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One-stage correction of high imperforate anus in the male neonate.

PURPOSE: The aim of this study was to examine the feasibility, safety, and short-term outcome of complete one-stage repair of high imperforate anus in the newborn boy. METHODS: A retrospective review was conducted of five full-term male infants who underwent posterior sagittal anorectoplasty without a colostomy within the first 48 hours of birth. Preoperative imaging was performed to assess associated anomalies. All infants underwent cystoscopy before the perineal operation to determine the level of the urinary tract fistula, if present. After completion of the anoplasty, all were turned supine and the colon irrigated free of meconium. Follow-up ranged from 10 to 24 months. RESULTS: Laparotomy was not required for any patient. Three patients had a rectoprostatic urethral fistula, one a rectovesical fistula, and one no fistula (common wall at level of prostate). Tapering rectoplasty was required for only the one patient with a rectovesical fistula. There were no intraoperative complications. All patients passed stool within 12 hours after operation and took full feeding by 48 hours. The average hospital stay was 7 days. Postoperative and stenosis occurred in one patient secondary to parental noncompliance with the postoperative dilation regimen. There were no perineal wound complications. All patients have a strong urinary stream and defecate spontaneously without the aid of oral medication or rectal stimulation or enemas. CONCLUSIONS: One-stage repair of high imperforate anus in the male neonate is feasible without short-term genitourinary or gastrointestinal morbidity. Whether it is preferable compared with a delayed (two or three stage) repair depends on ultimate long-term anorectal function, which cannot be assessed for several years.

Anal Canal↗

Physiotherapy to improve faecal control after Stephens' rectoplasty in high imperforate anus.

The role of physiotherapy for the achievement of faecal continence after Stephens' rectoplasty for high imperforate anus was studied. Clinical assessment and ano-rectal manometry were performed more than 7 years after surgery, both before and after one year of physiotherapy training of the perianal musculature. After the training period the faecal control improved. This effect was verified by the ano-rectal manometry findings. Faecal continence can be achieved by Stephens' rectoplasty. Furthermore, with any persisting incontinence, postoperative physiotherapy with training of the voluntary muscles in the perianal region can be valuable for the achievement of faecal continence.

Adolescent↗

Imperforate anus (anal agenesis) with rectal and sigmoid atresias in a newborn.

A male baby was born to a healthy mother after an uncomplicated 39-week pregnancy. He had finger and toe syndactyly, right hemidiaphragm eventration, and a high imperforate anus (anal agenesis). The kidneys and chromosomes were normal. A right transverse colostomy was done and 5 months later he underwent a posterior sagittal anorectoplasty (Pena procedure). An intermediate imperforate anus with no urethral fistula and a rectal atresia 2 cm proximal to the distal rectal pouch were found; the distal rectal pouch was resected and the standard Pena procedure completed. Dilatations were initiated 2 weeks postoperatively and continued until the colostomy was closed. Prior to closure of the colostomy, a distal colon loopogram demonstrated an obstruction in the sigmoid; constrast introduced via the rectum outlined the same obstruction separated by 2 cm. A low sigmoid atresia was then repaired. Three weeks later, a contrast loopogram showed a narrow but intact rectosigmoid anastomois, and the colostomy was closed. The anorectal dilatations were discontinued and he remains well at 7 years of age.

Abnormalities, Multiple↗

Repositioning the misplaced colostomy for high imperforate anus: report of a new technique.

The case of a 2-year-old boy with high imperforate anus and an incorrectly positioned colostomy (in the left upper quadrant of the abdomen) is presented. To relieve the resulting tension on the distal rectosigmoid colon and to allow for its use in a combined pull-through and posterior sagittal anorectoplasty, the colostomy was moved to the subumbilical area.

Anus, Imperforate↗

The various types of anorectal fistula in male imperforate anus.

The purpose of this paper is to provide a review of the anorectal fistulas occurring in male imperforate anus, with a suggested classification based on the site of termination of the fistula, namely, the bladder, the urethra, and the perineum. The fistulas included in these categories are discussed in some detail, with reference to previous cases or descriptions in the literature and with some personal observations.

Anus, Imperforate↗

Imperforate anus associated with a recto-bulbar-cutaneous fistula.

This is the report of two newborn boys with imperforate anus associated with a long rectocutaneous fistula running deep into the scrotum and communicating, in its midportion, with the bulbar urethra. The findings are compared with those of a somewhat similar case in the literature, with some speculations as to the possible embryogenesis of the lesion.

Anus, Imperforate↗

The genitourinary system in patients with imperforate anus.

The incidence and significance of associated genitourinary abnormalities was reviewed in a series of 484 infants with imperforate anus encountered over a 17-year period. Fourteen percent of the entire series had significant bilateral upper tract urinary abnormalities (either asymmetric or symmetric). Of 67 such patients only 14 had combinations of renal agenesis and hypoplasia-dysplasia representing uncorrectable renal pathology. A high incidence of vesicoureteral reflex and neurovesical dysfunction represent additional important sources of preventable renal deterioration.

Anus, Imperforate↗

Congenital gastrointestinal anomalies: psychosocial functioning of children with imperforate anus, gastroschisis, and omphalocele.

This study assessed the psychosocial status of 56 children aged 6-16 years with imperforate anus, gastroschisis, or omphalocele. Results indicate that (a) children were of average intellectual ability; (b) approximately 9% of the sample had learning disabilities; (c) although the mean maternal ratings of behavioral adjustment did not differ from those reported for children without anomalies, approximately 18% of the children had deviant adjustment scores; (d) a multivariate risk and resistance model accounted for a significant amount of the variance in children's adjustment; and (e) child, maternal, and family utilitarian and psychological risk and resistance resources contributed independently to children's overall behavioral adjustment.

Abdominal Muscles↗

Sonography of imperforate anus.

Real-time sonography provides a noninvasive method of determining the level of obstruction in patients with imperforate anus. The authors describe one technique for evaluating this anomaly which was employed in 6 neonates. A pouch-perineum distance of less than 1.5 cm is consistent with a low lesion, while a pouch that terminates above the base of the bladder is indicative of a high lesion.

Anus, Imperforate↗

Imperforate anus in male children: a new operation of primary perineal rectourethroanoplasty.

From experience in managing 348 patients with imperforate anus and after considering the existing literature, there appeared to be the possibility of performing a safe primary correction of supralevator anorectal anomalies via a perineal approach in the neonatal period in males. A total of 39 patients have been treated by the new operation, and in 31 of these patients the operation was carried out at birth. With a stiff catheter in the urethra, via a horizontal 'H'-shaped perineal incision and through the puborectalis sling, the rectum was mobilised and the fistula transfixed. The rectum was brought through the sling and sutured to the perineum so that perineal skin entered the new anal canal. In a few patients an abdominal approach was required at the same time. There were few postoperative complications. Subsequent X-rays showed good urethral and pelvic floor anatomy and function. Twenty-five of the babies are now over 2 years old. Of these, 84% are continent for solid faeces and 68% for liquid faeces. The perineal approach permits satisfactory identification of the puborectalis, rectum and fistula in most babies. Cutting the pelvic floor is not satisfactory. Introducing perineal skin into the anal canal is important. Early operation enables the child to learn to use its perineum. It is difficult to know whether the results in this series are attributable to the type of operation or its timing.

Anus, Imperforate↗

Artificial sphincter with colonic reservoir for severe anal incontinence because of imperforate anus and short-bowel syndrome: report of a case.

Anal sphincter replacement is a treatment option for severe anal incontinence that is not amenable to direct repair. We describe the unusual case of a 25-year-old male who has been successfully treated by implantable artificial sphincter for severe anal incontinence caused by imperforate anus and concomitant short-bowel syndrome. In early life, the patient underwent several surgical procedures, including a perineal colostomy for a high imperforate anus associated with a rectourethral fistula. At aged 21 years, he underwent a subtotal small-bowel resection and a restorative jejunotransverse anastomosis for acute intestinal necrosis related to intestinal malrotation with volvulus. The length of remnant jejunum was 90 cm. Consequently. the patient became permanently incontinent and required nutritional therapy. An artificial sphincter, after the creation of colonic reservoir, was implanted around the pulled-through colon. At two-year follow-up after implantation, despite short remnant bowel, the patient was fully continent without medication. Normal nutritional status was maintained under 100 percent oral nutrition. The patient's quality of life improved dramatically. He has returned to work and had no major restriction in his level of social functioning. Our case illustrates for the first time the original concept of artificial anal artificial sphincter implantation around a pulled-through colon, which constitutes a different situation from disease with rectum in place.

Adult↗

Continence and reflex pressure profile after surgery to correct the imperforate anus.

To clarify the cause of constipation which follows surgery for the supralevator type of disorder associated with the imperforate anus, rectal compliance, percentage maximum static anorectal pressure, and reflex profile were measured by anorectal manometry in 108 normal controls and 42 patients. Patients with constipation had a low percentage anorectal pressure (50%), high rectal compliance, associated with megarectum, and defecation of the staining type with constipation due to a reaction in anorectal motility.

Adolescent↗

Performance of the EuroQol in children with imperforate anus.

OBJECTIVE: To investigate the feasibility and validity of a proxy version of the EuroQol in children treated for imperforate anus. METHODS: Patients included were between 1 and 51 years of age. Instruments included were the EuroQol, the TACQOL and a disease specific questionnaire, the Langemeijer Stool Questionnaire. Patients older than 15 years filled in all questionnaires themselves, in the age groups 5-10 and 11-15 a parent administered the questionnaires. Feasibility was judged on the number of missing values. In search of validity, EuroQol scores were compared with the prevalence of disease symptoms (convergent validity) and with the TACQOL (construct validity). RESULTS: The number of missings was not related to age. The disease specific questionnaire correlated significantly with the EQvas from 11 years on and with the EQ-5Dindex from 5 years on. The mean correlation between contextual similar domains of the EuroQol and the TACQOL was -0.55. The correlation between different domains was -0.32. CONCLUSION: The results support the idea that the use of a proxy version of the EuroQol is feasible and valid. The convergent validity of the EQvas was supported from 11 years on. The EQ-5D showed good construct and convergent validity from 5 years on.

Adolescent↗

Laparoscopic muscle electrostimulation during laparoscopy-assisted anorectal pull-through for high imperforate anus.

PURPOSE: Our technique for laparoscopic muscle electrostimulation during laparoscopy-assisted anorectal pull-through (LAARPT) for high imperforate anus (HIA) in 3 patients is described. METHODS: The distal rectum and rectourethral fistula is dissected laparoscopically. A muscle stimulator is passed through one of the trocars and used to identify the center of contraction of the levator ani. The same muscle stimulator is used to identify the center of the external sphincter muscle transcutaneously. An intravenous cannulation device (SURFLO Flash IV catheter, TERUMO, CO, Yamanashi, Japan) is inserted through this proposed anus and observed piercing the center of the levator ani. A guide wire is passed through the SURFLO, and a series of dilators are passed along it to create a canal for the colonic pull-through. An anoplasty then was performed. RESULTS: Our technique was successful in all patients. Laparoscopic electrostimulation produced good levator ani contraction in patients I and II and weak contraction in patient III. Patients I and II have symmetrical anal contraction during rectal examination, but patient III has poor contraction. Stool frequency is decreasing in all. CONCLUSION: Direct laparoscopic observation of levator ani contraction allows intraoperative assessment of functional contractility and assists in the accurate placement of the colonic pull-through.

Anal Canal↗

High and intermediate imperforate anus: functional results and postoperative manometric assessment.

Between 1983 and 1989, 21 patients with high (12) and intermediate (9) imperforate anus were operated on according to the author's procedure. The anatomical results are satisfactory (20/21). The records of the clinical and paraclinical assessments of 13 of these patients with a follow-up of at least 5 years, is reported. The patient history demonstrates that 12 out of 13 are clean at night, with 1 to 3 bowel movements during the day. The intermediate forms have no accidental defecation and almost no soiling. Conversely, the high forms often soil but usually do not wear thick protections. Criteria of successful reconstruction as rectal examination, defecogram, CT scan or MRI, yield limited data but no formal conclusions. Manometric studies provide a better understanding of the bowel function after pull-through procedures, by studying the rectoanal inhibitory reflex threshold (RAIRT), the maximal anal resting closure (MARCP), the conscious rectal sensitivity threshold (CRST) and the maximal rectal compliance (MRC). Technical aspects of these recordings are detailed. Manometric recording from 13 of these 21 patients show that RAIRT is present in 3 out of 6 intermediate forms and 3 out of 7 high forms. CRST is normal in all the cases except 1. MARCP was normal in 5 out of 6 intermediate lesions and 5 out of 7 high lesions. MRC is good in 5 intermediate forms and 3 high forms.(ABSTRACT TRUNCATED AT 250 WORDS)

Anal Canal↗

Imperforate anus in females: frequency of genital tract involvement, incidence of associated anomalies, and functional outcome.

Of 162 female patients with imperforate anus, 21% had a noncommunicating and 79% a communicating anomaly of the rectum or anus. Associated anatomical abnormalities were found in the lower urinary tract (15%), upper urinary tract (25%), lower genital tract (27%), upper genital tract (35%), and additional organ systems (51%). Death occurred in 26 patients and in 19, this was attributed to the associated abnormalities. Functional outcome was assessed in those patients 13 years of age or older. Bowel function was normal or near normal in 85%, as was urinary and renal function. In 44% of patients evaluated, there was persistent vaginal abnormality or scarring and in 25%, this was severe enough to require future surgery.

Abnormalities, Multiple↗