Surgical Correction Of Anorectal Malformations Research Articles

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.

Complications after Surgical Correction of Anorectal Malformations

Complications after Surgical Correction of Anorectal Malformations

COMPARATIVE ASSESSMENT OF SPINCTER MUSCLE COMPLEX IN CHILDREN AFTER SURGICALCORRECTION OF ANORECTAL MALFORMATION

A comprehensive study of the state of the spincter muscle complex in children after surgical correction of anorectal malformations was carried out,including MRI of the structure of the spincter muscle complex,fecal continence on the Rintala scale, manometric studies of the anal function.The poor development of the spincter muscle complexwas characterized by a decrease (p < 0,05) of the average values of the relative width of the puborectal muscle (PRWR) to 0,14 ± 0,03, for the relative width of the external anal sphincter (EASWR) to 0,12 ± 0,02, which turned out to be 2,4 times lower than patients with excellent and good results. Values of PRWR 0,15.

Vaginal Reconstruction with Native Rectovestibular Fistula in an Infant with Vaginal Agenesis and Imperforate Anus

Vaginal agenesis is a congenital anomaly which occurs as isolated developmental defect or as a part of complex anomalies. Vaginal anomalies associated with anorectal malformations are usually diagnosed and repaired in time of surgical correction of anorectal malformations during the infancy period. Herein a case with vaginal agenesis and recto-vestibular fistula, who underwent a neo-vaginal reconstruction with distal segment of fistula during the posterior sagittal anorectoplasty was presented. The use of recto-vestibular fistula as a neo-vagina in cases with vaginal agenesis combined imperforate anus seems to be a feasible and effective approach that has satisfactory anatomical and functional outcomes for vaginal reconstructions.

Healthcare Utilization and Comorbidities Associated with Anorectal Malformations in the United States

To determine nationwide prevalence and healthcare utilization in children with anorectal malformations and associated anomalies over a 6-year period. We used the Kids' Inpatient Database for the years 2006, 2009, and 2012 for data collection. International Classification of Diseases, Ninth Revision codes were used to identify patients with anorectal malformations and associated anomalies. A total of 2396 children <2 years of age with anorectal malformations were identified using weighted analysis; 54.3% of subjects were male. The ethnic subgroups were 40.1% white, 23.6% Hispanic, 9.3% African American, and 27% other ethnicity. Other congenital anomalies were reported in 80% of anorectal malformations and were closely associated with increased length of stay and costs. A genetic disorder was identified in 14.1% of the sample. Urogenital anomalies were present in 38.5%, heart anomalies in 21.2%, and 8.6% had vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and limb defects association. Anorectal malformations with other anomalies including vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and limb defects association incurred significant hospital charges when compared with anorectal malformations alone. The average annual healthcare expenditure for surgical correction of anorectal malformations and associated anomalies for the 3 years was US $45.5 million. This large, major nationally representative study shows that majority of children with anorectal malformations have additional congenital anomalies that deserve prompt recognition. The high complexity and need for lifelong multidisciplinary management is associated with substantial healthcare expenditure. This information complements future healthcare resource allocation and planning for management of children with anorectal malformations.

Sphincter‐saving anorectoplasty for correction of anorectal malformations

AimPosterior sagittal anorectoplasty (PSARP) popularized by de Vries and Peña has become the preferred technique for surgical correction of anorectal malformations (ARM). Despite performing a technically perfect operation, it involves midline division of the sphincter with a subset of patients requiring significant lifelong bowel management for constipation or incontinence. Pratap et al. introduced sphincter‐saving anorectoplasty (SSARP) trying to spare the sphincter. The aim of this study is to preserve the neurophysiological function of the sphincter through SSARP in ARM patients and evaluate the outcome of these patients through this approach.Patients and MethodsThis prospective study conducted on ARM cases presented with high or intermediate anomalies. Patients with sacral agenesis and rectovesical fistula were excluded from the study. All patients underwent pre and postoperative electrophysiological studies of the external anal sphincter. Also a guide wire was introduced through the middle of the sphincter under sonographic guidance in all patients immediately before the operation.ResultsDuring the study period from January 2011 to September 2014 18 cases underwent surgical correction with SSARP technique. Their age ranged from 6 months to 5 years and weight ranged from 7 kg to 20 kg. The total duration of the SSARP procedure ranged from 65 to 90 min. There were no intra‐operative complications or mortality. No conversion to classic PSARP approach was necessary in any case. During the follow‐up period no recurrent urethral fistula or urinary retention was observed.ConclusionSSARP technique is a safe and effective approach in correction of ARM with acceptable postoperative complications.

Effects of Reconstructive Surgery on Bladder Function in Patients with Anorectal Malformations

Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. The charts of all 341 patients who underwent surgery atour center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in24(63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latestfollowup. Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

Validation of the Baylor Continence Scale in children with anorectal malformations

Background Anorectal malformations (ARMs) often result in lifelong problems with defecation. There have been no studies performed to develop scoring systems in children with fecal incontinence following repair of ARM. This study was designed to develop and validate a tool (Baylor Continence Scale [BCS]) to measure social continence in children after surgical correction of ARMs. Methods The BCS is a 23-question survey, was administered to children who have had repair of an ARM, children with enuresis, and normal children. Results Children in the ARM group had an average BCS score of 28.0, which was statistically different from an enuresis control group (16.5) and normal controls (11.5) ( P < .05). A significantly greater proportion of children in the ARM group had higher Impact on Family Scores (>30) than either the enuresis or normal control group ( P < .05). Conclusions The BCS tool should be useful in prospective studies of interventions to improve incontinence after repair of an ARM. An important secondary result of this study was to demonstrate the effect on families of caring for a child with imperforate anus, even years after the repair.

Use of biofeedback (BFB) in the treatment of fecal incontinence after surgical correction of anorectal malformations by posterior sagittal anorectoplasty (PSARP).

To evaluate biofeedback (BFB) responses to rehabilitation techniques and physical exercises in incontinent or partially continent anorectal malformations patients after posterior sagital anorectoplasty (PSARP). Prospective study. Pediatric Surgery-Department of Surgery-UNIFESP-EPM. The authors report on 14 patients with anorectal malformations (4 with partial fecal incontinence after primary PSARP; 6 with fecal incontinence after primary PSARP; 3 with partial fecal incontinence after secondary PSARP; and 1 with fecal incontinence after secondary PSARP). All patients were rehabilitated via a BFB program of exercises in order to improve the function of the anal sphincteric muscular complex for a period of 1-3 years. Clinical and manometric control. After BFB, of 4 partially continent patients after primary PSARP, 3 became continent; of 6 incontinent patients after primary PSARP, 4 became continent; of 3 partially continent patients after secondary PSARP, 1 became continent, 1 showed no improvement and 1 became incontinent (infection + abscess + fibrosis + important anorectal stenosis). The incontinent patient after secondary PSARP showed no improvement. The authors concluded that BFB, used at the appropriate time with patient collaboration, is an important complement to the anatomical reconstruction of anorectal malformations in order to achieve good development and contractile functioning of the sphincteric muscular complex.

Operative trauma to the genitourinary tract in the treatment of anorectal malformations: 1 5 years' experience

To determine the incidence of urologic trauma during surgical correction of anorectal malformations (ARM). The records of 228 children with ARM were retrospectively reviewed. Operative trauma to the genitourinary tract was seen in 12 patients, all male. Of 133 patients with a high ARM, 92 underwent an abdominoperineal pull-through and 41 underwent a posterior sagittal anorectoplasty (PSARP); the incidence of urologic trauma was 12% and 0%, respectively. In the subgroup of male infants with a high ARM (99 patients), the incidence of genitourinary trauma was 11%; in those with a low anomaly, the incidence was 1 of 43 (2%). There were 3 urethral tears, 4 urethral strictures, 5 urethral diverticula, 2 vas deferens injuries, and 1 ureteric injury. Nine of the 12 patients do not have any long-term sequelae resulting from the injuries; however, 2 are now infertile and 1 has urinary incontinence. Four patients are additionally compromised because of an absent or nonfunctioning kidney on one side. In children with ARM, those undergoing a conventional pull-through repair are more likely to have a genitourinary injury than those undergoing the PSARP, and boys with high ARM are at greatest risk.

Voluntary anal continence after surgery for anorectal malformations

Electromyography (EMG) and measurement of voluntary contraction pressure of the external sphincter muscle were performed in 28 patients, aged 5 to 14 years, to assess the function of the external sphincter after surgical correction of anorectal malformations. Ten normal children, aged 5 to 15 years, served as controls. External sphincter function in patients with high-type anomalies was disturbed in the areas of tonic activity, inflation reflex, and activity during further rectal filling. In patients with low-type or intermediate-type anomalies, function was preserved and was equal to that in normal controls. Phasic activity was observed in patients with all types of anomalies and in normal subjects. Among those with high-type anomalies, the three patients with Kelly's score of less than 2 had voluntary contractions of 20 cmH2O or less. However, the mean voluntary contraction pressures were not significantly different among the three types of anomalies. Therefore, patients with high-type anomalies may acquire compensatory voluntary continence through bowel training.

Results of surgical correction of anorectal malformations. A 10-30 year follow-up.

Of 119 patients with surgical correction of anorectal malformations, 47 who were 10-30 years of age were interviewed personally and had manometric studies to evaluate postoperative continence. This clinical study included not only long-term anorectal function but also sexual function. Patients with low type anomalies or with intermediate type anomalies were more likely to be continent, whereas patients with high type lesions had some problems with continence. However, only two of the 16 patients (12%) with high type anomalies were classified as having poor results. This rate is perhaps lower than might be expected. Thus, incontinent patients may become continent even if they were classified as having fecal incontinence before 6 years of age. Most patients who were 15-30 years of age had normal sexual function except for two females with irregular menstruation. These results indicate that achievement of fecal continence and sexual function in patients with high type anomalies treated by abdominoperineal rectoplasty depends on careful dissection as close as possible to the rectal wall and bringing the terminal bowel down exactly within the sling of the puborectal muscle.

Comparison of results of anorectal manometry performed after surgery for anorectal malformations and repeated three years later.

Anorectal manometry was performed in 27 patients 2 to 16 years after surgical correction of anorectal malformations and again 3 years after the initial examination. Further clinical improvement from faecal incontinence to normal bowel habits was observed in 3 of 11 patients with high anomaly. All of these 3 patients received special physiotherapeutic training from 3 months to 1 year during the two examinations. At the second examination these 3 patients had an anorectal pressure difference of more than 10 cmH2O, which is almost normal, and one of the 3 patients had developed an anorectal reflex. These results suggest that the functioning puborectalis muscle, which had been left intact during surgery, might be reinforced by physiotherapeutic training.

Long-term results after surgical correction of anorectal malformations.

Our surgical experience with 101 cases of anorectal malformations during the past 21 years includes staged abdominoperineal rectoplasty in 49 patients, perineoplasty in 35 patients, and permanent colostomy in 2 patients. The observations of this clinical study are especially focused on long-term anal continence. The functional results were assessed clinically on the basis of Kelly's criteria (5), and by manometric and radiologic studies. Fifty-one patients, aged 5-25 years, were personally interviewed and 36 of these 51 had manometric and radiologic studies to evaluate postoperative continence. Patients with low-type lesions were more likely to be continent, while patients with high-type lesions had more problems with continence, because a functional internal sphincter is absent in high-type lesions. However, continent patients with high-type lesions as well as those with low-type lesions characteristically had a marked high-pressure zone in the anal canal and an acute anorectal angulation in the defecogram . On the other hand, in the patients with fair or poor results, neither a high-pressure zone in the anal canal nor marked anorectal angulation was found. These results indicate that achievement of continence in patients with a high-type anomaly treated by abdominoperineal rectoplasty depends upon bringing the terminal bowel down exactly within the sling of the puborectalis and careful dilatation of the new anal canal after the creation of a cosmetically satisfactory anal opening.

A clinical and manometric correlation for assessment of postoperative continence in imperforate anus

Functional results after surgical correction of anorectal malformations were assessed on a clinical basis following Kelly score and by manometric study. Forty-six patients, aged 2--17, were personally interviewed and 25 of these 46 had manometric studies to evaluate postoperative continence. The manometric study was also performed on 35 normal children as a control group. Continent patients characteristically had marked high pressure zones as did the normal subjects. On the other hand, in the patients with fair or poor results, the anorectal pressure profile had no marked high pressure zone in the anal canal. The presence of normal anal pressure at rest as well as adequate ano-rectal pressure difference was found to correlate well with continence. In patients with perineoplasty, the ano-rectal reflex correlated well with continence but not in patients treated by abdominoperineal rectoplasty.

A clinical evaluation of the surgical treatment of anorectal malformations (imperforate anus).

The surgical correction of anorectal malformations remains a serious problem despite their relatively frequent occurrence. Our surgical experience with 90 patients, seen in the past ten years, includes abdominal-perineal or sacro-abdominal-perineal repair in 28 patients, complete perineal anoplasty in 13 patients, and "cut-back" perineal anoplasty in 38 patients. Secondary surgical procedures for "fecal reservoir syndrome" (seven patients), revision for stricture (11 patients) and excision of redundant mucous membrane (ten patients). The observations made from this clinical study are: 1) The importance of thorough urological and neurological evaluation of "high" abnormalities and the value of the cremasteric and bulbocavernosus reflexes as indicators of sacral innervation. 2) Increasing satisfaction with the "cut-back" anoplasty as a definitive procedure or as a temporary stage in low recto-vaginal or recto-vestibular fistulas. The technique for the "cut-back" is improved by the use of Burow's triangles and the use of nonabsorbable sutures. 3) The advisability of the complete perineal anoplasty with posterior positioning of the anus and construction of a perineal body in patients with low recto-vaginal and recto-vestibular fistulas. 4) A concern over the functional capacity of the distal bowel segment in high abnormalities. This is emphasized by the experience with six secondary resections of the rectosigmoid or left colon for "fecal reservoir syndrome." 5) Heartening results with secondary operations for stricture and redundant mucous membrane which suggest the desirability of an earlier surgical approach to these complications.

The Anomanometer: Its Use in the Assessment of Continence and in the Diagnosis of Hirschsprung's Disease

The purpose of this paper is to describe the anomanometer, a versatile instrument, portable and easy to use. It has been used for (a) assessment of continence after surgery for anorectal malformations; and (b) the diagnosis of Hirschsprung's disease in children. The measurement of the resting and voluntary squeeze pressures provides the criteria upon which it is possible to quantify continence after surgical correction of anorectal malformations. The anomanometer records the biphasic response of the sphincters to rectal distension in normal children and the lack of the relaxation phase in Hirschsprung's disease, thus providing a convenient and simple method of diagnosis.SummaryA portable anomanometer has been devised and has proved useful for the purposes of (a) assessment of continence after surgery for anorectal anomalies, and (b) the diagnosis of Hirschsprung's disease.It consists of a single air‐filled tambour‐type balloon mounted on a Perspex probe and connected through a transducer to an analogue readout, so that changes of pressure in the balloon induced by the sphincter muscles of the anal canal are registered as deflections of the needle of the meter.Two measurements obtained by the anomanometer provide criteria of postoperative anal sphincter function, namely the resting tone of the sphincter muscle and the voluntary squeeze pressure. Children who had had surgical reconstruction of the anal canal for supralevator, intermediate and translevator abnormalities were tested. A combination of a resting tone of 20 mm Hg with a voluntary squeeze pressure of 30 mm Hg or more was associated with an excellent state of continence, whereas the combination of a resting tone below 15 mm Hg and a voluntary squeeze less than 30 mm Hg corresponded with a clinical state of socially unacceptable incontinence. Those children with borderline resting tone of the sphincter and voluntary squeeze pressures above 30 mm Hg have varied degrees of impaired sphincter function, which may be improved in some by hardening of the fæces, education or the lapse of time.The anomanometer detected in a very reliable manner the anal responses to rectal distension, and in particular the lack of reflex relaxation of the internal sphincter in patients with Hirschsprung's disease. It proved to be a simple economical apparatus not only for the detection of Hirschsprung's disease, but also for eliminating this possibility in many patients.