Repair Of Imperforate Anus Research Articles

Adult residual rectourethral fistula and diverticulum presenting decades after imperforate anus repair: a\xa0case report

BackgroundThis report describes a rare surgical case of an intraabdominal mass in a middle-aged patient 40 years after imperforate anus repair.Case presentationA 44-year-old Latino male with history of repaired anorectal malformation presented with recurrent urinary tract infections and rectal prolapse with bothersome bleeding and fecal incontinence. During his preoperative evaluation, he was initially diagnosed with a prostatic utricle cyst on the basis of magnetic resonance imaging findings, which demonstrated a cystic, thick-walled mass with low signal contents that extended inferiorly to insert into the distal prostatic urethra. However, at the time of surgical resection, the thick-walled structure contained an old, firm fecaloma. The final pathology report described findings consistent with colonic tissue, suggesting a retained remnant of the original fistula and diverticulum.ConclusionsAlthough rare, persistent rectourethral fistula tracts and rectal diverticula after imperforate anus repair can cause symptoms decades later, requiring surgical intervention. This is an important diagnostic consideration for any adult patient with history of imperforate anus.

Gracilis Muscle and Myocutaneous Flap

Gracilis muscle is type II muscle flap. Total 16 cases were operated on with Gracilis muscle or myocutaneous flap. Group A – loss of scrotal skin following Fournier’s gangrene. Group B - Traumatic avulsion of the scrotal skin, Group C – Anal incontinence following Surgical repair of imperforate anus. Group D – Carcinoma penis operated for total amputation of the penis. Amputation of the penis. All the patients from Group A did well except for necrosis at the margin of the flaps in two patients. In Group B patient did well without any complication in Group C Three out of five patients did well. Two patients were still incontinent. In Group D, the first patient had a good flap for seven days. Later there was discolouration and on a ninth day, there was complete discolouration of the skin pedicle. Debridement was done, the muscle underneath was viable. A split-thickness skin graft was applied. The graft takes up was good. Within 10 days the muscle contracted too much an extent that the size of the phallus became almost nil.

Can complex surgical interventions be standardized? Reaching international consensus on posterior sagittal anorectoplasty using a modified-Delphi method

Background/PurposeIn an effort to standardize educational experience, address future physician shortages, and improve quality of care to patients, many surgical specialties are discussing how to maximize exposure to index cases. One solution being explored is telementoring, which requires a well-developed educational curriculum with intraoperative objectives. The American College of Surgery Telementoring Task Force selected anorectal malformation and posterior sagittal anorectoplasty (PSARP) for the repair of imperforate anus as the initial educational focus for this pilot. The purpose of this study was to obtain international consensus on intraoperative learning objectives for a complex surgical procedure. MethodsA multidisciplinary team of medical educators and pediatric surgery experts created an outline of essential curricular content and intraoperative learning objectives for PSARP in three clinical scenarios. Twelve international subject matter experts were identified meeting strict inclusion criteria. Intraoperative checklists were revised using the modified-Delphi process. ResultsAfter five rounds of modifications to the intraoperative checklists, international consensus was achieved for three different clinical scenarios requiring a PSARP: perineal or vestibular fistula, low prostatic fistula, and bladder neck fistula. ConclusionsA modified-Delphi approach was successful in generating guidelines for surgical techniques that can be used to standardize intraoperative teaching and expectations for trainees. Type of studyDiagnostic study Level of evidenceLevel V (expert opinion)

Robotic Rectopexy for Rectal Prolapse in Pediatric Patients

Rectal prolapse is the protrusion of the rectum out of the anus. Surgical correction can be accomplished via open and minimally invasive abdominal approaches, as well as from the perineum. Robotic rectopexy is an option for minimally invasive treatment of rectal prolapse. There are no studies that have established the efficacy of robotic rectopexy for rectal prolapse in the pediatric population. The aim of this study was to review the experience of robotic rectopexy at a single institution. This is a retrospective review of our pediatric robotic rectopexy experience from 2012 to 2015. Information was obtained from chart review of both operative notes and clinic visits. Four pediatric patients underwent a robotic rectopexy for rectal prolapse from 2012 to 2015. Three patients were male and one was female. The mean age was 15.5 years (range 13-17). Two patients had rectal prolapse with chronic constipation. One patient had rectal prolapse from Ehlers Danlos syndrome, and the last had rectal prolapse after imperforate anus repair as an infant. Three patients received a bowel preparation. Three patients were completed robotically, and one patient required conversion to an open procedure. The average postoperative length of stay was 3.25 days (range 2-4). There were no episodes of recurrent prolapse. Two patients had improvement in constipation, one had no improvement, and one had no documented change. Average postoperative follow-up was 11.5 months (range 3-29). This study was a review of one institution's experience with pediatric robotic rectopexy. With short-term follow-up, there was no recurrence of prolapse. Robotic rectopexy provided a safe, reliable, and short-term resolution of rectal prolapse in pediatric patients.

Intestinal-type Adenocarcinoma Possibly Arising from a Remnant of the Rectovaginal Fistula Tract Formed after Intermediate-level Imperforate Anus Repair

A 45-year-old Japanese woman, gravid 0, was referred to our hospital with a tentative diagnosis of primary vaginal adenocarcinoma. She complained of mucinous vaginal discharge, intermittent genital bleeding and long-term anal discomfort. She had a history of surgery for intermediate-level imperforate anus in her neonatal period, but we could not get any detailed information about her clinical history because of the loss of her parents in childhood. Pelvic examination revealed an exophytic hemorrhagic tumor filling the vagina. The tumor did not extend into the uterus, and no parametrial tissues or other pelvic organs were involved. She had experienced anal prolapse, which had been recognized about 25 years previously (Fig. 1). Barium enema study exhibited no abnormal findings. Pelvic magnetic resonance imaging studies showed a demarcated tumor 8 4 3 cm in size in a cavity just behind the vagina (Fig. 2, white arrowheads). The tumor filled the vaginal cavity, reaching the introitus (Fig. 2, closed star), but did not contact the rectum (Fig. 2, black asterisk). Positron emission tomography-computed tomography (PET-CT) studies depicted no distant metastasis. Serum carcinoembryonic antigen and sialyl Tn antigen levels were elevated as high as 6.4 ng/ml and 140 U/ml, respectively. The histopathological diagnosis of biopsy specimens was adenocarcinoma. Immunohistochemical staining demonstrated that the tumor tissues were positive for CK7 and CK20. With the preoperative diagnosis of bulky primary adenocarcinoma of the vagina with FIGO stage I and severe anal prolapse, the patient underwent total pelvic exenteration followed by ileal conduit and permanent colostomy. We adopted this procedure including radical hysterectomy with extended total vaginectomy, cystourethrectomy and pelvic lymphadenectomy, firstly, because there is no well-established alternative or adjuvant therapy for vaginal adenocarcinoma and, secondly, because radical hysterectomy with extended total vaginectomy or posterior exenteration frequently denervates the urinary bladder resulting in severe voiding dysfunction. Histopathological diagnosis of the tumor was intestinal-type adenocarcinoma with invasion into the vagina and pelvic adipose tissue. The excisional margin was negative. The tumor was considered to have originated from the intestinal epithelium overlaying a cavity located behind the vagina. The cavity was partly surrounded by muscle layers similar to those of the intestine (Fig. 3). Therefore, we concluded that the tumor had arisen from a remnant of the rectovaginal fistula tract formed 45 years previously after the intermediate-level imperforate anus repair. PET-CT 1 year after the surgery revealed multiple lung metastases. She has been undergoing systemic chemotherapy since then for about 3 years.

A case of successful vaginal delivery in a patient with a repair of an imperforate anus

As a general recommendation, women with a repair of an imperforate anus are candidates for vaginal deliveries if no contraindications exist. The adequacy (size and scarring) of the perineal body should be assessed by the obstetricians before making that decision. Together with a brief review of literature, we report a successful case of vaginal delivery in a woman with a repair of imperforate anus.

Time Course of C-Reactive Protein and Inflammatory Mediators after Neonatal Surgery

To characterize the perioperative course of C-reactive protein (CRP) and inflammatory mediators in neonates ≤44 weeks' corrected gestational age. Prospective study of CRP and inflammatory mediators interleukin (IL)-1β, IL-6, IL-8, IL-10, and tumor necrosis factor-α in 55 neonates undergoing thoracic or abdominal surgery. In the absence of infection, CRP increased after surgery, peaking on post-operative day 2. The perioperative patterns of CRP differed by diagnosis and inflammatory state. Surgery alone did not cause an increase in CRP because in 13 of 55 infants (24%), CRP remained <1.0 mg/dL at all time points. For thoracic procedures, patent ductus arteriosus ligation showed the least post-operative increase in CRP, and patients undergoing repair of congenital diaphragmatic hernia or tracheoesophageal fistula had a greater response. Abdominal procedures with low CRP response included repair of imperforate anus and pyloric stenosis, while gastroschisis repair and bowel reanastomosis after necrotizing enterocolitis were accompanied by a robust CRP response. IL-6 concentrations peaked on post-operative day 1 and correlated with the post-operative day 2 CRP peak (r=0.398, P=.004). The additional inflammatory mediators measured were not informative. The range and time course of perioperative CRP differ by diagnosis. Serial measurements may be more informative than CRP magnitude.

Transvesical resection of a mucocele after laparoscopically assisted anorectal pull-through for imperforate anus with rectobulbar urethral fistula

Mucocele formation is a rare complication after imperforate anus repair. Abdominal cyst enucleation or resection, transrectal resection by a posterior sagittal approach, and perineal cyst resection are the standard surgical options. The authors report the first patient who underwent transvesical mucocele resection.

Myoblast transplantation to defecation muscles in a rat model: a possible treatment strategy for fecal incontinence after the repair of imperforate anus

Infants with higher anorectal anomalies often develop fecal incontinence after surgical reconstruction mainly due to the incomplete development of defecation muscles. We investigated the possibility of defecation muscle regeneration by myoblast transplantation to improve fecal continence. Myoblasts from F344 female rats at ages of 1 day, 1, 2, 3, 4, 8, and 12 weeks were prepared by a preplating method. In vivo muscle differentiation of myoblasts was evaluated using immunofluorescence after transplantation of GFP-positive myoblasts into nude mice, the damaged thigh muscles, and the levator ani muscle of GFP-negative rats. The ratios of myoblasts obtained from 1 day, 1, 2, 3, 4, 8, and 12-week-old rats were 35, 71, 65, 61, 52, 44, and 23%, respectively. Myotube formation by transplanted myoblasts was observed in the back of nude mice. Myoblasts transplanted into damaged thigh muscles were integrated into recipient muscles with myofiber formation. Transferred myoblasts formed myotubes surrounding the levator ani muscle, although myofiber formation was not observed. Myoblasts were most efficiently obtained from juvenile rats. Myoblast transplantation may provide a novel treatment strategy for improving fecal continence after repair of anorectal anomalies in infants.

Minimal Access Surgery in the Neonate

After completing this article, readers should be able to: 1. Review physiologic, anatomic, and technical issues related to minimal access surgery in the neonate. 2. Describe conditions in which minimal access surgery can be used in neonates. Historically, pediatric surgeons were slow to adapt to minimal access surgery (MAS) techniques compared with the adult surgical community. However, MAS now is widely established in infants and children. Differences in size and physiology have necessitated a number of surgical and technological modifications to apply MAS techniques to this population. This review outlines the important differences between children and adults with respect to MAS and provides examples of the use of MAS for diseases seen in infants. ### Physiologic Issues The physiologic response to pneumoperitoneum in children is more pronounced than in adults. Peritoneal insufflation with carbon dioxide has been shown to cause hypercarbia, acidemia, and decreased oxygenation in the pediatric piglet model. (1) In infants, however, there is no detrimental effect on blood pressure, heart rate, or oxygen saturation during short periods of pneumoperitoneum, and elevations in end-tidal CO2 can be counteracted readily by increasing the minute ventilation. (2) Hypercarbia-induced pneumoperitoneum in infants is associated with changes in cerebral blood flow and cardiac output. Nevertheless, laparoscopic procedures have been performed safely with proper anesthetic management in children who have even severe congenital cardiac abnormalities such as hypoplastic left heart syndrome. (3) Hypothermia also can be a concern due to rapid insufflation of unwarmed CO2 gas, mandating the moderation of flow rates. (4) ### Anatomic Issues A number of anatomic factors set children apart from adults and must be accounted for when performing laparoscopic surgery in this age group. Young children and infants have thinner abdominal walls than adults, so extreme care must be exercised when placing trocars. Due to the small size of the abdomen, the working …

Thoracoscopic Repair of Esophageal Atresia and Tracheoesophageal Fistula

For the past 60 years, successful repair of esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) has been performed via a thoracotomy. However, a number of reports have described adverse musculoskeletal sequelae following thoracotomy in infants and young children. Until now, only a few scattered case reports have detailed an individual surgeon's success with thoracoscopic repair of EA/TEF. This multi-institutional review represents the largest experience describing the results with this approach. A cohort of international pediatric surgeons from centers that perform advanced laparoscopic and thoracoscopic operations in infants and children retrospectively reviewed their data on primary thoracoscopic repair in 104 newborns with EA/TEF. Newborns with EA without a distal TEF or those with an isolated TEF without EA were excluded. In these 104 patients, the mean age at operation was 1.2 days (+/-1.1), the mean weight was 2.6 kg (+/-0.5), the mean operative time was 129.9 minutes (+/-55.5), the mean days of mechanical ventilation were 3.6 (+/-5.8), and the mean days of total hospitalization were 18.1 (+/-18.6). Twelve (11.5%) infants developed an early leak or stricture at the anastomosis and 33 (31.7%) required esophageal dilatation at least once. Five operations (4.8%) were converted to an open thoracotomy and one was staged due to a long gap between the 2 esophageal segments. Twenty-five newborns (24.0%) later required a laparoscopic fundoplication. A recurrent fistula between the esophagus and trachea developed in 2 infants (1.9%). A number of other operations were required in these patients, including imperforate anus repair in 10 patients (7 high, 3 low), aortopexy (7), laparoscopic duodenal atresia repair (4), and various major cardiac operations (5). Three patients died, one related to the EA/TEF on the 20th postoperative day. The thoracoscopic repair of EA/TEF represents a natural evolution in the operative correction of this complicated congenital anomaly and can be safely performed by experienced endoscopic surgeons. The results presented are comparable to previous reports of babies undergoing repair through a thoracotomy. Based on the associated musculoskeletal problems following thoracotomy, there will likely be long-term benefits for babies with this anomaly undergoing the thoracoscopic repair.

Psychosocial liaison-consultation for the children who have undergone repair of imperforate anus and Hirschsprung disease

Objective The aim of this study was to investigate the psychological status of Japanese children with congenital anorectal malformation and their mothers to develop appropriate psychiatric interventions. Method The subjects comprised 50 children with congenital anorectal malformation aged 0 to 16 years and their mothers. The psychology of children aged 7 to 16 years was investigated by Kovacs' Children's Depression Inventory (CDI). The psychology of their mothers was assessed by Spielberger's State-Trait Anxiety Index (STAI) and Zung's Self-rating Depression Scale (SDS). Results Depression was more marked in the children aged 12 to 16 years than in those aged 7 to 11 years according to the CDI. The mothers of preschool children showed higher levels of anxiety and depression than those of school-aged children, according to the STAI and SDS. Significant correlations between the CDI score of the children and the STAI or SDS score of the mothers were observed only among children aged 7 to 11 years. Conclusions The frequency of depression and anxiety among children with congenital anorectal malformation and that among their mothers was associated with the age of the child. Long-term postoperative psychosocial support for the children and their mothers may be required, taking into account the age of the child.

Hirschprung’s disease and imperforate anus in pallister-hall syndrome: a new association

Hirschprung’s disease and imperforate anus are described concurrently in a newborn with Pallister-Hall syndrome as well as the difficulties in making this diagnosis. Awareness of this new association should prompt the exclusion of Hirschprung’s disease before repair of imperforate anus in infants with Pallister-Hall syndrome. The known genetic parallels between these conditions is discussed briefly in terms of etiology.

Colonic motility in children with repaired imperforate anus.

Following surgical correction of imperforate anus, voluntary bowel control is frequently poor because of abnormal anorectal function. Using colonic manometry we investigated the role of colonic motility in the pathogenesis of fecal soiling in children following imperforate anus repair. Thirteen children with repaired imperforate anus and fecal soiling underwent motility testing 2-12 years after anoplasty. All had fecal incontinence unresponsive to conventional medical treatment. Colonic manometry was performed using water-perfused catheters. Anorectal manometry was undertaken in 10 patients. Motility study results, treatment and outcomes were compared. All patients had high-amplitude propagating contractions (HAPCs) with an average of 80% propagation into the neorectum. There was no correlation between HAPC number or morphology and any variable. Internal anal sphincter resting pressure was low in 6/10 patients. Relaxation of the internal anal sphincter was present in 6/10 children. Only 1 of 5 patients able to cooperate was capable of generating a normal maximal squeeze pressure. Therapeutic regimens were changed in 11 patients with clinical improvement in five. Fecal soiling in patients with repaired imperforate anus is a multifactorial problem including propagation of excessive numbers of HAPCs into the neorectum as well as internal anal sphincter dysfunction. Colonic manometry in conjunction with anorectal manometry aids in the understanding of the pathophysiology of fecal soiling and guides clinical management in children with repaired imperforate anus.

Simplified technique for antegrade continence enemas for fecal retention and incontinence

Fecal incontinence and chronic constipation occur with annoying frequency after reconstructive colorectal operations for Hirschsprung’s disease and imperforate anus in childhood. Chronic constipation is a frequent occurrence that complicates the care of children with spina bifida and of neurologically impaired patients of all ages. Fecal incontinence is devastating to the self-esteem of a child, depriving him or her from socializing with peers and limiting participation in most vigorous sports activities. A bowel-management program with use of a daily enema, manipulation of the diet, and use of some medications has been helpful in reducing incontinence in several children after repair of imperforate anus. Chronic use of rectal enemas, suppositories, laxatives, or lubricants, however, is often uncomfortable, poorly accepted, and intolerable to the patient, and cumbersome and disruptive to other members of the family who are responsible for the child’s care. Since its first description by Malone and associates in 1990, the antegrade continence enema (ACE) has been used with increasing frequency by surgeons for the management of children with fecal incontinence or retention. The technique entails construction of a permanent, nonrefluxing appendiceal stoma in the abdominal wall through which a catheter can be inserted to deliver enema solutions in an antegrade manner on a daily basis, as desired. This operative procedure is somewhat extensive for the patient who requires only the instillation of enema solutions into the proximal colon and has caused skin irritation, granulation tissue with stenosis, and occasional fecal leakage in five of the children in whom it was used in our hospital. Griffiths and Malone reported that 80% of their patients had some type of complication, either minor or major, and that 5 of 21 patients required a colostomy. Other investigators have noted several late complications after the Malone procedure. To reduce these complications, several modifications of the continent appendicostomy have been reported by other groups. To simplify the operation, Webb and associates recommended laparoscopic construction of a cutaneous appendicostomy for antegrade enemas. Chait and associates have performed percutaneous insertion of a low-profile cecostomy catheter or “button” under local anesthesia as a two-stage procedure for antegrade colonic cleansing in several children, with good success. The present study summarizes our clinical experience at the University of California, Los Angeles Medical Center in the management of 24 children with fecal incontinence or constipation using a simplified surgical technique. In this procedure, a Broviac silicone elastomer intravenous (IV) catheter was placed in the cecum for ACE infusions.

Bowel management for fecal incontinence in patients with anorectal malformations

Background/Purpose: Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. Methods: Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. Results: Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. Conclusion: Bowel management consisting of enemas, laxatives, and medications is successful when administered in an organized manner. It is vital to determine the type of fecal incontinence from which the patients suffer and to target their treatment accordingly.

The use of augmented-pressure colostography in imperforate anus.

Colostomy is recommended in most newborns with imperforate anus (IA), except those with low-type anorectal malformations (ARM). Accurate demonstration of the anatomy of any associated fistula between the rectum and the urogenital tract is essential for optimal surgical management. Augmented-pressure distal colostography is recommended to fulfill this requirement prior to definitive surgical repair of IA. We examined 12 cases of high or intermediate-type ARM using this technique, which clearly demonstrated the distal perineal anatomy. All were confirmed at posterior sagittal anorectoplasty.

Hirschsprung's disease, imperforate anus, and Down's syndrome: A case report

Patients with trisomy 21 have a higher incidence of several gastrointestinal anomalies. However, the coexistence of imperforate anus, Hirschsprung's disease, and trisomy 21 had not been reported previously. This report describes the case of an infant girl born with trisomy 21 and imperforate anus, without a fistula, who presented with bowel obstruction 3 months after anoplasty. The obstruction was attributable to Hirschsprung's disease. This was managed by a leveling colostomy in the descending colon, followed by an endorectal pull-through after 4 weeks. She has a normal stooling pattern 11 months after colostomy closure. Hirschsprung's disease should be suspected in infants with trisomy 21 who have constipation after repair of imperforate anus. The authors believe that the endorectal pull-through is the safest technique to use for Hirschsprung's disease after a previous anoplasty.

The atonic baggy rectum: A cause of intractable obstipation after imperforate anus repair

Three children with successful imperforate anus repairs had chronic, intractable obstipation and recurrent fecal impaction refractory to diligent medical therapy. All had massive stool-filled rectums up to 14 cm in diameter on abdominal radiographs. They responded well to resection of the baggy atonic rectum with low anterior anastomosis. The massively dilated atonic rectal pouch in some children with repaired imperforate anus can demonstrate abnormal motility characterized by poor peristalsis and propulsion. This results in recurrent obstipation, impaction, and soiling refractory to the usually successful medical management.

Advantages of performing the sagittal anoplasty operation for imperforate anus at birth

The development of the posterior sagittal anorectoplasty approach has been a major recent advance in the repair of imperforate anus. It has been found that sagittal anoplasty can easily and preferably be carried out in the newborn period without the need for colostomy or “tapering”. It is suggested that the perineal sagittal approach be attempted first, with the infant positioned so that the abdominal part of the abdominoperineal approach can be used if necessary—this seldom may be required. Neonatal closure of urinary tract fistulas in boys is an added attractive feature of this approach. The importance of optical magnification (microsurgery) and excellent, intense lighting of the perineal area with a headlamp is stressed for this approach in the newborn, particularly for the management of high pouches and high fistulas. This operation at birth relieves alimentary tract obstruction at birth, eliminates urinary tract contamination (when it exists) at birth, establishes anorectal continuity and maximum potential for “normal” defecation reflexes at birth, and achieves all of this in one rather than three operations.