Cases reported "Situs Inversus"

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1/319. 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. ( info)

2/319. Coronary stenting in patients with situs viscerum inversus.

    Atherosclerotic coronary disease may develop even in patients with complex cardiovascular anomalies who reach adult life. Coronary revascularization (surgical or interventional) may present potential difficulties related to the unusual anatomy. We describe two cases of situs viscerum inversus who underwent cardiac catheterization and angiography as adults because of ingravescent angina. The study identified the cardiac relations and connections, and diagnosed severe coronary disease that was successfully treated with coronary angioplasty and stent implantation. The implications of performing such procedures in patients with similar cardiac anomalies are discussed. ( info)

3/319. New syndromic entity of situs inversus totalis.

    A 22-year-old Bedouin female with MCA/MR has been recently ascertained. She showed profound mental retardation, proportionate short stature, facial dysmorphism, spastic quadreparesis, bilateral taliper equinovarus, brachydactyly, situs inversus totalis, and MRI findings of cerebellar/midbrain migration defects. The described phenotype represents a new syndromic situs inversus with a characteristic Facio-Cerebro-Skeleto-Cardiac phenotype. ( info)

4/319. 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. ( info)

5/319. A case of malrotation and situs ambiguus.

    Isolated levocardia is a rare condition in which intestinal malrotation may be encountered. The case herein reported is particularly uncommon and raises a number of questions about the development of abdominal symmetry. ( info)

6/319. urinary diversion following cystectomy in a patient with situs inversus totalis.

    situs inversus totalis is a rare syndrome. The association of malignancies with situs inversus totalis is rare, and only 9 cases were reported. The management of invasive bladder cancer in the presence of situs inversus totalis has not been reported previously, and herein we report the 1st case. ( info)

7/319. 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. ( info)

8/319. New syndrome?: Three sibs diagnosed prenatally with situs inversus totalis, renal and pancreatic dysplasia, and cysts.

    Recently we described a previously apparently undescribed autosomal recessive syndrome in two sib fetuses with situs inversus totalis, cystic dysplastic kidneys and pancreas, bowing of the lower limbs and clavicles, severe intrauterine growth retardation, and oligohydramnios. This syndrome differs from that of Ivemark and related syndromes due to lack of liver involvement. After these two sibs, this consanguineous family had a third child and an early prenatal diagnosis of pancreatic and dysplastic renal cysts was made in the 19.5-week-old fetus. The last case supports the genetic hypothesis. ( info)

9/319. situs inversus with cholelithiasis.

    situs inversus totalis is a form of heterotaxia which is usually detected accidentally while investigating for any associated condition. If undetected, this condition can create a diagnostic puzzle. We report one such case in which situs inversus was associated with cholelithiasis. ( info)

10/319. Renal dysplasia and situs inversus totalis: an autopsy case report and literature review.

    Renal dysplasia has rarely been reported to be associated with situs inversus. Only 4 cases were reported previously with a spectrum of anomalies including bilateral renal dysplasia, situs inversus totalis, and pancreatic and hepatic fibrosis. Recently we encountered another case. A 22-year-old mother, gravida 1 para 0, was in her 23rd week of gestation when she was found to have oligohydramnios. A dead baby was delivered by extraovular induction. On autopsy, Potter's sequences including flattened, low-set ears, receding chin, upturned nose, and club hands and feet were noted. The posterior fontanel was obliterated. Internal examination showed situs inversus totalis, bilateral renal dysplasia, and agenesis of bilateral ureters. There was increased interstitial fibrosis in the pancreas. None of the family members was known to have any renal anomalies. Pinar and Rogers suggested a new syndrome when situs inversus totalis was combined with bilateral renal dysplasia as well as multisystem fibrosis. In our case, 2 major abnormalities, i.e., situs inversus totalis and bilateral renal dysplasia, were present though the increase of interstitial fibrosis within the pancreas was not as significant. This case is likely to be within the spectrum of anomalies mentioned by Pinar and Rogers. ( info)
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