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Combination of annular pancreas and partial situs inversus: a multiple organ malrotation syndrome associated with duodenal obstruction.

A combination of annular pancreas and partial situs inversus has been encountered in three babies who presented as duodenal obstruction. All the abnormalities can be explained on the basis of multiple organ malrotation involving the duodenum, pancreas, liver, stomach, and indirectly, the spleen; hence, the term multiple organ malrotation syndrome (MOMS). The heart maintains its normal left side location in this condition. Similarly the remaining small bowel, large bowel, and appendix are normally located. It is proposed that the triad of intestinal obstruction as suggested by vomiting and visible peristalsis, left-sided liver in the presence of laevocardia, and radiographic appearance of reversed double bubble, be considered as indicating MOMS.

Abnormalities, Multiple↗

A routine liver transplantation in a patient with situs inversus: a case report and an overview of the literature.

Liver transplantation (LT) in an adult with situs inversus (SI) is extremely rare and requires precise pre-operative management. A 48-yr-old male with SI suffering from alcoholic liver cirrhosis underwent LT at our institution in March 2003. Pre-operatively, liver anatomy was determined by CT scan, three-dimensional liver reconstruction and angiography. LT was performed using the Belghiti technique with side-to-side cavo-caval anastomosis, transplanting a graft from a donor with normal anatomy. Post-operatively, the patient recovered without major complications, except an epileptic event because of a central pontine myelinolysis, and he was discharged on the 25th post-operative day. Three months after surgery, the T-drain placed intra-operatively into the donor bile duct was removed; transplant perfusion and function were stable with an actual follow-up period of 24 months. LT in patients with SI is feasible. Pre-operative imaging with three-dimensional reconstruction is a beneficial tool for operation planning in patients with rare anatomic variations.

Hepatectomy↗

Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C-TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Vessel Prosthesis↗

Acute appendicitis in complete situs inversus.

An acute appendicitis was diagnosed in a 29-year-old patient with situs in inversus totalis. Appendectomy was carried out. The incidence, etiology and the diagnosis of complete situs inversus as well as the diagnosis and pitfalls of acute appendicitis in this rare congenital abnormally are described.

Adult↗

Heart-lung transplantation in situs inversus and chest wall deformity.

Heart-lung transplantation in the presence of complex congenital heart disease including situs inversus and significant chest wall deformity can be accomplished successfully. However, the postoperative course is apt to be prolonged because of mechanical respiratory problems, which will respond to a protocol of weaning and nutritional supplementation.

Adult↗

[Laparoscopic cholecystectomy in a patient with situs inversus].

Laparoscopic cholecystectomy is the standard approach to manage symptomatic gallbladder stones. However, only twelve patients with total situs invertus have been previously reported in the literature. We report a new case of a 58-year-old patient hospitalized for acute pain of the left hypochondrium with fever. The diagnosis of acute cholecystitis with situs inversus totalis was made following clinical examination and radiological investigations. Laparoscopic cholecystectomy was subsequently performed through a modification of the technique to adapt to the mirror image anatomy.

Cholecystectomy, Laparoscopic↗

Surgical approach to corrected transposition of the great vessels and situs inversus [I, D, D] with ventricular septal defect and systemic atrioventricular valve regurgitation.

Successful surgical repair of a 4-year-old boy with situs inversus [I, D, D] and corrected transposition of the great vessels (TGV) as well as hemodynamically significant ventricular septal defect (VSD), systemic atrioventricular (tricuspid) valve regurgitation, and atrial septal defect is described. Unique technical aspects of VSD and tricuspid valve repair in association with corrected TGV and situs solitus or inversus are discussed and clarified.

Child, Preschool↗

Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers-Manes Kartagener and Bjorn Afzelius.

The relationship of Kartagener's syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartagener's triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome.

Canada↗