1/13. 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) |
2/13. Imperforate anus in Feingold syndrome.A father and daughter had the characteristic findings of Feingold syndrome including microcephaly, short palpebral fissures, brachydactyly with clinodactyly of fifth fingers, and bilateral syndactyly of second to third and fourth to fifth toes. The infant presented with long-gap esophageal atresia without fistula (type A). Her father, who had short stature and learning disabilities, had congenital imperforate anus with a recto-vesical fistula. This is the first report of distal intestinal atresia in Feingold syndrome.- - - - - - - - - - ranking = 0.4keywords = atresia (Clic here for more details about this article) |
3/13. Rectal cancer in anorectal malformation with rectovestibular fistula: sphincter-restoring surgery by anterior resection and dynamic vaginoanograciloplasty.PURPOSE: The aim of this study was to describe and discuss the anosphincter-restoring procedure adopted in an adult patient with rectal cancer, affected by anorectal atresia and rectovestibular fistula not previously treated. methods: After anterior resection and transverse dissection of the rectovaginal septum, a straight double-staple low colorectal anastomosis was performed. A subcutaneous tunnel was extended from the inter-rectovaginal space, surrounding the pseudoanal orifice. The tunnel housed a left gracilis muscle arranged counter-clockwise and connected to an implanted stimulator. The vaginal and anorectal flaps were sutured longitudinally, thereby spacing out the two orifices and creating an anorectal angle. A temporary loop right colostomy was performed. RESULTS: The postoperative course was uneventful. After continuous electrostimulation training and colostomy takedown, the patient had improved continence (stimulator on) and normal defecation (stimulator off). CONCLUSIONS: The solution described seemed a suitable alternative to a pull-through procedure, which would have involved a colocutaneous anastomosis and the loss of the anorectal sensory function. Any previous anal transposition or pull-through procedures would have prevented the saving of the pre-existing "anus," because of the interruption of the residual mesenteric vascular supply to the anorectal remnant secondary to mesorectal excision.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
4/13. oligohydramnios and megacolon in a fetus with vesicorectal fistula and anal-urethral atresia: a case report.Severe oligohydramnios and extremely dilated bowel filled with hyperechogenic material floating in fluid were the ultrasonographic findings in a fetus at 27 weeks' gestation. Vesicorectal communication and urethral-anal atresia permitted urine to empty into the colon, causing megacolon, oligohydramnios, and markedly increased intraabdominal pressure resulting in pulmonary hypoplasia.- - - - - - - - - - ranking = 20.083941821408keywords = anal atresia, atresia (Clic here for more details about this article) |
5/13. A case report: vesico-rectal fistula with ano-urethral atresia.Vesico-rectal fistula is a rare congenital abnormality causing severe early second trimester oligohydramnios. prenatal diagnosis of such a case is reported here. Ultrasound diagnosis could be aided by transabdominal amnio-infusion and, if necessary, fetal intraperitoneal saline installation. In a karyotypically normal fetus with normal somatic growth, demonstration of normal fetal kidneys together with a functioning urinary bladder, in presence of severe oligohydramnios, is very suggestive of the diagnosis. Since pulmonary hypoplasia is the major cause of neonatal mortality in these cases, restoration of normal amniotic fluid volume by serial amnio-infusion was attempted. Although amnio-infusion is an important diagnostic aid in the evaluation of severe midtrimester oligohydramnios, the role of multiple therapeutic amnio-infusion in improving lung growth remains to be evaluated.- - - - - - - - - - ranking = 0.8keywords = atresia (Clic here for more details about this article) |
6/13. 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) |
7/13. An exceptional type of anorectal malformation: anorectal atresia with posterior fistula in a newborn male.The authors report the case of a newborn male with an exceptional type of anorectal atresia with a fistula that passes posterior to the muscle complex and exits at a site just to the tip of the coccyx. This anorectal malformation was associated with other abnormalities including agenesis of the right kidney, pyelic dilatation of the left kidney, duplicity, and left megaureter. There was no defect in the spine. The anorectal malformation was managed successfully by a posterior sagittal approach according to the Pena procedure.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
8/13. 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) |
9/13. Congenital anal fistula with normal anus.Three patients with a congenital anovestibular/perineal fistula were treated at the Montreal Children's Hospital. Two females (one of East Indian and the other of Japanese origin) had anovestibular fistulae that became symptomatic in the first few months of life. The third patient, a boy of Korean descent, presented at 9 months of age with a chronically draining perineal fistula. During surgery, a small fistula tract was easily dissected out and excised. Microscopic examination showed a well-preserved rectal mucosa throughout the tract. Most male patients described to date had anourethral fistulae, often accompanied by other major anomalies such as esophageal atresia or renal malformations. We believe our patient is the first male to be described with a congenital perineal fistula; this suggests that some fistula-in-ano in male infants may be due to a congenital sinus that secondarily becomes infected and drains to the skin.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |
10/13. 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 = 115.70365092845keywords = anal atresia, atresia (Clic here for more details about this article) |
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