1/12. Pyloric atresia associated with epidermolysis bullosa, malrotation, and high anorectal malformation with recto-urethral fistula: a report of successful management.Pyloric atresia (PA) is an uncommon anomaly that may be associated with many other congenital anomalies, the commonest of which is junctional epidermolysis bullosa (JEB). Most of the cases of PA associated with JEB (Herlitz syndrome) reported have been fatal. A case of PA associated with JEB, malrotation, and a high anorectal malformation with a rectourethral fistula, which was hitherto undescribed, was successfully managed at our institution.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/12. The prenatal diagnosis of imperforate anus with rectourinary fistula: dilated fetal colon with enterolithiasis.The finding of extraluminal calcifications is commonly reported and usually indicates intrauterine intestinal perforation with intraperitoneal extravasation of meconium, most often associated with intestinal obstruction and/or atresias. Intraluminal calcification of meconium is more rare and appears to result from the mixing of stagnant urine and meconium in utero. The presence of the intraluminal calcifications in a dilated loop of intestine, particularly with an associated urinary tract abnormality, should suggest a rectourinary fistula. Two cases of prenatally diagnosed imperforate anus with rectourinary fistulae are reported.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
3/12. A case of rectal atresia associated with recto-bulbar urethral fistula.Rectal atresia is a rare condition in which the anus and sphincter muscles are normally developed, with usually no fistulous communication with the urinary tract. An unusual case of rectal atresia associated with recto-bulbar urethral fistula treated by a combination of colo-anal anastomosis and mucosal proctectomy via a posterior sagittal approach is reported for the first time.- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |
4/12. Augmented-pressure colostogram in imperforate anus with fistula.Most newborns with imperforate anus, except for those with very low varieties, undergo a diverting colostomy performed in the postnatal period, with definitive surgical repair at a later age. Accurate demonstration of the anatomy of any associated fistula between the rectum and urogenital tract is essential for optimal surgical management. An augmented-pressure distal segment colostogram is recommended prior to definitive repair, both to confirm the level of rectal atresia and to define any associated fistulous communication. We report a case of high imperforate anus with rectourethral fistula in which the fistulous tract was not identified on the conventional contrast colostogram but was readily delineated when an augmented-pressure modification of the technique was utilized. The technical aspects of augmented-pressure colostography are presented.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
5/12. Hyperchloremic metabolic acidosis as a clue to recto-urethral fistula in an infant with anal atresia.An infant with high anal atresia and transverse colostomy, in whom initial radiologic evaluation of the urinary tract had been normal, developed hyperchloremic metabolic acidosis at 24 days of age. gastroenteritis and renal tubular acidosis as possible causes for this metabolic disturbance were excluded, which prompted a repeat investigation of the possibility of a communication between the urinary tract and the rectum. A recto-urethral fistula was demonstrated by urethrography. Analysis of the fluid obtained from the left colon as compared to urine in the bladder and voided urine demonstrated that electrolyte exchange was taking place in the colon, resulting in hyperchloremic hypokalemic acidosis. Treatment with oral sodium bicarbonate and daily lavage of the left colon resulted in normalization of the acid-base status and catch-up growth of the baby. Hyperchloremic acidosis associated with anal atresia and recto-urinary communication appears to be uncommon. However, early diagnosis and treatment of the metabolic derangement are of importance as it may determine the infant's overall prognosis.- - - - - - - - - - ranking = 86.635027773327keywords = anal atresia, atresia (Clic here for more details about this article) |
6/12. Use of the recto-urethral fistula for reconstruction of the anal canal in high anal atresia.A 6-month-old boy with high anal atresia was operated upon via a sacro-perineal approach. After division of the rectourethral fistula, pressure recordings from the rectal part of the fistula during electrical stimulation of the rectal mucosa showed a resting pressure and relaxations resembling internal anal sphincter activity. The fistula was used for the reconstruction of the anal canal and anastomosed to the skin in the anal incision. The postoperative course was uneventful. Six weeks after the operation ano-rectal manometry showed a high resting anal pressure and normal internal sphincter relaxations. It is concluded that the presented surgical technique may provide a functioning internal sphincter in at least some patients with high anal atresia with recto-urethral fistula.- - - - - - - - - - ranking = 86.635027773327keywords = anal atresia, atresia (Clic here for more details about this article) |
7/12. epidermolysis bullosa dystrophica with urinary tract involvement.A case of epidermolysis bullosa dystrophica ( EBD ), neonatally corrected atresia of the pylorus and early infantile development of urinary tract manifestations is reported. Owing to misinterpretation of radiological findings, instrumental treatment of the urethra was employed. It resulted in complete and permanent obliteration of the lumen. While the skin disorder has remained slight to moderate in severity, recurrent keratitis has resulted in impaired vision.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
8/12. Genital abnormalities in females with bardet-biedl syndrome.The major manifestations of the bardet-biedl syndrome are digital anomalies, tapetoretinal degeneration, obesity, renal abnormalities, and hypogenitalism (described mainly in males). We report on 2 girls with bardet-biedl syndrome who also had vaginal atresia. A similar association in females with bardet-biedl syndrome was suggested in published reports of 11 affected individuals who had structural genital abnormalities, (some of which were missed in childhood), including persistent urogenital sinus, ectopic urethra, hypoplasia of the uterus, ovaries and fallopian tubes, uterus duplex, and septate vagina. The association of atresia of the vagina and other malformations of female genital structures in individuals with bardet-biedl syndrome has often been missed in childhood and should be looked for more systematically.- - - - - - - - - - ranking = 0.4keywords = atresia (Clic here for more details about this article) |
9/12. Posterior urethral diverticula: a complication of surgery for high anorectal malformations.We describe five boys, all of whom presented with urinary tract infection or acquired urinary incontinence some years after surgery for a high anorectal malformation (arm). All were found to have a posterior urethral diverticulum thought to represent the remains of the original rectourethral fistula accompanying the high rectal atresia. Excision of the diverticula resulted in complete relief of symptoms. The clinical features and radiological appearances of the diverticula are described. A urethral diverticulum is one treatable cause of urinary symptoms in children with high anorectal malformations, and should be considered when symptoms recur late after definitive surgical correction of the malformation.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
10/12. hydrocolpos, vaginal atresia, and urethrovaginal fistula in a neonate: abdomino-perineal-vaginal pull-through.The combination of high maternal estrogenic stimulation of the secretory glands of the reproductive tract of the newborn female and vaginal obstruction results in an abdominal mass due to vaginal distention (hydrocolpos). Prior experience with two infants with hydrocolpos (LG) led us to perform abdomino-perineal-vaginal pull-through, as advocated by Ramenofsky and Raffensperger, in the newborn period. So far as we can ascertain this is only the second infant so reported in the literature.- - - - - - - - - - ranking = 0.8keywords = atresia (Clic here for more details about this article) |
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