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Because segmental dilatation of the colon causes severe constipation, it has to be included in the differential diagnosis of every constipated child. A case of segmental dilatation of the colon involving the rectosigmoid area is presented. The differential diagnosis and the treatment of this rare entity are discussed.
Intestinal neuronal dysplasia (IND) was initially described as a developmental abnormality of the submucous plexus in children. In recent years this abnormality has also been observed in adults with chronic constipation. The aim of this study was a morphometric characterization of this disease. The investigation was performed with 10 adults with IND, compared with 10 healthy control probands. The best diagnostic indicator of IND proved to be the detection of 6-10 giant ganglia with more than 7 nerve cells in 15 biopsy sections. IND is an interesting cause of chronic constipation which can be histologically verified.
BACKGROUND: Colon motility disorders require reliable methods for calculating segmental colonic transit time. This study evaluated bowel transit time by means of a safe, easy, cheap, non-digestive, and non-disintegrating radionuclide-filled capsule that provided accurate and clear images. METHODS: Radionuclide-filled mini-containers (MCs) were prepared from infusion sets by an apparatus used for sealing blood bags or plasmapheresis sets. In vitro stability studies were performed by immersing 5% methylene blue dye-filled MCs in buffers of variable pH and enzymes simulating the conditions in the stomach and the small bowel. Colon transit scintigraphy was performed with MCs filled with iodine 131 (n = 5) and thallium 201 (n = 8) that were placed in a commercially available capsule. RESULTS: By in vitro acid, base, and intestinal enzyme resistance tests, no methylene blue leakage was determined visually and by spectrophotometric analysis. Accurate and clear images were obtained for colon transit study in constipated patients. After excretion of MCs in the feces, abdominal, myocardial, thyroid, and urinary bladder region counts were found to show the same activity as the background. Radionuclide leakage from MCs was not determined in vivo by gamma camera. CONCLUSIONS: This is a suitable, safe, easy, and cheap method to provide accurate and clear images for colon transit study in constipated patients.
Presacral ganglioneuroma in a 70-year-old man with persistent constipation and its features on computed tomography, magnetic resonance, and endoscopic ultrasonography are presented. Barium enema study and laparotomy showed that constipation was caused mainly by extrinsic compression from this tumor. Computed tomography, magnetic resonance, endoscopic ultrasonographic features such as well-defined solid tumor with a cystic component and punctate calcifications may facilitate early diagnosis of this rare tumor.
Desmosis coli is a disturbance of the intramural connective tissue mesh network of the colonic wall which can lead to a hypoperistalsis syndrome with chronic constipation in the absence of any anomaly of the vegetative gut innervation. The condition typically occurs in infants and adolescents; however, as an incomplete form, desmosis coli can also cause chronic constipation in adults, as demonstrated in this case report.
To describe manifestations of acetylcholinesterase (AchE) activity in the bowel of patients presenting with refractory constipation and correlate them with outcome, rectal biopsy specimens (RBS) from 165 patients who presented with refractory constipation between 1988 and 1999 were examined. Age at biopsy ranged from 4 days to 17 years; 45 subjects were excluded because they satisfied diagnostic criteria for Hirschsprung's disease, intestinal neuronal dysplasia, or hypoganglionosis. Thirty-five autopsy subjects were used as controls. All RBS were compared and AchE activity was assessed in the lamina propria (LP), muscularis mucosae (MM), and around the submucosal vessels (V). Variations in AchE distribution were classified as grade I (no AchE-positive nerve fibers in the LP or MM), grade II (some positive fibers in the LP or MM), grade III (moderate positive fibers in the LP or MM), grade IV (many positive fibers in the LP, MM, or V), or grade V (fibrillar, foamy, or amorphous staining for AchE). All grade I (11/120) and V (12/120) subjects achieved normal bowel control with laxatives alone and all grade II subjects (58/120) did with laxatives and enemas. Grade III subjects (34/120) required addition of cisapride. All grade IV subjects (5/120) were unresponsive to conservative management and 4/5 were found to have a megarectum, which was treated surgically. AchE distribution correlated well with eventual outcome and requirement for surgery. AchE distribution could also be used to classify bowel motility disorders, and we suggest the term AchE-positive disease be used to describe them.
Idiopathic chronic constipation (ICC) is one of the most common clinical conditions in children. The pathophysiology is multifactorial and differs from case to case. To investigate the relationship between anorectal motility (ARM) and clinical course in children with ICC, anorectal function was evaluated using fecoflowmetry in nine children aged 2-14 years (mean 6.1). Three were boys and six were girls. Pressure fluctuations in the rectum and anal canal were simultaneously recorded during saline (250-500 ml) infusion into the rectum. The dynamics of defecation were evaluated using recordings of the saline evacuation curve from the rectum in each patient. Seven patients showed periodic contractions of the rectum accompanied (five) or unaccompanied (two) by relaxations of the anal canal during saline infusion. These patients achieved comfortable spontaneous defecation during follow-up periods ranging from 5 to 20 months. The other two exhibited no rectal contractions in spite of relaxations of the anal canal, and did not respond well to long-term medical management. In eight patients segmental fecoflowmetric curves showed a significantly lower flow rate and longer evacuation time than those of controls. Fecoflowmetry is a simple and non-invasive technique for evaluation of the ability to defecate. Disturbances of ARM may play an important role in patients with severe ICC. When evaluating anorectal function in children with chronic constipation, more attention should be paid to ARM and fecodynamics.
The Malone antegrade enema (MACE) and the caecostomy button (CB) are two methods of achieving colonic lavage in constipated children with faecal soiling. We reviewed our experience with the MACE and CB, aiming to compare results, complications, and outcomes. Between June 1998 and August 2002, 37 children (15 boys) underwent MACE and 12 children (9 boys) underwent CB for idiopathic constipation that had failed conventional treatment. Rectal biopsy was ganglionic in all cases. Mean age at surgery was 9.9 years for the MACE patients and 9.8 years for the CB patients. All children are under continuous review, and mean follow-up is 18 months. Statistical analysis of proportions used Fisher's exact test. Soiling stopped completely in 30 children with MACE and in 9 with CB. Occasional soiling is still present in two children with a CB and in one with MACE. One child with a CB had resumed regular bowel activity, and the CB was removed. MACE failed in 5 (14%) patients because of ineffective colonic lavage, and in one patient (3%) the appendix was replaced by a CB because of perforation of the appendicostomy. CB failed in one patient (8%) because of faecal leak around the button; the child was subsequently converted to MACE (P = >0.5). Complications requiring operative intervention were seen in 9 (24%) of the 37 patients who underwent MACE and none of the 12 patients who underwent CB (P = 0.09). The main complication requiring surgical intervention was stoma stenosis (11%). Complications not requiring operative intervention were seen in 7 (19%) patients after MACE and 11 (92%) of the 12 patients who underwent CB (P < 0.001). The MACE and CB procedures are reliable and effective with high success rates. The MACE has a higher incidence of complications requiring operative intervention. Conversely, complications not requiring operative intervention are more frequent with CB. CB is a safe and effective alternative to MACE in children with faecal soiling.
BACKGROUND AND AIMS: The c-kit protooncogene receptor and its ligand stem cell factor regulate the proliferation and survival of germ cells as well as interstitial cells of Cajal (ICCs). Decreased numbers of ICCs and defects in its networks have been reported in the colon of patients with slow transit constipation (STC). However, little information about the c-kit messenger ribonucleic acid (mRNA) and protein expression in the constipated colon is available. The aim of this study was to determine whether the expression of c-kit mRNA and c-kit protein declined in the colon in STC. PATIENTS AND METHODS: The sigmoid colonic samples from 12 patients with STC and from eight age-matched patients with non-obstructed colorectal cancer were used for this study. Expression of c-kit mRNA was detected by reverse transcriptase-polymerase chain reaction (RT-PCR), and expression of c-kit protein was detected by Western blot analysis. RESULTS: Decreased expression of c-kit mRNA was demonstrated in the STC group compared with the control group. The ratio of c-kit and beta-actin was 1.26+/-0.32 in controls and 1.17+/-0.41 in the STC group (U=0.500, P=0.029). c-kit protein expression significantly declined in the STC group. The mean value of optical density was 162.97+/-5.43 in the control group and 96.64+/-8.80 in the STC group (U=0.000, P=0.021). CONCLUSIONS: The data indicate that the expression of c-kit mRNA and c-kit protein significantly decreased in the colon of STC, suggesting that the c-kit signal pathway may play an important role in ICC reduction in STC.
UNLABELLED: A paraneoplastic syndrome is occasionally the first clinical symptom seen with tumours. We report on two children who initially presented with paraneoplastic syndromes due to ganglioneuroblastomas: the first with severe watery diarrhoea caused by a ganglioneuroma producing vasoactive intestinal peptide, the second with non-treatable constipation, caused by ganglioneuroma-produced anti-neuronal nuclear antibodies. CONCLUSION: Either severe diarrhoea or chronic constipation may represent rare paraneoplastic syndromes in ganglioneuroblastomas.
BACKGROUND: When medical therapy fails, slow-transit constipation (STC), a condition seen almost exclusively in women, can be treated surgically. The aim of this study was to describe our results with laparoscopically assisted subtotal colectomy (LASC) for STC. METHODS: Over a 22-month period, four female patients underwent LASC with ileorectal anastomosis for STC at our hospital. The preoperative workup included clinical assessment, evacuation proctography, rectoanal manometry, and colonic transit measurement. Mobilization of the whole colon and ligation and division of colonic mesenteric vessels with preservation of the omentum were achieved laparoscopically. Through a small Pfannenstiel incision, the colon was removed from the abdomen, leaving a 15-cm rectal stump in situ. The rectal stump was fully mobilized posteriorly, a side-to-end ileorectal anastomosis was fashioned, and the rectal stump was fixed onto the presacral fascia with sutures. RESULTS: The operating time ranged from 150 to 260 min and blood loss was minimal. There were no conversions to open surgery. The postoperative course was uneventful in all patients but one, a 47-year old woman who had transient severe abdominal distension, pain, and frequent small-volume bowel movements. All patients were discharged by the 4th to 10th postoperative day. At 9-month follow-up, all of our patients had normal anorectal function with two to four solid bowel movements per day. CONCLUSION: Although it is a technically demanding procedure, laparoscopically assisted colectomy for slow-transit constipation, can be achieved safely.
Anterior displacement of the anus (ADA), otherwise known as "anterior ectopic anus," is considered to be a common congenital abnormality of anorectal region. However, the true incidence of this condition is not well known. We evaluated the incidence of ADA and its relationship to constipation in 357 children (191 boys and 166 girls) who were admitted to our hospital with unrelated disorders. For this purpose, the anal index was defined as the ratio of the scroto-anal distance to the scroto-coccygeal distance in boys, and as the ratio of the fourchette-anal distance to the fourchette-coccygeal distance in girls. An anal index of less than 0.34 in girls and less than 0.46 in boys was determined to be an ADA. The incidence of ADA was 43.4% in girls and 24.6% in boys (P < 0.01). However, the incidence of constipation in children with a normal anal index and those with a low anal index indicative of an ADA was not significantly different. These findings suggest that ADA is a common variant of the normal anatomical location of the anus, especially in girls.
Laparoscopic surgery has had a remarkable impact on the practice of colorectal surgery. However, most operations are performed using a technique of laparoscopic assistance, whereby extracorporeal bowel division and anastomosis are made following laparoscopic mobilization of the bowel. To our knowledge, this is the first report to describe a case of chronic constipation managed by total colectomy with ileorectal anastomosis, performed completely laparoscopically. The diagnosis of slow transit constipation was made by a transit time study. After dissection of the entire colon, the colon to be resected was delivered through the open rectal stump and brought out transanally. The anvil of an intraluminal circular stapler was passed through the rectum into the peritoneal cavity and the end of the open distal rectum was closed with a linear cutting stapler. The anvil of the circular stapler was inserted into the end of the open terminal ileum and fixed with an Endo-Loop, following which an intracorporeal double-stapling anastomosis was performed. By 3 months following surgery, the patient was passing 3-4 stools a day. Thus, we highly recommend this technique as it eliminates the need for a small incision to deliver the resected colon, thereby minimizing the operative time and risk of wound infection.
Chronic constipation is one of the more frequent functional gastrointestinal symptoms. The more severe cases not infrequently undergo a major surgical approach. However, there is still a need for a better diagnostic and therapeutic approach, since the results are still unpredictable, probably due to a poor selection of candidates. This article focuses on the current diagnostic approach to chronic constipation, with an emphasis on the possible role of colonic manometry to help better choose those patients that may benefit from surgery.
BACKGROUND: Fibre is often recommended as the first-choice treatment but its effects can be uneven. The aim of the study was to compare the clinical efficacy and tolerability of fibre versus lactulose in outpatients with chronic constipation. METHODS: In a prospective randomized crossover trial, patients were randomized to receive fibre or lactulose for four weeks. Between treatments, patients had at least one week free of laxatives. RESULTS: 50 patients, of median age 50 years (range, 18-85) were recruited and 39 patients completed the trial. Compared to fibre, lactulose resulted in significantly higher mean bowel frequency (7.3, 95% CI 5.7 to 8.9 vs. 5.5, 95% CI 4.4 to 6.5; p=0.001) and stool consistency score (3.4, 95% CI 3.1 to 3.7 vs. 2.9, 95% CI 2.5 to 3.3; p=0.018). Scores for ease of evacuation were similar. The frequencies of adverse effects were not significantly different, but greater in the lactulose group. Mean patients' recorded improvement score was significantly higher after taking lactulose than fibre (6.2, 95% CI 5.5 to 7.0 vs. 4.8, 95% CI 4.0 to 5.9; p=0.017). Of the 39 patients who completed the trial, 24 (61.5%) preferred lactulose and 14 (35.9%) preferred fibre. CONCLUSIONS: Lactulose had better efficacy than fibre for chronic constipation in ambulant patients, although both treatments were equally well tolerated in terms of adverse effects.
The diagnosis of significant rectocele is currently made on the basis of cinedefecographic findings. Clinical examination alone will only allow assessment of the presence but not the significance of a rectocele. Therefore, the aim of this study was to determine if anal manometric findings can predict the significance of a rectocele. All patients with a diagnosis of constipation and rectocele confirmed on cinedefecography between 1992 and 1998 were retrospectively reviewed. Significant rectocele was defined as the presence of three of the following five parameters: rectocele >4 cm in diameter as measured during the evacuatory phase of cinedefecography, rectal and/or vaginal symptoms present for longer than 12 months, persistence of rectal or vaginal symptoms for at least four weeks, despite increased dietary fiber (up to 35 g/day), need for rectal and/or vaginal digitation or perineal support maneuvers for rectal evacuation. Statistical analysis was performed using the Mann-Whitney test and Fisher's exact test. A logistic regression model with stepwise selection was used to determine significant prognostic factors. A total of 305 patients (31 men) with rectocele, with a median age of 68 years (range, 12-89) were identified. Of these, 89 (29.2%) had significant rectoceles. There was no difference in the frequency of significant and non-significant rectoceles with respect to gender or age. However, patients with a significant rectocele compared to those with a non-significant rectocele had higher median first sensation volume (45 vs. 30 ml, p=0.0005), median capacity (160 vs. 120 ml, p<0.0001), and median compliance (10 vs. 8 ml H(2)O/mmHg, p=0.05). Calculations based on a logistic regression model determined that with a first sensation of 100 ml, a capacity of 400 ml, and a compliance of 50 ml/mmHg, the probability of a significant rectocele would be 85%. In conclusion, anal manometric findings may be useful in predicting significant rectocele in constipated patients.
PURPOSE: Severe idiopathic constipation with rectal inertia represents a challenging medical problem that, in extremis, might warrant surgery. We studied a group of patients who have undergone proctocolectomy and ileal pouch-anal anastomosis for this problem. The purpose was to assess the functional success of this procedure and its impact on the social function of the patients. METHODS: Patients with functional, intractable constipation have one motion or less per week and are laxative-dependent. The indication for surgery was based on failure of long-term medical therapy. The selection criteria were normal colonoscopy, normal anal manometry and pudendal nerve latency test, and abnormal transit study and abnormal video proctography. The functional outcome after surgery was assessed by anal manometry and stool frequency. Quality of life was analyzed using the Rand health survey 1.0 consisting of a comprehensive questionnaire used preoperatively and postoperatively with emphasis on physical function, role limitation because of functional/emotional problems, social function, pain, and general health. RESULTS: Fifteen patients (14 females) underwent ileal pouch-anal anastomosis over a seven-year period (1993-1999). The mean age was 41 (range, 25-47) years. All had a temporary defunctioning loop ileostomy fashioned, and there were no anastomotic leaks. Two patients required pouch excision within 18 months because of intractable pelvic pain. The mean stool frequency was eight (range, 3-12) per day at the first follow-up. At the second follow-up, this had improved to five (range, 2-6) per day. The mean resting and squeeze anal pressures preoperatively were 82 cm H2O and 104 mmHg, respectively, and were unchanged after surgery. Significant improvement in lifestyle scores were recorded in the categories of physical function, social function, and pain at the first follow-up and in all categories at the second follow-up (P < 0.05). CONCLUSION: Restorative proctocolectomy is an option in selected patients and leads to progressive improvement in quality of life.
BACKGROUND: The pathophysiological basis of slow transit constipation are scarcely understood. Some recent evidence suggests that increased apoptotic phenomena in the colonic enteric neurons may play a role. However, the best method to assess these phenomena has not been evaluated. AIMS: To compare three different methods to detect enteric neuronal apoptosis in these patients. METHODS: Serial colonic tissue sections obtained in 10 patients with intractable slow transit constipation were evaluated with immunohistochemical methods aimed at evaluating apoptotic phenomena: the formamide-MAb method, the TUNEL, and the caspase-3. RESULTS: The highest yield of apoptotic neurons was obtained by means of the formamide-MAb method, compared to the other two, whereas the lowest yield was observed with the caspase-3. CONCLUSIONS: The formamide-MAb method, which is able to distinguish apoptosis from necrosis and is not influenced by DNA breaks, may prove useful to assess neuronal apoptotic phenomena in the human enteric nervous system. This represents a relevant method to detect enteric neuronal apoptosis.