Surgery For Anorectal Malformations Research Articles

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021.

Anorectal malformations (ARMs) are complex congenital anomalies. The corrective operation is demanding and schedulable. Based on complete national data, patterns of care have not been analyzed in Germany yet. All cases with ARM were analyzed (1) at the time of birth and (2) during the hospital stay for the corrective operation, based on the national hospital discharge data (DRG statistics). Patient's comorbidities, treatment characteristics, hospital structures, and the outcome of corrective operations were analyzed with respect to the hospitals' caseload. From 2016 to 2021, 1,726 newborns with ARM were treated at the time of birth in 388 hospitals. Of these hospitals, 19% had neither a pediatric nor a pediatric surgical department. At least one additional congenital anomaly was present in 49% of cases and 7% of the newborns had a birthweight below 1,500 g.In all, 2,060 corrective operations for ARM were performed in 113 hospitals in the same time period. In 24.5% of cases, at least one major complication was documented. One-third of the operations were performed in 56 hospitals, one-third in 20 hospitals, and one-third in 10 hospitals with median annual case numbers of 2, 5, and 10, respectively.Hospitals with the highest caseload operated cloacal defects more often than hospitals with the lowest caseload (7 vs. 2%) and had more early complications than hospitals with the lowest caseload (30 vs. 21%). This difference was not statistically significant after risk adjustment. Children with ARM are multimorbid. Early complications after corrective surgery are common. Considering the large number of hospitals with a very low caseload, centralization of care for the complex and elective corrective surgery for ARM remains a key issue for quality of care.

A Predictive Model to Identify the Effects of Transcutaneous Sacral Nerve Stimulation With Pelvic Floor Exercises in Fecal Incontinence After Surgery for Anorectal Malformation.

Although the combination of transcutaneous sacral nerve stimulation (tSNS) and pelvic floor exercises (PFEs) has shown significant effectiveness in treating fecal incontinence (FI) after surgery for congenital anorectal malformation (CARM), not all patients achieve satisfactory continence. Therefore, identifying which individuals will benefit from this method is crucial. A prospective cohort study enrolled 92 children with FI. All patients underwent tSNS with PFE treatment, and an improved outcome was defined as a Wexner score ≤4. A predictive model to identify the effects of tSNS with PFEs in FI was developed based on the analysis of magnetic resonance imaging and high-resolution anorectal manometry with area under the receiver-operating characteristic curve to evaluate the predictive value of external anal sphincter (EAS) thickness index and anal squeezing pressure (ASP). tSNS with PFEs improved outcomes in 72 patients and led to poor outcomes in 20 (4 had their rectums deviate from the puborectalis muscle center or puborectal muscle ruptures while 16 lacked EAS with a lower ASP). The areas under the receiver-operating characteristic curve for EAS thickness index and ASP in predicting the effects of tSNS with PFEs were 0.915 (95% confidence interval 0.846-0.983, P = 0.000) and 0.886 (95% confidence interval 0.819-0.952, P = 0.000), respectively. By applying cutoff values of 0.076 for EAS thickness index and 21.95 mm Hg for ASP, tSNS with PFEs was found to be ineffective. tSNS with PFEs is effective for most patients with FI after CARM surgery, except when the rectum deviates from the puborectal muscle center, puborectal muscle rupture occurs, or EAS is absent with a low ASP.

The candle as an improvised anal dilator following surgery for anorectal malformation.

Anal dilation with Hegar dilator is recommended as part of the post-operative management following surgical correction of anorectal malformation (ARM) in children to prevent strictures. Parents usually continue dilation at home with a personal set of dilators. However, in low-resource settings, many parents cannot afford the Hegar dilator. Devices that have been improvised as dilators include appropriately sized candles, spigot from Foley catheter, rounded ball pen ends and mothers' fingers. We aimed to report our preliminary experience with the use of candle as an improvised anal dilator following surgeries for ARM in our practice. A review of records of children who had surgery for ARMs and commenced on candle dilation between February 2018 and July 2019 at our centre was done. Relevant demographic information, type of anomaly, duration of dilation, availability of health insurance coverage, complications and parents' acceptance of improvised dilator (evaluated during scheduled follow up visits) were retrieved. The data were analysed using SPPSS version 21 (Chicago, Illinois). Nineteen patients underwent anal dilation with improvised candles during the period. Rectobulbar and rectovestibular fistulas were most frequent anomaly in boys and girls, respectively. The median age at surgery was 6 months. The median duration of dilation (completed in 9 patients) was 7 months. Candle dilation was well tolerated, and all parents were comfortable using the device. The candle as an improvised anal dilator following anorectal surgeries in children is a useful alternative to the conventional dilator. It is more affordable and gives satisfactory results.

Hemi-circumferential mucosal resection and anastomosis procedure for rectal prolapse following anorectoplasty for anorectal malformations.

Rectal prolapse after radical surgery for anorectal malformations (ARMs) is a common postoperative complication that causes bleeding and stenosis, and sometimes requires surgical treatment. Different surgical techniques have been reported, but most are associated with high recurrence rates and a long postoperative stay. We performed the hemi-circumferential mucosal resection and anastomosis procedure (HCMR) to preserve anal muscle and anal function. Here, we report the success of our minimally invasive surgical approach to correct rectal prolapse in patients with ARMs. The subjects of this retrospective review were patients who underwent HCMR for rectal prolapse after radical surgery for ARMs between January, 2014 and August, 2021. HCMR involves rectal mucosal resection without muscle plication. A collective 15 HCMR procedures were performed in 10 patients. The median age at repair was 1.8years (range, 10months-18years). The median operation time and postoperative length of stay were 46min (range, 17-85min) and 3days (range, 1-7days), respectively. The median postoperative observation period was 3years 4months (range, 8months-7years 10months) and no complications or recurrences were observed. HCMR is a safe and effective surgical treatment for rectal prolapse after radical operation for ARMs, which is minimally invasive and preserves rectal muscle and function.

Analysis of the efficacy of biofeedback for faecal incontinence after surgery for anorectal malformation

Objective To explore the therapeutic effects of biofeedback in the treatment of faecal incontinence (FI) after surgery for anorectal malformation (ARM). Methods Clinical data were collected from paediatric patients for postoperative biofeedback due to FI caused by ARM between May 2017 and November 2021. The data included the duration of symptoms, the integrity of the anal sphincter, anorectal manometry parameters, and FI scores. These patients were divided into the low ARM group (group A) and the high ARM group (group B). Results A total of 45 paediatric patients were enrolled in the study. There were 28 cases in group A and 17 cases in group B. The differences in age, gender, and body weight were not statistically significant between the two groups (p > 0.05). The differences in the clinical indicators were also not statistically significant between the two groups at the time of the initial evaluation. The duration of symptoms was 2.21 ± 0.71 years and 4.14 ± 1.89 years in groups A and B. There were 16 cases with an intact anal sphincter in group A and only two cases with an intact anal sphincter in group B. This difference was statistically significant between the two groups (p < .05). The anal resting pressure, initial sensitivity threshold, defaecation sensitivity threshold, defaecation urge threshold, and FI scores were significantly improved in both groups post-treatment compared to pre-treatment (p < .001). Strong impulses improved significantly in group A, while strong impulses did not improve significantly in group B. The multivariate logistic regression analysis with these variables further showed that symptom duration and anal sphincter integrity were the main factors influencing the therapeutic effects of biofeedback. Conclusion Biofeedback plays a positive role in the treatment of FI in paediatric patients following surgery for ARM. Symptom duration and anal sphincter integrity were found to be the main factors influencing the therapeutic effect of biofeedback.

Діагностика та лікування анальної інконтиненції після хірургічної корекції вродженої аноколоректальної патології в дітей

Fecal incontinence (FI) in children is a serious medical and social problem. FI leads to severe limitations in a child’s development and social adaptation. Pediatric surgeons mainly deal with the mechanical type of FI that occurs after surgical correction of anorectal malformations (ARM) or Hirschsprung's disease (HD). Violation of defecation control is observed in 53-89% of patients who underwent surgery for ARM and HD. Purpose - to study the causes and effectiveness of the proposed complex treatment of FI in children operated on for HD and ARM. Materials and methods. Clinical examination and treatment of 92 children with congenital pathology of the distal colon and anus (55 (59.78%) patients with HG and 37 (40.22%) with ARM) was carried out at the Pediatric Surgery Clinic of Ivano-Frankivsk National Medical University. Among the surveyed children were 64 boys and 28 girls (ratio - 2.29:1). In terms of age, newborns and children of the first year of life predominated among those who underwent primary surgery - 57 (61.95%) children. FI of varying severity was detected in 48 (52.17%) patients operated on for HD and ARM at different terms after surgical correction - from 6 months to 3 years. The types, causes, and severity of FI in each child were studied. In addition to conventional clinical and instrumental studies, patients underwent anoscopy and rectoscopy, transanal ultrasound, anorectal manometry. The severity of AI was assessed on a S.D. Wexner score. Results. FI occurred in 34 (70.8%) children after HD correction and in 14 (29.2%) patients after ARM surgery. True FI was found in 33 (68.75%) children. Pseudoincontinence was detected in 15 (31.25%) patients it was more common in patients operated on for HD. For conservative treatment of patients with FI a Bowel Management Program was implemented. In 24 (50.0%) patients treated according to this program, the control of defecation was significantly improved, the incontinence severity index was significantly reduced according to the Wexner score. In true AI due to severe anal sphincter damage, we performed minimally invasive surgical correction of postoperative insufficiency or congenital defect of the internal sphincter of the anus using a volume-forming implant in 14 (29.17%) children. Immediate and long-term results of the operation were good, which was reflected in improved control of defecation, a significant increase of basal pressure and a decrease of the incontinence severity index. Conclusions. Tactics of the FI treatment depends on the cause and severity. The implementation of the Bowel Management Program can significantly improve the control of defecation and improve the quality of life of patients. In case of insufficiency of the internal anal sphincter, minimally invasive correction by anal submucosal implantation of a volume-forming gel is effective. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: fecal incontinence, anorectal malformations, Hirschsprung's disease, children.

Результати реконструктивних операцій при вродженій аноколоректальній патології в дітей

Fecal incontinence (FI) in children is a serious medical and social problem. FI leads to severe limitations in a child’s development and social adaptation. Pediatric surgeons mainly deal with the mechanical type of FI that occurs after surgical correction of anorectal malformations (ARM) or Hirschsprung's disease (HD). Violation of defecation control is observed in 53-89% of patients who underwent surgery for ARM and HD. Purpose - to study the causes and effectiveness of the proposed complex treatment of FI in children operated on for HD and ARM. Materials and methods. Clinical examination and treatment of 92 children with congenital pathology of the distal colon and anus (55 (59.78%) patients with HG and 37 (40.22%) with ARM) was carried out at the Pediatric Surgery Clinic of Ivano-Frankivsk National Medical University. Among the surveyed children were 64 boys and 28 girls (ratio - 2.29:1). In terms of age, newborns and children of the first year of life predominated among those who underwent primary surgery - 57 (61.95%) children. FI of varying severity was detected in 48 (52.17%) patients operated on for HD and ARM at different terms after surgical correction - from 6 months to 3 years. The types, causes, and severity of FI in each child were studied. In addition to conventional clinical and instrumental studies, patients underwent anoscopy and rectoscopy, transanal ultrasound, anorectal manometry. The severity of AI was assessed on a S.D. Wexner score. Results. FI occurred in 34 (70.8%) children after HD correction and in 14 (29.2%) patients after ARM surgery. True FI was found in 33 (68.75%) children. Pseudoincontinence was detected in 15 (31.25%) patients it was more common in patients operated on for HD. For conservative treatment of patients with FI a Bowel Management Program was implemented. In 24 (50.0%) patients treated according to this program, the control of defecation was significantly improved, the incontinence severity index was significantly reduced according to the Wexner score. In true AI due to severe anal sphincter damage, we performed minimally invasive surgical correction of postoperative insufficiency or congenital defect of the internal sphincter of the anus using a volume-forming implant in 14 (29.17%) children. Immediate and long-term results of the operation were good, which was reflected in improved control of defecation, a significant increase of basal pressure and a decrease of the incontinence severity index. Conclusions. Tactics of the FI treatment depends on the cause and severity. The implementation of the Bowel Management Program can significantly improve the control of defecation and improve the quality of life of patients. In case of insufficiency of the internal anal sphincter, minimally invasive correction by anal submucosal implantation of a volume-forming gel is effective. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local ethics committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Key words: fecal incontinence, anorectal malformations, Hirschsprung's disease, children.

Pathology and Surgical Management of Urinary Retention Manifesting After Anorectal Malformation Surgery.

Introduction:Associated congenital anomalies, operative structural injury, and postoperative neurovesical dysfunction combine to cause urinary retention in children with a history of surgery for anorectal malformation (ARM).Aim:To study the presentation and management of urinary retention in patients with a history of ARM surgery.Methodology:Retrospective study.Results and Discussion:Twenty-five children presented with urinary retention with a history of ARM surgery performed elsewhere. There were 17 males (high – 14, intermediate – 2, and low – 1) and 8 females (cloaca). Sixteen children had an abnormal spine. Eight children had urethral injury (total transection – 3) and three had a large residual rectal stump. 41/48 renal units were dilated and 27 refluxing. Seventeen children had abnormal estimated glomerular filtration rate and five had undergone urinary diversion.Management:Definitive surgical management was individualized, the most pertinent consideration being whether normal voiding would be feasible or whether a continent low pressure urinary reservoir with clean intermittent catheterization (CIC) would be a safer option. Operative management included excision of the rectal stump (3), urethral reconstruction (2), bladder augmentation (17), Mitrofanoff port (22), bladder neck closure (2), and antireflux surgery (13). Follow-up estimated glomerular filtration rate had improved/normalized in all but two patients. HUN resolved/improved in all and 25/27 refluxing units ceased refluxing. All are socially continent with ten voiding normally and the rest on CIC.Conclusion:Urinary retention after ARM surgery is multifactorial and requires prompt recognition and possibly urinary diversion. Final reconstruction aims at achieving continence with safe upper tracts. Urethral voiding is possible in the selected cases.

Outcomes following the establishment of Qatar’s quaternary neonatal general surgical service in a hospital with a “Tabula Rasa” for service development

Background: Sidra Medicine is the first dedicated Children and Maternity hospital in the State of Qatar. This paper describes the preparations for and results of activating quaternary neonatal surgical services in a brand new, "greenfield" hospital. We believe that these are the first published national results of neonatal general surgical services from the Gulf region. Methods: A review of surgical babies below four weeks corrected age from 1st of April 2018 – 31st of March 2020 was undertaken. Patient demographics, primary diagnosis, surgical procedures, 30-day postoperative mortality, overall mortality, and cause of death were recorded. Results: One hundred and sixty-nine babies (169) were identified (44.4% term and 55.6% preterm). Major surgery included laparotomy (76), congenital diaphragmatic hernia repair (20), surgery for anorectal malformations (25), and esophageal atresia (13). One set of conjoined twins were also separated successfully. Fourteen babies died, resulting in overall mortality of 8.3 %. Excluding babies who died of life-limiting conditions, two babies died within 30 days of surgery, resulting in 30 days postoperative mortality rate of 1.2 %. Conclusions: The neonatal surgical mortality rate is comparable with those from top international centers. This low rate could be attributed to the high level of expertise preceded by months of preparations.

Role of urodynamics in male patients of high-anorectal malformations: a prospective study.

Association of spinal or vertebral anomalies and the iatrogenic denervation during surgical correction of anorectal malformation patients especially in boys can lead to neurogenic bladder inthese subset of patients. The paucity of literature with regard to urodynamic studies focusing exclusively in male children with high-anorectal malformations (HARM) lead us to analyze the urodynamic changes. The objective was to study urodynamic profile in male patients who have undergone surgery for anorectal malformation. Male high-anorectal malformation patients who had completed all the stages of repair were prospective studied. Following the basic work up, all patients based on the urodynamics were categorized into 2 groups as safe or unsafe bladders. Unsafe bladder was defined as detrusor pressure > 40cm (high detrusor pressure) or pressure variability of 15cm of water (detrusor overactivity) or significant post-void residue. MRI was limited to patients with only abnormal urodynamics to rule out spinal causes of neurogenic bladder and due to financial constraints, it could not be offered to all patients. 41 HARM meet the exclusion criteria. All patients were asymptomatic with none having history of urinary tract infections. Ultrasound showed bladder wall thickening in 31.7% patients. UDS revealed reduction in bladder capacity and compliance was noted in 31.7% and 30% patients, respectively. Elevated detrusor pressures (> 40cm of water) were noted in 10% (4/41), detrusor overactivity with concomitant elevated detrusor pressures in 19.5% (8/41) and normal UDS in 70% (29/41). 13 patients (31.7%) had abnormal cystometric parameters with 12(30%) having unsafe bladders. MRI confirmed sacral agenesis in 1 patient with unsafe bladder. Urodynamics can demonstrate occult neurovesical dysfunction in patients with HARM. This would help in early renal protective therapy and prevent the burden of long-term sequelae of neurovesical dysfunction in HARM patients.

Constipation after surgery for anorectal malformations: Unrecognised problem until it is a problem.

Background:Constipation is a common problem after surgery for anorectal malformations (ARMs), especially in patients having preserved rectosigmoid after pull-down surgery. Here, we present our experience with patients having constipation after surgery for ARMs and briefly discuss its management.Materials and Methods:We retrospectively reviewed electronic operative and follow-up data from January 2015 to December 2019 of all patients having constipation after surgery for ARMs.Results:A total of 77 patients were included in the study. The median age was 60 months, and thirty patients were male. Primary diagnoses were rectourethral fistula (26; 33.77%), vestibular fistula (25; 32.47%), perineal fistula/ectopic anus (18; 23.38%), rectovaginal fistula (4; 5.19%) and imperforate anus without fistula (4; 5.19%). Anal stenosis was found in 20 (25.97%) patients, posterior ledge with an adequate anal opening in 5 (6.49%), displaced anus in 4 (5.19%) but no obvious problem found in 48 (62.34%) patients. Non-operative management was successful in 75.44% (43 out of 57) of patients. Surgery was performed in 32 out of 77 patients (41.56%) with no mortality. Forty-three (72.88%) patients are continent, stayed clean, but 16 (27.19%) patients still need some sort of laxative/bowel wash/enema intermittently (Grade I/II constipation). Incidence of constipation was higher in patients operated through anterior sagittal route (27.58%) than posterior sagittal route (23.94%), but it was not statistically significant (P = 0.479). Follow-up ranged from 3 months to 5 years.Conclusion:Constipation is a common problem after ARMs surgery, especially in patients having preserved rectosigmoid after pull-down surgery. It is advisable to create relatively larger neoanus after pull through with early practice of neoanus dilatation, especially in redo cases. Repeated counselling, laxatives and dietary manipulations should begin at the first sign of constipation.

Management of postoperative fecal incontinence by pediatric surgeons in Nigeria: a pilot study

BackgroundFecal incontinence is a challenging problem in pediatric surgical practice as it occurs in 25 to 50% of patients who have undergone surgery for anorectal malformation and in 5 to 23% after transanal pull-through for Hirschsprung disease. Fecal incontinence impacts on the quality of life of patients; hence, it is important that optimum management is instituted. The management of fecal incontinence in children in sub-Saharan Africa is largely unreported. The study, therefore, evaluated the management practices regarding fecal incontinence by pediatric surgeons in Nigeria.ResultsA total of 37 pediatric surgeons participated in the cross-sectional pilot study. The mean length of practice as specialists was 9.7 (± 7.8) years. The majority, 33 (89.2%), were males; 23 (62.2%) were ≤ 45 years of age, and 14 (37.8%) had practiced as consultant pediatric surgeons for ≥ 10 years.When presented with a patient with anorectal malformation and recto-bladder neck fistula or Hirschsprung disease who developed fecal incontinence after surgery, 25 (67.6%) and 22 (59.5%), respectively, will combine modalities in managing the patient. Bowel management (81.1% and 83.8% respectively) was the most commonly selected option of managing postoperative fecal incontinence. Other options selected for the management of postoperative fecal incontinence were as follows: examination under anesthesia (43.2% and 37.8%), constipating diet (43.2% and 35.1%), re-operation (24.3% and 27.0%), and diverting colostomy (21.6% and 16.2%) for anorectal malformation and Hirschsprung disease respectively. There were no statistically significant associations between the length of practice as specialist pediatric surgeons and the selection of single vs. multiple treatment options for a patient with fecal incontinence after surgery either for anorectal malformation or Hirschsprung disease.ConclusionsA combination of modalities will be adopted by at least 60% of pediatric surgeons in Nigeria to manage postoperative pediatric fecal incontinence with bowel management favored by over 80% of specialists surveyed.

Does clinical score accurately support fecoflowmetry as a means to assess anorectal motor activity in pediatric patients after anorectal surgery?

PurposeWe investigated the relationship between Krickenbeck score (KS) and fecoflowmetry (FFM) parameters and assessed the characteristics of this new questionnaire test by comparing Kelly's clinical score (KCS) in pediatric patients with anorectal surgery for anorectal malformation (ARM) and Hirschsprung's disease (HD). MethodsWe enrolled pediatric patients who underwent anorectal surgery for ARM or HD. Bowel function was assessed with KS and KCS thereafter, FFM and anorectal manometry (AM) were conducted. Patients were divided into subgroups according to each parameter of the scoring system and each FFM parameter was compared among the KCS or KS subgroups, respectively. Moreover, correlation analyses were conducted between FFM and AM parameters. ResultsThe comparison of FFM parameters among the subgroups of KCS showed that Fmax in the KCS staining 2 group was significantly higher than that in KCS staining 1 group and the Fmax in KCS sphincter squeeze 1 group was significantly higher than that in KCS sphincter squeeze 0 group. Moreover, Fmax in the KCS “good” group was significantly higher than that in the KCS “fair” group. The comparison of FFM parameters among the subgroups of KS parameters showed that TR in the no soiling group was significantly higher than that in the KS grade 2 soiling group. FFM and AM parameters showed a significant positive correlation between Fmax and voluntary squeezing anal pressure. ConclusionFFM clarified the different characteristics of two scoring systems, namely, KCS reflects the anal sphincter performance, whereas the KS soiling score might reflect the tolerance and evacuation ability.

General complications after surgery for anorectal malformations.

The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals.

REOPERATIONS IN CHILDREN WITH ANORECTAL MALFORMATIONS

The aim of the study was to assess the possibility of using a surgical approach – anterior sagittal anoproctoplasty in children who had previously been operated with various techniques for anorectal malformations. Material and methods . The analysis of postoperative results was performed using a scale for assessing the long-term results of treatment of anorectal malformations before and after surgery. A retrospective study of the patients who was previously operated for various forms of anorectal malformations for the period from 2016 to 2019 in pediatric surgery department of State Novosibirsk district hospital was performed. The early and long-term outcomes were assessed. Results. The criteria for repeated surgical treatment were: rectal mislocated outside of the sphincter mechanism, low social adaptation, secondary changes from the perineum and external genital organs. After investigation (identification of the sphincter mechanism using a myostimulator, irrigoscopy, MRI or MSCT of the pelvic organs), all patients underwent surgical correction – the anterior sagittal anoproctoplasty. Postoperative results were assessed using Holschneider score. The results obtained in the course of this study indicate the possibility of using this method of surgical treatment. Discussion. Children previously underwent surgery for anorectal malformations but having severe functional disorders in the anorectal region, are a complex group of patients who must be determined specific criteria for the reoperations need. The efficiency of surgical treatment depends on the presence of associated congenital malformations (myelodysplasia, pathology of the sacrum and tailbone), as well as the severity of cicatricial changes in the sphincter mechanism. Surgical intervention in 33.3 % of cases led to satisfactory and in 66.7 % of cases to good results.

How much do we know about constipation after surgery for anorectal malformation?

To evaluate the occurrence of constipation after anorectal malformations (ARM) repair and the results of laxative treatment. Between August 2012 and July 2017, the clinical data of patients with ARMs was prospectively collected. The patients were divided into two groups, good types and poor types. Good types included rectoperineal, rectovestibular, rectourethral bulbar, and no fistula. Risk factors were defined as spinal cord anomalies, sacral ratio <0.4, or cognitive impairment. Success was defined as that laxative could be tapered. Eighty-four patients were enrolled with mean age of 6.3±7.8 (0.6-59.9) years. The mean age of onset of constipation was 12.8±8.3 months and the mean interval was 5.9±5.1 months after reconstructions. The interval was not significantly different between patients with good types and poor types. In 23 patients with severe constipation being treated for >6 months, 14 of 18 (77.8%) patients with good types were classified as success, whereas only 1 of 5 (20%) patients with poor types was (p=0.02). In patients with good types, 9 of 9 (100%) patients with no risk factors were successful; however, only 5 out of 9 (55.6%) patients with risk factors were successful (p=0.02). Constipation occurs shortly after operations. Patients with good types and no risk factors are susceptible to weaning laxatives.

Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations

PurposeA complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. MethodsWe performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. ResultsOf 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. ConclusionPatients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF.Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. Level of evidenceLevel III.

VY anoplasty for ectropion of anal mucosa in an adult with anorectal malformation

Abstract Ectropion of anal mucosa (AME) is a possible complication following anorectal surgery and it is often cause of symptoms such as soiling, pain and bleeding affecting the patients' quality of life. Two-flaps anoplasty has been previously described for the correction of AME. We herein report a case of an adult patient who underwent a successful double V/Y-flaps for mucosal ectropion as consequence of repair of anorectal malformation (ARM). A 25 year-old woman with history of ARM presented with an extensive AME causing bleeding, pain, soiling, and dyspareunia. This condition prevented her from an effective rectal nursing and a satisfactory social life. The patient underwent the resection of ectopic mucosa and the creation of two V-shaped skin flaps to cover the skin gap. A minor dehiscence of one of the flaps was treated conservatively. At two years follow up the patient is symptoms free, has a good quality of life and can perform an effective bowel management. AME is a possible complication after anorectal surgery for ARM and the multiple V-Y plasty is a viable procedure for its treatment. A multidisciplinary approach in these cases is recommended. Level of Evidence V.

Screening practices and associated anomalies in infants with anorectal malformations: Results from the Midwest Pediatric Surgery Consortium

BackgroundThis study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM). MethodsWe performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed. ResultsAmong 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p<0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%). ConclusionScreening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients. Type of studyMulti-institutional retrospective cohort study. Level of evidenceIII

Long term follow up of proximal hypospadias repair-urethral stricture should be excluded in adults who present with epididymo-orchitis.

Epididymo-orchitis (EO) is a well-known complication of urinary tract infections (UTI) in children. This is commonly seen in children who had hypospadias repair or in those who had surgery for anorectal malformation especially when it results in urethral stricture. However EO occurring as a complication of urethral stricture in adults operated for hypospadias in childhood is not well documented in the literature. This is a retrospective review of four adults who had proximal hypospadias repair in childhood. All four men presented to us with EO. They were thoroughly investigated to rule out presence of urethral stricture. Three patients had urethral stricture of which two responded well to dilatation and one required cystoscopy and visual internal urethrotomy. The fourth patient did not have urethral stricture. EO in this patient is thought to be due to excessive straining during micturition causing reflux into seminal vesicles. Urethral stricture should be excluded in any adult who had a hypospadias repair and presents with EO. Urethral strictures after hypospadias surgery respond well to dilatation and to endoscopic urethrotomy.