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One-stage correction of intermediate imperforate anus in males.

This prospective study was designed to assess the safety, cost-effectiveness, and advantages of performing posterior sagittal anorectoplasty (PSARP) without colostomy on males with intermediate imperforate anus in a developing country. Fifteen consecutive males with intermediate imperforate anus were entered into the study. Chest and abdominal x-rays, skeletal surveys, renal ultrasound scans, and invertograms were done. Patients were resuscitated and Pena's PSARP done in prone positions. A 2-ml syringe vent was inserted into the new anus for 10 days. Babies were nursed prone postoperatively. Cephalosporin and metronidazole were given as perioperative antibiotics. All patients had intermediate anomalies. There were no other major associated congenital anomalies. A urethral catheter could not be inserted in one patient, and one patient who presented with septicemia and jaundice was deemed too ill to withstand a major operation; these two patients therefore had diverting colostomies. There were no problems with PSARP in the other 13 patients. One patient's father discharged him against medical advice on the 5th postoperative day; the mother had had postpartum hemorrhage, so they opted for traditional treatment because they could not provide blood donors. The skin wounds of 10 patients healed completely with removal of stitches; two boys had superficial wound infection. Parents who lived far from the hospital were taught how to dilate the anus. Follow-up has ranged between 3 months and 2 years. This prospective study shows that it is feasible for males with intermediate imperforate anus to have safe PSARP without colostomy. The advantages of one instead of three major operations are many, especially in developing countries. If this result can be reproduced in cases of high anomalies, colostomy may be unnecessary in many cases of anorectal malformations, with many benefits to these children and their families.

Anus, Imperforate↗

Comprehensive assessment of long-term postoperative continence in pediatric imperforate anus. Clinical, manometric and contrast studies.

109 patients with imperforate anus were followed up postoperatively for 5 to 28 years. Postoperative continence was evaluated by anorectal manometry, barium enema and clinical symptoms. A comprehensive assessment system, combining the clinical and objective systems was designed which can reflect postoperative anal continence function completely and objectively. It is useful for determining the causes of incontinence and its treatment.

Adolescent↗

Hirschprung's disease and imperforate anus in Pallister-Hall syndrome: a new association.

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

Abnormalities, Multiple↗

One-stage correction of imperforate anus and rectovestibular fistula in girls: Preliminary results.

BACKGROUND/PURPOSE: This prospective study was designed to assess the safety, cost effectiveness, and advantages of performing posterior sagittal anorectoplasty without colostomy on girls with imperforate anus and rectovestibular fistula. METHODS: Four girls with imperforate anus and rectovestibular fistula were entered into the study. Chest x-ray, renal ultrasound scan, and lateral fistulogram were done. Rectal pouches were washed through the fistula with NaCl and aqueous povidone-iodine solutions. Peña's posterior sagittal anorectoplasties were done in the prone positions. Cephalosporin and metronidazole were given as perioperative antibiotics. RESULTS: All patients had intermediate anomalies. There were no other major associated congenital anomalies. Washout through the fistula was easy. There were no particular problems with posterior sagittal anorectoplasty in the prone positions. Two patients had perianal skin excoriations; one had superficial infection of the posterior sagittal wound. Two patients have undergone follow-up for a year. All are having monthly dilatations. All patients pass stool without need of stool softeners or enemas. CONCLUSIONS: This preliminary study shows that it is feasible for girls with imperforate anus and rectovestibular fistula to have safe posterior sagittal anorectoplasty without colostomy. The advantages of one, instead of 3 major operations, are many, especially in developing countries.

Abnormalities, Multiple↗

Challenges in imperforate anus: I--Primary perineal rectourethroanoplasty for anatomical and functional correction in males with supralevator anomalies.

Out of a total of 372 patients with imperforate anus, 99 males with high imperforate anus have been treated. Of these patients 34 have had a new operation through the perineum; 26 of these operations were done as a one stage procedure in the neonatal period. During the operation, through the perineum the puborectalis sling is identified, the recto urethral fistula divided, and the rectum mobilised and brought through the pelvic floor to be sutured to H flaps at the site of the original perineal incision in such a way that these flaps are drawn up to form part of the new anal canal. Post operative clinical and radiological evaluation show good urethral and rectal contours with good functional results. The good functional results are probably partly due to the timing of surgery and partly due to the type of operation.

Anal Canal↗

Imperforate anus in 700,000 consecutive liveborn infants.

We have studied the incidence of imperforate anus (anorectal atresia and stenosis) occurring in almost 700,000 consecutive liveborn infants in British Columbia (B.C.) from 1964-1982 using the records of a health surveillance registry which uses multiple sources of ascertainment. The estimated incidence rate was 1 in 2,524 live births (273 cases out of 689,118 consecutive liveborn infants). Data pertaining to sex ratio, additional anomalies, recurrence, and mortality were also analyzed over the period 1952-1983. Additional anomalies were common in infants with anorectal atresia and stenosis; approximately 6 out of 10 cases had anomalies outside the anorectal canal. Skeletal and limb anomalies were frequent, occurring in almost one-third of patients. Also common were genitourinary anomalies, occurring in one-third of patients. Details of associated anomalies occurring in these infants are given, and clinical implications arising from the study regarding the neonatal assessment of infants with imperforate anus are discussed.

Anus, Imperforate↗

Endoscopic management of infected enlarged prostatic utricles and remnants of rectourethral fistula tracts of high imperforate anus.

PURPOSE: Infected enlarged prostatic utricles and infected remnant fistula tracts of high imperforate anus are usually managed by a suprapubic, transtrigonal or posterior sagittal approach. We describe a minimally invasive endoscopic approach to these entities. MATERIALS AND METHODS: We treated 12 patients with infected enlarged prostatic utricles and 4 with infected remnant fistula tracts using endoscopic techniques. Specifically a resectoscope with a bulb electrode or a cystoscope with a Bugby electrode was used to fulgurate circumferentially the dilated utricle or remnant fistula. After fulguration a Councill catheter was placed in the lesion for 3 to 5 days and urine was diverted via a suprapubic tube for 2 to 3 weeks. Obliteration of the abnormality was verified by a voiding cystourethrogram. RESULTS: Using this technique median postoperative hospital stay was 2 days (range 0 to 7). The enlarged prostatic utricle or remnant fistula tract was completely obliterated in 87% of the cases (62% after 1 and 25% after 2 treatments). Of our patients 13% had a significant (greater than 50%) decrease in utricular cyst size although a urethral abnormality persisted. Postoperative morbidity was minimal. One patient (6%) had a fever for 3 days postoperatively and none has had a urethral stricture during a median followup of 2 years (range 3 months to 4 years). CONCLUSIONS: Although it is not a panacea, electrofulguration of an enlarged prostatic utricle and/or remnant fistula of imperforate anus is a simple procedure that has a high rate of success, does not require prolonged hospitalization and is associated with minimal morbidity.

Adolescent↗

[Duplication of the femur, imperforate anus and polydactyly in a stillborn infant].

An unusual case of stillborn fetus with imperforate anus and left lower limb malformation is described. The principal features were: femoral duplication, muscular atrophy below the knee, heel malformation with absence of calcaneous bone, presence of nine toes of the left foot associated with imperforate anus and with presence of cutaneous tag on the left buttock. No similar case reports are known by the Authors at the present time.

Abnormalities, Multiple↗

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

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

Abnormalities, Multiple↗

Multiple-flap anoplasty in the treatment of rectal prolapse after pull-through operations for imperforate anus.

Rectal prolapse is a frequent complication after pull-through operations for high imperforate anus. Mucosal prolapse causes soiling, occasional bleeding, and pain. Simple resection of the redundant mucosa is unsatisfactory and leads to frequent recurrences or strictures. In 1982, Millard and Rowe reported a technique designed to correct rectal prolapse using two perineal flaps, thus providing a skin-lined anal canal. We have operated on two patients using the same technique. A three-flap anoplasty was used in nine other patients. With an average follow-up of 13 months, none of out patients presented recurrence of the prolapse or a significant stenosis. This procedure is safe and physiologically sound. The skin-lined anal canal provides some sensation where it is lacking. The functional and esthetic results are gratifying and we are now using the three-flap anoplasty as a primary procedure in the correction of high imperforate anus.

Adolescent↗

Transperineal ultrasonography in imperforate anus: identification of the internal fistula.

The purpose of this study was to assess the usefulness of transperineal ultrasonography in identifying the internal fistula in cases of imperforate anus. Transperineal ultrasonography was performed in 19 infants (13 neonates and 6 older infants; 13 were male and 6 were female) with imperforate anus to identify the internal fistula. Sagittal plane images were obtained through the anal dimple, and the internal connection of the rectal fistula was traced. The ultrasonographically traced internal fistula was compared with that observed on distal loopography after colostomy or with surgical findings. The internal fistula was identified as a hypoechoic linear tract, containing linear echogenicity in some cases. Of 19 patients, internal fistulas were correctly identified in 16 patients; these were rectourethral (n = 12), rectovaginal (n = 1), rectovestibular (n = 1), rectovesical (n = 1), and rectocloacal (n = 1). In three patients, internal fistulas were incorrectly defined; these cases consisted of rectovestibular (n = 2) and rectovaginal (n = 1) fistulas. Internal fistulas were correctly identified in all of the 13 male patients and in 3 of 6 female patients. Transperineal ultrasonography is an excellent diagnostic modality to define the type of the internal fistula in imperforate anus.

Anus, Imperforate↗

Long-term anorectal function in imperforate anus treated by a posterior sagittal anorectoplasty: manometric investigation.

Thirty imperforate anus patients were investigated by anorectal manometry 5 to 10 years after a posterior sagittal anorectoplasty. Anal resting tone (ART) and anal squeezing pressure (ASP) were subnormal in most patients. Rectal volume (RV) and sensation to balloon distension were within the normal range. Rectoanal reflex inhibition was demonstrated in 9 of 30 patients. Soiling was more common in patients with a very low ART (less than 40 cm H2O) and a low ASP (less than 100 cm H2O). Constipation was more common in patients with a large RV (greater than 150 mL). Still, the correlation to clinical results was incomplete. As regards to the correlation to type of malformation the rectal atresia patients showed near normal results. The vestibular fistula patients were next in results showing rectoanal reflex inhibition in 5/6 patients. There was no difference in the results between bulbar and prostatic fistula patients.

Adolescent↗

Imperforate anus, delayed presentation in a 7-year-old girl.

Most of the cases of imperforate anus should be discovered at birth, during the initial physical examination. This case represents the status of Third World rural perinatal care, wherein delivery of an infant by a midwife in poverty stricken area of mountainous Jipijapa, Ecuador, failed to reveal the anomaly during the cursory examination. Moreover, the cultural taboos of the Jipijapan agricultural community further delayed the diagnosis. A 7-year-old girl with imperforate anus presenting with a rectovaginal fistula is reported. Specific findings in the case, therapy, and an approach to the diagnosis are discussed.

Anus, Imperforate↗

Hyperchloremic acidosis and imperforate anus.

The predominant electrolyte imbalance associated with enterourinary fistulas is hyperchloremic acidosis. The mechanism is the absorption of urinary electrolytes across the colonic mucosa. One of the genitourinary associated anomalies of a high imperforate anus is a rectourinary fistula. There have been 5 cases of hyperchloremic acidosis as a complication of an imperforate anus with a rectourinary fistula reported in the literature to date. An additional case is presented with a clinical analysis of the previously reported cases. The important factors in the development of hyperchloremic acidosis in patients with an imperforate anus are 1) the presence of a rectourinary fistula, 2) an initial diverting colostomy permitting a long segment of colonic mucosa for the absorption of urinary electrolytes, 3) distal urinary tract obstruction allowing significant volumes of urine to flow into the colonic segment and 4) the presence of urinary tract infection contributing to the urinary obstruction. Management should consist of vigorous electrolyte therapy, decreasing the retrograde flow of urine into the colon by temporary catheterization and early repair of the fistulous tract.

Acidosis↗