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.

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.

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.

Is intestinal stasis sufficient by itself in promoting enterocolitis in a non-genetic rat model of Hirschsprung's disease?

BACKGROUND: Hirschsprung's disease-associated enterocolitis (HE) is a life-threatening septic complication of Hirschsprung's disease (HD), leading to bacterial translocation (BT) and sepsis. Many factors, such as intestinal stasis, HD-related inherited immune disorders and abnormal mucosal secretion have been implicated in its pathogenesis. OBJECTIVES: To investigate the effect of intestinal stasis as an independent factor in the pathogenesis of HE intestinal lesions and its systematic effects. MATERIAL AND METHODS: The rectal ganglion cells of 46 Wistar rats were chemically ablated through local benzalkonium chloride (BAC) injection, in order to create a HD model (megacolon rats) that does not carry the possible genetic burden of HD. The animals were sacrificed either on the 20th or 25th day after ablation and were examined for histopathological changes on the wall of the small intestine, presence of bacterial translocation in body organs, body biometrics, and white blood cell count (WBC) and hemoglobin concentration. The results were compared to control animals. RESULTS: In the megacolon rats, severe damage on the small intestine as well as BT proportional to the extent of the intestinal damage and to the time elapsed after ablation was observed. Significant effects on the WBCs, hemoglobin concentration and biometric parameters were also observed. CONCLUSIONS: In megacolon rats, intestinal stasis can lead by itself to a full-blown HE. The HE lesions that promote BT are present even in regions distant from the aganglionic bowel and are proportional to the time elapsed under the influence of intestinal stasis. Systematic effects such as growth retardation are also produced.

Megacolon agangliónico en el adulto. Urgente y sorprendente causa de oclusión intestinal / Aganglionic megacolon in the adult. Urgent and surprising cause of intestinal occlusion

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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.

Abdominal compartment syndrome secondary to megarectum and megasigmoid.

A 31-year-old male patient with chronic constipation of unknown aetiology presented emergently with worsening nausea, vomiting and abdominal distension of one week duration. On examination, his abdomen was distended with minimal tenderness. A plain film of the abdomen demonstrated severe faecal loading. The patient was haemodynamically unstable on admission and appeared sick. An urgent CT abdomen and pelvis was conducted showing extensive rectal dilatation and associated proximal colonic stercoral perforation. The patient proceeded straight to theatre for laparotomy as his general condition was deteriorating rapidly. On transfer to the operating table, the patient suffered cardiopulmonary arrest. Resuscitation was immediately commenced. Abdominal compartment syndrome was suspected. Cardiac output was re-established following a midline laparotomy which acted relieve the abdominal pressure. The rectosigmoid faecal content was decompressed via an enterotomy. The perforated segment of transverse colon was resected and an end colostomy fashioned. A year later, the continuity of the bowel was re-established.

Symptoms and diagnostic criteria of acquired Megacolon - a systematic literature review.

BACKGROUND: Acquired Megacolon (AMC) is a condition involving persistent dilatation and lengthening of the colon in the absence of organic disease. Diagnosis depends on subjective radiological, endoscopic or surgical findings in the context of a suggestive clinical presentation. This review sets out to investigate diagnostic criteria of AMC. METHODS: The literature was searched using the databases - PubMed, Medline via OvidSP, ClinicalKey, Informit and the Cochrane Library. Primary studies, published in English, with more than three patients were critically appraised based on study design, methodology and sample size. Exclusion criteria were studies with the following features: post-operative; megarectum-predominant; paediatric; organic megacolon; non-human; and failure to exclude organic causes. RESULTS: A review of 23 articles found constipation, abdominal pain, distension and gas distress were predominant symptoms. All ages and both sexes were affected, however, symptoms varied with age. Changes in anorectal manometry, histology and colonic transit are consistently reported. Studies involved varying patient numbers, demographics and data acquisition methods. CONCLUSIONS: Outcome data investigating the diagnosis of AMC must be interpreted in light of the limitations of the low-level evidence studies published to date. Proposed diagnostic criteria include: (1) the exclusion of organic disease; (2) a radiological sigmoid diameter of ~ 10 cm; (3) and constipation, distension, abdominal pain and/or gas distress. A proportion of patients with AMC may be currently misdiagnosed as having functional gastrointestinal disorders. Our conclusions are inevitably tentative, but will hopefully stimulate further research on this enigmatic condition.

Death Caused by Hepatodiaphragmatic Interposition of the Colon.

Hepatodiaphragmatic interposition of the colon is a rare, usually asymptomatic, anomaly and is typically an incidental radiologic finding. There are few cases in the literature describing the symptomatic form of the condition, known as Chilaiditi syndrome. In some cases, it may be accompanied by various severe complications. If symptoms are present, usually conservative treatment is given. However, conservative treatment only addresses the symptoms but does not prevent their recurrence and possible complications. Our present report shows that this anomaly may not only cause symptoms, but may also progress and cause severe complications, in our case-megacolon leading to right heart failure and, ultimately, death. To date, however, there have been no literature reports of death caused by colonic interposition. Therefore, it is important to draw attention to the importance of this anomaly and its appropriate diagnosis and treatment to ensure the most favorable patient outcomes.

[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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Enteric Neuronal Damage, Intramuscular Denervation and Smooth Muscle Phenotype Changes as Mechanisms of Chagasic Megacolon: Evidence from a Long-Term Murine Model of Trypanosoma cruzi Infection.

We developed a novel murine model of long-term infection with Trypanosoma cruzi with the aim to elucidate the pathogenesis of megacolon and the associated adaptive and neuromuscular intestinal disorders. Our intent was to produce a chronic stage of the disease since the early treatment should avoid 100% mortality of untreated animals at acute phase. Treatment allowed animals to be kept infected and alive in order to develop the chronic phase of infection with low parasitism as in human disease. A group of Swiss mice was infected with the Y strain of T. cruzi. At the 11th day after infection, a sub-group was euthanized (acute-phase group) and another sub-group was treated with benznidazole and euthanized 15 months after infection (chronic-phase group). Whole colon samples were harvested and used for studying the histopathology of the intestinal smooth muscle and the plasticity of the enteric nerves. In the acute phase, all animals presented inflammatory lesions associated with intense and diffuse parasitism of the muscular and submucosa layers, which were enlarged when compared with the controls. The occurrence of intense degenerative inflammatory changes and increased reticular fibers suggests inflammatory-induced necrosis of muscle cells. In the chronic phase, parasitism was insignificant; however, the architecture of Aüerbach plexuses was focally affected in the inflamed areas, and a significant decrease in the number of neurons and in the density of intramuscular nerve bundles was detected. Other changes observed included increased thickness of the colon wall, diffuse muscle cell hypertrophy, and increased collagen deposition, indicating early fibrosis in the damaged areas. Mast cell count significantly increased in the muscular layers. We propose a model for studying the long-term (15 months) pathogenesis of Chagasic megacolon in mice that mimics the human disease, which persists for several years and has not been fully elucidated. We hypothesize that the long-term inflammatory process mediates neuronal damage and intramuscular and intramural denervation, leading to phenotypic changes in smooth muscle cells associated with fibrosis. These long-term structural changes may represent the basic mechanism for the formation of the Chagasic megacolon.

Acute colonic complications in a patient with Chagas disease.

We present the case of a young bolivian woman who suffered two acute and impressive colonic complications due to a Chagasic megacolon.

Fístula cólon-jejunal em paciente com fecaloma e volvos jejunal e sigmoideano devido à dolicomegassigmoide: relato de caso / Colon fistula-jejunal in patient with fecaloma and volvos jejunal sigmoideano and due to dolicomegassigmoide: case report

O dolicomegacólon consiste no aumento do comprimento e diâmetro dos segmentos do cólon, causando alteração da motilidade e interferindo no funcionamento do trânsito intestinal. Pode ser classificado em: Aganglionose Congênita ou Doença de Hirschsprung; o Megacólon Chagásico ou Adquirido e o Idiopático. Suas principais complicações são fecalomas, volvos, úlceras por estase fecal com isquemia da parede intestinal e perfurações. Relatamos o caso de uma paciente com quadros recorrentes de suboclusões intestinais que melhoravam com tratamentos clínicos conservadores. Os preparos mecânicos para realização de uma colonoscopia sempre foram inadequados devidos aos fecalomas, volvo sigmoideano e a dor forte apresentada pela paciente durante tais tentativas. Indicado laparotomia exploradora, evidenciou-se volvo de sigmoide com fecaloma impactado, aderido e fistulizado para um volvo de jejuno distal.

The dolicomegacólon is to increase the length and diameter of the parts of the colon, causing changes in motility and interfering with the operation of the intestinal transit. Can be classified into: Aganglionosis Congenital or Hirschsprung disease; the Chagas Disease or Acquired megacolon and idiopathic. Its main complications are fecalomas, volvulus, fecal stasis ulcers with ischemia of the bowel wall and perfurações. We report the case of a patient with recurrent episodes of intestinal sub-occlusion that improved with conservative medical treatment. Mechanical preparation for performing a colonoscopy were always inadequate due to fecalomas, sigmoideano volvo and severe pain presented by the patient during such attempts. Suitable exploratory laparotomy, revealed a sigmoid volvulus with impacted fecal impaction, and joined fistulized for volvo distal jejunum.

Abdominal X-ray in Pediatric Acute Severe Colitis and Radiographic Predictors of Response to Intravenous Steroids.

BACKGROUND: Abdominal x-ray (AXR) can identify complications in acute severe colitis (ASC) and may assist in selecting high-risk children for early aggressive treatment. We aimed to describe AXR findings in pediatric ASC and to explore radiological predictors of response to intravenous corticosteroid (IVCS) therapy. METHODS: A total of 56 children with ASC were included in a multicenter, retrospective 1-year cohort study (41% boys, mean age 12.1 ±â€Š4.2). Radiographs of responders to IVCS and those requiring second-line salvage therapy by discharge were analyzed independently by 2 blinded radiologists. RESULTS: A total of 33 responders to IVCS were compared with 23 nonresponders. The day-3 Pediatric Ulcerative Colitis Activity Index (PUCAI) score was significantly higher in nonresponders (63 ±â€Š16 vs 46 ±â€Š21, P = 0.001). The mean transverse colon luminal diameter was 30 ±â€Š16 mm in responders and 38 ±â€Š16 mm in nonresponders (P = 0.94). The upper range of transverse colonic diameter in children <12 years was ∼40 mm, whereas in older children it was 60 mm as accepted in adults. Ulcerations and megacolon seen on AXR were associated with nonresponse to IVCS (P = 0.006 and 0.064, respectively). CONCLUSIONS: The presence of mucosal ulcerations and megacolon on AXR could be considered in the risk stratification of children with ASC for early aggressive treatment, together with the previously known day-3 and day-5 Pediatric Ulcerative Colitis Activity Index scores, albumin, and C-reactive protein.