Long-term follow-up results of the treatment of high and intermediate anorectal malformations using a modified technique of posterior sagittal anorectoplasty.

The aim of this study was to evaluate the long-term follow-up result of a modified technique in the operation for high and intermediate anorectal malformations. The operation was performed by a posterior sagittal approach with three modifications: The external sphincter complex was not opened on the posterior side, the rectal pouch was not tapered, the dissection was performed outside the rectal pouch. From January 1984 to December 1992, 70 cases were operated on including 55 boys and 15 girls. There was one postoperative death during the first 48 hours due to malignant hyperthermia and one late death due to an unrelated disease. A follow-up result was obtained in 48 of 68 survivors (71 %) with follow-up ranging from 13 months to 10 years 11 months (mean: 72 months). According to the modified Wingspread criteria, 2 patients (4 %) had very good anorectal function, 26 children (54 %) had good anorectal function, 18 children (38 %) had fair anorectal function, and only two children (4 %) had poor anorectal function. All children had urinary continence and all boys had a normal erection. The long-term follow-up result was satisfactory. Two important factors affecting the anorectal function were the type of malformations and the thickness of the external sphincter complex.     

Long-term continence after surgery for anorectal malformations

Out of a total of 1030 infants treated for anorectal malformations, 403 were diagnosed as having high and intermediate types of anomaly: 190 completed all stages of reconstruction; of these for 139 it had been 5 years to more than 15 years since reconstruction. They were recalled for follow-up examinations.Faecal continence was assessed in 84 of these children who came for review. Assessment was by clinical examination and manometry. Sixteen children had abdominoperineal reconstruction and 25 sacroabdominoperineal and 43 sacroperineal reconstruction. Sacroperineal reconstruction resulted in good fecal continence. The results after sacroabdominoperineal operation were slightly poorer. There was a direct correlation between manometric assessment and clinical assessment. Furthermore, manometry allowed a prognosis to be established and the need for reoperation to be determined. Offprint requests to: K. K. Varma     

Bowel function after surgery for anorectal malformations in patients with tethered spinal cord

Tethered spinal cord (TC) is an anomaly frequently recognized in association with anorectal malformations (ARM). However, the influence of TC on bowel function in children with ARM remains unknown. Furthermore, there are few studies that have assessed anorectal function in children with ARM and TC. The aim of this study was to evaluate anorectal function in ARM patients with TC using clinical assessment and anorectal manometry. Among 258 patients with ARM, this retrospective investigation included 35 patients who underwent spinal magnetic resonance imaging (MRI) after surgery for ARM. The patients were divided into two groups based on the presence or absence of TC, and bowel function was assessed by Kelly’s clinical score and anorectal manometry. Tethered cord was found in nine of the 35 patients (26%) with ARM. Of the ARM patients, TC was noted in four of 11 (36%) with high type anomalies, one of 8 (13%) with intermediate type anomalies, two of 14 (14%) with low type anomalies, and two of two patients (100%) with cloacal anomalies. Kelly’s clinical score did not significantly differ between the two groups. However, two of the nine patients with TC had poor bowel function (Kelly’s score; 2–0 points). On the contrary, patients without TC did not have poor bowel function. Anorectal manometry did not show a significant difference between patients with and without TC. However, the two patients with TC who had poor bowel function by Kelly’s score had low anal resting pressure, which was essential for achieving fecal continence. In conclusion, the present study showed that tethered cord was more frequently found in patients with more severe anorectal anomalies. Patients with TC were more likely to have poor bowel function, but this did not reach statistical significance. Presented at the 14th International Paediatric Colorectal Club, York, UK, 14–16 July 2007.     

Postoperative anorectal manometric evaluation of patients with anorectal malformation

BACKGROUND: Fecal incontinence is a common problem after reconstructive surgery for anorectal malformations. The aim of this study was to investigate the effectiveness of clinical scores and anorectal manometry in patients, who have been operated on for anorectal malformations. METHODS: In total, 18 patients who underwent surgery for anorectal malformation between 1999 and 2004 were investigated for anal continence. For the assessment of the patients' continence, Kelly's clinical scoring, Kiesewetter-Chang scoring, and anorectal manometry were used. RESULTS: In the intermediate level anorectal malformations, average anal resting pressure was found as 58.16 +/- 8.14 cmH(2)O and in high level anorectal malformations was found as 40.16 +/- 17.4 cmH(2)O. In the continence score, good according to Kelly and Kiesewetter-Chang scoring systems was an average anal resting pressure value of 57.92 +/- 8.57 cmH(2)O and in fair or bad was found as 32 +/- 12.83 cmH(2)O. There were significant differences between the scoring systems anorectal malformation level, and average anal resting pressure values (P < 0.05). CONCLUSIONS: Anorectal manometric evaluation of the patients in postoperative period with anorectal malformation can give more realistic information about the patient continence status in anorectal malformations.     

Anorectal malformations — results of treatment and long-term follow-up in 208 patients

During a 20-year period (1964–1983) 208 patients with anorectal malformations (143 males, 65 females) were treated; 98 had low and 110 had high or intermediate malformations. Associated anomalies were detected in 67.8%. The overall mortality in the series was 14.4%; most deaths were related to associated anomalies and/or prematurity. Four different procedures were used for high and intermediate anomalies: (1) abdominoperineal (18 cases); (2) sacroabdominoperineal (38); (3) sacroperineal (8); and (4) posterior sagittal anorectoplasty (7). Neonatal anoplasty was used to correct male low anomalies. Female low and intermediate anomalies with vestibular fistula were treated with anal transposition. More than one-half of the patients with high or intermediate anomalies had complications related to surgical therapy. Major secondary surgery was performed in 41 cases (12 resections of megarectum, 29 late sphincter reconstructions). Of the living patients, 90% (159/178) were followed up for 5–25 years. A majority (93%) of the patients with low anomalies had a good outcome at the last follow-up examination. In patients with high and intermediate anomalies continence improved with time; the greatest improvement occurred after 10 years of age. At the last follow-up examination 73% of the patients with high or intermediate anomalies had acceptable social continence. Manometric evaluation revealed a rectoanal inhibitory reflex in all assessed patients with a low anomaly, indicating a functioning internal sphincter. In patients with intermediate and high anomalies a rectoanal inhibitory reflex was found only when full-thickness terminal bowel had been used in the reconstruction (by perineal and posterior sagittal repair); presence of the reflex correlated with superior fecal control. Acceptable fecal continence may be achieved in the majority of patients with anorectal anomalies. Operative methods that optimally utilize the voluntary sphincters and preserve the potential internal sphincter in the terminal rectal pouch seem to facilitate earlier development of fecal continence. Offprint requests to: R. Rintala     

Secondary posterior sagittal anorectoplasty for anorectal malformations

Sixteen patients who had undergone secondary posterior sagittal anorectoplasty (PSARP) for fecal incontinence were clinically followed for 3 to 10 years (mean 5.8). In addition to preoperative manometric evaluation, postoperative anorectal manometry was performed on all patients 1–3 times. Fourteen of the 16 patients underwent postoperative biofeedback training. At the last follow-up visit, the mean age of the patients was 18.3 years (range 16–26). Fectal continence improved in 13 patients. Manometrically measured, both the squeeze and resting pressures in the anal canal were significantly increased. Biofeedback facilitated the improvement of continence in 10 of the 14 patients. However, at the last follow-up, only 4 had good fecal continence with voluntary bowel movements and only occasional soiling. Three patients had voluntary bowel movements but frequent soiling; 8 used daily enemas to stay clean and 1 has a permanent colostomy. Secondary PSARP improves fecal continence in patients with intractable incontinence following primary repair of anorectal anomalies, but in adulthood it is good in only a minority of cases. Most patients require adjunctive measures to maintain socially acceptable bowel function.     

Three-flap anoplasty for imperforate anus: results for primary procedure or for redoes.

Three-flap anoplasty was first described in 1987 by Yazbeck for the treatment of rectal prolapse after pull-through operations for imperforate anus, and in 1992 in a case of anterior perineal approach. It is intended to reproduce the normal anatomy of a sensitive anal canal. The purpose of this study is to evaluate 14 children (9 boys and 5 girls) operated for imperforate anus. METHOD: Eleven children had an intermediate or high imperforate anus and 3 had a low imperforate anus. Nine were operated for the first time with a three-flap anoplasty (GrA), and 5 were reoperated with this technique because of fecal incontinence and/or rectal prolapse (GrB). Seven patients had associated malformations: one Hirschsprung's disease, one cloacal defect with renal insufficiency, three complex caudal malformations with tethered cord, one Down syndrome, and two psychological and psychomotor troubles. The patients (average age = 6 years) have been evaluated every year since 1997, with a questionnaire based on a clinical score validated with 30 healthy children, used as a control group. Ten anal manometries were carried out. RESULTS: In 1998, the GrA score was 16.1 (control = 22.5) and the GrB score was 11.5 (p = 0.25). In 1999, GrA and GrB score were approximately the same. The score of those without associated anomalies was 19.6 whereas the score of the children with other malformations or anomalies was 10 (p = 0.02). Anal manometry is almost normal in patients with low or intermediate imperforate anus (rectoanal relaxation reflex for 10 cm H2O, and basal resting pressure more than 40 cm H2O). Even though anal manometry is subnormal in patients with Down syndrome or psychomotor troubles, the clinical score remains low (score = 10). In cases of complex caudal malformations or high imperforate anus, the results of anal manometry and clinical score are bad (score 9.7). CONCLUSION: The three-flap anoplasty can be carried out in newborns without colostomy and often represents the only possible surgical approach to avoid a laparotomy. This plasty, proposed secondarily to correct a defect of continence, can be performed without colostomy, with satisfactory results.     

Electromyographic studies on the external anal sphincter in children with operated anorectal malformations.

Fecal incontinence is a common problem after surgical repair in patients with anorectal malformations. A normally functioning external anal sphincter (EAS) mechanism is a major factor for continence. The aim of this study was to assess the function of the EAS in children with operated anorectal malformations by electromyography (EMG) and to refine the sphincter function further. The study group consisted of 8 patients with incontinence problems who were operated for anorectal malformations. The electrical activity of EAS was evaluated by EMG using concentric needle electrodes in all subjects. The presence, localization, integrity and activity of the sphincter were assessed. Seven of 8 patients showed electrical activity of the EAS. Sphincter localization was found to be appropriate in six children. Five children showed a well preserved sphincter integrity and 2 of them disclosed normal EMG activity. In cases of localization or integrity abnormalities, secondary corrective surgery was planned, while in patients showing only abnormal EMG activity, biofeedback treatment was given. In cases of fecal incontinence in patients with operated anorectal malformations, electromyographic study shows the type of sphincter abnormality and further helps to make a decision whether to improve the EAS function with conservative treatment or surgically.     

Evaluation of outcome of anorectal anomaly in childhood: the role of anorectal manometry and endosonography

The aim of this study was to evaluate role of anorectal manometry (ARM) and anal endosonography (ES) in assessment of the internal anal sphincter (IAS) quality on continence outcome following repair of anorectal anomalies (ARA). We devised a scoring system to evaluate the quality of the IAS based on ARM and ES and correlated the scores with clinical outcome, using a modified Wingfield score (MWS) for faecal continence. We also assessed the implication of megarectum and neuropathy on faecal continence. Of 54 children studied, 34 had high ARA and 20 had low ARA. Children with high ARA had poor sphincters on ES and ARM, and also poor faecal continence compared to those with low ARA. The presence of megarectum and neuropathy was associated with uniformly poor outcome irrespective of the IAS quality. The correlations between MWS on one hand, and ES and ARM scores for IAS on the other hand were weak in the whole study group, ES r = 0.27, P < 0.04, and ARM r = 0.39, P < 0.004. However, the correlations were strong in those who had isolated ARA without megarectum or neuropathy, ES r = 0.51, P < 0.02 and ARM r = 0.55, P < 0.01, respectively. In conclusion, the ARM and ES are valuable in evaluation of continence outcome in children after surgery for ARA and those with good quality IAS had better faecal continence. The IAS is a vital component in functional outcome in absence of neuropathy and megarectum.     

Biofeedback conditioning for fecal incontinence in anorectal malformations

Twenty children aged 6–18 years who had deficient fecal continence after primary and secondary surgery for anorectal malformations were treated by biofeedback conditioning. The original malformation was high in 17 cases and low in 3. Pretreatment manometric assessments and training were performed with a perfused open-tip catheter connected to a recording unit where the manometric tracings could be inspected digitally or from the pressure curve. A latex balloon was used to stimulate the rectum. The patients were taught to develop maximal sphincter contraction and synchronize the contraction with rectal distention. Rectal sensitivity was conditioned by gradually decreased balloon volumes. There were 4–11 training sessions. The follow-up time was 2–24 months. Sixteen of the 20 patients responded favorably to the training. Fecal continence and the ability to take part in a normal social life improved significantly in the responders. In the responders, the contractile power of the sphincter increased significantly while other manometric parameters (basal anal canal pressure, rectal sensitivity, rectal capacity, rectoanal inhibitory reflux) could not be influenced. Biofeedback is a safe, simple, and effective method in treating fecal incontinence associated with anorectal malformations.     

Urological problems or fecal continence during long-term follow-up of patients with anorectal malformation

Introduction  

Anorectal malformations comprise a wide spectrum of diseases. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. The aim of this study was to evaluate fecal continence together with additional urinary anomalies in patients with anorectal malformations (ARM) and determine which of them is more troublesome for life quality in long-term follow-up.     

Management and outcome of low anorectal malformations

Low anorectal malformation comprises about half of all anorectal anomalies. Most of the literature concerning management of anorectal anomalies is centred around the treatment and outcome of high anomalies. The management of low anomalies has been considered significantly less challenging than high anomalies. Also, the outcome of low anomalies has traditionally been considered good. However, recent more critical long-term follow-up reports show a different picture. Many patients with low anomalies suffer from long-term anorectal functional problems, especially constipation but also soiling that occurs in a significant percentage of patients. In this review, we compile the recent views on the diagnosis, surgical treatment and outcome of low anorectal anomalies. We also present an algorithm for the management of these anomalies. The emphasis on the surgical management of low anorectal anomalies is to use as minimally invasive operative methods as possible and preserve the native mechanisms of continence that usually are much better preserved than in more severe high anomalies.     

Congenital gastrointestinal anomalies in anorectal malformations: What relationship and management?

To date, no systematic review of gastrointestinal anomalies associated with anorectal malformations has been performed. We report the management of three challenging cases of neonates affected by congenital gastrointestinal and anorectal malformations and we review the pertinent published reports to disclose the incidence and outcome of this combination. In 3951 anorectal malformations, the incidence of digestive tract anomalies was approximately 15%. The association of multiple congenital gastrointestinal defects strongly increased the case complexity and worsened the outcome. Based on our study, the application of a rational and staged management plan provides easier diagnosis and treatment of complex cases. Moreover, keeping in mind the likely co-occurrence of anorectal and gastrointestinal anomalies could help avoid misdiagnosis and prevent life-threatening complications. Finally, the knowledge of this association and its successful management can help the multi-specialist team to become more confident in counselling, diagnosis and treatment.     

Levatorplasty using a posterior sagittal approach in secondary faecal incontinence

Levatorplasty has been used successfully in children with secondary faecal incontinence. A posterior sagittal approach in anorectal anomalies gives excellent delineation of the anatomy of the pelvic floor and perineum. Between August 1985 and December 1989, 13 children underwent levatorplasty using a posterior sagittal approach for faecal incontinence following primary operations for imperforate anus. There were 9 boys and 4 girls; 7 of them had a major associated anomaly. Twelve patients had a high anorectal anomaly and the primary operation was a sacro-perineal or sacro-abdomino-perineal pull-through. One had an intermediate anomaly treated by a perineal operation. All patients had persistent soiling and among them had 27 further unsuccessful operations to improve continence prior to posterior sagittal levatorplasty. Six patients had good and 7 had poor to moderate voluntary squeeze pressures prior to levatorplasty. Age at posterior sagittal levatorplasty ranged from 4 to 17 years (mean 10.7 years). All patients were followed up for periods ranging from 2 years 2 months to 6 years 5 months (mean 4.7 years). At least review 6 patients (46%) had excellent results with full continence and no soiling; 5 (39%) showed significant improvement compared with the pre-operative state with occasional soiling only. Of these, 3 patients opted for total colonic irrigation once a day to remain clean at all times. Two patients (15%) had poor results with frequent soiling. The posterior sagittal approach provides excellent exposure for levatorplasty. Best results were obtained in patients who had good voluntary squeeze pressures on anorectal manometry prior to the operation. Correspondence to: P. Puri     

Is posterior anal transfer a good treatment for vestibular anus?

Eleven females who had a posterior anal transfer (PAT) for a vestibular anus were reviewed in order to: (1) assess the long-term functional success of this operation clinically; (2) evaluate the anorectal manometry profile; and (3) assess defaecation by video proctography. The cosmetic appearance was excellent in all patients. Seven had a good result, 2 a fair result, and 2 a poor result when assessed clinically (Kelly score) in terms of constipation and soiling. The anorectal manometry profile remained within normal limits except in the 2 with a poor result who had diminished sensory awareness of stool in the rectum. PAT does not appear to damage the muscle-sphincter complex, and the children with a large rectum full of faeces seemed to behave like children with acquired megacolon and constipation. Video proctography showed normal defaecatory patterns in all patients. Patient selection is important for this procedure. When PAT was used as the initial treatment a good result was obtained; when it was used to relieve constipation following previous surgery the result was less satisfactory.     

Occult spinal dysraphism: neurogenic voiding dysfunction and long-term urologic follow-up

From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days–21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.     

Effects of herbal medicine Dai-Kenchu-to on anorectal function in children with severe constipation.

AIM: We administered the herbal medicine Dai-Kenchu-To (DKT) to children with severe chronic constipation or with severe constipation after surgery for anorectal malformations. We then objectively assessed the effect of DKT on anorectal function by manometric study in addition to using a clinical scoring system. PATIENTS AND METHODS: Ten children with severe chronic constipation and 5 children with severe constipation after surgery for anorectal malformations were assessed. These 15 children received 0.3 g/kg/day of DKT for periods ranging from 3 months to 1 year. We objectively assessed their bowel function, sphincter function and rectal reservoir function by anorectal manometry and clinical scoring. RESULTS: In 10 children with severe chronic constipation, the clinical score after administration of DKT (7.2 +/- 0.8) improved significantly compared with that before administration of DKT (4.6 +/- 2.9) (p < 0.02). The threshold sensation volume and the maximum tolerable volume after administration of DKT significantly (p < 0.05; p < 0.01) decreased (128 +/- 63 ml vs. 69 +/- 18 ml; 229 +/- 99 ml vs. 144 +/- 47 ml), and rectal compliance after administration of DKT also significantly (p < 0.05) decreased (12.4 +/- 10.9 ml/cmH(2)O vs. 4.7 +/- 3.9 ml/cmH(2)O). CONCLUSION: The present study demonstrated that DKT had a favorable clinical effect on severe constipation in children, and anorectal manometry showed an improvement in their rectal reservoir functions. It appears that the results were secondary to DKT-stimulated peristalsis of the intestine, which promoted regular bowel habits.     

Posterior sagittal approach with perirectal dissection for reconstructive surgery of severe urogenital anomalies

There are many approaches for surgery of complicated congenital and acquired urogenital anomalies in children with intact rectum. Pena advocates the mid-sagittal division of the sphincter mechanism posterior and anterior to the rectum, along with opening of the posterior and anterior rectal walls. The aim of this study is to determine whether the posterior sagittal approach with perirectal dissection (PSAPD) and elevation of the mobilized rectum would impair fecal continence when used for correction of complicated urogenital anomalies in children with normal rectum. Between 1988 and 1994 the authors performed PSAPD in eight infants and children with an intact anorectum. Indications for PSAPD were high vaginal atresia, Mullerian duct remnants, prostatic rhabdomyosarcoma, and traumatic vesicovaginal fistula. After a mean 10-year follow-up the bowel habits were assessed. Anorectal and uromanometric studies and a detailed questionnaire (modified Holschneider’s scoring) sent to children or parents were evaluated. Three patients who preoperatively were clinically fecal continent had soiling only at the time of diarrhea. Early postoperative low anorectal pressure profile normalized during the follow-up. Seven patients had a fecal continence score above 23, two of them with maximum points of 26. Only one girl had a low score of 15. The authors conclude the PSAPD which offers a few advantages over the sagittal division of the rectum provides an alternative approach for selected lesions of the genitourinary tract in children with a normal rectum. Our results suggest that fecal continence is either preserved or partially affected.     

Anorectal anomalies: anorectal manometric function and anal endosonography in relation to functional outcome

To assess the relation between continence and the manometric and endosonographic state of the anorectal segment after surgery for anorectal anomalies (ARA), 33 adolescents operated upon for ARA and 14 controls were examined. Seventeen patients had low and 16 intermediate or high ARA. Fecal continence was recorded, and anal canal manometry was performed by microtransducer. The sphincter muscle complex and its relation to the anal opening was visualized by anal endosonography (ES). Ten patients had perfect continence, 10 had staining, and 13 had soiling. The anal canal resting and squeeze pressures were significantly different in all three groups, and continence function was significantly correlated to anal canal pressures. By anal ES, the internal (IAS) and the external anal sphincters (EAS) were identified with various amounts of scar tissue in all patients. In patients with high or intermediate anomalies the IAS was missing in the lower part of the anal canal, and abundant scar tissue was identified in the EAS in all patients. In patients with good continence function, the EAS was better preserved than in patients with major incontinence. The position of the anus in the EAS muscle complex was assessed, and varying degrees of .eccentrically placed anal canals were identified. Continence function after surgery for ARA is thus correlated to anal canal pressures and ES images. ES, which is painless and suitable for use in children, is a valuable tool for assessing perianal structures, and the findings may serve as a helpful guide for corrective surgery.     

Outcomes from the correction of anorectal malformations

PURPOSE OF REVIEW: Anorectal malformations have been recognized and managed since antiquity, with surgical treatment evolving to maximize anatomic reconstruction, avoid complications, and understand mechanisms of incontinence, ultimately leading to improved quality of life for patients. This review describes recent advances in the management of anorectal malformations, including prenatal diagnosis, newborn treatment, surgical correction, and postoperative care. RECENT FINDINGS: Surgical treatment has improved with better understanding and exposure of anatomy and appreciation of the intimate relation between rectum and urinary tract. Repair of cloacal malformations has evolved to include the total urogenital mobilization and an appreciation of the complex associated Mullerian anomalies. The importance of associated urologic, gynecologic, neurologic, and orthopedic malformations has been recognized. Addition of a bowel management program to patients' postoperative care has increased dramatically the number of children who are clean and dry. SUMMARY: Management of anorectal malformations requires an accurate clinical diagnosis, proper newborn treatment, meticulous anatomic reconstruction, and comprehensive postoperative care with the goal of having a child who is clean and dry, with an excellent quality of life, because they either have the capacity for continence or can be kept artificially clean with a comprehensive bowel management program.