1/27. liver transplantation in patients with situs inversus.Two patients with situs inversus and biliary atresia were treated with hepatic transplantation, one with an auxiliary liver and the other with an orthotopic graft which was placed using a piggy-back technique. Both transplants functioned well initially. The auxiliary liver was rejected after 1 1/2 months, and the patient died after an attempt at retransplantation many months later. The recipient of the orthotopic liver has perfect liver function 10 months postoperatively.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/27. Microdeletion of chromosome sub-band 2q37.3 in two patients with abnormal situs viscerum.We report on two cases of microdeletion of chromosome sub-band 2q37.3 with abnormal situs viscerum. The first patient had dextrocardia, duodenal and jejunal atresia, and an abdominal hernia. The liver was in the left upper quadrant, stomach in the right upper quadrant. In contrast anema the ascending colon was in the left, and descending colon on the right, with an area of atresia in the mid-jejunum. The second patient had malrotation and malposition of large and small bowel, with most of the bowels positioned above the liver and spleen. There was incomplete rotation of the cecum. The right kidney was malrotated and mal-positioned. The finding of 2q37.3 deletion in both patients implies that a locus or loci involved in the development of normal body situs lies within this chromosome region. Molecular cytogenetic evaluation for a possible 2q37.3 deletion should be considered in patients with abnormal situs viscerum.- - - - - - - - - - ranking = 2keywords = atresia (Clic here for more details about this article) |
3/27. Combined Rastelli and atrial switch procedure: a 10-year follow-up.We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
4/27. liver transplantation from situs inversus to situs inversus.Congenital anatomic anomalies often present technical obstacles during liver transplantation. biliary atresia (BA) is the most common indication for liver transplantation in children, and up to 28% of children with situs inversus are complicated by BA. A boy aged 2 years 11 months with BA, situs inversus, and dextrocardia received a liver transplant from his father. The donor also had situs inversus and dextrocardia without other anomalies. Graft function was excellent postoperatively, and no significant complications were encountered. This is only the second report of the successful use of a living related donor graft for a patient with BA and situs inversus. This case was particularly rare because the donor also had situs inversus, which made the present procedure more feasible.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
5/27. esophageal atresia with distal tracheoesophageal fistula associated with situs inversus.The presence of situs inversus (SI) causes difficulty in the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We report two cases: the first infant had situs SI abdominis with a right-sided aortic arch and the second had situs inversus totalis. To our knowledge, these are the first reported cases of repair of EA/TEF in a patient with SI.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
6/27. A new variant of esophageal atresia with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus.esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is the most common anatomic pattern within congenital anomalies of the esophagus. Also, more than 50% of the infants with esophageal atresia have other congenital malformations. To our knowledge, this is the first case report of EA with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus (SI).- - - - - - - - - - ranking = 6keywords = atresia (Clic here for more details about this article) |
7/27. Late presentation of a duodenal web in a patient with situs inversus and apple peel jejunal atresia.A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
8/27. A case of agnathia, situs inversus, and a normal central nervous system.We report here a premature female infant with agnathia, low-set but normally formed ears, a downward eye slant, choanal atresia and a cleft palate. She had severe respiratory distress and died despite maximum intervention at 5 days of age. autopsy revealed situs inversus totalis; crossed fused renal ectopia; agnathia; normal thyroid, larynx, trachea, and bronchi; incomplete lobation of the lungs; immature pulmonary development with early hyaline membranes; and a normal central nervous system. This lack of significant central nervous system abnormalities distinguishes this infant from the majority of previously reported infants with agnathia and situs inversus.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
9/27. Case of living donor liver transplantation in a patient with biliary atresia combined with situs inversus.Until recently, situs inversus was considered to be an absolute contraindication for liver transplantation. However, recent reports have suggested that situs inversus should not be considered a contraindication. This study presents a successful living donor liver transplantation performed in a 4-month-old male infant with biliary atresia and situs inversus. The surgical findings revealed abdominal situs inversus with polysplenia and an absent retrohepatic inferior vena cava and intestinal malrotation.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
10/27. Living donor liver transplantation using a right lobe graft in an adult with situs inversus.situs inversus totalis is a rare anatomic variant in which there is a complete mirror image of the thoracic and abdominal viscera. The common association of situs inversus and biliary atresia has led to a variety of modifications of surgical techniques utilizing both living donor and deceased donor liver grafts, with mixed results in the pediatric liver transplant population. The use of a living donor liver graft in an adult with situs inversus has not yet been described. However, living donor liver transplantation (LDLT) has produced excellent results in the adult population, particularly in the cholestatic population, which may be disadvantaged by the model for end-stage liver disease system. This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
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