Ileo-anal S-Pouch reconstruction in patients with total colonic aganglionosis after failed pull-through procedure

Total colonic aganglionosis (TCA) occurs in 3% to 12% of Hirschsprung’s disease patients. Although numerous surgical techniques have been utilized for the treatment of these patients, little information is available regarding optimal surgical management of their frequent complications or failured procedures. The ileoanal S pouch (IASP) technique has been utilized in the treatment of children with familial adenomatous polyposis and ulcerative colitis. The authors present the results of salvage IASP in 3 TCA patients who had poor results after total colectomy and Soave pull-through.     

Histochemical acetylcholinesterase reactions in total colonic aganglionosis

Reliability of the histochemical diagnosis in total colonic aganglionosis is controversial. We studied histochemical acetylcholinesterase (AChE) reactions in three children with total colonic aganglionosis. In all three, there was an increase in AChE positive nerve fibers in the rectal mucosa, obtained when the patients were over the neonatal period. However, a proximal extension of the increase in AChE positive nerve fibers was noted in the distal end of the resected bowel obtained at the definitive operation in two cases out of three. In one, there was no proximal extension in the resected bowel, and in another, there was an increase in AChE positive nerve fibers in the mucosa of the ganglionic ileum. Our findings indicate that the histochemical study of AChE activity in rectal suction biopsy is useful, even in cases of total colonic aganglionosis.     

Laparoscopic-assisted pull-through for Hirschsprung's disease,a prospective repeated evaluation of functional outcome

Aim of the study

To evaluate the functional outcome of laparoscopic-assisted endorectal pull-through (LAP) for Hirschsprung's disease (HSCR) over time.

Methods

Thirty-five children with HSCR underwent laparoscopic-assisted pull-through at our institution between 1998 and 2009. The diagnosis was histologically confirmed in all cases. Clinical data was extracted from the case records. A prospective assessment of the functional outcome was performed in 2009 and 2012. Exclusion criteria were a follow-up of less than 6 months after treatment (1 case) and total colonic aganglionosis (1 case). An independent examiner, not involved in the clinical care of the patients, performed interviews using a semi-structured questionnaire. Four patients could not be traced for the first interview. Two cases were lost for the second interview. Altogether twenty-seven patients completed the study. Data from the two interviews were compared. The regional ethical review board approved the study.

Main Results

The median patient age was 4 years old (range 2–16) at the time of the first interview and 7 years old (range 5–19) at the time of the second interview . There were 23 males and 4 females in the study group. The median age at laparoscopic-assisted pull-through was 104 days old (range 29 days–8 years). In the first interview 11 patients reported constipation, 18 patients reported soiling more frequently than once per week when they had loose stools and 16 patients when they had solid stools. Laxatives or irrigations were used by 13 of the patients. In the second interview 4 patients reported constipation, 16 patients reported soiling when they had loose stools and 15 patients reported soiling when they had solid stools. Eight patients used laxatives or irrigations. The decrease in constipation was statistically significant (p = 0,023).

Conclusions

Our study shows a statistically significant reduction of constipation over time. There is a high risk of incontinence after laparoscopic-assisted pull-through, with few signs of short-term improvement.     

Motility of the anorectum after “Rectoplasty with posterior triangular colonic flap” in Hirschsprung’s disease

Clinical assessment of fecal continence and anorectal manometry were carried out in 22 patients with Hirschsprung’s disease who had undergone “Rectoplasty with posterior triangular colonic flap”. Satisfactory postoperative continence was achieved in 18 out of these 22 patients. The result of anorectal manometry disclosed that normal rectal compliance (reservoir function of the rectum), together with normal resting pressure of the anorectum and rectoanal reflex, is indispensable for an adequate postoperative continence.     

Effect of intravenous erythromycin on the colonic motility of children and young adults during colonic manometry

Purpose

Erythromycin is successfully used as a gastroduodenal prokinetic agent. Given the limited available treatments for colonic dysmotility, further investigation into erythromycin's effect on colonic motility is warranted. We aimed to study the effect of erythromycin on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders.

Methods

Patients referred for colonic manometry were eligible for enrollment. Fasting motility was recorded for 1 to 2 hours, then erythromycin lactobionate (EL), 3 mg/kg, was administered intravenously, and colonic motility was monitored for 1 to 2 hours after erythromycin. Manometry was then continued per routine. The motility index (MI) of pressure tracings at each pressure transducer was calculated for each patient for a period of 15 and 60 minutes before and after EL infusion. Change in MI was compared by Wilcoxon signed rank test.

Results

Twenty patients were enrolled. The most common indication was constipation with encopresis. Seventy percent of patients had normal colonic manometry, and 30% of patients demonstrated a neuropathy. Average MI for the 60-minute period before and after EL infusion were 254 ± 74 mm Hg/h and 253 ± 94 mm Hg/h, respectively (P = .55). Average MI for the 15-minute period before and after EL infusion were 64 ± 23 mm Hg/15 min and 69 ± 32 mm Hg/15 min, respectively (P = .45).

Conclusions

Administration of intravenous EL resulted in no changes in colonic MI in pediatric patients referred for colonic manometry. Further studies on potential colokinetic agents are warranted in this population of patients.     

Reliability of intraoperative frozen sections in the management of Hirschsprung's disease

Background

The use of a 1-stage pull-through for Hirschsprung’s disease (HD) is dependent on accurate identification of the normally innervated bowel on intraoperative frozen sections (IOFS). The authors wished to determine the incidence and sources of error during this process.

Methods

All HD patients undergoing IOFS over a 15-year period were reviewed.

Results

Three hundred four patients underwent a total of 700 IOFS. In 9 cases (3%), there was discrepancy between IOFS and permanent sections. Two of these were false-positive (ganglion cells incorrectly believed to be present at IOFS); both required a second operation as a result of the error. Seven were false-negative (presence of ganglion cells not recognized at IOFS); none required a subsequent operation, but 2 had a significantly more extensive colonic resection than was necessary. Responsible factors included sampling from the transition zone, freezing artifact, and misinterpretation of ganglion cells in very young patients owing to pathologist inexperience. There was significant variability in the error rate among the 11 pathologists. However, the numbers were too small for statistical analysis to determine whether there was a correlation between the rate of errors and the volume of cases done or years of experience.

Conclusions

Error in reading of IOFS is rare but can have significant repercussions in patient care. Multiple factors, including technical issues and pathologist experience, may have a role in contributing to these errors.     

Hirschsprung's disease discordant in monozygotic twins: a study of possible environmental factors in the production of colonic aganglionosis.

The occurrence of Hirschsprung's disease, with histologically verified colonic aganglionosis, in only 1 of 2 prematurely born and presumed identical (monozygotic) twins is reported. The occurrence of monozygotic twinning was supported by the observed sharing of a single and common placenta and by ABO and HLA identity of peripheral blood erythrocytes and leukocytes. The affected twin was of a slightly lower birth weight and experienced early respiratory distress, necrotizing enterocolotis, and more prolonged umbilical artery catheterization (no encountered in the unaffected twin). This, to the authors' knowledge, is the first reported occurrence of Hirschsprung's disease discordant in monozygotic twins. The literature relating to genetic and environmental factors in clinical and experimental colonic aganglionosis is reviewed and speculation is presented regarding the occurrence of colonic aganglionosis discordant in monozygotic twins as reported here.     

SEMS置入术治疗急性结肠癌性梗阻效果分析

目的：临床分析SEMS置入术治疗急性结肠癌性梗阻的可行性。方法选取2011年1月～2013年12月在我院接受治疗的急性结肠癌性梗阻患者76例,对其临床治疗成功率进行分析。结果76例患者支架成功植入率为94.7％,置入成功患者的梗阻症状全部得到有效缓解,有效缓解率为100％。结论 SEMS置入术治疗急性结肠癌性梗阻效果显著,具有可行性。     

老年结直肠癌并肠梗阻的外科治疗（附112例报告）

目的探讨老年结直肠癌并肠梗阻的外科治疗有关问题。方法回顾性分析1991年1月至2011年1月间112例老年结直肠癌并急性肠梗阻外科处理的临床资料。结果术后痊愈出院109例,死亡3例,围手术期并发症43例（38.4%）。结论老年结直肠癌并急性肠梗阻一经确诊,应积极争取手术,术式选择应根据患者具体病情决定。完善围手术期处理,是降低术后并发症和病死率的关键。     

Chronic idiopathic intestinal pseudo-obstruction caused by acquired visceral neuropathy localised in the left colon: Report of two cases

Two cases of chronic idiopathic intestinal pseudo-obstruction (CIIP) are reported. One is a 51-year-old man, and the other is a 47-year-old woman. Both patients presented with severe constipation and barium enema showed a marked dilation of the right colon, and a narrowing in the left colon. Studies done on the motility of the colon and anorectum revealed normal resting pressure profiles of the anorectom, a normal recto-anal reflex, and a normal resting tone of the collapsed colon. Administration of methacholine chloride, however, provoked large, non-propulsive movements in the collapsed colon, which were inhibited by the administration of atropin sulfate. Histologic examination disclosed a marked decrease in neurons and an increase of Schwann cells in the myenteric plexus of the collapsed colon. CIIP due to acquired visceral neuropathy localised in the left colon, was diagnosed as a result of manometric and histologic findings. One case was cured surgically, by a left hemi-colectomy, and the other was cured medically using choline antagonists and laxatives.     

镍钛记忆网管在膀胱颈功能性梗阻治疗中的应用(附4例报告)

目的 寻找膀胱颈功能性梗阻的理想治疗方法。方法 回顾分析4例镍钛合金记忆网管支撑治疗膀胱颈功能性梗阻的临床资料。结果 3例1次置管成功,1例2次失败后第3次置管成功。随访6～47个月,平均21个月,2例排尿困难症状消失,2例症状明显改善。结论 镍钛记忆网管支撑是治疗膀胱颈功能性梗阻的一种简单、安全、有效的手段。     

Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung’s disease

Background/purpose

Hirschsprung’s disease (HSCR), characterized by the absence of ganglia in the distal colon, results in functional obstruction. Despite surgical resection of the aganglionic segment, around 40% of patients suffer recurrent life threatening Hirschsprung’s-associated enterocolitis (HAEC). The aim of this study was to investigate whether gut microbiota and intestinal immunity changes contribute to the HAEC risk in an HSCR model.

Methods

Mice with neural crest conditional deletion of Endothelin receptor B (EdnrB) and their littermate controls were used (EdnrB-null and EdnrB-het). Bacterial DNA was prepared from cecal contents of P16-18 and P21-24 animals and pyrosequencing employed for microbiome analysis. Ileal tissue was isolated and secretory phospholipase A₂ (sPLA₂) expression and activity determined. Enteroinvasion of Escherichia coli into ileal explants was measured using an ex vivo organ culture system.

Results

EdnrB-het and EdnrB-nulls displayed similar flora, sPLA₂ expression and activity at P16-18. However, by P21-24, EdnrB-hets demonstrated increased Lactobacillus and decreased Bacteroides and Clostridium, while EdnrB-nulls exhibited reciprocal changes. EdnrB-nulls also showed reduced sPLA₂ expression and luminal activity at this stage. Functionally, EdnrB-nulls were more susceptible to enteroinvasion with E. coli ex vivo and released less sPLA₂ than EdnrB-hets.

Conclusions

Initially, EdnrB-het and EdnrB-nulls contain similar cecal flora but then undergo reciprocal changes. EdnrB-nulls display dysbiosis, demonstrate impaired mucosal defense, decreased luminal sPLA₂ and increased enteroinvasion of E. coli just prior to robust colonic inflammation and death. These findings suggest a role for the intestinal microbiome in the development of HAEC.     

Experience in the diagnosis of Hirschsprung's disease using the anomanometer

Results of using electromanometry as the sole preoperative diagnostic test for Hirschsprung's disease are presented. Out of a total of 113 infants and children diagnosed to be suffering from the disease, manometry was the initial diagnostic test in 92 infants and children. In nine, manometry confirmed the diagnosis after a colostomy has been done. In 11, barium enema had diagnosed the condition at a peripheral hospital and manometry confirmed the diagnosis. In one, manometry was interpreted as normal, but laparotomy revealed Hirschsprung's disease. Histologic corroboration has been obtained in 97 instances. In 14, no operative treatment was undertaken and, hence, no histologic examination was made; in 2, histologic examination is still pending.     

Isolated colonic inertia is not usually the cause of chronic constipation

Aim Chronic constipation is classified as outlet obstruction, colonic inertia or both. We aimed to determine the incidence of isolated colonic inertia in chronic constipation and to study symptom pattern in those with prolonged colonic transit time. Methods Chronic constipation patients were classified radiologically by surgeon‐reported defaecating proctography and transit study into four groups: isolated outlet obstruction, isolated colonic inertia, outlet obstruction plus colonic inertia, or normal. Symptom patterns were defined as stool infrequency (twice weekly or less) or frequent unsuccessful evacuations (more than twice weekly). Results Of 541 patients with chronic constipation, 289 (53%) were classified as isolated outlet obstruction, 26 (5%) as isolated colonic inertia, 159 (29%) as outlet obstruction plus colonic inertia and 67 (12%) as normal. Of 448 patients (83%) with outlet obstruction, 35% had additional colonic inertia. Only 14% of those with prolonged colonic transit time had isolated colonic inertia. Frequent unsuccessful evacuations rather than stool infrequency was the commonest symptom pattern in all three disease groups (isolated outlet obstruction 86%, isolated colonic inertia 54% and outlet obstruction plus colonic inertia 63%). Conclusion Isolated colonic inertia is an unusual cause of chronic constipation. Most patients with colonic inertia have associated outlet obstruction. These data question the clinical significance of isolated colonic inertia.     

The research on screening differentially expressed genes in Hirschsprung’s disease by using Microarray

Objective

To study the differential expression of genes between Hirschsprung’s disease (HSCR) and normal tissue by using microarray for exploring the mechanism of HSCR development and establishing the gene expression profiles of HSCR.

Methods

Colon tissues (aganglionic and normal segments) of 4 patients with HSCR were detected by the Agilent SurePrint G3 Human GE 8x60K Microarrays. RT-PCR was used to verify the results of Microarray test. Then, immunohistochemistry was used to demonstrate the expression of HAND2 in the myenteric plexus of the colon from 46 patients with HSCR to further explore the relationship between HAND2 and development of HSCR.

Results

A total of 12,125 meaningful expressed genes were screened out. 4 pairs of specimens had 622 differentially expressed genes, 584 (93.89%) of which were up-regulated while 38(6.11%) were down-regulated. 6 of the 622 genes were tested by RT-PCR, which were consistent with the results detected by Microarray. The average optical density of positive expression of HAND2 in myenteric plexus was compared between the aganglionic, transitional, dilated, normal segments and control group. The average optical density in the aganglionic segments was obviously reduced. Statistical analyzed data showed that it has significant deviation (P<0.01).

Conclusion

1. A set of differentially expressed genes between aganglionic and normal segments of HSCR was obtained. Our data may provide significant information to research the pathogenesis of HSCR. 2. Reduced protein expression of HAND2 in the myenteric plexus of the aganglionic would suggest that HAND2 was involved in the pathogenesis of HSCR.