Diagnosis of Hirschsprung’s Disease: an age‐related approach in children below or above one year

Aim The aim of this study was to evaluate the effectiveness of a differential diagnostic approach to Hirshchsprung’s Disease (HD) on the basis of age. Method Data on 185 consecutive children with suspected HD were subjected to an age‐related diagnostic approach. The patients were divided into two groups according to age (A < 1 year; B > 1 year). Children in Group A had rectal suction biopsy (RSB) and contrast enema (CE), and in Group B anorectal manometry (ARM) was performed. Patients with a normal recto‐anal inhibitory reflex (RAIR) underwent bowel disimpaction and medical treatment. Only selected cases in Group B underwent RSB and CE. Results In Group A (18 patients) CE showed a colonic transitional zone in three patients, whereas RSB led to the diagnosis of HD in nine. In Group B (167 patients) ARM was not possible in seven patients and it was normal in 140 (normal anal sphincter pressure: 83; hypertonia of the internal anal sphincter: 57). The RAIR was negative in 20 patients. RSB performed in 31 children in Group B confirmed HD in three patients. Conclusions For patients with a neonatal onset of constipation RSB is the best diagnostic technique. Chronic constipation is rarely due to HD and ARM is a useful non‐invasive screening tool.     

Crucial role of rectoanal inhibitory reflex in emptying function after anoplasty in infants with anorectal malformations

Constipation is a common problem after reconstructive surgery for anorectal malformations. The underlying pathophysiology of the constipation in these patients is unclear. The objective of this study was to compare manometric disturbance in infants with and without post-anoplasty constipation. Anorectal manometry studies were performed within 12 months of anoplasty, as a part of the follow-up protocol, in 24 infants aged less than 3 years who had anorectal malformations. The manometric profiles studied were mean resting anal pressure (ArP), mean resting rectal pressure (RrP), mean resting rectoanal pressure gradient (RRPG), peak squeeze pressure (PSP), and the presence of the rectoanal inhibitory reflex (RAIR). Eight of 24 infants (33%) experienced constipation during the examination period. There was no difference in pressure profiles between low and non-low anomalies. In the non-constipation group, RrP was 5.1 mmHg, ArP was 21.0 mmHg, RRPG was 16.0 mmHg, and PSP was 88.4 mmHg. In the constipation group, RrP was 7.3 mmHg (p = 0.37), ArP was 37.5 mmHg (p = 0.03), RRPG was 3.05 mmHg (p = 0.05), and PSP was 81.7 mmHg (p = 0.77). RAIR was present in 93.75% of cases without constipation and 12.5% of cases with constipation (p < 0.01). One patient who had clinical conversion from constipation to a good result also showed positive conversion of the RAIR. RAIR and anal resting tone play important roles in emptying function. As far as possible, these functions should be preserved during reconstruction.     

Anorectal sleeve micromanometry for the diagnosis of Hirschsprung's disease in newborns

Background/Purpose

An accurate diagnosis is mandatory for surgery in newborns with Hirschsprung's disease (HD). Acetylcholinesterase staining of rectal suction biopsy specimens is widely performed in the diagnosis of HD, but results are sometimes incorrect or atypical in newborns. We report the usefulness of our method of anorectal manometry using a specially designed sleeve microassembly for the diagnosis of neonatal HD.

Methods

Anorectal manometry was conducted without sedation in 41 newborns, aged 2 to 30 days (19 newborns were within the first week of life), with abdominal distension. A silastic assembly with a 2-cm-long sleeve sensor and 5 side holes arrayed along the sleeve was designed to reduce the effects of displacement of pressure sensors relative to the anal sphincter. Rectoanal inhibitory reflex (RAIR) was examined with rectal balloon distension.

Results

Thirty-two subjects who showed falls of anal sphincter pressure fulfilling the criteria for RAIR were diagnosed to be without HD. Nine patients without an appropriate RAIR were subsequently confirmed to have HD based on operative pathologic findings. Parameters of anal sphincter function did not differ significantly between the subjects with and without RAIR.

Conclusions

An anorectal sleeve micromanometric technique is useful in the diagnostic workup of newborns suspected of having HD.     

Anorectal functions in patients with lumbosacral spina cord injury

OBJECTIVE: To investigate the anorectal status in patients with lumbosacral spinal cord injury (SCI). METHODS: Twenty six patients (23 males, 3 females) with lumbosacral SCI and 13 normal volunteers were enrolled into this study as controls. The median age was 43.7 years (ranging 17-68 years) and the median time of patients since injury was 59.1 months (ranging 8 months-15 years). They were diagnosed as complete lumbosacral SCI (n =2, American Spinal Injury Association (ASIA) score A), or incomplete lumbosacral SCI (n=24, ASIA score B-D) with mixed symptoms of constipation and/or fecal incontinence, and were studied by anorectal manometry. None of the patients had any medical treatments for neurogenic bowel prior to this study. RESULTS: The maximum anal resting pressure in lumbosacral SCI patients group was slightly lower than that in control group (One-way ANOVA: P=0.939). During defecatory maneuvers, 23 of 26 (88.5%) patients with lumbosacral SCI and 1 of 13 (7.7%) in the control group showed pelvic floor dysfunction (PFD) (Fisher's exact test: P<0.0001). Rectoanal inhibitory reflex (RAIR) was identified in both patients with lumbosacral SCI and the controls. The rectal volume for sustained relaxation of the anal sphincter tone in lumbosacral SCI patients group was significantly higher than that in the control group (Independent-Samples t test: P<0.0001). The mean rectal volume to generate the first sensation was 92.7 ml+/-57.1 ml in SCI patients, 41.5 ml+/-13.4 ml in the control group (Independent-Samples t test: P<0.0001). CONCLUSIONS: Most of the patients with lumbosacral SCI show PFD during defecatory maneuvers and their rectal sensation functions are severely damaged. Some patients exhibit abnormal cough reflex. Anorectal manometry may be helpful to find the unidentified supraconal lesions. RAIR may be modulated by central nervous system (CNS).     

Anorectal functions in patients with lumbosacral spinal cord injury

Objective: To investigate the anorectal status in patients with lumbosacral spinal cord injury (SCI). Methods: Twenty six patients (23 males, 3 females) with lumbosacral SCI and 13 normal volunteers were enrolled into this study as controls. The median age was 43.7 years (ranging 17-68 years) and the median time of patients since injury was 59.1 months (ranging 8 months-15 years). They were diagnosed as complete lumbosacral SCI (n=2, American Spinal Injury Association (ASIA) score A), or incomplete lumbosacral SCI (n =24, ASIA score B-D) with mixed symptoms of constipation and/or fecal incontinence, and were studied by anorectal manometry. None of the patients had any medical treatments for neurogenic bowel prior to this study. Results: The maximum anal resting pressure in lumbosacral SCI patients group was slightly lower than that in control group (One-way ANOVA: P =0.939). During defecatory maneuvers, 23 of 26 (88. 5%) patients with lumbosacral SCI and 1 of 13 (7.7%) in the control group showed pelvic floor dysfunction ( PFD) ( Fisher' s exact test: P<0.0001). Rectoanal inhibitory reflex (RAIR) was identified in both patients with lumbosacral SCI and the controls. The rectal volume for sustained relaxation of the anal sphincter tone in lumbosacral SCI patients group was significantly higher than that in the control group (Independent-Samples t test: P <0.0001). The mean rectal volume to generate the first sensation was 92.7 ml±57.1 ml in SCI patients, 41. 5 ml±13. 4 ml in the control group (Independent-Samples t test: P < 0.0001). Conclusions: Most of the patients with lumbosacral SCI show PFD during defecatory maneuvers and their rectal sensation functions are severely damaged. Some patients exhibit abnormal cough reflex. Anorectal manometry may be helpful to find the unidentified supraconal lesions. RAIR may be modulated by central nervous system (CNS).     

直肠肌部分切除在便秘治疗中的探讨

目的：探讨直肠肌部分切除治疗顽固性便秘的适应证，手术方法及疗效。方法：经肛门直肠肌部分切除术，治疗便秘患儿64例。其中先天性短段型巨结肠（HD）36例；HD根治术后便秘复发10例；乙状结肠冗长症7例；特发性便秘11例。结果：随访38例（60％），随访时间最长7．5年，最短2个月，平均26．4个月。疗效满意，能自行排便32例（88％）；欠满意，偶而需要辅助方法排便2例（5．2％）；疗效差，便秘复发4例（10．5％）。结论：本手术方法简单，容易掌握，损伤小，并发症少，效果好。     

The effect of abdominal ventral rectopexy on faecal incontinence and constipation in patients with internal intra‐anal rectal intussusception

Aim Optimal treatment of anal incontinence in a patients with a normal anal sphincter is controversial, as is the role of intra‐anal rectal intussusception in anal incontinence. We evaluated the results of abdominal ventral rectopexy on anal continence in such patients. Method Forty consecutive patients with incontinence and intra‐anal rectal intussusception without a sphincter defect were treated by abdominal ventral mesh rectopexy without sigmoidectomy. The Cleveland Clinic Incontinence Score (CCIS), patient satisfaction and constipation before and after surgery and recurrence were recorded. Results The mean CCI scores were 13.2 (=/?4.25) preoperatively and 3 (±3.44) postoperatively (P<0.0001). Patient assessment was reported as ‘cured’ in 26 (65%), ‘improved’ in 13 (32.5%) and ‘unchanged’ in one (2.5%) patient. Constipation was induced in two (5%) patients and was cured in 13 of 20 (65%) patients who were constipated before surgery. One case of recurrent prolapse occurred after a mean follow‐up of 38 months. Conclusion Intra‐anal rectal intussusception may be associated with anal incontinence. For these patients, abdominal ventral mesh rectopexy appears to be an adequate treatment.     

Use of anorectal manometry for evaluation of postoperative results of patients with anorectal malformation: a study from Kuwait

Purpose

The objective of this study is to use anorectal manometry for functional assessment of early postoperative results after corrective surgery for anorectal malformations (ARMs) in children and compare manometric observations with age-matched controls. Parents were counseled and management strategies were planned according to the manometric assessments.

Methods

From August 2005 to September 2009, 32 patients who underwent surgery for ARM were assessed postoperatively with anorectal manometry using a water-perfused anorectal motility catheter to record anal canal length or high-pressure zone, resting pressure of anal canal (RP), and rectoanal inhibitory reflex (RAIR). These patients were divided in 2 groups (infants, <1 year; children, >1 year) according to the age at the time of performance of anorectal manometry that was done at 6 months or later following stoma closure or anoplasty.

Results

Out of these 32 patients, high anomaly was present in 13, whereas 19 had low type of defect. Manometric anal canal length of the children with high and low ARM was 2.10 ± .44 and 2.25 ± .53 cm, respectively, which was significantly shorter than that of their age-matched controls(P < .05). In patients with high ARM, RP in infants (17 ± 7.7 mm of Hg) and children (21 ± 9.4 mm of Hg) was lower than that of controls (RP in infants = 42.43 ± 8.19 mm of Hg, RP in children = 43.43 ± 8.79 mm of Hg, P < .001). In patients with low ARM, RP in infants (34 ± 8.6 mm of Hg, P = .002) and children (26 ± 9.9 mm of Hg, P = .001) was lower than that in controls. Presence of RAIR was demonstrated in 5 (38.4%) of 13 patients with high ARM and in 11 (57.9%) of 19 cases with low ARM. Parental counseling was done after this early evaluation, and management strategies like bowel management program and biofeedback training were planned according to the results of the tests.

Conclusion

Our anorectal manometric results suggest that patients with ARM had short anal canal with lower RP and impaired RAIR, which could affect the ultimate functional outcome in these patients. Thus, postoperative anorectal manometric evaluation of the patients with ARM can give more realistic information about future continence and might help in planning future treatment strategies like bowel management program or biofeedback training.     

Botulinum toxin in the treatment of refractory constipation associated with anal sphincter hypertonicity: A pilot prospective study in Chinese children (CME paper)

Objective: Idiopathic constipation in children can be difficult to manage with conventional therapies. The present study aimed to evaluate the efficacy of adjunctive botulinum toxin injection in children attending our regional centre with refractory idiopathic constipation associated with internal anal sphincter hypertonicity. Methods: Children suffering refractory constipation after a minimum of 3 months bowel training, dietary and laxative treatments were evaluated by anorectal manometry. Those with idiopathic anal sphincter hypertonicity (resting pressure > 60 mmHg with normal recto‐anal reflex) were recruited for botulinum toxin injection. Pretreatment evaluation also included bowel‐function score and the degree of megarectum as measured transpubically by ultrasound. Each child received 60 units of Botox^® intrasphincterically under general anaesthesia. Follow‐up evaluations were carried out at 6 weeks and 6 months. Results: Eleven Chinese children, mean age 6.1 years, underwent botulinum toxin treatment. The mean sphincter pressure decreased from 73 mmHg to 55 mmHg and the mean symptom score improved from 2.5 to 6.3 (P = 0.003, Wilcoxon signed rank‐sum test). The mean rectal diameter decreased from 3.7 cm to 3.4 cm. Findings were sustained at 6 months with no treatment complications. Conclusion: Botulinum toxin intrasphincteric injection is a safe, non‐invasive and effective adjunct to conventional therapies in managing children with refractory idiopathic constipation associated with sphincter hypertonicity. Timely definitive therapy to facilitate pain‐free habitual defecation plays an important role in these children in their potty training, as demonstrated by the sustained ‘sphincterotomy’ effects outlasting the transient toxin paralysis.     

Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery

IntroductionAnorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function.MethodologyThis was a prospective observational study. Convenience sampling method was used to recruit all HD patients who had definitive pullthrough from January 2019 to December2020 in our institution. High-resolution anorectal manometry (HRAM) was used to record anal resting pressure (ARP), length of high-pressure zone (HPZ), and presence/absence of recto-anal inhibitory reflex (RAIR). The Paediatric Incontinence/Constipation Scoring System (PICSS) was scored for all participants. PICSS is a validated questionnaire with scores mapped to an age-specific normogram to denote constipation, incontinence, and their combinations. Non-parametric and chi-square tests at significance p<0.05 were conducted to examine the relationship between PICSS categories and manometry findings. Ethical approval was obtained.ResultsThere were 32 participants (30 boys). Median age at participation was 26.5 months (range: 13.8–156). Twenty-four (75%) had transanal pullthrough, 8(25%) underwent Duhamel procedure. PICSS scored 10(31.3%) as normal, 8(25%) as constipation, 10(31.3%) as incontinent, and 4(12.5%) as mixed. RAIR was present in 12 patients (37.5%). HPZ, maximum ARP, mean ARP were comparable across all PICSS groups without statistically significant differences. Presence of RAIR was not significantly associated with any PICSS groups (p = 0.13).ConclusionBowel function does not appear to be significantly associated with HRAM findings after definitive pullthrough for HD, but our study is limited by small sample size. RAIR was present in 37.5% patients after pullthrough.Level of EvidenceLevel II     

盆底痉挛21例肛直肠动力学研究

目的研究盆底痉挛(pelvic floor dyssynergia,PFD)的肛管直肠动力学变化。方法对有便秘症状并经排粪造影诊断为PFD的21例患者用肛管直肠动力检测仪行肛管直肠压力测定,并测定会阴下降和直肠排空率。以无排便功能紊乱症状的12例健康志愿者作对照组。结果与对照组相比,PFD肛管静息压升高(P<0.05),力排时直肠-肛管压力差明显下降(P<0.01),且此压力差为负值,引起直肠初始感觉和初始便意感觉的容积均明显升高(P<0.01),直肠肛门抑制反射(rectal anal inhib-itory reflex,RAIR)阈值容积和直肠排空率均明显下降(P<0.01)。PFD的RIAR阈值容积与引起直肠初始感觉的容积呈正相关(P<0.01)。PFD力排时的会阴下降幅度下降(P<0.05)。结论PFD存在明显的直肠感觉功能下降和盆底肌肉协调运动障碍。     

Role of 3D high resolution anorectal manometry compared to conventional technique in management of constipation and fecal incontinence in children

BackgroundAnorectal manometry is a valuable tool for objective assessment of motility motor and sensory function of the anorectum. The aim of this study was to investigate role of water-perfused (WP) three dimensional high-resolution anorectal manometry (3D-HRARM) compared to WP conventional manometry (CM) in the management of chronic idiopathic constipation (CIC) and faecal incontinence (FI) in children.MethodsThis was a retrospective review of 122 consecutive children, who had WP 8-channel CM or 24 channel 3D-HRARM and endosonography under ketamine anaesthesia from September 2012 to February 2019. All patients had a validated symptom severity score questionnaire ranging from 0 (best) to 65 (worst). Mann-Whitney-U test and Spearman rank test were used and p<0.05 was considered significant.ResultsSubjects were divided according to investigation: CM group (n = 75) and 3D-HRAM (n = 47), who were otherwise comparable. Median anal resting pressure was 50 mmHg and rectoanal inhibitory reflex (RAIR) threshold volume was 10mls across the entire cohort. There were no significant differences in resting pressure or RAIR threshold when using conventional or 3D-HRARM. Rectal capacity was significantly higher in the CM group (p = 0.002). Rectal capacity and internal anal sphincter (IAS) thickness positively correlated with symptom severity, duration and patient age. 3D-HRARM provided a more detailed depiction of the anorectal pressure profile. Botulinum toxin was injected into the external anal sphincter (EAS) muscle (n = 75, 61%) and an anterograde colonic enema (ACE) stoma was needed subsequently in 19 (16%) to treat recurrent constipation and soiling symptoms. Rectal biopsy was done in 43(35%) patients, if RAIR was absent or inconclusive.ConclusionCM and 3D-HRARM are comparable in terms of resting pressure and RAIR threshold. 3D-HRAM is safe and provides detailed functional morphology of anal sphincters and it is useful to understand underlying pathophysiology of constipation and faecal incontinence in children and plan further treatment.Level of EvidenceLevel I     

Anterior or posterior sagittal anorectoplasty without colostomy for low-type anorectal malformation: how to get a better outcome?

Background/Purpose

Usually, anorectal malformations (ARM) are treated in 2 or 3 stages for fear of disturbed wound healing and subsequent damage to the anal sphincter complex. The aim of this study was to assess the feasibility, safety, advantages, and follow-up of an anterior or posterior sagittal anorectoplasty in low-type ARM (rectoperineal or rectovestibular), performed without colostomy.

Materials and Methods

Prospective collection of data regarding demographics, VACTERL (Vertebral-, Anal-, Cardiac-, Tracheo-Esophageal-, Renal-, Limb malformations) screening, perioperative measurements, surgeons, and complications.

Results

In 35 consecutive children (13 boys, 22 girls), repair of a low-type ARM was performed without colostomy. There were 13 boys and 10 girls with a rectoperineal and 12 girls with a rectovestibular fistula. The median age at operation was 4 months (range, 0-73 months); 34% being performed in the newborn period. Seventeen children had one or more other congenital anomaly. Preoperatively, all patients had rectal washouts with oral and rectal neomycin, and perioperative antibiotics, either 24 h (prophylaxis) or for 2 to 5 days. An anterior or posterior sagittal anorectoplasty was performed.Postoperatively, 9 children had no enteral feeding and total parenteral nutrition (TPN). All children had postoperative anal dilatations according to the Peña scheme. Two children (both with rectoperineal fistula) had a wound abscess; in the first child (with renal insufficiency), a colostomy was performed and in the other child a successful correction of the anoplasty was done. In 7 children (4 rectoperineal, 3 rectovestibular fistulae), the anus eventually healed after minor wound dehiscence. There was 1 anal stricture, after a median follow up of 14 months (range, 1-84 mo). After therapeutic antibiotics (2-5 days), 11% (2/18) had some degree of wound infection, versus 41% (7/17) after either no antibiotics or after prophylactic antibiotics (24 hours). Patients with TPN did not seem to profit with regard to wound healing and one patient experienced a central line related sepsis. At last follow-up, 12 children needed regular laxatives and/or enemas. Anal dilatations were well accepted above 6 months, and a trend was seen towards less need for laxatives when dilatations were continued longer.

Conclusion

Repair of a low-type ARM without colostomy, with therapeutic antibiotics, and followed by a long period of postoperative anal dilatations has low morbidity and good outcome, which does not seem to be improved with TPN.     

Diagnosing Hirschsprung's disease: increasing the odds of a positive rectal biopsy result

Background/Purpose: Rectal biopsies are performed as a definitve means of diagnosing Hirschsprung's Disease (HD) in children presenting with constipation. The authors hypothesized that key features in the history, physical examination, and radiographic evaluation would allow us to avoid unnecessary rectal biopsies. Methods: A retrospective analysis was conducted on patients undergoing rectal biopsy between 1995 and 2001. Patients with HD were identified (n = 50), and a concurrent cohort of patients with idiopathic constipation (IC; n = 50) was selected. Pertinent features in patients with HD versus those with IC were cross tabulated using Pearson [Chi ]² testing (significance was P [lt ] .05). Results: Sixty percent of patients with HD and 15% of patients with IC experienced onset of symptoms in the first week of life. HD patients more frequently experienced delayed passage of meconium (P [lt ] .05), abdominal distension (P [lt ] .05), vomiting (P [lt ] .05), and transition zone on contrast enema (P [lt ] .05). All patients with HD had one or more of these significant features. In contrast, only 64% of patients with IC had one or more of these features. The classic triad of symptoms (ie, delayed passage of meconium, vomiting, and abdominal distension) was present in 18%, and one or more of these symptoms was present in 98% of HD patients. In contrast, only 60% of patients with IC had a history of delayed passage of meconium, vomiting, or abdominal distension. Conclusions: A history of delayed passage of meconium, abdominal distension, vomiting or the results of a contrast enema identified all patients with HD and excluded HD in approximately 36% of patients with idiopathic constipation. The authors have shown that key features in a patient's history, physical examination, and radiologic evaluation can differentiate between HD and IC. In a child presenting with constipation and none of the above features, it is not necessary to perform a rectal biopsy to exclude HD. J Pediatr Surg 38:412-416.     

Internal anal sphincter achalasia: outcome after internal sphincter myectomy

BACKGROUND: Internal anal sphincter achalasia (IASA), also referred to as ultrashort segment Hirschsprung's disease (HD), is a clinical condition with presentation similar to HD, but with the presence of ganglion cells on rectal biopsy. The diagnosis of IASA is made on anorectal manometry, which shows the absence of rectosphincteric reflex on rectal balloon inflation. Altered intramuscular innervation has been reported in IASA. The purpose of this study was to review the outcome after internal sphincter myectomy in patients with IASA. METHODS: Fifteen consecutive patients (age range, 2 years to 12 years) with IASA underwent posterior internal sphincter myectomy. All patients presented with severe constipation with or without soiling. The diagnosis of IASA was made by anorectal manometry. HD was excluded in these cases by the presence of ganglion cells and normal acetylcholinesterase activity on suction rectal biopsies. Internal sphincter (IS) specimens were examined using immunohistochemistry for the general neuronal marker PGP 9.5 and synapsin 1 (a presynaptic marker) and using general histochemistry for NADPH-diaphorase. All patients underwent follow-up for periods from 2 years to 6 years. RESULTS: PGP 9.5, synapsin 1 and NADPH-diaphorase positive nerve fibers were either absent or markedly reduced in IASA specimens compared with controls, confirming previous reports of defective intramuscular innervation in IASA. At the time of follow-up, 7 patients have regular bowel motions and are not on any laxatives. Six patients have normal bowel habits but are on small doses of laxatives. One patient is able to stay clean with regular enema regimen. One patient required resection of dilated and redundant sigmoid colon and now has normal bowel habits with laxatives. CONCLUSION: The majority of patients with internal anal sphincter achalasia can be treated successfully by internal sphincter myectomy.     

Rectal myectomy in the treatment of chronic constipation in children

Rectal myectomy was used with the positive result in 22 children. In patients with the supra-anal form of the Hirschsprung's disease, rectal myectomy was performed as an independent intervention. In patients with the residual zone of agangliosis after radical intervention, idiopathic constipation after radical myectomy, the pressure in the anal canal reduced, rectal emptying improved.     

Chronic Constipation Due to Delayed Diagnosis of a Congenital Anal Web

Congenital anal web is a rare form of anorectal malformation. In cases of delayed diagnosis, patients can present with signs ranging from mild constipation to complete bowel obstruction. The diagnosis is made by thorough anorectal inspection and a digital rectal exam. We present the case of a 9-month-old boy with Down's syndrome with chronic constipation secondary to an anal web. To our best knowledge, this is the first report of an anal web in a patient with Down's syndrome presenting with severe chronic constipation.     

Clinical results of abdominal rectopexy for rectal prolapse.

Abdominal Marlex-mesh rectopexy was used for surgical treatment of rectal prolapse in 54 consecutive patients. Anal incontinence was observed in 43 patients (80%) before surgical treatment. The degree of anal incontinence was more severe in women as compared with men. Operative treatment corrected the pathologic anatomy effectively as only one recurrent prolapse developed. At the follow-up examination three patients had symptomless anal mucosal prolapse during maximal straining. 75% of the incontinent patients regained continence for faeces and the rest had some improvement in continence. Seventeen patients (31%) had postoperative constipation, that required lactulose treatment. In conclusion, abdominal Marlex-mesh rectopexy can be recommended as safe and effective treatment for rectal prolapse, despite some patients developing constipation and some remaining incontinent.     

Differential effects of sacral anterior root stimulation on anal sphincter and colorectal motility in spinally injured man

The motility responses of the sigmoid colon, rectum and external anal sphincter to sequential electrical stimulation of the anterior sacral roots (S2, S3 and S4) were studied in five patients with traumatic spinal cord injury. Identical and reproducible results were obtained. S2 stimulation provoked isolated low-pressure colorectal contractions. S3 stimulation initiated high-pressure colorectal motor activity which appeared peristaltic and was enhanced with repetitive stimuli. This response appeared to be frequency-dependent. S4 stimulation increased colonic and rectal tone. External sphincter activity was stimulated in increasing order from S2 to S4. These observations directly elucidate the central control of colorectal motility and may have implications in the treatment of severe constipation following spinal injury.     

Hirschsprung disease and anorectal malformations — An uncommon association

BackgroundThe simultaneous occurrence of Hirschsprung disease (HD) and anorectal malformation (ARM) is extremely rare, with only a very limited amount of cases published in the literature.Constipation is a major problem in patients operated for ARM, and biopsies from the distal rectum in patients with ARM may not show ganglion cells owing to different reasons, leading to a false positive diagnosis of HD. A pull-through procedure for HD after previous anorectoplasty for ARM always leads to fecal incontinence.The aim of the present study was to assess the incidence of simultaneous diagnoses of ARM and HD in a single large cohort of ARM patients and to demonstrate that biopsies from the anal canal, which are negative for ganglion cells, may mislead to a diagnosis of HD.Materials and methodsA retrospective review of our database from 1980 to 2018 identified 164 patients with HD and 2397 patients with ARM. Four patients suffered from both HD and ARM.ResultsThe incidence of HD in ARM patients was 4/2397 = 0.17%, and the incidence of ARM in HD patients was 4/164 = 2.4%.ConclusionOur results strongly suggest that the association of ARM and HD is less common than previously reported.Type of studyTherapeuticLevel of evidenceIV