Adult Megacolon Research Articles

Conservative versus surgical therapy for idiopathic and secondary megacolon or megarectum in adults - a retrospective multicentre controlled study.

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. 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. 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). Surgical treatment may be considered earlier if idiopathic or secondary MC or MR is correctly diagnosed, and conservative treatment has been attempted.

Total collectomy in adult woman with Hirschsprung's Disease: a case report

Hirschsprung's disease (HD) is a congenital anomaly of innervation of the lower intestine, limited to the colon, resulting from partial or total obstruction. The main symptom is the inability of a newborn to evacuate within 48 hours after birth, which can cause intestinal obstruction in the neonate and megacolon in children and adults. Therefore, this study reported the case of a 30-year-old female patient with HD affecting the entire colon, where we opted for surgical treatment. In the present study, it was emphasized the medical need to have a better understanding of the clinical picture of HD, as well as its complications and the importance of early diagnosis. In the report presented, it was discussed a late diagnosis of HD and how it affects the patient's life. It presented the improvement in the quality of life that can be obtained by offering an accurate diagnosis and adequate treatment for the patient.

Etiological analysis and surgical method selection of adult megacolon

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

Effect of subtotal proctocolectomy with modified Duhamel anastomosis on anal function in patients with slow transit constipation complicated with adult megacolon

目的： 探讨慢传输型便秘合并成人巨结肠行结肠全切或次全切除+改良Duhamel吻合术对肛门功能的影响。 方法： 收集2007年12月至2017年12月期间，湖南省人民医院慢传输型便秘合并成人巨结肠行结肠全切或次全切除改良Duhamel吻合术治疗80例患者的临床及随访资料，其中28例行全结肠、直肠上段切除，回肠储袋J-Pouch与直肠下端改良Duhamel吻合术+回肠造口术（结肠全切除术组），52例行结肠次全切除（保留回盲部及部分升结肠）、直肠上段切除，升结肠与直肠下端改良Duhamel吻合术+回肠造口术（结肠次全切除术组）。观察全组患者术前、术后3、6、12、24个月时主观肛门功能（Wexner便秘评分、肛门功能Kirwan分级）和客观肛门功能（直肠肛门测压）。 结果： 两组患者基线资料的比较，差异均无统计学意义（均P>0.05）。两组患者均顺利完成手术，术后无严重并发症的发生。随着时间的推移，两组患者Wexner便秘评分逐渐降低，肛管静息压和肛管最大缩榨压逐渐升高（均P<0.001）。与结肠全切除术组比较，结肠次全切除术组患者的术后6~24个月Wexner评分较低，术后3~6个月的肛门功能Kirwan分级更优，术后3~6个月的肛管静息压和肛管最大缩榨压更强，术后6~24个月的直肠肛门抑制反射阳性率更高，差异均有统计学意义（均P<0.05）。两组间术后12~24个月Kirwan分级、肛管静息压和肛管最大缩榨压的差异均无统计学意义（均P>0.05）。 结论： 慢传输型便秘合并成人巨结肠患者行结肠全切或次全切除+改良Duhamel吻合术患者，自主排粪功能和自主控便功能基本在术后1年得到恢复，其中保留回盲部及部分升结肠的手术患者术后肛门功能总体恢复情况优于结肠全切除患者。.

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

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.

Choice of surgical procedures and control of surgical risks in chronic constipation

Surgery, as one of the methods for the treatment of chronic constipation, is the final choice after the failure of non-surgical treatment with its specific particularity. The history of surgical treatment of chronic constipation is complex and tortuous. How to select operation among many kinds of surgery, and control risk is difficult for clinician. The choice of surgical procedure depends mainly on the patient's conditions, the objective examination basis and the experience of physician teams. Based on the previous reports and the team's experience, this paper discusses the choice of surgical treatment for the following types of chronic constipation: (1) Slow transit constipation: subtotal colorectal resection plus ileorectal anastomosis or ascending colon rectum anastomosis is widely used at present in the domestic, and its efficacy is quite good. (2) Outlet obstructive constipation: surgical treatment needs to be cautious with no consensus, and surgeons must follow the advice of "minimally invasive first" principle. (3) Mixed constipation: there is no clear and unified surgical treatment, while Jinling surgery is a promising way of operation. (4) Adult Hirschsprung's disease: surgery is the only treatment, and removing the stenosis segment, transitional segment and obvious expansion segment is the basic principle, and preventive ileostomy at the same time is also recommended. (5) Adult idiopathic megacolon: subtotal colectomy with ileorectal anastomosis or ascending colon rectum anastomosis is highly recommended. (6) Hypoganglionosis: it is rare, and no consensus has been reached in surgical treatment. How to select the proper timing and mode of operation, and how to control the operation risk are the contents that clinicians must master. With the development of laparoscopic surgical technology, minimally invasive surgery is becoming the main direction of constipation treatment.

Preliminary exploration on accurately preoperative evaluation of colonic lesions in slow transit constipation complicated with adult megacolon

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. 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. 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. 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.

Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults.

Chronic megacolon is a rare disease of the colonic motor function characterized by a permanent increase in colonic diameter. We reviewed electronic medical records of all patients diagnosed with chronic megacolon from 1999 to 2014 at Mayo Clinic. Our aim was to summarize clinical and motility features, including colonic compliance and tone measured by colonic barostat-controlled 10-cm-long infinitely compliant balloon. Colonic compliance curves were compared to healthy control (40) and disease (47) control groups. Among 24 identified patients, the mean maximal colonic diameter on abdominal radiograph was 12.7±0.8cm. The cause of megacolon was idiopathic in 16 of 24 and secondary in 8 of 24. A relatively high prevalence (10/24) of comorbid pelvic floor dyssynergia was identified. At the time of this report, 16 patients had undergone colectomy. In general, megacolon presented high fasting colonic volume at relatively low pressures (16-20mmHg), suggesting high colonic compliance; similarly, volumes at operating pressures that ensured apposition of the balloon to the colonic wall suggested low colonic tone. Median balloon volume at 44mmHg distension was 584mL (IQR 556.5-600) in patients with megacolon compared to 251mL (212-281) in healthy, 240mL (207-286) in functional constipation, and 241mL (210.8-277.5) in diarrhea-predominant irritable bowel syndrome controls. Colon's tonic response to feeding was generally intact, and there was frequently maintained phasic contractile response to feeding. Chronic megacolon is a severe colonic dysmotility, manifesting radiologically with increased colonic diameter; it can be proven by measuring colonic compliance and typically requires colectomy because of failed medical therapy.

Sa1384 Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults

Sa1384 Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults

Possible Involvement of Hyperactive Internal Anal Sphincter in Adult Megacolon

Background: Adult megacolon comprises a heterogeneous group of conditions. This study is a retrospective review of a series of adult megacolon aimed to discriminate a subpopulation of this condition by elucidating shared physiopathology. Methods: Eight adult megacolon patients without Hirschsprung’s disease were treated over 15 years. Their clinical characteristics were evaluated with an epmphasis on manometric studies. Results: There were three females and five males aged 60.9 years on average. Seven of the eight patients had central nervous system disorders including epilepsy (4), Parkinson’s syndrome (2) and others (5). Three patients had hemorrhoids. All of these eight patients suffered from chronic constipation, and six of them had a history of sigmoid volvulus. Manometric study revealed anal Ultra Slow Waves, a manometric finding representing hyper-active internal anal sphincter, in five patients. Conclusion: Anal ultraslow waves and central nervous disorders were highly prevalent in adult megacolon. Hyperactive internal anal sphincter may be responsible for long standing constipation. Persisting hyperactivity of the internal anal sphincter after surgical treatment is a possible explanation for the recurrence of sigmoid volvulus or megacolon in these patients. More attention should be paid for the physiopathology of the anorectum in adult megacolon.

A case of adult megacolon with special clinical manifestations

A case of adult megacolon with special clinical manifestations

Total Laparoscopic Modified Duhamel Operation in Combination With Transanal Endoscopic Microsurgery

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. 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. 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.

Use of antiperistaltic cecoproctostomy in colorectal reconstruction

To explore the feasibility and functional outcome of antiperistaltic cecoproctostomy in colorectal reconstruction. Fifty-six patients who underwent antiperistaltic cecoproctostomy were retrospectively studied. Indications for antiperistaltic cecoproctostomy included slow transit constipation(n=44), synchronous colon cancer or colonic polyps(n=5), acute obstructing left colon carcinoma(n=4), and adult megacolon(n=3). Short-term postoperative complications included wound infections(n=5), 3 lymphatic leakages(n=3), and inflammatory small bowel obstruction(n=1). One month after antiperistaltic cecoproctostomy, the median frequency of daily bowel movement was 4.0(range, 2-6). After a median follow-up of 4 years(range, 1 month to 7 years), the median daily bowel frequency was 2.5(range, 0.5-4.0). Five patients suffered from long-term postoperative complications including small bowel obstruction(n=3), incision hernia(n=1), and mild cecal dilatation(n=1). The mean Wexner incontinence score was 4.2±1.1. Antiperistaltic cecoproctostomy is safe and effective for colorectal reconstruction.

Congenital aganglionic megacolon in Nigerian adults: Two case reports and review of the literature

Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, in infants. Despite modern pediatric practice, with emphasis on early diagnosis, Hirschsprung's disease is seen in adults in regions where perinatal care is limited. We report two cases of Nigerian adults with longstanding, recurrent constipation, getting relieved by laxatives and herbal enemata, and then presented to our Emergency Department with a history of progressive abdominal distention, colicky pain, occasional vomiting, and weight loss. Per rectal examination revealed a gripping sensation in the rectum, 10 cm from the anal verge, with rectal fecal load. Barium enema showed a grossly distended proximal large colon, with high fecal retention, with the transition zone at the middle one-third of the rectum. Due to difficulty in bowel preparation of these patients, emergency laparotomy was done. The first case had a diverting sigmoid colostomy and later had a low anterior resection. The second case had a one-stage procedure. Histology of both the cases showed aganglionosis of the stenotic segment and a normal distal rectum. Both patients had complete resolution of the symptoms, without complications, in a three-year follow-up. The related literatures were reviewed. Hirschsprung's disease should be considered in adults patient presenting with chronic constipation. Low anterior resection of the rectum would be a surgical option for the treatment of short and zonal segment of adult Hirschsprung's disease.

Phenotypic alteration of interstitial cells of Cajal in idiopathic sigmoid megacolon

Interstitial cells of Cajal (ICCs) are detected as a pacemaker of gastrointestinal movement and express c-kit and CD34. Recently, ICCs have implicated pathogenesis in several human diseases presenting gastrointestinal motor dysfunction. This study was performed to clarify the role of ICCs in idiopathic sigmoid megacolon using histological and immunohistochemical examinations. Four adult patients with idiopathic sigmoid megacolon and 11 controls were studied. Histology and immunocytochemistry using NSE, S100, c-kit, and CD34 were performed in conjunction with quantitative analysis using the public domain NIH image program. Little histological change in neuromuscular structures in megacolon was observed. Immunohistochemistry demonstrated remarkable decrease of c-kit expressing ICCs without reduction of CD34 expression in the similar interstitial cell population. This observation was further supported by quantitative assessment using public domain NIH image program. A specific downregulation of c-kit in ICCs may be a cause of idiopathic sigmoid megacolon in adults.

W1642 Laparoscopic and Open Anterior Resection and Low Colorectal Anastomosis for Adult Megacolon: Surgical Outcomes

W1642 Laparoscopic and Open Anterior Resection and Low Colorectal Anastomosis for Adult Megacolon: Surgical Outcomes

Acute and chronic megacolon

Megacolon, defined as dilation of the abdominal colon, may occur acutely or in a chronic form. Acute megacolon that occurs in association with severe inflammation of the colon is known as toxic megacolon, whereas acute megacolon without obvious colonic disease is known as Ogilvie's syndrome. The pathophysiology and management of toxic megacolon, Ogilvie's syndrome, and chronic megacolon in adults differ significantly, and it is critically important to distinguish among these entities. Toxic megacolon is a medical emergency that requires coordinated intensive medical and surgical management. In addition to vigorous resuscitation with fluids, electrolytes, and blood products, medical treatment consists of parenteral corticosteroids, broad-spectrum antibiotics, and close monitoring of the patient. Surgical intervention is required if there is no improvement, or deterioration after 12 to 24 hours of intensive medical management, or if there is evidence of colon perforation. Ogilvie's syndrome usually occurs in hospitalized patients with serious underlying medical or surgical illnesses. Management is directed at preventing ischemia and perforation of the distended colon. Supportive therapy includes nasogastric suction, correction of fluid and electrolyte imbalances, stopping potentially aggravating medications, and decompressing the colon with a rectal tube and positional changes. Intravenous neostigmine is the only pharmacologic agent of proven efficacy; colonoscopic decompression is an alternative in patients who do not respond to neostigmine or who have conditions that contraindicate its use. Daily oral administration of polyethylene glycol electrolyte solutions appears to decrease the relapse rate after initial decompression is achieved. Chronic megacolon in adults represents advanced colon failure that does not respond to pharmacologic stimulation. Goals of therapy are to cleanse the colon, prevent impaction, and minimize stool volume and gas buildup. For patients with disabling symptoms, surgical exclusion of the colon, decompression and antegrade enemas via cecostomy, or subtotal or segmental resection may be palliative.

An assessment of anorectal motility in the management of adult megacolon.

Anorectal motility measurements have been performed on 19 patients with adult megacolon and the results compared with 12 normal subjects. Adult Hirschsprung's disease (4 patients) was recognized by the absence of anal canal inhibition on rectal distension (normal mean inhibition 21.9 +/- 2.3 mmHg). Seven patients with non-Hirschsprung's megacolon (idiopathic) had an elevated anal pressure (mean 90.4 +/- 7.0 mmHg). These patients were treated with repeated anal dilatation. Eight further patients had normal anal canal pressures (mean 33.2 +/- 11.7 mmHg) and were treated with enemas and regular suppositories. Little overall clinical improvement resulted in this group and 1 patient underwent subtotal colectomy. Measurements of anorectal motility have been found to assist in the management of adult megacolon.

Preoperative assessment in cases of adult megacolon suffering from moderate malnutrition

Acquired megacolon is a chronic disease associated with constipation and malnutrition. Surgical treatment may be required for the alleviation of the intestinal symptoms, emphasizing the interest of nutritional assessment in this population. In a prospective study of 33 patients suffering from acquired megacolon and requiring either anterior resection or pull-through operation, standard anthropometric and biochemical measurements as well as the PNI of Buzby and Mullen were preoperatively assessed. Mean age of the population was 49 ± 13 y, with 17 males and 16 females. The combined nutritional score indicated 63.6% of the population were malnourished ( 21 33 ) of the patients, whereas the Prognostic nutritional index (PNI) revealed 3 high-risk cases (9.1%), 9 with moderate risk (27.3%), and reduced risk for all others. Indeed, 39.4% ( 13 33 ) of the patients displayed surgical or septic problems. Chi-square analysis confirmed that both studied criteria were significantly associated with complications ( p < 0.05), as morbidity was restricted predominantly to cases with unfavorable Prognostic nutritional index (PNI) results or clear signs of nutritional deficit. Both PNI and conventional nutritional assessment are valuable tools for the screening of these surgical candidates. Artificial alimentation was not used in this experience, but deserves consideration in selected patients. Nutritional status improved in the late postoperative period, with normalization of bowel function.

Mutations of the RET proto-oncogene in Hirschsprung's disease.

Hirschsprung's disease (HSCR) is a common condition (1 in 5,000 live births) resulting in intestinal obstruction in neonates and megacolon in infants and adults. This disease has been ascribed to the absence of autonomic ganglion cells, which are derived from the neural crest, in the terminal hindgut. Segregation analyses have suggested incompletely penetrant dominant inheritance in familial HSCR. Recently, a gene for HSCR has been mapped to chromosome 10q11.2 (refs 6, 7). No recombination was observed between the disease locus and the locus for the RET proto-oncogene, a protein tyrosine kinase gene expressed in the cells derived from the neural crest. Here we report nonsense and missense mutations in the extracellular domain of RET protein (exons 2, 3, 5 and 6) in six unrelated probands and show that the mutant genotypes segregate with the disease in HSCR families. Mutations of RET have been previously reported in multiple endocrine neoplasia type 2A (MEN 2A). Thus, germ-line mutations of the RET gene may contribute either to developmental anomalies in HSCR or to inherited predisposition to cancer in MEN 2A.