1/29. Successful treatment of tracheomalacia associated with esophageal atresia without a tracheoesophageal fistula by aortopexy: report of a case.tracheomalacia (TM) is well known as a complication associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF); however, the occurrence of TM requiring surgical treatment in a patient having EA without a tracheoesophageal fistula has never been reported. We describe herein a rare case of TM associated with EA without TEF. Respiratory distress was caused by compression of the trachea by a severely dilated upper esophageal pouch with weakness of the tracheal wall. Aortopexy was performed, and an excellent postoperative result was achieved.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/29. Multiple gastrointestinal atresias with cystic dilatation of the biliary duct.A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.- - - - - - - - - - ranking = 1.4keywords = atresia (Clic here for more details about this article) |
3/29. Postsurgical use of amplatzer septal occluder in cyanotic patients with pulmonary atresia/intact ventricular septum: significance of cor triatriatum dexter and dilated right atrium.Percutaneous closure of secundum atrial septal defects (ASDs) has been shown to be safe and effective. However, its role after surgery in patients with cyanotic congenital heart disease who may have associated cor triatriatum dexter and a dilated right atrium has not been established. This article reports on successful closure in such patients, including precautions and results.- - - - - - - - - - ranking = 0.8keywords = atresia (Clic here for more details about this article) |
4/29. A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome.A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest x-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
5/29. Distal foregut atresias in consecutive siblings and twins in the same family.The distal foregut comprises the antrum of the stomach and supra-ampullary region of the duodenum. In this part of the gut, intraluminal mucosal diaphragms, webs, membranes (type I), and rarely solid cord (type II) atresias are occasionally seen in clinical practice. Due to increased awareness, the number of reported cases has increased in recent years. These foregut lesions are known to have a strong genetic association. Both antral (AA) and duodenal atresias (DA) may occur as potentially curable sporadic cases or in combination with lethal malformations such as hereditary multiple gastrointestinal atresias affecting the fore-, mid-, and hindgut. The association of AA and junctional epidermolysis bullosa lethalis is well-known. We describe an unusual occurrence of foregut atresias in two consecutive siblings and a pair of monozygous twins. The involvement of four siblings in a single family is extraordinary and as yet unreported. This cluster of cases not only lends a strong support to a genetic origin, but also the mode of transmission, despite the absence of a history of consanguinity. It indicates that atresia of the gastric antrum and supra-ampullary DA are etiologically closely related. It is also possible that the discordant expression in the twins could be a pleiotropic effect of the same genetic factor. Attempts are made to explain the possible pathogenesis of this unusual atresia and relate it to other associated anomalies.- - - - - - - - - - ranking = 2keywords = atresia (Clic here for more details about this article) |
6/29. Rapid progression of intrapulmonary arteriovenous shunting in polysplenia syndrome associated with biliary atresia.This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absent retrohepatic inferior vena cava, and azygous connection. She was diagnosed postnatally as BA because of persistent acholic stool and neonatal jaundice. She underwent hepatic portoenterostomy at age 158 days. The gallbladder and the hepatic ducts were hypoplastic, and the common bile duct was absent. Magnetic resonance image and operative findings also identified polysplenia and an absent portal trunk. Liver histology showed cirrhotic changes and bile duct proliferation. Postoperatively, she achieved good bile secretion, with gradual decrease of total bilirubin. However, she had repeated febrile episodes, and computerized tomography at age 7 months showed multiple liver cysts. Thereafter, she presented with exertional dyspnea. Contrast-enhanced echocardiography showed IPS with a degree of 2/III at age 8 months and 3/III at 10 months. (99m)technetium-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy revealed a shunt ratio of 25.5% at 9 months and 39.7% at 10 months. Percutaneous transhepatic drainage of the bile cysts was performed without success. Sludged bile was obtained. However, respiratory distress rapidly progressed, and she died at age 11 months. In the present patient, the association of polysplenia syndrome and absent portal vein with BA, as well as liver cirrhosis, seemed to be contributing factors to rapid progression of IPS in early life.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
7/29. prenatal diagnosis of esophageal atresia with the pouch sign.A 19-year-old primipara was referred to our center for a routine scan at 33 4 weeks' gestation. A visible but small stomach bubble (18 mm) was detected in the fetal abdomen, associated with a dilation of the esophagus with a tapering distal part. Direct visualization of filling and emptying of the proximal esophagus suggested the presence of obstruction. A cystic pouch in the region of the esophagus was observed to be full and empty in accordance with fetal swallowing. No other fetal anomalies were detected. Fetal biometric measurements were on the 10th percentile and polyhydramnios was detected. esophageal atresia type I was suspected. Fetal karyotyping was declined by the parents, no therapy was given and the pregnancy continued until week 37 when a female baby was delivered following spontaneous labor. esophageal atresia type I was radiographically confirmed. Bougienage was used for 8 weeks and esophageal anastomosis was successfully performed when the infant was 11 months old. No other anomalies were found and the child is currently in excellent health. The present case shows that the upper neck pouch sign may be a delayed manifestation present only in certain types of esophageal atresia. However, when it occurs it should prompt careful fetal examination as it is an important step in the diagnosis of esophageal atresia.- - - - - - - - - - ranking = 1.6keywords = atresia (Clic here for more details about this article) |
8/29. Brown bowel syndrome: a late complication of intestinal atresia.Two children, aged 11 years, who originally had jejunal atresia corrected in the neonatal period, developed massive dilatation of the proximal small intestine. This resulted in circular muscular hypertrophy with lipofuscin deposits giving the typical appearance of "brown bowel." The condition was associated with malnutrition and vitamin e deficiency. Because of relatively short bowel, the condition was treated by limited resection and extensive tapering of the dilated segment, end-to-end reanastomosis, vitamin E supplementation, and intensive nutritional support.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
9/29. biliary atresia with associated complicated anorectal and urogenital malformations.We report on a girl with biliary atresia (BA) who also suffered with anorectal agenesis without fistula and complicated urogenital malformation. The outcome of patients with these severe anomalies is poor, but she has survived without liver and/or renal transplantation for more than 3 years. A careful treatment plan for each anomaly in addition to prevention of cholangitis and urinary tract infection is indispensable for managing these complicated anomalies.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
10/29. A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum.BACKGROUND/PURPOSE: The authors experienced 3 cases of persistent pulmonary hypertension of the neonate (PPHN) associated with duodenal atresia with paradoxical dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association. methods: The medical charts of the patients were reviewed retrospectively. RESULTS: Case 1 was a 2,862-g male infant with a prenatal diagnosis of duodenal atresia. The dilated intestine was identified as the distal blind end of the duodenum during duodeno- duodenostomy. PPHN developed after surgery, and the patient died despite treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). lung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment of pulmonary vessels. Case 2 was a 2,244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in death. autopsy results showed ACD with misalignment of pulmonary vessels and duodenal atresia associated with dilatation of the distal blind end. Case 3 was a 2,462-g female infant with a prenatal diagnosis of duodenal atresia associated with dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully under combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrasonographic findings showed enlarged echogenic fetal lungs as in the first 2 cases. CONCLUSIONS: The characteristics of this novel association are enlarged echogenic fetal lungs, duodenal atresia associated with paradoxical dilatation of the distal blind end, and refractory PPHN resistant to iNO.- - - - - - - - - - ranking = 2keywords = atresia (Clic here for more details about this article) |
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