In this prospective cohort study, we evaluated features of \"adult-onset megacolon with focal hypoganglionosis.\"
We assessed the radiologic, endoscopic, and histopathologic phenotyping and treatment outcomes of 29 patients between 2017 and 2020. Data from community controls, consisting of 19,948 adults undergoing health screenings, were analyzed to identify risk factors. Experts reviewed clinical features and pathological specimens according to the London Classification for gastrointestinal neuromuscular pathology.
The median age of the patients with adult-onset megacolon with focal hypoganglionosis at symptom onset was 59 years (range, 32.0-74.9 years), with mean symptom onset only 1 year before diagnosis. All patients had focal stenotic regions with proximal bowel dilatation (mean diameter, 78.8 mm; 95% confidence interval [CI], 72-86). The comparison with community controls showed no obvious risk factors. Ten patients underwent surgery, and all exhibited significant hypoganglionosis: 5.4 myenteric ganglion cells/cm (interquartile range [IQR], 3.7-16.4) in the stenotic regions compared to 278 cells/cm (IQR, 190-338) in the proximal and 95 cells/cm (IQR, 45-213) in the distal colon. Hypoganglionosis was associated with CD3+ T cells along the myenteric plexus. Colectomy was associated with significant symptom improvement compared to medical treatment [change in the Global Bowel Satisfaction score, -5.4 points (surgery) vs. -0.3 points (medical treatment); p < 0.001].
Adult-onset megacolon with focal hypoganglionosis has distinct features characterized by hypoganglionosis due to inflammation. Bowel resection appears to benefit these patients.

UNASSIGNED: To explore the treatments and short-term effects of different types of adult Hirschsprung\'s disease.
UNASSIGNED: 89 patients treated in Shanghai Changhai Hospital were retrospectively analyzed. According to the patient\'s medical history, clinical manifestations, auxiliary examination and postoperative pathological results, the patients were divided into adult congenital megacolon, adult idiopathic megacolon, ganglion cell deficiency (types I and II), toxic megacolon and iatrogenic megacolon, The Treatment methods and short-term prognosis of patients in each group were summarized.
UNASSIGNED: 41 cases of Hirschsprung\'s disease in adults and low anterior resection or pull-out low anterior resection was performed, and 35 patients with idiopathic Megacolon were treated with one-stage subtotal colon resection under the condition of adequate preoperative preparation. Some patients admitted for emergency intestinal obstruction received conservative treatment first or underwent elective surgery after colonoscopic decompression was improved; two patients with ganglion cell deficiency subtotal colectomy were performed to remove the dilated proximal bowel segment and the narrow distal bowel segment; three patients with toxic Hirschsprung\'s disease underwent colostomy in mild cases, while subtotal colorectal resection was required in severe cases; Iatrogenic megacolon was diagnosed in eight cases and the optimum operation should be selected according to the specific conditions of patients.
UNASSIGNED: Adult Hirschsprung\'s diseases were divided into adult congenital hirschsprung\'s disease, idiopathic Hirschsprung\'s disease, ganglion cell deficiency, toxic hirschsprung\'s disease, and iatrogenic Hirschsprung\'s disease. Different types of surgical treatments for Hirschsprung\'s disease in adults should be selected according to the specific diagnosis. All patients with adult Hirschsprung\'s diseases have good short-term outcomes after surgical treatment.

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.
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.
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.
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.

Introduction: Hirschsprung\'s Disease (HD) is a motor disorder of the gut caused by the failure of neural crest cells to migrate craniocaudally into the bowel during intestinal development, resulting in a functional obstruction. The majority of patients with HD are diagnosed in the neonatal period when they present with symptoms of distal intestinal obstruction. Aim: This study aims to identify the clinic-pathological characteristic of HD patients in our institution in KSA and comparing it with local and international data. Materials and Methods: This retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC), a tertiary care center in Riyadh, Kingdome of Saudi Arabia (KSA). Results: A total of 54 patients (72% male) were diagnosed with HD. Forty-eight patients (89%) were born at term, and 6 were pre-term. Sixty-three percent of the patients presented in the neonatal period. Twenty-two patients (41%) underwent one-stage endorectal pull-through procedure, 23 patients (43%) two-stage endorectal pull-through, and 9 patients (16%) had three-stage endorectal pull-through. Five out of 54 patients had ganglion cells seen on FS but were absent in the permanent section. Therefore, the concordance rate was 90.8%. Conclusion: FS biopsy is a necessary method to determine the level of aganglionosis intraoperatively in HD, but the definitive diagnosis should be with permanent section. Also, the choice of surgical operation type (single-stage or multi-stage pull-through) depends on the patient\'s clinical condition.

OBJECTIVE: To describe the clinical findings, management and outcome of colonic impaction in dogs and report the effectiveness of medical treatment.
METHODS: Case records of 58 dogs with colonic impaction were reviewed. Telephone contact with the owners was used to obtain long-term outcome.
RESULTS: Twenty-nine dogs (50%) were mixed-breed, and 45 (78%) were entire males. Median age at presentation was 7 years, and median bodyweight was 22 kg. The degree of radiographic colonic distension did not appear to be related to long-term outcome. Fifty-five dogs (95%) received medical treatment including enemas alone, hyperosmotic and/or bulk-forming and/or lubricant laxatives, enemas combined with laxatives or enemas and/or laxatives combined with manual evacuation of faecal material under anaesthesia. Median survival time of the 58 dogs was 2 years. Overall, 36 of 41 dogs with available long-term follow-up had a favourable outcome.
CONCLUSIONS: Medical treatment of colonic impaction in dogs with a single agent or a combination of agents has a high success rate. Marked colonic dilation is not necessarily indicative of megacolon in dogs.

BACKGROUND: Researches on Chagas disease still use several animals and rats, due to size and susceptibility were preferred by many authors.
OBJECTIVE: To develop an experimental model of megacolon in rats inoculated with the strain Y of Trypanosoma cruzi.
METHODS: Thirty male Wistar rats were distributed in three groups inoculated with different inoculants: Group A: 600000, Group B: 1000000 and Group C: 1500000 blood trypomastigotes of T. cruzi. Animals were sedated intramuscularly at zero inoculation time (T0) and 60 days after inoculation (T60), to perform the barium enema in order to evaluate the dilatation of the different segments of colon in a comparative study of the measurements obtained, using a digital caliper. Evidence of infection was performed by blood smear collected from the animal\'s tail 18 days after inoculation with observation of blood forms.
RESULTS: Comparing the intestinal diameter of the inoculated animals with 60,0000 trypomastigotes in the T0 of infection with T60 days after the inoculation, significant dilatation was observed between the proximal, medial and distal segments (p<0.01), indicating the establishment of the megacolon model. In addition, comparing intestinal diameter between the different segments, with in the T0 of infection and the T60 after inoculation, significant alterations were observed (p<0.05).
CONCLUSIONS: The proposed model was possible for in vivo studies of alterations due to infection by T. cruzi and functional alterations of the colon. In addition, the changes manifested in the colon are not directly proportional to the size of the inoculum, but to the time of infection that the animals were submitted, since the animals inoculated with 60,0000 blood forms were the ones which presented the most significant alterations.

BACKGROUND: Chronic intestinal idiopathic pseudo-obstruction (idiopathic CIPO) is a rare heterogeneous condition for which the different phenotypes are difficult to be established. Oesophageal motility has shown to be impaired in patients with idiopathic CIPO at traditional manometry, whereas no studies have assessed it by high resolution manometry (HRM).
OBJECTIVE: To evaluate oesophageal motility by HRM in patients with idiopathic CIPO.
METHODS: 14 patients with idiopathic CIPO underwent oesophageal HRM. Multiple rapid swallows (MRS) were performed in order to evaluate contraction reserve. The Chicago Classification 3.0 was used to classify the oesophageal motility disorders.
RESULTS: One idiopathic CIPO patient had type-II achalasia, one aperistalsis and 12 had minor disorder of peristalsis (11 ineffective oesophageal motility and one fragmented peristalsis). These minor disorders were not significantly different from those of 50 other consecutive patients who underwent HRM for dysphagia or GERD and received the diagnosis of ineffective oesophageal motility. Three of the 12 idiopathic CIPO patients with minor disorder of peristalsis had no contraction reserve after MRS.
CONCLUSIONS: HRM is able to identify different grades of oesophageal motor impairment in patients with idiopathic CIPO. Presence of major oesophageal dismotility or absent contraction reserve suggest a more severe and widespread motor disorder.

Chronic constipation (CC) and idiopathic megacolon (IMC) occur frequently in cats. The aim of the study was to investigate the effects of a multi-strain probiotic (SLAB51™) in constipated cats (n=7) and in patients with megacolon and constipation (n=3). Ten pet cats with a diagnosis of chronic constipation, non-responsive to medical management received orally 2×1011 bacteria daily for 90 days. For microbiota analysis, selected bacterial groups were analysed by qPCR. Histological samples in megacolons were evaluated for interstitial cells of Cajal (ICC), enteric neurons, and neuronal apoptosis. Biopsies were compared at baseline (T0) and after the end of treatment (T1), and with those obtained from healthy control tissues (archived material from five healthy cats). Constipated cats displayed significantly lower ICC, and cats with idiopathic megacolon had significantly more apoptotic enteric neurons than controls. After treatment with SLAB51™, significant decreases were observed for feline chronic enteropathy activity index (FCEAI) (P=0.006), faecal consistency score, and mucosal histology scores (P<0.001). In contrast, a significant increase of ICC was observed after probiotic therapy. Lactobacillus spp. and Bacteroidetes were increased significantly after treatment (comparing constipated cats before and after treatment, and control healthy cats to constipated cats after treatment), but no other differences in microbiota were found between healthy controls and constipated cats. Treatment with SLAB51™ in cats with chronic constipation and idiopathic megacolon showed significant clinical improvement after treatment, and histological parameters suggest a potential anti-inflammatory effect of SLAB51™, associated with a reduction of mucosal infiltration, and restoration of the number of interstitial cells of Cajal.

Hirschsprung\'s disease (HD) is an important colon disease in children. The aim of this study is to describe the epidemiological features of HD in Taiwanese children.
We conducted a study from the Taiwan National Health Insurance Research Database and analyzed cases who received surgical intervention between 1998 and 2010 due to HD (International Classification of Diseases, 9(th) Revision, Clinical Modification 751.3) or megacolon (International Classification of Diseases, 9(th) Revision, Clinical Modification 564.7). The incidence, sex ratio, age at the surgical intervention, associated complication, and medical expenditures were analyzed.
There were a total of 629 HD cases, including 458 boys and 171 girls, with an overall incidence of 2.2 per 10,000 live births. The male-to-female incidence ratio was 2.38. There was no secular trend of incidence across the years. Seventy-two percent of cases received surgical treatment before the age of 1 year. The younger cases had higher operation-related medical expenditures. Those patients with preoperative enterocolitis (EC) had a higher possibility of postoperative EC than those patients without preoperative EC (34.6% vs. 24.3%, p = 0.013). There were 169 (26.9%) HD cases with additional anomalies, the most common being gastrointestinal and circulatory system anomalies. Of these, 12 (1.9%) cases were Down syndrome.
The incidence of HD in Taiwanese children, a majority Chinese population, was one per 4545 live births with a male predominance. Preoperative EC was a significant factor that was associated with postoperative EC. The percentage associated with Down syndrome was relatively low, probably due to a prenatal screening program.

Chagasic megacolon is accompanied by extensive myenteric and, simultaneously, moderate submucosal neuron loss. Here, we examined changes of the innervation pattern of the lamina propria (LP) and muscularis mucosae (MM). Two alternating sets of cryosections were taken from seven non-chagasic colonic and seven chagasic megacolonic specimens (the latter included both the dilated megacolonic and the non-dilated transitional oral and anal zones) and were immunohistochemically triple-stained for smooth-muscle actin (SMA), synaptophysin (SYN) and glial acid protein S100 and, alternatively, for SMA, vasoactive intestinal peptide (VIP) and somatostatin (SOM). Subsequent image analysis and statistical evaluation of nervous tissue profile areas revealed that, in LP, the most extreme differences (i.e. increase in thickness or decrease in nerve, glia and muscle tissue profile area, respectively) compared with control values occurred in the dilated megacolonic zone itself. In contrast, the most extreme differences in the MM were in the anal-to-megacolonic zone (except the profile area of muscle tissue, which was lowest in the megacolonic zone). This parallels our previous results in the external muscle coat. A partial and selective survival of VIP-immunoreactive in contrast to SOM-immunoreactive nerve fibres was observed in both mucosal layers investigated. Thus, VIPergic nerve elements might be crucial for the maintenance of the mucosal barrier. The differential changes of neural tissue parameters in LP and MM might reflect a multifactorial rather than a pure neurogenic development of megacolon in chronic Chagas\' disease.