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.

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.

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.

Chagasic megacolon: enteric neurons and related structures.

Megacolon, the irreversible dilation of a colonic segment, is a structural sign associated with various gastrointestinal disorders. In its hereditary, secondary form (e.g. in Hirschsprung's disease), dilation occurs in an originally healthy colonic segment due to an anally located, aganglionic zone. In contrast, in chronic Chagas' disease, the dilated segment itself displays pathohistological changes, and the earliest and most prominent being found was massive loss of myenteric neurons. This neuron loss was partial and selective, i.e. some neurons containing neuronal nitric oxide synthase and/or vasoactive intestinal peptide (VIP) were spared from neuron death. This disproportionate survival of inhibitory neurons, however, did not completely correlate with the calibre change along the surgically removed, megacolonic segments. A better correlation was observed as to potentially contractile muscle tissue elements and the interstitial cells of Cajal. Therefore, the decreased densities of α-smooth muscle actin- and c-kit-immunoreactive profiles were estimated along resected megacolonic segments. Their lowest values were observed in the megacolonic zones itself, whereas less pronounced decreases were found in the non-dilated, transitional zones (oral and anal to dilation). In contrast to the myenteric plexus, the submucosal plexus displayed only a moderate neuron loss. Neurons co-immunoreactive for VIP and calretinin survived disproportionately. As a consequence, these neurons may have contributed to maintain the epithelial barrier and allowed the chagasic patients to survive for decades, despite their severe disturbance of colonic motility. Due to its neuroprotective and neuroeffectory functions, VIP may play a key role in the development and duration of chagasic megacolon.

Could the complications of megacolon be avoided by monitoring the risk patients? cases report.

We report 2 cases of megacolon associated with cerebrovascular accident and neuropsychiatric drug consumption. Case report 1: a 75-year-old woman with diabetes mellitus, hypertension, tachycardia with atrial fibrillation, bilateral pleural effusions and previous cerebral hemorrhage was admitted in our hospital. She presented clouded sensorium and abdominal distension, with closed alvus. The CT scan showed a distension of the colon, with severe fecal impaction. A volvulus of the sigma was found at surgical intervention.Case report 2: a 59-year-old man with a medical history of oligophrenia was admitted to our hospital for acute abdomen.He presented stupor and closed alvus with abdominal distension. The abdominal CT scan showed a dolichosigma, with fecal impaction. The patient was submitted to a laparotomy and a two millimetres perforation of the sigma was found.The sigma had a diameter of 28 cm and a length of 75 cm.Even if a clear correlation has not been found yet, anomalies of the regulation of the gastro-intestinal motility can occur at different levels in patients with psychiatric or cerebrovascular diseases and drug consumption with anticholinergic properties,and they should be carefully monitored. The purpose is an early diagnosis of colon function anomalies in order to avoid potentially fatal complications.

Megacolon diagnosis in pregnancy: A case report and literature review.

Megacolon is a rare clinical condition consisting of an abnormally dilated colon in the absence of mechanical obstruction. Megacolon can complicate pregnancy in terms of maternal morbidity and mortality (volvulus, ileus, systemic toxicity, bowel perforation, sepsis) and obstetrical outcomes (preterm birth, premature rupture of membranes, dystocia). Pregnancy, on the other hand, can exacerbate chronic constipation through hormonal and mechanical mechanisms. A case of megacolon, first detected during pregnancy in an otherwise healthy nulliparous woman, is reported. The diagnosis was suspected on observation of a pelvic mass of unknown aetiology (mean diameter > 10 cm) constricting and dislocating the gravid uterus contralaterally during a routine mid-trimester fetal ultrasound. The diagnostic work-up and management are discussed. Chronic constipation in women of reproductive age should receive greater clinical attention during pre- and periconception care. A multi-disciplinary approach, timely diagnosis and delivery planning are fundamental to ensure favourable outcomes for both the mother and fetus when dealing with megacolon during pregnancy.

Multiple endocrine neoplasia type 2B diagnosed after small intestinal volvulus with progressive megacolon in an adolescent.

Multiple endocrine neoplasia type 2B is a rare autosomal dominant disease characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, Marfan-like fatigue, a peculiar face with thickening of the lips, mucosal neuromas on the lips and tongue, and gastrointestinal phenomena. Most patients harbor pathological variants of the RET gene. Herein, we present the first case of a 14 year-old boy who experienced small intestinal volvulus along with a megacolon, and he was diagnosed with multiple endocrine neoplasia type 2B. The patient complained of constipation since he was 2 years old and slowly progressive abdominal distension at school age. At 14 years of age, he presented with remarkable megacolon mimicking Hirschsprung's disease and complicated with small intestinal volvulus. The volvulus was successfully repaired, and the particularly dilated transverse colon was resected following a rectal biopsy. Histopathological evaluation of the resected transverse colon revealed to be compatible with ganglioneuromatosis. After emergency surgery, the patient was diagnosed with multiple endocrine neoplasia type 2B with medullary thyroid carcinoma, and a de novo variant of RET was confirmed. Gastroenterologists should consider it when treating patients with constipation, especially those with megacolon. Therefore, timely diagnosis may lead to appropriate treatment of medullary thyroid carcinoma and improve mortality.

[Plication of diaphragm for eventration of diaphragm caused by megacolon; report of a case].

A 70-year-old woman with severe respiratory distress was admitted to our hospital by ambulance. Chest X-ray revealed marked elevation of left diaphragm and invagination of megacolon. Colectomy was performed to improve the respiratory distress, but continuous mechanical ventilation was necessary after operation due to hypoxia and hypercapnea. Therefore, plication of diaphragm was additionally performed. With 10 rows of nonabsorbable sutures, 6 to 8 pleats were formed. The following day of operation, she was successfully relieved from the ventilator.

[Anesthetic management of a patient with diaphragmatic relaxation and megacolon].

We managed an 87-year-old man with diaphragmatic relaxation under general anesthesia. He had dyspnea and severe constipation. The chest X-ray revealed that two thirds of the left chest cavity were compressed by the megacolon gas. The Spo2 before the operation was 93%. The colon gas was deflated before and after the induction of anesthesia. There was no significant improvement in the tidal volume and the arterial oxygen tension. The postoperative chest X-ray showed that the shift of the left diaphragm was improved. He was able to walk 100 meters and the severe constipation disappeared after the operation.

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.

Preponderance of inhibitory versus excitatory intramuscular nerve fibres in human chagasic megacolon.

INTRODUCTION: Megacolon, chronic dilation of a colonic segment, is a frequent sign of Chagas disease. It is accompanied by an extensive neuron loss which, as shown recently, results in a partial, selective survival of nitrergic myenteric neurons. Here, we focused on the balance of intramuscular excitatory (choline acetyltransferase [ChAT]-immunoreactive) and inhibitory (neuronal nitric oxide synthase [NOS]- as well as vasoactive intestinal peptide [VIP]-immunoreactive) nerve fibres. MATERIALS AND METHODS: From surgically removed megacolonic segments of seven patients, three sets of cryosections (from non-dilated oral, megacolonic and non-dilated anal parts) were immunhistochemically triple-stained for ChAT, NOS and VIP. Separate area measurements of nerve profiles within the circular and longitudinal muscle layers, respectively, were compared with those of seven non-chagasic control patients. Additionally, wholemounts from the same regions were stained for NOS, VIP and neurofilaments (NF). RESULTS: The intramuscular nerve fibre density was significantly reduced in all three chagasic segments. The proportions of inhibitory (NOS only, VIP only, or NOS/VIP-coimmunoreactive) intramuscular nerves were 68 %/58 % (circular/longitudinal muscle, respectively) in the controls and increased to 75 %/69 % (oral parts), 84 %/76 % (megacolonic) and 87 %/94 % (anal) in chagasic specimens. In the myenteric plexus, NF-positive neurons co-staining for NOS and VIP also increased proportionally. The almost complete lack of dendritic structures in ganglia of chagasic specimens hampered morphological identification. DISCUSSION AND CONCLUSION: We suggest that preponderance of inhibitory, intramuscular nerve fibres may be one factor explaining the chronic dilation. Since the nerve fibre imbalance is most pronounced in the anal, non-dilated segment, other components of the motor apparatus (musculature, interstitial cells, submucosal neurons) have to be considered.

[Emergency anesthesia in a woman with mitochondrial neurogastrointestinal encephalopathy]. / Anestesia de urgencia en paciente adulto con síndrome de MNGIE.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is characterized by leukoencephalopathy, peripheral neuropathy, ptosis, ophthalmoplegia, and gastrointestinal dysmotility. Mitochondrial myopathies are rare diseases and little is known of how to manage them when the patient requires anesthesia. We describe the anesthetic procedure used during emergency surgery for megacolon in a 26-year-old woman with MNGIE. Variables monitored were electrocardiogram, invasive arterial pressure, oxygen saturation by pulse oximetry, end-tidal carbon dioxide pressure, neuromuscular block, and depth of anesthesia (entropy). Rapid sequence induction was accomplished with midazolam, fentanyl, propofol, and rocuronium as an alternative to succinylcholine. Anesthesia was maintained with intravenous propofol; a second dose of the neuromuscular blocker was not required. No intraoperative problems developed and extubation was possible 2 hours after arrival in the postoperative critical care unit, once we had checked the level of block to confirm that reversion was not required.

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.

Recurrent cytomegalovirus colitis with megacolon in an immunocompetent elderly man.

Gastrointestinal infection with cytomegalovirus (CMV) is uncommon in immunocompetent hosts. The case of a 70-year-old male with CMV colitis, who has no history of chronic inflammatory bowel disease or immunodeficiency is described. Diagnosis was aided by the identification of inclusion bodies that reacted positively for CMV by immunohistochemical testing in biopsy specimens from the colonic mucosa. His hospital course was characterized by poor improvement of his symptoms after the CMV infection was treated with ganciclovir, and the occurrence of megacolon. A repeat colonoscopy with biopsy revealed a recurrence of the CMV infection. Although CMV colitis is common in immunocompromised patients, we believe this is the first case of CMV colitis with megacolon and recurrent CMV infection in an immunocompetent patient. Colitis caused by CMV colitis should be considered in elderly people with diarrhea.

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.

Characterization of the presence and distribution of Foxp3(+) cells in chagasic patients with and without megacolon.

Patients with Chagas's disease in the chronic phase regularly present with the chagasic megacolon. This form is characterized by inflammation, neuronal destruction, and organ dilatation. Chagasic patients with megacolon always present with inflammatory process near the enteric plexuses of the colon, as previously demonstrated. The aim of this study is to characterize the presence and distribution of Foxp3(+) cells in the muscle layers and neuronal plexuses area of the colon from chagasic patients with and without megacolon. Our results demonstrated that chagasic patients without megacolon presented with an increased concentration of Foxp3(+) cells in all colon layers compared with chagasic patients with megacolon and noninfected individuals. These cells were situated mainly near the blood vessels and rarely were associated with the inflammatory foci. We believe that the presence of Foxp3(+) cells may help to control the inflammatory process through the management of lymphocyte migration and, consequently, prevent neuronal destruction and chagasic megacolon development.

Medical management of ulcerative colitis.

Ulcerative colitis (UC) is a chronic and relapsing inflammation limited to the colonic mucosa and always involving the rectum with variable extension towards the cecum. The aim of medical treatment is to induce and maintain clinical remission. In contrast to Crohn's disease for which a 'top-down' or 'early aggressive' therapy is discussed, in UC the concept of a step-up treatment is still valid. This step-up approach includes local or systemic administration of 5-aminosalicylic acid as first-line therapy followed by topical or systemic steroid administration as well as azathioprine, 6-mercaptopurine, cyclosporine, and more recently anti-tumor necrosis factor monoclonal antibodies as options in refractory or chronic active disease. Colectomy may be necessary if medical treatments are unsuccessful or if complications develop. The decision about the individual therapy of UC is dependent on both disease activity and on disease location. Different therapy strategies are applied in ulcerative proctitis, left-sided colitis, pancolitis and fulminant colitis as well as in chronic active disease and maintenance of remission. This overview presents important concepts in the treatment of UC based on the published guidelines.

[Megacolon and arthritis]. / Megacolon és ízületi gyulladás.

In the present report the authors describe four cases with megacolon and arthritis. The etiology of these unique associations is known in only one case. The musculoskeletal pictures belong to the group of seronegative spondyloarthritis. A huge resistance with great scibalas was detected in the left iliac region by physical examination in all cases. Surgical procedures of the colon resulted in complete remission of arthritis in one case, in the others, chronic obstipation with intermittent relapse of arthritis persisted.