Posterior Sagittal Anorectoplasty Procedure Research Articles

A Promising Future for Hands-On At-Home Training in Pediatric Surgery.

Sufficient training is needed to acquire and retain the procedural skills needed for the surgical correction of congenital anatomical malformations. This study aims to assess the opinions of trainees and pediatric surgeons on the use of simulation-based continued at-home training, which can help to acquire these skills. This study consisted of two parts. First, an international survey among trainees and pediatric surgeons assessed their opinions on simulation-based at-home training for pediatric surgical procedures (5-point Likert scale). Second, participants of pediatric colorectal courses were instructed to practice the posterior sagittal anorectoplasty procedure at-home on a simulation model and, subsequently, complete a questionnaire regarding their opinions on continuous at-home training (5-point Likert scale). A total of 163 participants (83% pediatric surgeons) completed the international survey (response rate 43%). Overall, participants considered the training useful for both laparoscopic (mean 4.7) and open procedures (mean 4.2) and agreed that it may be used at home after a hands-on course (mean 4.3).Twenty participants completed the questionnaire on continued training (response rate 36%). All agreed that at-home training was of added value (mean 4.5) and that the skills were transferable to the clinical setting (mean 4.3). At-home training was regarded a suitable exercise after a hands-on workshop (mean 4.3), but less so without a workshop (mean 3.7, p=0.017). Participating trainees and pediatric surgeons were of the opinion that simulation models and at-home training have added value. This implies that simulation-based training may be used more often in pediatric surgical training.

Validation of a Newly Developed Competency Assessment Tool for the Posterior Sagittal Anorectoplasty

The correction of an anorectal malformation (ARM) is complex and relatively infrequent. Simulation training and subsequent assessment may result in better clinical outcomes. Assessment can be done using a competency assessment tool (CAT). This study aims to develop and validate a CAT for the posterior sagittal anorectoplasty (PSARP) on a simulation model. The CAT-PSARP was developed after consultation with experts in the field. The PSARP was divided into five steps, while tissue and instrument handling were scored separately. Participants of pediatric colorectal hands-on courses in 2019 and 2020 were asked to participate. They performed one PSARP procedure on an ARM simulation model, while being assessed by two objective observers using the CAT-PSARP. A total of 82 participants were enrolled. A fair interobserver agreement was found for general skills (intraclass correlation coefficient [ICC] = 0.524, p < 0.001), a good agreement for specific skills (ICC = 0.646, p < 0.001), and overall performance (ICC = 0.669, p < 0.001). The experienced group scored higher on all steps (p < 0.001), except for "anoplasty (p = 0.540)," compared with an inexperienced group. The CAT-PSARP is a suitable objective assessment tool for the overall performance of the included steps of the PSARP for repair of an ARM on a simulation model.

The Use of an Inanimate Simulation Model for the Correction of an Anorectal Malformation in the Training of Colorectal Pediatric Surgery.

An anorectal malformation (ARM) is a congenital malformation that requires surgical correction. To acquire the skills needed to perform this complex procedure, an affordable simulation model has previously been developed and validated. The aim of this study is to evaluate the suitability of this ARM model (with perineal fistula) for training in hands-on workshops. The ARM model consists of a wooden casing with disposable perineal body. Participants in several international pediatric colorectal hands-on workshops in 2019 and 2020 were asked to participate. They were divided in a target group and an experienced group based on experience. All practiced the posterior sagittal anorectoplasty procedure on the model with multimodality guidance. Subsequently, statements on the suitability of the model for use during hands-on workshops were scored on a 5-point Likert scale. A total of 80 participants were included (43 surgical specialists, 13 pediatric surgery fellows, and 25 residents). Nearly, all statements scored at least a mean of >4.0, all scored significantly better than a neutral opinion. The target group (n = 58) scored higher compared with the experienced group (n = 22) on "transferability of the skills to the clinical setting" (means 4.4 vs. 4.0, p = 0.038); however, the "suitability as a replacement for an animal model" scored significantly lower (means 3.6 vs. 3.9, p = 0.049). No other differences were found. This affordable ARM model was regarded a suitable model for training during preclinical hands-on workshops and could be used for the specified steps of the procedure.

Challenges, constraints and failures that are related to the posterior sagittal anorectoplasty approach to anorectal malformations in a low-resource context: An experience from a sudanese tertiary referral centre.

Background:Anorectal malformations (ARMs) in the sub-Saharan Africa are a common cause of neonatal referral for intestinal obstruction, and the posterior sagittal anorectoplasty (PSARP) approach is rapidly spreading. The small number of paediatric surgeons and the low-resource context limit children's access to care and constrain the quality of results. A retrospective, observational study has been done on a consecutive series of ARM cases admitted to a Sudanese tertiary paediatric surgical centre within the framework of a partnership between Italian and Sudanese academic institutions addressed to review and upgrade the standard of care of major congenital anomalies.Materials and Methods:The authors collected 94 ARM cases in a 3 years' period. Conditions on referral, operative procedures, post-operative course and follow-up were recorded and examined. Their correlations with complications and outcome were analysed.Results:The male/female ratio was 47/47. Eighty patients presented with an untreated ARM; 66 had a divided stoma and 14 had already a PSARP procedure, followed by a poor outcome or sequelae. In 25% of the cases, colostomy required re-doing. In 57 cases, a staged PSARP (primary or re-do) was done. Surgical-site infections occurred in nine patients. Some patients were lost to follow-up after preliminary colostomy. Post-operative dilatation programme suffered from the lack of systematic follow-up, and colostomy closure was possible in 46% of the cases due to problems in travelling and accessing hospital care. Anal stenosis was frequently observed among unfollowed patients.Conclusion:Despite PSARP's widespread adoption in Africa, the risk of complications and failures is high. Primary management is often inappropriate, and a high rate of colostomy-related complications is observed. Poverty and lack of transportation reduce attendance to follow-up, hampering the final results. Investments in healthcare facilities and retention of trained health providers are needed to improve the standard of care.

Laparoscopic-assisted Anorectoplasty: A Single-center Experience.

Aim:To assess the modifications in the technique of laparoscopic-assisted anorectal pull-through (LAARP) practiced at our institute and to analyze the postoperative outcome and associated complications.Materials and Methods:A retrospective study was done to analyze the results for LAARP procedure done for high anorectal malformations (ARMs) from January 2001 to May 2016. A total of 68 patients had undergone LAARP, with 62 male and 6 female children. Staged procedure was done in 55 patients and one child with rectovestibular fistula of 5 months of age had a single-stage procedure. Technical modifications such as traction over the fistula which helps in identification and dissection of the puborectalis muscle, dividing the fistula without ligation, railroading with Hegar's dilators over the suction cannula which creates adequate pull-through channel, have helped save time and make the procedure simpler. The patients were followed up with clinical evaluation and continence scoring.Results:All the patients withstood surgery well. One patient with rectovesical fistula required conversion to gain adequate length of the distal rectum, for whom the colostomy was closed and relocated at splenic flexure level. The complications were mucosal prolapse (8 cases), anal stenosis (5), adhesive obstruction (2), distal rectal necrosis (3), and urethral diverticulum (2). The progress has been satisfactory and weight gain is adequate. 71.15% patients had good continence on follow-up.Conclusion:LAARP procedure is safe for high ARMs with good continence and correctable side effects. It has completely replaced posterior sagittal anorectoplasty procedure for high anomalies in our practice.

Cystoscopic-assisted excision of rectourethral fistulas in males with anorectal malformations

IntroductionWe report a novel technique to label rectourethral fistulas in males with anorectal malformations who are undergoing posterior sagittal anorectoplasty (PSARP) to facilitate complete excision of the fistula. MethodsPrior to performing PSARP in 21 male patients with rectourethral fistulas, cystoscopy was carried out to identify the orifice of the fistula within the urethra. A 3Fr ureteral catheter with calibrations was then inserted into the orifice to label the fistula. During the PSARP procedure, the rectourethral fistula was dissected to the junction of the urethra, as identified by the presence of the ureteral catheter, and the fistula tract was completely excised. ResultsSix prostatic and 15 bulbar rectourethral fistulas were found by cystoscopy. The orifices of the rectourethral fistulas were all located in the midline along the dorsal wall of the posterior urethra. The average length of the rectourethral fistulas was 10mm (range=5–15mm). During the PSARP procedure, the rectourethral fistula could be clearly identified, easily dissected and completely excised. Patients were followed up for 7–24months. During the length of follow-up, micturition was normal and no urethral complications were found in any of the 21 patients. ConclusionIntraoperative cystoscopy with placement of a ureteral catheter in the fistula tract facilitates complete excision of rectourethral fistulas in males with anorectal malformations without risking injury to the urethra.

Undiagnosed H‐type tracheo‐oesophageal fistula resulting in failed ventilation in an infant with Down's syndrome

SummaryWe present a 6‐month‐old male infant with Down's syndrome with an undiagnosed H‐type tracheo‐oesophageal atresia discovered on induction of general anaesthesia for an elective posterior sagittal anorectoplasty procedure for imperforate anus. Mechanical ventilation was not possible after endotracheal intubation. Rigid bronchoscopy after three failed attempts to ventilate the lungs revealed a large tracheo‐oesophageal fistula.

Assessment of postoperative results in anorectal malformations

Surgery for the correction of anorectal malformations (ARM) is performed by almost all pediatric surgeons. A number of operative procedures are practiced. The posterior sagittal anorectoplasty procedure has found wide acceptability and although it provides the most accurate anatomical reconstruction of the neoanorectum and the sphincters, the results are not in keeping with this technical advantage. In addition, there is no uniformity in describing the results of surgery and hence comparative evaluation of different series is difficult. This review describes the various methods that are available for the assessment of postoperative results following surgery for ARM, with a special emphasis on clinical methods keeping in mind the limitations in various parts of the country. Clinical examination and clinical scoring systems hold an important place in the post-operative evaluation of these patients. Imaging modalities are useful in the diagnosis of misplaced bowel and damage to the muscle complex and are necessary before re-do surgery is planned. Objectivity in the evaluation is provided by anorectal manometry combined with electromyography and these corelate well with clinical scoring systems. A consensus is required for uniformity in the methods of assessment.

The treatment of high and intermediate anorectal malformations: One stage or three procedures?

Background/Purpose The aim of this study was to examine the safety, feasibility, and the long-term outcome of complete 1-stage repair of high and intermediate anorectal malformation using posterior sagittal anorectoplasty (PSARP) in a neonate. Methods One hundred thirteen patients with high-type and intermediate-type anorectal malformations (ARM) underwent follow-up. Of 113 cases, 48 cases entailed a divided colostomy, definitive operation, and colostomy closure (group I); the other 65 patients underwent 1-stage PSARP (group II). Anorectal function was measured by the modified Wingspread scoring, including “excellent,” “good,” “fair,” and “poor.” In barium enema studies, anorectal angulation was judged as “clear,” “unclear,” and “not present,” and leakage of barium was observed in the meantime. For anorectal manometric studies, anal resting pressure (ARP), anal squeezing pressure (ASP), and positive anorectal reflex (PAR) were measured. Results In group I, the rate of excellent and good scores was 58.3% (28 of 48). In the barium enema examination, 85.4% (41 of 48) was clear and 14.6%(7 of 48) unclear or not present. The rate of barium leakage was 10.4% (5 of 48). In group II, the rate of excellent and good was 53.8% (35 of 65). Anorectal angulations were clear in 83.1% of patients (54 of 65). Barium leakage happened in 7.69% of patients (5 of 65). Early operative complications occurred in 56.3% (27 of 48) of patients in group I and 29.2% (19 of 65) in group II. The incidence of colostomy complications in group I was 39.6% (19 of 48). Soiling and constipation were the major complications after the PSARP operation. The respective rates of constipation in the 2 groups were 47.9% (23 of 48) and 44.6% (29 of 65), and the respective rates of soiling were 47.9% (23 of 48) and 50.8% (33 of 65). There was no significant difference in the mean ARP between the 2 groups. Conclusions The 1-stage PSARP procedure in the neonate not only achieves the same long-term outcome as the conventional PSARP procedure but also involves fewer short-term complications. Complete 1-stage repair using the PSARP to treat high-type and intermediate-type anorectal malformations is safe and feasible.

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations.

To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies. NVD may occur even in the absence of sacral anomalies. A 4 h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations

Aim: To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. Methods: All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. Results: Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies. Conclusion: NVD may occur even in the absence of sacral anomalies. A 4h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.

Fecal continence in patients having undergone posterior sagittal anorectoplasty procedure for a high anorectal malformation improves at adolescence, as constipation disappears

Background/Purpose: Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient's growth period. Methods: The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord. Results: At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result. Conclusion: In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome. J Pediatr Surg 36:1218-1221. Copyright © 2001 by W.B. Saunders Company.

Preoperative Enzymo-Histochemical Diagnosis of Dysganglionoses Associated with Anorectal Malformations (ARM) with Recto-Vestibular and Recto-Perineal Fistula

The posterior sagittal anorectoplasty (PSARP) is widely recognized as the best technique available today for the surgical treatment of anorectal malformations (ARM). However, different retrospective studies on the functional results of PSARP in the treatment of ARM have shown different postoperative degrees of constipation. In particular, even in patients with normal sacrum, about 70% of operated ARM with vestibular fistula and about 50% with perineal fistula can be complicated by fecal constipation and pseudoincontinence. In order to identify preoperatively whether ARM patients present abnormal innervation patterns of rectal pouch and fistula (as reported by Holschneider et al [7]), we decided to study suction rectal biopsies performed by introducing SBT-100 rectal suction biopsy tool into the fistula at 6, 4, 3 and 2 cm from the meatus. To date, this approach has been adopted in 22 ARM cases (15 females and 7 males, age range 7 days-4 years), 6 of them with recto-vestibular fistula and 13 with recto-perineal fistula. Biopsies were frozen in isopentane at liquid nitrogen temperature and cryostatic sections were studied by acetylcholinesterase (AChE), succinic-dehydrogenase (SDH) and alpha-naphthyl-esterase (ANE) enzymo-histochemical techniques. The results concerning the innervation-type of fistula and proximal rectal pouch were confirmed by the biopsies obtained during PSARP. Our overall incidence of rectal innervation intrinsic disorders was 81.82%. In particular, all our cases of vestibular fistula presented associated dysganglionoses. The incidence of associated Hirschsprung's disease was high, corresponding to 18% of cases. Our results suggest that the high frequency of constipation in low forms of ARMS depends on primary intestinal neuronal malformations and it cannot be ascribed to a denervation secondary to rectal dissection and to PSARP procedure. We propose the introduction of this type of preoperative enzymo-histochemical diagnosis in ARM cases because it can select those patients with severe associated dysganglionoses. In our opinion, if this diagnosis is available preoperatively, PSARP can be performed without using abnormally innervated structures and reducing postoperative functional complications.

A case of ectopic anus localized on the right gluteus

A case of a gluteus-localized ectopic anus on the right side is described. A 5-month-old boy had a severe sacral anomaly with atrophy of the right lower extremity and a pes equinus deformity. Transcutaneous muscle stimulation showed a well-developed muscle complex, and parasagittal muscle fibers have been localized obliquely. Although he has severe sacral anomalies, the posterior sagittal anorectoplasty procedure could be performed. This unique case provides a good demonstration of not only the ectopically localized anus but also the ectopically localized muscle complex.

Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence?

The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors' goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Children's Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a “fecal continence score” (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patient's family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 ± 1.4 (5 = excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents' responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors' results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors' series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

Internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations ? technical considerations

Posterior sagittal anorectoplasty (PSARP) has gained overall acceptance as a standard surgical approach for the repair of high and intermediate anorectal malformations. PSARP allows superior exposure for the identification of the spectrum of these defects and enables anatomic reconstruction of the anal canal and voluntary sphincter muscle complex. In the original PSARP procedure, the involuntary internal anal sphincter, which in embryologic and clinical studies has been shown to be present in the region of the fistulous bowel termination, is not preserved. Internal sphincter-saving PSARP was performed in 65 patients with high or intermediate anorectal malformations. Manometrically, a positive rectoanal inhibitory reflex was noted in 82% of the patients, suggesting the presence of a functional internal sphincter. There was a statistically significant difference in the anorectal resting pressures between patients with a functional internal sphincter and patients without this structure. The technical details of internal sphincter-saving PSARP are described and illustrated in the present communication.