 safety measures and a patient who needs urgent treatment can be very challenging and stressful.

Etiological analysis and surgical method selection of adult megacolon / 中华胃肠外科杂志

Adult megacolon is a rare disease with heterogeneneous etiology. The treatment schemes of megacolon caused by different causes are also different, but surgery is the final and the most effective method. Due to the lack of early understanding of the disease, many patients have not been clearly diagnosed as adult megacolon and have not been properly treated. This article classifies adult megacolon according to the etiology and summarizes its surgical options. For adult Hirschsprung's disease, modified Duhamel, the Jinling procedure, low anterior resection, or pull-through low anterior resection can be used. For patients with idiopathic megacolon, one-stage subtotal colorectal resection can be selected with adequate preoperative preparations. Some patients admitted to the hospital with emergency intestinal obstruction can be treated with conservative treatment or decompression under colonoscopy followed by selective surgery. For patients with aganglionosis, the procedure is subtotal colorectal resection, the same as that of idiopathic megacolon. The procedure is to remove both the dilated proximal intestine and the stenotic distal intestine, then an ileorectal anastomosis or ascending colon rectal anastomosis is performed. For toxic megacolon, colostomy can be done for mild cases, and for severe infections, subtotal colorectal resection is required. Latrogenic megacolon is mostly caused by segmental stenosis or lack of peristalsis, resulting in chronic dilatation of the proximal end and the formation of megacolon. It is necessary to choose a reasonable surgical procedure according to the specific conditions of the patient. The first choice for the treatment of acute colonic pseudo-obstruction syndrome is decompression under colonoscopy. For those with the secondary changes in the intestine, ostomy is still the most effective surgical procedure, but should be performed with caution.

Unusual case of cholecystocolonic fistula secondary to megabowel.

Cholecystocolonic fistula with associated idiopathic megabowel (megacolon and megarectum) is a rare presentation as acute large bowel obstruction. Frequently presenting with chronic constipation, acute bowel obstruction is rarely encountered in the presence of concomitant cholecystocolonic fistula. This presents diagnostic and management difficulties with no consensus on appropriate surgical approach. This case highlights the outcomes following emergency total colectomy and subtotal cholecystectomy as a single-stage procedure for a 68-year-old man presenting with cholecystocolonic fistula secondary to idiopathic megabowel as acute large bowel obstruction.

Laparoscopic-assisted colectomy with Duhamel procedure for idiopathic megacolon in adults: a retrospective study.

BACKGROUND: Idiopathic megacolon (IMC) is an uncommon disease in adults. To date, only a few laparoscopic experiences and functional outcomes of IMC have been reported. This study was to retrospectively analyse our 12 year surgical experience and functional outcomes in adult patients with IMC. METHODS: A 12-year retrospective study from October 2006 to November 2018 was performed for patients with IMC who underwent surgical interventions. Patients who underwent laparoscopic-assisted colectomy and Duhamel procedure with ileorectal or colorectal anastomosis were collected. Clinical data of surgery and functional outcomes were analysed. RESULTS: A total of 13 patients who underwent surgical interventions were included in the study. Seven patients underwent laparoscopic total colectomy with ileorectal anastomosis (Duhamel procedure), one patient underwent laparoscopic total colectomy with end ileostomy because of acute intestinal obstruction, while five other patients underwent laparoscopic segmental colectomy with colorectal anastomosis (Duhamel procedure). The mean operative time was 181.6 min (range 150-246). The mean estimated blood loss was 75.6 ml (range 40-200). The mean postoperative hospital stay was 8.2 days (range 6-13). There was no conversion to an open procedure and no surgical mortality. Postoperative diarrhoea was the most prominent complaint during the early period after total colectomy. All patients showed adaptation to the defaecation frequency 3-6 months postoperatively, and had a good quality of life in long-term follow-up. CONCLUSION: Laparoscopic-assisted colectomy with Duhamel procedure is a safe and efficient technique for IMC in adults. The scope of colon resection and the type of anastomosis should be individually selected.

Chagasic megacolon in Venezuela - case report / Megacólon chagásico na Venezuela

ABSTRACT Chagasic megacolon is the second most frequent cause of manifestation of the digestive forms of Chagas disease (trypanosoma cruzi parasitosis), characterized by progressive pseudo-occlusive symptoms or chronic constipation, caused by an alteration in the functioning of the colonic wall musculature. In Venezuela, cases of chagasic disease reported in the past are referred to chagasic heart disease, this being the first documented case of chagasic megacolon. We broach herein a case of chagasic megacolon in which early diagnosis and prompt surgical treatment led to a successful outcome.

RESUMO O megacólon chagásico é a segunda causa mais frequente de manifestação das formas digestivas da doença de Chagas (parasitose do Trypanosoma cruzi), caracterizado por sintomas pseudo-oclusivos progressivos ou constipação crônica, causado por uma alteração no funcionamento da musculatura da parede do cólon. Na Venezuela, os casos da doença chagásica relatados no passado são referidos como doença cardíaca chagásica, sendo este o primeiro caso documentado de megacólon chagásico. Neste artigo, abordamos um caso de megacólon chagásico no qual o diagnóstico precoce e o tratamento cirúrgico imediato levaram a um resultado bem-sucedido.

Megacolon en la enfermedad inflamatoria intestinal: respuesta a infliximab / No disponible

El megacolon es una complicación grave de la enfermedad inflamatoria intestinal que con frecuencia requiere colectomía. Infliximab sería una alternativa terapéutica cuando fracasa el tratamiento convencional, antes de la cirugía. En la actualidad, su uso se basa en la publicación de casos aislados. Presentamos nuestra serie de 12 pacientes con megacolon (cinco con signos de toxicidad sistémica) tratados con infliximab. El 75% de los pacientes evitaron la colectomía durante el episodio agudo tras la instauración precoz del tratamiento con infliximab (2,45 días desde el diagnóstico del megacolon), apreciando un mayor riesgo de cirugía entre los pacientes con colitis ulcerosa y criterios de toxicidad. Pese a mantener infliximab a largo plazo, dos pacientes más requirieron cirugía en el seguimiento. Ningún paciente sufrió efectos adversos relevantes en relación con el tratamiento ni complicaciones posquirúrgicas significativas

No disponible

Pediatric sigmoid volvulus due to Chagas disease.

Exposure and infections by Trypanosoma cruzi are the fourth cause of loss of potential life years between parasitic and infectious diseases. We describe the case of a 11-year-old patient with intestinal occlusion, surgically treated with intestinal volvulus, the surgical specimen is sent to histopathology reporting Chagasic megacolon. The age range of presentation is a challenge in the absence of nonspecific symptoms. There is no pediatric statistical data that define trypanosomiasis in a latent or chronic state and will be diagnosed in adult stages due to the physiopathological alterations that they will present.

La exposición y las infecciones por Trypanosoma cruzi ocupan el cuarto lugar entre las causas de pérdida de años de vida potenciales por enfermedades parasitarias e infecciosas. Se describe el caso de un niño de 11 años, con cuadro de oclusión intestinal, intervenido quirúrgicamente con datos de vólvulo intestinal. La pieza quirúrgica se envió a histopatología, que reportó megacolon chagásico. El rango de edad de presentación es un reto ante la falta de síntomas inespecíficos. No se cuenta con datos estadísticos pediátricos que definan la tripanosomiasis en estado latente o crónico, y estos niños serán diagnosticados en la etapa adulta por las alteraciones fisiopatológicas que presentarán.

An unusual cause of idiopathic megacolon in a juvenile: A case report.

RATIONALE: Idiopathic megacolon (IMC) is a rare condition in young children. The association of indigestible food and IMC has never been mentioned in literature. PATIENT CONCERNS: An 11-year-old boy reported with a 1-year duration of intermittent constipation and abdominal distension after having eaten a large amount of fried sticky rice in 1 consumption. DIAGNOSES: Chronic low colonic obstruction, IMC and malnutrition. INTERVENTIONS: This patient was managed conservatively for 1 week at first. Then he underwent loop ileostomy since conservative therapy was poorly tolerated. Enteral decompression, gut biopsy, peritoneal lavage, and drainage were performed in the same procedure. OUTCOMES: Rapid weight gain was observed 4 months after operation. LESSONS: IMC is difficult to diagnose due to the lack of specific clinical manifestations and pathological features. The protocols for management of IMC remains controversial. To achieve a good long-term outcome, early intervention is recommended.

Transanal proximal rectosigmoidectomy. A new operation for severe chronic idiopathic constipation associated with megarectosigmoid.

BACKGROUND: At the severe end of the idiopathic constipation spectrum exist patients with chronic idiopathic constipation associated with an enormous megarectosigmoid, among whom few require surgery. We performed transanal proximal rectosigmoidectomy, involving preservation of a 5 cm rectal reservoir, to ameliorate inconsistent and unpredictable outcomes and fecal incontinence occurring in some patients operated on with other techniques. METHODS: We retrospectively observed patients with chronic idiopathic constipation with megarectosigmoid who underwent transanal proximal rectosigmoidectomy during July 2017-May 2018. We analyzed sex, age, operation indication, complications, functional outcome, and time of follow-up. We statistically compared the dosage of laxative before and after the procedure. RESULTS: Thirteen patients were included in the study. Median age during surgery was 8 years. In 9 cases, the indication for surgery was chronic intake of a daily high dose of Senna with failed weaning trials, and 4 had persistent fecal impaction with laxative-intolerance and refusal of rectal enemas. No intra/postoperative complications occurred. Currently, all 13 patients have daily voluntary bowel movements and no fecal accidents. Laxative dosage was significantly reduced (p = 0.007). Follow-up ranged 6-16 months. CONCLUSION: Preliminary results suggest transanal proximal rectosigmoidectomy as an adequate alternative for patients requiring surgery for chronic idiopathic constipation with megarectosigmoid. TYPE OF STUDY: Clinical research. LEVEL OF EVIDENCE: Level III.

Constipation with megacolon in a 36-year-old man: a rare presentation of MEN2B from Sri Lanka.

Diffuse intestinal ganglioneuromatosis is a rare condition associated with MEN2B. It is also seen in conditions like neurofibromatosis type 1 and Cowden syndrome. This is a report of a patient who underwent total colectomy with end ileostomy creation for a megacolon. He was diagnosed to have diffuse ganglioneuromatosis on histological examination of the resected segment of colon. The definitive management of diffuse ganglioneuromatosis is to resect and anastomose.