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Background: Idiopathic and secondary megacolon (MC) and megarectum (MR) in adults is associated with persistent bowel dilatation and reduced intestinal motility. Little is known about the optimal treatment of this rare disease. Therefore, we retrospectively analysed long-term data from these patients in 5 community and university hospitals, focusing on conservative versus surgical treatment., Methods: Patient records from 7/2004 to 9/2021 were screened for colorectal diseases with severe constipation and persistent megacolon ≥ 9.0 cm and/or megarectum ≥ 6.5 cm. Follow up-data was collected through telephone interviews and written surveys. ClinicalTrialsgov NCT04340856., Results: Sixty-seven patients with idiopathic (n=10) and secondary (n=57) MC or MR were identified with only 20 of 67 patients (29.9%) correctly diagnosed. Mean/median age was 64/69 (range 18-93) years. Thirty-two patients were treated with laxative regimens, and 35 underwent surgery (colostomy: n=12, segmental resection, or hemicolectomy: n=10, (sub)total colectomy: n= 13) after conservative treatment attempts in 32/35 (91.4%). The mean/median follow-up was 4.2/2.7 (range 0.1-17.0) years. The readmission rate for MC-associated symptoms was significantly higher after conservative treatment than after surgical therapy at 12 (0.84 vs. 0.36 per patient, p=0.036), 24 (1.00 vs. 0.52, p=0.048) and 36 (1.13 vs. 0.58, p=0.047) months, as was the number of patients with persistent laxative dependence (28/32 (87.5%) vs. 19/33 (57.6%); p = 0.007). Therapy-associated adverse events (Clavien-Dindo classification) were documented more often in surgically treated patients (11/35, 31.4%) (p=0.025)., Conclusion: Surgical treatment may be considered earlier if idiopathic or secondary MC or MR is correctly diagnosed, and conservative treatment has been attempted., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

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., (© 2024. The Author(s).)

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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

PURPOSE: Chagasic megacolon occurs frequently in central Brazil. There are several surgical techniques for the treatment of megacolon and no consensus on which one is the best. Our goal is to present a new surgical treatment for chagasic megacolon. METHODS: One hundred forty-seven patients with chagasic megacolon were submitted to surgical treatment between January 1985 and December 1995. Fourteen patients were lost to follow-up because they could not be reached. Patients were evaluated preoperatively and submitted to rectosigmoidectomy with interposition of an isoperistaltic segment of ileum between the descending colon and the rectum. RESULTS: There were three deaths in the postoperative period, two because of complications caused by septic peritonitis and one because of hypovolemic shock. Major complications occurred in 5.4 percent of the cases: four dehiscences at the ileocolic anastomosis and two at the ileorectal anastomosis. Minor complications corresponding to 3.1 percent of the total included two with abdominal wall infection, one with a temporary infection, and one with a cardiac arrhythmia. Four patients (3.1 percent) had recurrence of constipation. CONCLUSION: Rectosigmoidectomy with interposition of an ileal segment is a new therapeutic option for chagasic megacolon that has several advantages in relation to the other techniques because of its ease of execution, lack of complications such as those seen in pull-through procedures, and a low recurrence rate. [ABSTRACT FROM AUTHOR]

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.

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 (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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© 2020 Royal Australasian College of Surgeons.)

Competing Interests: All authors have disclosed no conflicts of interest.

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., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

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 ± 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 (χ 2  = 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%) (χ 2  = 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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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.

Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH., Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung's Disease (ADHD) were collected., Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding., Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (Copyright: © 2019 Permanyer.)

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To report the use of skin staples alone, and in combination with sutures, for closure of gastrointestinal incisions in cats and to describe outcomes., Materials and Methods: Retrospective study of cats that underwent gastrotomy, jejunotomy, jejunal or colonic anastomosis using skin staples alone or in combination with sutures to close the enteric wounds at one referral hospital between 2001 and 2016. Data regarding patient signalment, duration of clinical signs, indication for surgery, diagnosis, haematological and biochemical values, surgical time, procedure, complications and outcome were collected. All the gastrotomies and some of the large intestine incisions were closed in a hybrid technique using two layers (monofilament suture and skin staples). All the small intestine and some of the large intestinal incisions were closed in a single layer using skin staples., Results: Twenty-nine cats were included in the study. Indications for surgery included foreign body (14/29), neoplasia (6/29) and idiopathic megacolon (9/29). Overall, 26 of 29 (~90%) of cases survived to discharge. One cat had postoperative cardiopulmonary arrest, and 2/29 cats were euthanased at the owner's request. There was no evidence of incisional dehiscence in any case., Clinical Significance: Skin staplers are safe, reliable, affordable and effective for closure of gastrointestinal incisions in cats. We found skin staples can be a useful tool in closure of gastrotomies and large intestinal resection and anastomosis procedures in cats., (© 2018 British Small Animal Veterinary Association.)

Objective: The present study describes the medical and surgical management of sigmoid volvulus due to Andean dolicomegacolon in a hospital at an altitude above 3000 m., Material and Methods: A descriptive, observational, crosssectional study of 418 patients diagnosed with sigmoid volvulus; Admitted initially due to intestinal obstruction, in the Hospital of Juliaca Carlos Monge. Puno-Perú, during the period 2008-2012. The data were processed through the SPSS software version 21., Results: A total of 418 patients were enrolled, the mean age was 60 years, range 18-89 years, and the male/female ratio was 3.5/1. Nonsurgical management was done in 64 (15.4%), the treatment used was saline enema 20 cases (31%) and rectal catheter 44 (69%), recurrence was present in 27 patients (45%), who had surgery with primary anastomosis resection, of which the mortality corresponded to 8 patients (30%). Of the 354 patients undergoing emergency surgical management, 325 were submitted to sigmoidectomy with primary anastomosis (92%), while 29 had Hartmann's colostomy (8%), the morbidity for both procedures was 52 cases (14.7%), Mortality for both procedures was 45 cases (12.7%)., Conclusions: In patients with sigmoid volvulus due to Andean megacolon the mean age was 60 years. The 15.4% had non-surgical management, the recurrence rate was 45%, and mortality 30%. Patients with surgical management was 84.7%, from this group; 92% had primary anastomosis resection and 8% Hartmann colostomy, morbidity was 14.7% and mortality was 12.7%.

Patients suffering from chagasic megacolon must have an intact mucosal barrier as they survive this chronic disease for decades. A key structure of the mucosal barrier are epithelial cells. Vasoactive-intestinal-peptide (VIP)-positive nerve fibres are involved in influencing, e.g., epithelial cell proliferation, mucus secretion (e.g., mucin 2 and trefoil factor 3 of goblet cells) and inflammation or autoimmunity, all putative and/or known factors altered in chagasic megacolon. We analyzed qualitatively and quantitatively goblet cells, their specific markers, such as mucin 2 (MUC2) and trefoil factor 3 (TFF3) and enterocytes, the relation of VIP-immunoreactive nerve fibres to the epithelia, the distribution of gelsolin, a protein involved in chronic inflammation processes in the epithelia, and the proliferation rate of epithelial cells by combined 4',6-diamidino-2-phenylindole (DAPI) and phosphohistone-H3 (PHH3) staining. Goblet cells were the dominating epithelial cell type. They accounted for 38.4% of all epithelial cells in controls and changed to 58.9% in the megacolonic parts. In contrast to the overall expression in goblet cells of control epithelia, TFF3 was confined to goblet cells at the base of the crypts whereas MUC2 was found only in luminal goblet cells. Gelsolin-positive goblet cells were predominantly recognized within the controls. Finally, the mean value of mitosis increased from 1.5% within the controls up to 2.6% in the anal parts of the chagasic sepcimens. Taken together, increased cell proliferation, preponderance of goblet cells, differential MUC 2, and TFF 3 expression might all be factors maintaining an intact mucosal barrier within chagasic megacolon.

Ileoanal pouch anastomosis is the procedure of choice for patients with drug refractory ulcerative colitis, indeterminate colitis and familial adenomatous polyposis (FAP). In selected patient groups this procedure is a treatment option for patients with Crohn's disease, hereditary nonpolyposis colorectal cancer (HNPCC), synchronous colorectal cancer and for severe colorectal constipation refractory to conservative drug treatment. The pouch procedure provides the opportunity to avoid a permanent ileostomy. The majority of surgeons prefer the ileal J‑pouch as the construction is the easiest to perform and complications and dysfunction rates are low. Due to functional reasons most pouch surgeons favor a circular stapled ileoanal pouch anastomosis. The more radical proctocolectomy can produce sensory defects in the anal canal with subsequent soiling and incontinence. Studies have shown that even after proctocolectomy residual rectal mucosa was found in the anal canal. Therefore, the functionally important anorectal transitional zone should be preserved if possible. Ulcerative colitis can be "healed" with proctocolectomy; however, pouchitis can still occur in one third of the patients. Patients must be informed about the risk of pouchitis and a multidisciplinary monitoring and treatment strategy must be available. In Crohn's disease the ileoanal pouch survival rate of 80% in the long-term follow-up is surprisingly good despite an increased postoperative complication rate. The anal pouch anastomosis is the standard operation in patients with drug refractory ulcerative colitis, indeterminate colitis and FAP. Synchronous colorectal cancer, HNPCC and severe therapy refractive constipation represent rare indications for proctocolectomy where decisions must be made on an individual basis.

Aim: To evaluate the results of Rehbein procedure with extraperitoneal colorectal anastomosis combined with complex conservative treatment in children with secondary megacolon due to chronic constipation., Material and Methods: We carried out surgical interventions in 78 children aged 7-12 years with chronic colostasis resistant to conservative treatment. All patients underwent clinical, laboratory and instrumental examination. All patients were divided into 3 groups depending on type of surgery: group I - colon resection followed by intraperitoneal colorectal anastomosis (28 patients), group II - open Rehbein procedure (29 patients), group III - laparoscopic Rehbein procedure (21 patients)., Results: Absence of dyspeptic disorders and enterocolitis in remote postoperative period in patients receiving comprehensive treatment enhanced with new additions is the cause of improved outcomes. Type of surgery should be individualized in view of anatomical colon changes and secondary pathological conditions. Some pathological symptoms observed at preoperative period may be recurrent in long-term postoperative period due to wrong selection of surgery. The risk of recurrent chronic constipation is high after colon resection followed by intraperitoneal colorectal anastomosis., Conclusion: Rehbein procedure may be preferred for management of secondary megarectum and megasigma. Laparoscopic technique reduces surgical trauma and requires less postoperative analgesia.

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.

Purpose: To present our experience in patients with chronic idiopathic constipation complicated by megarectosigmoid and fecal incontinence who underwent a primary sigmoidectomy and appendicostomy., Methods: We reviewed eight patients referred to the Colorectal Center from 2014 to 2016 with chronic idiopathic constipation and undergoing a sigmoidectomy and appendicostomy. We analyzed the previous medical treatment, indications for the surgical procedure, and outcomes., Results: Age at operation was 5-19 years. Time with constipation was 4-15 years. All patients received multiple laxatives, mainly polyethylene glycol, and all had severe social problems. Four patients have autism. The indication for surgery was an unsuccessful laxative trial, refusal to continue with rectal enemas or both, and social fear of continued fecal incontinence. Post-operatively, all patients were having daily bowel movements without fecal accidents., Conclusion: Selected patients with chronic idiopathic constipation complicated by megarectosigmoid and fecal incontinence can obtain great benefit from primary sigmoidectomy and appendicostomy.

Background: Primary posterior sagittal anorectoplasty is recommended to repair anorectal malformations with rectoperineal or rectovestibular fistula. The aim of this study was to identify the impact of the presence of megarectum on the relative frequency of complications related to posterior sagittal anorectoplasty., Methods: We performed a cross-sectional retrospective study including patients with anorectal malformation, preoperative rectogram and surgically treated with primary or staged posterior sagittal anorectoplasty. Only complications related to anorectoplasty were analyzed and compared with the presence of megarectum., Results: Thirty patients aged 1 day to 7 years were included, 60% had megarectum. Sixteen patients had primary repair: 6 with megarectum and 10 without megarectum; complications occurred in four of the six with megarectum, 66.7%, and no complication were observed in the 10 patients without megarectum (F p=0.008). Fourteen patients had staged repair and no complications related to posterior sagittal anorectoplasty occurred in these patients., Conclusions: Comprehensive preoperative evaluation in patients with anorectal malformation with rectoperineal or rectovestibular fistula could include a rectogram. Awareness of the presence of megarectum could be useful information in the decision to create a colostomy or perform a primary posterior sagittal anorectoplasty., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Chagas disease is a rare condition but with an increasing incidence. Megacolon is a known sequelae. Surgical management remains the only disease modifying treatment option; with variable long-term success. We highlight an interesting case to emphasize attention to this rare condition as a differential diagnosis in any patient presenting with massive intestinal dilatation.

Objective: To compare outcomes following totally transanal endorectal pull-through (TTERPT) versus pull-through with any form of laparoscopic assistance (LAPT) for infants with uncomplicated Hirschsprung's disease., Design: Systematic review and meta-analysis., Setting: Five hospitals with a paediatric surgical service., Participants: 405 infants with uncomplicated Hirschsprung's disease., Interventions: TTERPT versus LAPT., Primary Outcomes: mortality, postoperative enterocolitis, faecal incontinence, constipation, unplanned laparotomy or stoma formation, and injury to abdominal viscera., Secondary Outcomes: Haemorrhage requiring transfusion of blood products, abscess formation, intestinal obstruction, intestinal ischaemia, enteric fistula formation, urinary incontinence or retention, impotency and duration of procedure., Results: Five eligible studies comprising 405 patients were identified from 2107 studies. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. Operative duration was 50.29 min shorter with TTERPT (95% CI 39.83 to 60.74, p<0.00001). There were no significant differences identified between TTERPT and LAPT for incidence of postoperative enterocolitis (OR=0.78, 95% CI 0.44 to 1.38, p=0.39), faecal incontinence (OR=0.44, 95% CI 0.09 to 2.20, p=0.32) or constipation (OR=0.84, 95% CI 0.32 to 2.17, p=0.71)., Conclusions: This meta-analysis did not find any evidence to suggest a higher rate of enterocolitis, incontinence or constipation following TTERPT compared with LAPT. Further long-term comparative studies and multicentre data pooling are needed to determine whether a purely transanal approach offers any advantages over a laparoscopically assisted approach to rectosigmoid Hirschsprung's disease., Trial Registration Number: PROSPERO registry- CRD42013005698., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background: Migration from Latin American countries has increased the number of cases of chagasic megacolon in Western countries. Megacolon is a late complication of Chagas disease, resulting from irreversible destruction of the intramural intestinal nervous system with extensive loss of neurons, ganglionitis, and myositis at the sites of the myenteric and submucosal plexuses. Several surgical procedures involving partial or total resection of the dilated colon have been proposed for treating chagasic megacolon, but intra-operative evaluation of neuronal degeneration in the residual colon has not been commonly done., Methods: Case report and literature review., Case Report: We describe a case of chagasic megacolon treated successfully with a modified Habr-Gama technique, with the intra-operative examination of frozen sections of the residual segments of the colon for the presence of neurons., Conclusions: The recurrence of constipation after surgical treatment can result from the progression of chagasic neuronal degeneration in the preserved colon, and may be preventable by intra-operative evaluation of whether signs of neuronal degeneration or inflammation are absent in the anastomosed colonic tract.

Objective: To analyze our ten year experience of surgical treatment of children with chronic colonostasis of different origin., Material and Methods: The study covers 353 children aged from 1 day to 17 years with colonic stasis who undergo treatment at the Department of Children's Surgery, Bukovinian State Medical University, Cernăuţi, Ukraine. Study design included case history, physical examination, routine clinical and biochemical laboratory tests, endoscopy (sigmoidoscopy, colonoscopy), X-ray (irrigoscopy, irrigography, other radiopaque techniques) methods, and sphincterometry., Results: Non-surgical treatment was effective in 261 (73.94%) patients. The group of the operated patients consisted of 92 (26.06%) children. The outcome of surgical treatment of Hirschsprung's disease (49 children), dolichosigmoid (29 children), Payr's disease (12 children), and Chilaiditi syndrome in all cases is considered to be satisfactory. The optimal methods of surgical operations are offered according to pathology of colon., Conclusions: One-step Soave-Boley operation is considered to be the method of choice for rectal and rectosigmoid forms of Hirschprung's disease. Transversal colon resection and phrenico-colic ligament dissection in Payr's disease, hepatopexia and right colon fixation in Chilaiditi syndrome are effective treatment approaches.

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., (Celsius.)

The effectiveness of anterior resection for the surgical treatment of Chagasic megacolon and the advantages of laparoscopy for performing colorectal surgery are well known. However, current experience with laparoscopic surgery for Chagasic megacolon is restricted. Moreover, associated long-term results remain poorly analyzed. The aims of the present study were to ascertain the immediate results of laparoscopic anterior resection for the surgical treatment of Chagasic megacolon, to identify risk factors associated with adverse outcomes, and to settle late results. A retrospective review of a prospective database was conducted. Between November 2000 and September 2012, 44 patients with Chagasic megacolon underwent laparoscopic anterior resection with posterior end-to-side low colorectal anastomosis. Fifteen (34.1%) patients were male. Mean age was 51.6 years (31 to 77 y). The mean body mass index (BMI) was 22.9 kg/m (16.9 to 36.7 kg/m). Thirty-four previous abdominal operations had been performed. Mean operative time was 265 minutes (105 to 500 min). Four surgeons operated on all cases. Surgeon's experience with the operation was not associated with surgical time (P=0.36: linear regression). Mean operative time between patients with and without previous abdominal surgery was similar (237.7 vs. 247.5 min: P=0.78). There was no association between BMI and the duration of the operation (P=0.22). Intraoperative complications occurred in 2 (4.5%) cases. Conversion was necessary in 3 (6.8%) cases. There was no association between conversion and previous abdominal surgery (P=0.56) or between conversion and surgeon's experience (P=0.43). However, a significant association (P=0.01) between BMI and conversion was observed. Postoperative complications occurred in 10 (22.7%) cases. Anastomotic-related complications occurred in 4 cases. Two of them required diversion ileostomy. Restoration of transanal evacuation was achieved in all cases. Mean duration of postoperative hospital stay was 9.8 days (4 to 45 d). Of 19 patients with known clinical late follow-up, only 1 (5.3%) reported use of enemas and 5 (26.3%) reported use of laxatives. Thirteen (68.4%) patients reported daily bowel movements. There was no association between postoperative complications and use of laxatives (P=0.57). It was concluded that laparoscopic anterior resection for Chagasic megacolon is safe. Obesity was a risk factor for conversion. Restoration of transanal evacuation after surgical treatment of infectious complications was achieved. Minimally invasive surgery for Chagasic megacolon is associated with satisfactory late intestinal function with no significant constipation relapse.

Objective: To explore the efficacy of different procedures for refractory constipation complicated with megacolon., Methods: Clinical data of 112 patients of refractory constipation complicated with megacolon undergoing surgery in our institute from June 2007 to January 2013 were retrospectively analyzed. Of these 112 patients, the duration of constipation ranged from 4 to 22 years. Seventy-four patients had previous abdominal operations. Surgical procedures: (1)Jinling procedure (subtotal colectomy plus ascending colorectal posterior wall side-to-side anastomosis, n=81), including 24 laparoscopy-assisted procedures, 18 terminal ileostomies. (2)total colectomy plus ileorectal side-to-side anastomosis(n=18). (3)total colectomy plus end ileostomy, and ileorectal posterior wall side-to-side anastomosis 6 months later(n=13). The end ileostomy was reversed 6 months after operation., Results: The successful rate was 100%, and no surgery-related deaths were found. Postoperative complications included early diarrhea (90 cases, 80.4%), anal pain and incomplete evacuation (22 cases, 19.6%), urinary retention within 24-48 h after catheter removal (16 cases, 14.2%), anastomosis bleeding (9 cases, 8.0%), anastomosis leakage (6 cases, 5.4%), and intestinal obstruction (15 cases, 13.4%). Six patients with intestinal obstruction underwent adhesiolysis, and others were managed by conservative therapy. At the postoperative follow-up at 6 months, the Wexner constipation score was significantly reduced (5.8-8.3 vs. 21.4-28.7, P<0.01), and malnutrition improved as well., Conclusion: Surgical intervention results in good efficacy for refractory constipation complicated with megacolon.

A rare case of a severely constipated patient with rectal aganglionosis is herein reported. The patient, who had no megacolon/megarectum, underwent a STARR, i.e., stapled transanal rectal resection, for obstructed defecation, but her symptoms were not relieved. She started suffering from severe chronic proctalgia possibly due to peri-retained staples fibrosis. Intestinal transit times were normal and no megarectum/megacolon was found at barium enema. A diverting sigmoidostomy was then carried out, which was complicated by an early parastomal hernia, which affected stoma emptying. She also had a severe diverting proctitis, causing rectal bleeding, and still complained of both proctalgia and tenesmus. A deep rectal biopsy under anesthesia showed no ganglia in the rectum, whereas ganglia were present and normal in the sigmoid at the stoma site. As she refused a Duhamel procedure, an intersphincteric rectal resection and a refashioning of the stoma was scheduled. This case report shows that a complete assessment of the potential causes of constipation should be carried out prior to any surgical procedure.

Aim: Urgent colectomy for severe Clostridium difficile infection can be associated with increased morbidity and mortality. We aimed to use endoscopic methods for treatment., Method: We describe a technique of placing an intracolonic tube facilitating decompression and direct delivery of vancomycin to the proximal colon along with enemas on a regular and frequent basis that may not be possible with vancomycin enemas alone., Results: Successful resolution of the C. difficile infection and avoidance of surgery., Conclusion: While further long-term evaluation is required, our initial results have shown it to be effective in treating select patients with recalcitrant Clostridium difficile-associated megacolon., (Published 2013. This article is a U.S. Government work and is in the public domain in the USA.)

Introduction: Laparoscopic-assisted colonic resection has been well described for multiple surgical indications and typically requires an abdominal incision for specimen removal that is associated with most of the postoperative pain. We report the total laparoscopic modified Duhamel operation for megacolon in combination with transanal endoscopic microsurgery for transanal specimen retrieval and anastomosis to avoid the additional abdominal extraction incision., Case Description: Two cases are presented: case 1 was a 15-year-old boy who presented with intermittent abdominal distention, pain, and constipation for 3 years' duration and was diagnosed with Hirschsprung disease, and case 2 was a 60-year-old man who presented with repeated attacks of incomplete intestinal obstruction for 2 years' duration and was diagnosed with adult megacolon. They were treated by the total laparoscopic modified Duhamel operation without an abdominal extraction incision in combination with transanal endoscopic microsurgery. The operations were successfully accomplished without conversion to open surgery. The patients tolerated the procedure well, complained of minimal postoperative pain, and did not require narcotics beyond the day of the operation. No surgical complications occurred. Discharge from the hospital occurred on the ninth postoperative day in case 1 and the 13th postoperative day in case 2., Discussion: The total laparoscopic modified Duhamel operation in combination with transanal endoscopic microsurgery is a feasible and minimally invasive technique for idiopathic megacolon and adult megacolon. This advanced surgical technique was developed by combining laparoscopy with the concept of natural orifice transluminal endoscopic surgery.

Dural sac ectasia is a very infrequent anatomical abnormality, usually caused by connective tissue diseases, as Marfan syndrome. Very few cases have been described being a consequence of a previous spine surgical procedure. We describe the case of an elderly patient who should be operated on twice due to sub-occlusive colon disease. Surgery was performed under spinal anaesthesia. A dural sac ectasia was suspected after the first procedure and the abdominal X-ray was reviewed. The characteristics of the anatomical alteration and the course of both anaesthetic procedures were described. X-ray and CT images were provided., (Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.)

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.