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Anatomical features of liver in situs inversus.

The hepatic veins and the intrahepatic branches of both the portal vein and the hepatic artery have been dissected and studied in three specimens of liver obtained from bodies in situs inversus. The position of the hepatic veins was found generally to correspond to the intersegmental scissure planes of the liver. The intrahepatic branches of the portal vein were found to delineate the subdivision of the liver into lobes, segments and subsegments which correspond to the 'mirror image' of the normal liver. The arterial irrigation derives from the mirror image constituting peculiar patterns in each of the specimens. In the first specimen, the left hepatic artery irrigates the superior subsegments of the right lobe, which depends on the right branch of the portal vein. In the second specimen, a voluminous anastomotic intrahepatic channel was noted between the right hepatic artery and the subsegmental posterior inferior branch of the left hepatic artery. In the third specimen, the arterial branch which irrigated the inferior subsegments of the right lobe also contributed to the irrigation of the superior lateral subsegment.

Adult↗

Laparoscopic cholecystectomy in situs inversus totalis: The importance of being left-handed.

Since laparoscopic cholecystectomy has become the standard procedure for the treatment of gallstone disease, several cases have been reported in patients with situs inversus. These cases require more technically demanding procedures due to the symmetrical disposition of the anatomy. Thus, handedness could influence the performance of these operations. The two of us (L.M.O.) and (J.M.B.), a right-handed and a left-handed surgeon, respectively, placed the instruments in reverse mode from that used in orthotopic patients. The right-handed surgeon felt more impairment when dissecting with his left hand and decided to cross the instruments within the abdomen. The left-handed surgeon was able to alternate the performance of the dissection maneuvers between the right and left hands. Surgical procedures are apparently designed for right-handed surgeons and can be approached by the left-handed in alternative ways. In fact, the accommodation of laparoscopic cholecystectomy to left-handedness has been described in the literature. The rare opportunity to operate in a symmetrical way allows the right-handed surgeon to understand the absence of comfort and ergonomy often experienced by left-handed colleagues.

Aged↗

Familial clustering of situs inversus totalis, and asplenia and polysplenia syndromes.

We report on a family in which a male infant had the asplenia syndrome, a younger brother had the polysplenia syndrome, and their father had situs inversus totalis. The occurrence of the asplenia and the polysplenia syndromes in a sibship of the present family and in two other previously reported sibships indicates that the two syndromes are causally and pathogenetically related to each other. If it is assumed that the father had an incomplete form of the polysplenia complex, then the condition in this family either is an autosomal dominant trait or is multifactorially determined.

Adult↗

Situs inversus abdominus in association with congenital duodenal obstruction: a report of two cases and review of the literature.

This report describes two newborns with persistent bile-stained vomiting. Their radiological investigations revealed the existence of situs inversus and duodenal obstruction. In one, the duodenal obstruction was partial, secondary to a duodenal diaphragm with a central aperture, whereas the other child had complete duodenal atresia as well as Fallot's tetralogy. Such an association is extremely rare, with only 18 cases reported so far in the literature. Embryological aspects, investigations, and treatment are also discussed.

Duodenal Obstruction↗

Coronary arteriography in the presence of dextrocardia and situs inversus.

Given the rarity of dextrocardia, it is not unexpected that there are only a few reports of coronary arteriography in such a setting. We describe a case of successful coronary arteriography in a patient with mirror image dextrocardia and situs inversus, and the technique used, and review the literature.

Angina Pectoris↗

The versatility of the Amplatzer Septal Occluder for the management of multiple atrial septal defects in a patient with dextrocardia and situs inversus.

A 7-year-old male was referred for evaluation of a heart murmur heard on routine check-up. Transthoracic echocardiogram revealed the presence of a fenestrated atrial septal defect (ASD) in a dextrocardia with total situs inversus ( mirror image ). Two Amplatzer Septal Occluders (Cook Incorporated, Bloomington, Indiana) were used to successfully occlude the ASDs with no residual shunt.

Cardiac Catheterization↗

[D-transposition in situs inversus, atrial septal defect, open foramen ovale and tricuspid valve insufficiency].

This paper reports on a rare case of a so-called corrected d-transposition of the great vessels of a 62-year old woman with situs inversus. In addition the patient had a VSD, a patent foramen ovale, and a tricuspid-valve insufficiency. The topography of the ventricular cavities, the great vessels and the coronary arteries is described in detail. There is pointed to the surgical treatment of additional abnormalities to improve the prognosis.

Electrocardiography↗

Congenitally corrected transposition of the great arteries associated with hypoplasia of the morphological left ventricle in the setting of atrial situs inversus.

Hypoplasia of the morphological left ventricle associated with severe pulmonic stenosis and an intact ventricular septum in the setting of congenitally corrected transposition of the great arteries is a rare lesion. We report the clinical and echocardiographic findings of this lesion in a patient with atrial situs inversus.

Abnormalities, Multiple↗

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.

Adolescent↗

Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection.

Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 years.

Abnormalities, Multiple↗

Biliary atresia with situs inversus: an experience shared.

Biliary Atresia (BA) is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus (SI) has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis (HN) of left kidney and stasis at pelvi ureteric junction (PUJ). Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia (EHBA). The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium (SI) and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit.

Bile Ducts↗

Living donor liver transplantation for biliary atresia complicated by situs inversus: technical highlights.

Living-donor liver transplantation (LDLT) has become an established technique to treat children with end-stage liver disease. Biliary atresia (BA), one of the most common indications for liver transplantation in children, can be associated with situs inversus (SI). In the past, the presence of SI has been considered to be an absolute contraindication for liver transplantation because of the technical difficulties. Recently, some reports of successful diseased-donor liver transplantation in patients with BA complicated by SI have been published; however, few reports of that with LDLT exist. The technical difficulties involved with LDLT for such cases have not been described. Herein, we present 4 successful cases of LDLT for BA with SI. Complex anomalies associated with SI, such as a hepatic artery arising from the supraceliac aorta, a preduodenal portal vein, and absence of the retrohepatic inferior vena cava, increase the technical difficulties involved with the operation. Additional caution is required in LDLT because a living-donor graft has short vessels and the availability of vascular grafts from the donor is limited. In conclusion, LDLT for BA complicated by SI can be managed successfully with technical modifications and scrupulous attention. This series represents the largest reported group of patients with BA complicated by SI who underwent a successful LDLT procedure.

Abnormalities, Multiple↗

Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report.

Preduodenal portal vein (PDPV) is a rare anomaly described in only 82 patients in the literature. In a few patients, the PDPV was the direct cause of duodenal obstruction. The authors have treated a newborn with PDPV associated with total situs inversus, intestinal malrotation and polysplenia who presented clinically with duodenal obstruction.

Abnormalities, Multiple↗