Clinical and manometric postoperative evaluation of posterior sagital anorectoplasty (PSARP) in patients with upper and intermediate anorectal malformations

PSARP is currently the most widely-used surgical technique for surgical correction of high and intermediary anorectal malformations, but there is much controversy in the literature about the postoperative evaluation of these cases. We studied 27 cases of anorectal malformations operated with PSARP from clinical and manometric aspects in order to analyze: 1) fecal continence; 2) the relationship between fecal continence and the associated sacral anomalies and; 3) the relationship between the postoperative manometric evaluation and fecal continence. From the analysis of 27 cases of high and intermediary anorectal malformations, we concluded that: 1) fecal continence was achieved in 48.14 percent of the cases; partial fecal continence in 25.92 percent; and fecal incontinence in 25.92 percent of the cases; 2) the presence of fecal incontinence was directly related to the association of sacral anomalies and; 3) anorectal manometry is a useful test to evaluate the patients operated by PSARP, due to the existence of a relationship between the manometric results and the degree of fecal continence.     

The failed anoplasty: successful outcome after reoperative anoplasty and sigmoid resection

BACKGROUND/PURPOSE: Children with anorectal malformations often have less than optimal results after repair. The authors report on five patients (ages 3 to 17 years) born with imperforate anus and treated with anoplasty as a newborn. At presentation, all patients were completely incontinent of stool. None had ever experienced voluntary bowel movements, and all wore diapers continuously. METHODS: Perineal examination with the nerve stimulator showed the muscle complex was largely intact with good contraction, but the neoanus was outside of the muscle complex. Contrast enema showed massive dilation of the rectosigmoid colon and fecal impaction. A tethered spinal cord was excluded by magnetic resonance imaging (MRI). We treated these patients with a combined reoperative anoplasty via the posterior sagittal approach and sigmoid resection. RESULTS: Within 6 months after the procedure, all patients had achieved complete continence. They had from one to three voluntary bowel movements per day without soiling. CONCLUSIONS: Children with severe constipation and fecal incontinence after anoplasty should undergo evaluation by a surgeon. If examination shows a reasonably intact muscle complex and correctable anatomic defects, an excellent result can be achieved with appropriate reoperation.     

Gluteus maximus augmentation for the treatment of fecal incontinence

Fecal incontinence is a devastating problem for school-aged children and adults. Medical and biofeedback therapies are unsuccessful in most patients who have severely defective internal and external sphincters. Continued fecal incontinence frequently leads to social isolation and withdrawal. Gluteus maximus augmentation of the sphincter mechanism is one surgical method for treating fecal incontinence. The authors present their results with gluteus maximus augmentation of the anal sphincter and describe patient selection criteria. From 1992 through 1996, seven patients underwent gluteus maximus augmentation of the anal sphincter for fecal incontinence. Six of these patients were children 5 to 6 years of age who had major deficiencies of their anorectal sphincter demonstrated by manometry. One patient was a 56-year-old adult woman who had acquired idiopathic fecal incontinence. Four of the six children (67%) had imperforate anus and two had cloacal anomalies (33%). The augmentation was performed in three stages. A sigmoid-end colostomy with a Hartman's pouch was followed 1 month later by rotation of a portion of the gluteus maximus for anorectal sphincter augmentation. A colostomy take down was performed 2 to 4 months later. All patients underwent dilatation after sphincter augmentation and were taught muscle exercises for using their neosphincter during the period before colostomy take down. Four of six children and the adult are continent postoperatively (71%). Both patients who remain incontinent are unable to sense rectal distention clinically or on anal manometric analysis but have excellent voluntary sphincter tone. Fecal incontinence can be successfully treated with gluteus maximus augmentation in carefully selected patients. Patients unable to sense rectal distension are unlikely to benefit from this procedure. The presence of a rectal reservoir and a skin-lined anal canal also appear to be important in attaining fecal continence.     

Diagnosis and management of fecal incontinence in elderly patients

Fecal incontinence is a physically and psychologically disabling condition that affects millions of Americans, especially those over the age of 65 years. The pathophysiology is often multifactorial, with decreased anorectal sensation, reduced rectal compliance. anal sphincter dysfunction, altered stool consistency and immobility playing significant roles. A detailed history and a thorough physical examination are always necessary in patients with fecal incontinence and physiologic tests, including anorectal manometry, cinedefecography and electromyography, may be required for proper diagnosis and treatment. In most patients fecal incontinence is initially treated with conservative measures, such as biofeedback training or alteration of the stool consistency (if appropriate). If conservative management fails, surgical intervention, such as sphincteroplasty or gracilis muscle transposition, may be considered.     

Antegrade continent enema management of chronic fecal incontinence in children

BACKGROUND/PURPOSE: Fecal incontinence impedes social and psychological development in children. Conventional bowel management with rectal enemas, medication, and biofeedback frequently will provide intervals free of fecal soiling sufficient for children to be socially continent. METHODS: This report details the improvement achieved by antegrade irrigation of the colon when conventional bowel management programs have failed to provide satisfactory intervals free of fecal soiling. Twenty-three children had conduits performed to administer antegrade continent enemas in the colon from December 1993 to May 1997. RESULTS: Twenty of the children were available for evaluation. One child was lost to follow-up, and two were noncompliant with the irrigation program. Fourteen of the 20 children are out of diapers. Four of the six wearing diapers, do so for urinary, not fecal incontinence. Two patients (10%) still require diapers for fecal incontinence. Parents were pleased with the efficacy of antegrade irrigation and the decrease in family stress with the elimination of fecal soiling. CONCLUSIONS: Ninety percent of the children available for follow-up benefited from antegrade irrigations of the colon. The antegrade irrigations were an improvement over conventional bowel management programs because a predictable interval free of soiling could be established. This allowed families to plan activities and improved the self-esteem of their children.     

Fibroblast growth factor-2 stimulates cell proliferation and decreases sexually dimorphic cell death in an avian song control nucleus

Congenital anorectal malformations are found in many forms, and are frequently associated with other anomalies, especially of the spinal cord, spine, and urogenital system. Decisions concerning initial management of children with anorectal malformations can be made only after accurate determination of (a) the level and type of malformation, (b) the type of fistula, (c) the developmental state of the sphincter muscle complex, and (d) the presence of associated anomalies. Magnetic resonance imaging has proven to be the only modality to answer all these crucial questions, and has contributed to a better insight in the morphology and pathogenesis of such complex congenital malformations.     

Reproductive aging is an evolutionarily programmed strategy that no longer provides adaptive value

PURPOSE:The aim of this study was to assess the results of the Malone (antegrade colonic enema) procedure for fecal incontinence. METHODS: By a retrospective review of patients treated between 1990 and 1996 in a tertiary referral center, 36 patients were treated with a Malone procedure. Age at operation was 8.3 (range, 3 to 14) years, the mean period of follow-up was 39 (range, 9 to 72) months. The indication was fecal soiling in 35 and chronic constipation in one. The underlying diagnosis was an anorectal anomaly in the majority of patients. The appendix was used in 30 patients and a cecal flap in six, and a submucosal antireflux procedure was also performed in 10. In 35 patients, a circular stoma was created and in one a V flap was used. Antegrade colonic enemas were performed daily in 10, alternate days in 23, and in three patients the stoma was no longer used. Enemas were performed with a 10F catheter using a mixture of phosphate enema (or liquorice) and saline. RESULTS: Fecal soiling was completely controlled in 28 patients, and eight children soiled more than once a week. Complications occurred in 15 patients; the main problem was stenosis of the conduit, which occurred in 9 of 30 appendiceal stomas and three of six cecal stomas. Stomal stenosis was treated with surgical revision in eight patients. Additional complications were reflux through the stoma (n=2), pain on catheterisation (n=1), and small bowel obstruction (n=1). In one patient the Malone has been converted to a colostomy. CONCLUSIONS: The Malone procedure is a simple technique that can effectively control fecal incontinence in the majority of cases. It appeared to be better in older children. Stomal stenosis is a frequently encountered problem that may require surgical revision.     

Cloaca, the most severe degree of imperforate anus: experience with 195 cases

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.     

Percutaneous cecostomy: a new technique in the management of fecal incontinence

A pilot study on the percutaneous introduction of a cecostomy tube for colonic irrigations in the treatment of children with fecal incontinence is described. The results were good, and the technique is recommended for certain patients.     

Pelvic floor insufficiency as an interdisciplinary responsibility

Weakness of the pelvic floor is in the majority a disease of women. The most frequent complaints are urinary incontinence and visceral descent. The usual methods of gynaecological and urological operations to cure these impairments are demonstrated. Because several patients with a lax pelvic floor suffer from anorectal incontinence a simultaneous interdisciplinary treatment is proposed.     

Alveolar soft-part sarcoma: further evidence by FISH for the involvement of chromosome band 17q25

BACKGROUND/PURPOSE: Down's syndrome is a common association in patients with anorectal malformations. The purpose of this study was to determine whether the anorectal defect in patients with Down's syndrome had specific characteristics and whether the presence of Down's syndrome represented a serious detriment to the patient's functional prognosis. METHODS: Nine hundred eighty-seven patients with anorectal malformations were studied retrospectively. Twenty patients (2%) had Down's syndrome. Nineteen of these (95%) had the same specific type of anorectal defect: imperforate anus with no fistula. This defect has a good prognosis, the rectum is located about 2 cm above the perineal skin, the sacrum is normal, and the sphincter mechanism is good. For comparison, a group of 34 patients with the same defect but without Down's syndrome was also studied. All patients were operated on via posterior sagittal approach by the same surgeon. RESULTS: Imperforate anus without fistula occurs in 5% of all patients with anorectal malformations and in 95% of those patients who also suffer from Down's syndrome. The characteristics of the defect were the same in both groups of patients, and surprisingly, the prognosis was good in both groups (80% to 96% of patients had voluntary bowel movement, 100% had urinary continence). CONCLUSIONS: The association of Down's syndrome with imperforate anus without fistula is not coincidental. This particular benign defect can be predicted to occur in most patients with Down's syndrome. The presence of Down's syndrome in cases of anorectal malformations should not be a contraindication to repairing the imperforate anus and to closing the colostomy.     

Technic of overlapping sphincter anal repair in the treatment of traumatic anal incontinence

Faecal incontinence is an important disabling symptom in the affected patients. Classically, we divide faecal incontinence in two main types: neurogenic faecal incontinence and traumatic anal incontinence. Traumatic anal incontinence is due to causes damaging sphincteric mechanism directly. The aim of the present study was to evaluate the outcome of overlapping sphincter anal repair procedure in the management of traumatic anal incontinence. To this end we studied 27 patients with traumatic anal incontinence who underwent an overlapping sphincter anal repair procedure according to the method described by Parks and McPartlin in 1971. Mean follow up was up three years and was based mainly both on clinical evaluation with anorectal exploration and manometric values carried out on a 6 monthly basis. When the outcome was evaluated in terms of faecal continence our date were similar to those reported by Parks and Fang. In the subjects studied we haven't reported any major complications apart from one case of abscess, one case of wound's infection and one case of stenosis which were efficaciously treated. Our findings supported the view that overlapping sphincter anal repair procedure is the surgical approach of choice in the patients with traumatic anal incontinence.     

Prevalence and determinants of carotid atherosclerosis in healthy postmenopausal women

Fecal incontinence is an under-reported complication of scleroderma. Ten incontinent patients with scleroderma were evaluated through anorectal manometry and compared with 20 incontinent patients without scleroderma who were matched for age and sex as controls. The scleroderma patients had a higher voluntary external anal squeeze pressure, whereas the resting internal anal sphincter pressure was similar to that of the control group. The threshold for rectal sensation in the scleroderma group was significantly less than that in controls. Episodes of fecal incontinence, anal canal length, and maximal tolerable volume were not significantly different between the study groups. The rectoanal inhibitory response was abnormal in 80% of patients with systemic sclerosis but was normal in 70% of the controls. Stool consistency was significantly looser in the scleroderma patients. Treatment of fecal incontinence in scleroderma patients may be successful in many patients using a combination of dietary and pharmacologic manipulation because diarrhea is an important etiologic cofactor superimposed on reduced internal anal sphincter pressure.     

Video laparoscopic surgery and rectal prolapse. Our experience in rectopexy

BACKGROUND: Laparoscopy is gaining an important role in the treatment of benign colorectal disorders. The aim of this study is the evaluation of clinical and functional results in 4 patients submitted to a laparoscopy rectopexy according to Wells. METHODS: Four females (22-76, mean 53.7 years) affected from a total rectal prolapse with fecal incontinence underwent this procedure from 1993 through 1995. Six months after surgery, at the end of a rehabilitation program consisting of kinesitherapy, bio-feedback and electrostimulations, all patients have been re-evaluated by means of a clinical exam, anorectal manometry, defecography. RESULTS AND CONCLUSIONS: Preliminary results seem satisfactory and may allow to prefer this approach instead of the traditional open one.     

Functional results following the antegrade continence enema procedure

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.     

Management of neurogenic fecal incontinence in myelodysplastic children by a modified continent appendiceal stoma and antegrade colonic enema

OBJECTIVES: Antegrade colonic enemas for neurogenic fecal incontinence via reverse reimplanted appendices (Mitrofanoff principle) have been primarily reported by Malone and coworkers in 1990. We used a modification of the described surgical technique and treated the first 10 patients with neurogenic fecal incontinence due to spina bifida. The surgical procedure and the results are reported. METHODS: Since November 1991, we have used a surgical procedure similar to the appendiceal continence mechanism in urinary diversion to establish a continent colonic cutaneous stoma for antegrade enemas in 10 myelodysplastic patients (4 females, 6 males; median age 13.2 years [range 6 to 26]) with severe neurogenic fecal incontinence. The average follow-up is now 26.4 months (range 12.5 to 50). All patients had neurogenic bladder dysfunction successfully managed by clean intermittent catheterization, anticholinergic drugs, or artificial sphincter implantation. The surgical technique for fecal incontinence included the partial orthotopic submucosal imbedding of the appendix into a cecal tenia and the fixation of the ileocecal region at the inner side of the abdominal wall after creation of an appendicocutaneous catheterizable stoma. RESULTS: All patients reached fecal continence for at least 38 hours (median 45.3) by using antegrade colonic enemas with 1.5% saline solution (n = 9) or GoLYTELY solution (n = 1), 0.5 to 1.5 L every 2 to 3 days. All other therapies (diet, oral medication, rectal purgative, or enema) to reach fecal continence had previously failed. There were only two complications seen at the follow-up. One boy with an artificial urinary sphincter presented with infection of the sphincter system, which led to explantation. Another boy presented 15 months after creation of the colonic appendiceal stoma with saline intoxication possibly due to a homemade saline solution. CONCLUSIONS: We conclude that the antegrade colonic enema via an orthotopic continent appendiceal stoma is a safe and highly effective treatment modality for fecal incontinence in patients with neurogenic bowel dysfunction if nonsurgical management has failed.     

Hearing capacity and speech production in 417 children with facial cleft abnormalities

Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.     

Current management of anorectal anomalies

A significant amount of new information has been obtained concerning the early management of anorectal malformations. The surgical approach also has changed and improved the functional prognosis of children with these defects. Recommendations are made based on a series of 632 cases. A simplified approach to avoid the most common errors in the management of these defects is emphasized. Yet, there are still many children born with severe anatomic deficiencies who cannot expect normal bowel function; however, for this group, a bowel management program is available to help improve their quality of life.     

Fecal incontinence and rectal prolapse. Clinico-functional assessment

Rectal Prolapse is a rare and distressing condition, with a multifactorial etiopathogenesis. Often, this pathology is associated with fecal incontinence. The recommended approach to the patient with rectal prolapse and fecal incontinence is to repair the prolapse first, then deal particularly with fecal incontinence at a second operation. A retrospective, clinical and manometric study has varying degrees of fecal incontinence. Clinically five of their operation, and a further three patients improved, in two patients the degree of fecal incontinence remained invariable. One patient was worsened after surgery. Manometrically resting and pressure (RAP) was significantly higher in continent patients than in voluntary contraction pressure (MVCP) (p < 0.05) in preoperative testing. Postoperatively, there was a significant increase in the resting anal pressure as well as in maximum voluntary contraction pressure. Patients who remained incontinent had a significantly lower RAP and MVCP than patients who improved our regained continence. In conclusion this study shows an alteration of internal and external sphincteric function in patients with rectal prolapse. The surgical treatment of this disease improves sphincteric function. Incontinent patients with RAP < 10 mmHg and MCVP < 20 mmHg, probably they would be better treated simultaneously either for rectal prolapsus and incontinence. In this kind of patients the perianal proctectomy with total sphincteroplasty could be the elective treatment.     

Antegrade colonic enemas

Thirteen children aged 6-14 (mean 8) years in whom an antegrade colonic enema procedure was performed were reviewed retrospectively. All presented with refractory constipation or faecal soiling over a 3-year period. Nine of the children had previously undergone pull-through procedures for Hirschsprung's disease or high anorectal malformations. Two were suffering from spina bifida and two from idiopathic functional constipation. The operation was performed through a right iliac fossa incision. A catheterizable conduit was created. The appendix was brought out to the wound edge and made continent by intussuscepting the appendix base into the caecum. When the appendix was absent or unusable, a caecal tube was formed. Five patients suffered minor morbidity, six required a further operative procedure and two eventually required a sigmoid colostomy. However, the eventual outcome of a continent stoma was attained in 11 of the 13 children, all of whom would have been considered for sigmoid colostomy before introduction of the antegrade colonic enema procedure.