1/47. Multiple atresias in a low-birth-weight twin.This report describes a case of 16 small bowel atresias in a twin who was born at 31 weeks' gestation, weighing 1,690 g. All atresias and intestinal segments of 5 cm or less in length were resected, resulting in nine primary anastomoses, preserving 75% (107 cm) of his initial small bowel length. The baby went home on full oral feedings after 10 weeks. Multiple anastomoses in the low-birth-weight neonate can be tolerated with the functional benefit of maximal bowel length. The time taken to tolerate feedings appears to be independent of the number of anastomoses. Vascular anastomoses associated with monochorionic twinning may place both fetuses at risk of intestinal atresia in the event of an ischemic insult, either concurrently or with the demise of one affecting the other. Prenatal ultrasound scan appears to be useful for monitoring the evolution of intestinal atresia. However, the risks of extreme prematurity preclude the delivery of the affected baby at the time of initial diagnosis, and as yet it is unknown whether early delivery will alter the number, type, or prognosis of multiple atresias.- - - - - - - - - - ranking = 1keywords = intestinal atresia, atresia (Clic here for more details about this article) |
2/47. Intracranial malposition of a nasogastric tube following repair of choanal atresia.Intracranial penetration during attempted nasogastric intubation is a rare, often lethal occurrence. We report the inadvertent introduction of a nasogastric tube intracranially in a neonate following repair of unilateral choanal atresia. Following manual removal of the tube, the patient made a good recovery.- - - - - - - - - - ranking = 0.21054144884106keywords = atresia (Clic here for more details about this article) |
3/47. Jejuno-ileal atresia in identical twins: report of a case.Small intestinal atresia occurring in twins is extremely rare. The aims of this study are to report a case of identical twins with different types of jejunal atresia, to review six other pairs of twins, and discuss the etiology. Our case was the seventh in which both twins were diagnosed to have jejuno-ileal atresia. Furthermore, they are also only the sixth and seventh patients known to survive. All the other published cases involved identical twins except one. Three pairs had different types of atresia and four pairs did not have any other anomalies. In addition, to date there have been no further indications of any other members of these families being affected with intestinal atresia. Therefore, we conclude that most such cases might be due to environmental influences during gestation.- - - - - - - - - - ranking = 1keywords = intestinal atresia, atresia (Clic here for more details about this article) |
4/47. Effect of liver transplantation in a twin for biliary atresia on physical development and intellectual performance: report of a case.A case of twins, one of whom suffered from biliary atresia, is described herein. Although the patient had been doing well until 11 years of age after previously undergoing a primary hepatic portoenterostomy, she had to then undergo a liver transplantation due to severe refractory cholangitis at 14 years of age. Although the patient's intellectual performance had severely declined due to the progression of her illness for several years, it completely recovered after the liver transplantation. In cases where the physical development in childhood has been well preserved, liver transplantation might therefore offer the chance for a full recovery of deteriorated intellectual performance.- - - - - - - - - - ranking = 0.21054144884106keywords = atresia (Clic here for more details about this article) |
5/47. Monozygotic twins with chromosome 22q11 microdeletion and discordant phenotypes in cardiovascular patterning.Monozygotic twins with chromosome 22q11 microdeletions offer an ideal situation to observe the association of microdeletion and disrupted cardiovascular patterning. We report monozygotic twins concordant for 22q11.2 microdeletion but discordant for cardiovascular patterning. Both twins showed identical intracardiac defects including tetralogy of fallot with pulmonary atresia. Nevertheless, their great vessel patternings were variable. These twins show that the mispatterning of the great vessels may not correlate with intracardiac morphogenesis. The discordant development of the great vessels, especially in the pulmonary vascular system, has clinical significance for prognosis. The phenotypic variability of cardiovascular anomalies seen in 22q11 microdeletion cannot be explained on the basis of genotypic difference.- - - - - - - - - - ranking = 0.042108289768212keywords = atresia (Clic here for more details about this article) |
6/47. Unilateral choanal atresia in identical twins: case report and literature review.choanal atresia is postulated to be secondary to an abnormality of the rupture of the buccopharyngeal membrane during the embryological period. This condition usually occurs sporadically, but has been described in siblings and successive generations. The genetics remain unclear. We present monozygotic twins with identical findings of unilateral choanal atresia and no other associated anomalies. To our knowledge, this is the first report of such an occurrence.- - - - - - - - - - ranking = 0.25264973860927keywords = atresia (Clic here for more details about this article) |
7/47. twins with oesophageal atresia and the CHARGE association.twins are rarely concordant for oesophageal atresia and tracheo-oesophageal fistula. Such a case is presented and their similar associated anomalies described. The CHARGE association is considered as a possible diagnosis and the aetiological implications considered.- - - - - - - - - - ranking = 0.21054144884106keywords = atresia (Clic here for more details about this article) |
8/47. Concordance for hypoplastic left heart syndrome in a monochorionic twin pregnancy.The risk of structural heart disease is significantly higher in twin pregnancies than in singleton pregnancies, but the concordance rate has been found to be relatively low, even in monochorionic pregnancies. This is the first report of a monochorionic twin pregnancy concordant for hypoplastic left heart syndrome (HLHS), the diagnosis having been made by fetal echocardiography at 15 weeks' gestation. The findings were confirmed at necropsy at 17 weeks' gestation, following termination of pregnancy. Both twins had mitral and aortic atresia, with severely hypoplastic aortic arches. This report adds weight to there being a genetic component to the cause of HLHS in some cases and illustrates how the findings from early fetal echocardiography with postmortem follow up can help to extend the understanding of the aetiology of this condition.- - - - - - - - - - ranking = 0.042108289768212keywords = atresia (Clic here for more details about this article) |
9/47. 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 = 0.73159559372516keywords = intestinal atresia, atresia (Clic here for more details about this article) |
10/47. Jejunal atresia related to the use of toluidine blue in genetic amniocentesis in twins.Since 1990 avoidance of methylene blue as a dye in diagnostic amniocentesis is recommended. This is the result of the observation that a high incidence of jejuno-ileal atresia appeared in twin pregnancies following intraamniotic injection of methylene blue. We report a case of jejunal atresia in twins after injection of toluidine blue. We describe the clinical course, discuss possible teratogenic mechanisms and emphasize that no synthetic dyes should be used in second trimester amniocentesis.- - - - - - - - - - ranking = 0.25264973860927keywords = atresia (Clic here for more details about this article) |
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