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The treatment of portal hypertension by videolaparoscopy in situs inversus totalis.

A rare case of a 32-year-old male with situs inversus totalis viscerum admitted to hospital for hematemesis owing to portal hypertension of Laennec cirrhosis was treated for the first time by videolaparoscopic surgery. Situs inversus diagnosis was confirmed by thoracic radiography, electrocardiogram, echocardiogram, abdominal echography and computed tomography. Upper gastrointestinal endoscopy showed esophageal varices and large varices in the fundus of the stomach. A successful operation (azygo-portal disconnection, splenic artery ligation without splenectomy; transesophageal suturing of esophageal varices without opening the esophagus and cholecystectomy), was performed by videolaparoscopy. The uneventful postoperative evolution (4-day hospitalization) reinforces the viability of the videolaparoscopic approach and the possibility of the application of this procedure even to situs inversus totalis organorum.

Adult↗

Laparascopic sigmoid colectomy for diverticular disease in a patient with situs inversus.

We present the case of a 50-year-old man with situs inversus who underwent laprascopic colonic resection for diverticulitis. The patient, who had right-sided pain due to inversion of the viscera mimicking appendicitis, initially presented a diagnostic challenge. A barium meal confirmed situs inversus and the diagnosis of diverticulitis. We present an overview of the operative technique of what is the first documented laparoscopic colectemy in a patient with situs inversus. We then discuss the benefits of the laproscopic approach to colectomy, with reference to this interesting case with unusual anatomy.

Colectomy↗

Adult complex spinal dysraphism with situs inversus totalis: a rare association and review.

STUDY DESIGN: First published report of an adult complex spinal dysraphism with situs inversus. OBJECTIVES: To describe a previously asymptomatic adult patient of multiple vertebral anomalies with cervical split cord malformation type II, tethering of the spinal cord (cervical and lumbar), and intraspinal arachnoid cyst along with dextrocardia and situs inversus. SUMMARY OF BACKGROUND DATA: Only 5 cases (fetus, 1; neonates, 3; child, 1) of spinal dysraphism with dextrocardia or situs inversus have been reported. All these cases have had associated multiorgan developmental anomalies usually incompatible with survival and requiring multidisciplinary care. METHODS: The case has been described and relevant literature reviewed. RESULTS: The patient was operated for cervical and lumbar levels in the same sitting. A C4-C5 laminectomy was performed, 2 hemicords enclosed in the same dural sac were visualized, dorsal paramedian nerve roots and the tethering arachnoid bands were cut, and the arachnoid cyst wall was partially excised. This was followed by L4-L5 laminectomy and detethering by sectioning of the thickened filum terminale. The patient showed significant neurologic improvement after surgery. CONCLUSIONS: The present case is a rare instance in which there has been an association of adult onset occult spinal dysraphism along with situs inversus totalis. Successful management requires appropriate understanding of embryology, anatomy, and imaging and has implications in neurosurgical and perioperative anesthetic care.

Abnormalities, Multiple↗

Ultrasound diagnosis of situs inversus in utero. A case report.

A case of situs inversus diagnosed prenatally by ultrasonography is presented. The diagnosis was confirmed on ultrasound examination and radiography in the immediate postnatal period. Subsequent investigations and management are reported, and the incidence, aetiology and clinical features of situs inversus are briefly reviewed.

Adult↗

Renal dysplasia, situs inversus totalis, and multisystem fibrosis: a new syndrome.

Renal dysplasia was associated with situs inversus totalis and multisystem fibrosis in a severely hydropic stillborn female fetus. The ureters were duplicated and showed fibrous obliteration. The pancreas, thyroid, and uterus were fibrotic, and the lungs had small and large irregular pulmonary lobules bounded by fibrotic septa. An extensive fibrous pericarditis was considered the etiology of hydrops. A similar spectrum of anomalies including bilateral renal dysplasia, situs inversus totalis, and pancreatic and hepatic fibrosis has been described in three separate reports. Our case is similar with additional findings of fibrosis of the thyroid, uterus, pulmonary septa, and pericardial tissues. We believe the presence of situs inversus totalis, renal dysplasia, and multisystem fibrosis constitutes a new syndrome.

Abnormalities, Multiple↗

Laparoscopic cholecystectomy in a patient with situs inversus totalis.

Currently, laparoscopic cholecystectomy is an undoubtfully optimal treatment of cholelithiasis. What about performing this procedure on a patient with situs inversus totalis and what are the difficulties of this operation for a right-handed surgeon? We presented a 35-year-old man with unknown situs inversus totalis who was admitted with epigastric pain and digestive problems. Ultrasonography and computed tomography of the abdomen confirmed the diagnosis of a gallstone. Besides, the liver and gallbladder were on the left side and the spleen was on the right. All systems were left-right reversal as mirror image in all diagnostic studies. Laparoscopic cholecystectomy was safely performed, despite of difficulties of situs inversus. The patient was discharged on postoperative day 1. It should be considered that existence of other anomalies may easily cause uninvited injuries. In the patients with situs inversus, laparoscopic cholecystectomy can be safely managed by an experienced surgeon through laparoscopy, and also hepatobiliary surgery.

Adult↗

Isolated atrial inversion in situs inversus: a rare anatomic arrangement.

Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.

Coronary Circulation↗

[Myocardial revascularization in dextrocardia with situs inversus].

BACKGROUND: The incidence of coronary artery disease in patients with dextrocardia associated with situs inversus is similar to that of the general population: Nevertheless, there are few papers regarding surgical myocardial revascularization in these patients. METHODS: We report two patients with dextrocardia and situs inversus who underwent myocardial revascularization by means of coronary artery bypass grafting, with the right internal mammary artery to the left anterior descending coronary artery in one case and with the left internal mammary artery as a free graft to the left anterior descending coronary artery associated with the repair of an aneurysm of the right coronary sinus of Valsalva in the second patient. RESULTS: There were no surgical complications; the patients were discharged on the 4th and 7th postoperative days, respectively. They are alive and well after 24 and 60 months of follow-up respectively. CONCLUSIONS: This report suggests that durable myocardial revascularization in dextrocardia with situs inversus can be successfully achieved with right or left mammary artery bypass grafting to the left anterior descending coronary artery. The advantage of using an in situ mammary artery is discussed.

Aged↗

Liver transplantation in the presence of situs inversus totalis: application of reduced-size graft.

Because of the anatomical features associated with situs inversus, technical difficulties will be encountered during orthotopic liver transplantation. This report describes the case of a patient with situs inversus totalis and end-stage liver disease from biliary atresia who was treated by segmental orthotopic liver transplantation. The segmental graft was safely placed in the left subphrenic space, and a suitable orientation was obtained for anastomoses of the hilar vessels. Chronic rejection necessitated retransplantation, by the same method, 19 months later. This technique has potential advantages in coping with anatomical obstacles encountered in patients with situs inversus.

Biliary Atresia↗

Adrenergic neurotransmitters and calcium ionophore-induced situs inversus viscerum in Xenopus laevis embryos.

Xenopus laevis embryos at the blastula-early tail bud stage were exposed to norepinephrine or octopamine dissolved in culture saline until they reached the larval stage. The left-right asymmetry of the heart and gut was then examined. We found that these adrenergic neurotransmitters induced situs inversus in the heart and/or gut in up to 35% of tested neurula embryos. Norepinephrine-induced situs inversus was blocked by the alpha-1 adrenergic antagonist prazosin. Furthermore, A23187, a calcium ionophore, also increased the incidence of situs inversus up to 54% when late-neurula embryos were exposed to the solution. A23187 treatment initiated before neural groove formation was less effective. The incidence of situs inversus induced by these reagents decreased towards the control level (2.2%, 25 untreated embryos out of 1127 embryos in total) in embryos past the stage of neural tube closure. In the present experiments we obtained 22 gut-only situs inversus embryos having an inverted gut and a normal heart. In contrast, such embryos were not observed among the 1127 untreated embryos. An adrenergic signal mediated by an increase in intracellular free calcium may be involved in the asymmetrical visceral morphogenesis of Xenopus embryos.

Adrenergic alpha-Agonists↗

Renal hypoplasia and situs inversus totalis.

A spectrum of renal abnormalities of patients with situs inversus has been reported. Renal dysplasia is the most common. Herein is described for the first time, an association of situs inversus totalis, unilateral congenital renal hypoplasia and external ear cartilage deformity.

Adult↗

Staurosporine does not prevent adrenergic-induced situs inversus, but causes a unique syndrome of defects in rat embryos grown in culture.

Staurosporine, an alkaloid isolated from Streptomyces species, is commonly used as a protein kinase C (PKC) inhibitor in animal investigations. In the present study, we used this compound to determine whether alpha 1-adrenergic stimulation-induced situs inversus in rats is mediated by PKC. Embryos were explanted at 8 A.M. on day 9 of gestation. Those with a neural groove but with no visible neural folds (Stage 11a) were selected and were cultured in medium containing various concentrations of staurosporine with or without 50 microM of phenylephrine, an alpha 1-adrenergic agonist. At 10 A.M. on day 11 of gestation, embryos were examined for situs inversus and other abnormalities. Staurosporine, tested at 0.0001, 0.001, 0.01, 0.1, 0.375, and 0.5 microM (lethal concentration), did not block phenylephrine-induced situs inversus at any concentration. However, staurosporine alone produced situs inversus at concentrations above 0.1 microM. At 0.5 and 1.0 microM, staurosporine also caused cyst-like lesions projecting dorsally from the mesencephalon that we named "mesencephalic vesicles" and the formation of secondary somites. To confirm and further examine these unique effects of staurosporine both grossly and histologically, we conducted additional experiments using staurosporine from another source. Embryos were explanted between 6 A.M. and 9 P.M. on day 9 of gestation and were placed in one of the following groups according to their stage of development: 10b, 11a, 11b, 11c, 12/s1-2, 12/s3-4, and 12/s5-6. Embryos were then cultured with various concentrations of staurosporine. Those cultured from Stage 11a exhibited similar lesions to those seen in the initial experiment but at somewhat higher concentrations of staurosporine. Embryos cultured from Stage 10b showed a similar pattern of lesions as seen at Stage 11a, except that higher concentrations of staurosporine were required to cause mesencephalic vesicles and secondary somites formation. Embryos cultured from Stage 11b showed similar effects to those cultured from younger stages except that maximum incidences of situs inversus were much lower. Those cultured from Stage 11c showed similar dose-response to those cultured from Stage 11b except that the incidence of secondary somites formation was much higher. In addition, in approximately 40% (n = 25) of embryos treated with greater than 1.0 microM of staurosporine, the growing end of the allantois did not reach the chorion and remained unattached in the exocoelomic cavity.(ABSTRACT TRUNCATED AT 400 WORDS)

Abnormalities, Drug-Induced↗

[Coronary angioplasty in a case of dextrocardia with situs inversus and myocardial infarct].

Dextrocardia and situs inversus totalis is a rare congenital anomaly in adults. Usually, the patients have structurally normal hearts and experience normal longevity. The risk of coronary atherosclerosis is similar to that of the general population. However, the report of coronary angioplasty and/or coronary arteriography on patients with dextrocardia is scant. We describe herein a case of successful percutaneous transluminal coronary angioplasty to the right coronary artery. We comment the technical particularities of the procedure, and a review of the literature is made.

Angioplasty, Balloon, Coronary↗

[The vortex cordis is never reversely directed, even in situs inversus and L-loop anomaly].

In 1926, Taussig reported on two instances of complete situs inversus of the heart and stated that "the main anatomical structure and the deep muscle bundles of the ventricles presented the mirror image of the normal, while the direction of the superficial muscle bundles remained unchanged". In an attempt to analyze whether her statement always held true, we examined the myocardial architecture of five specimens of situs inversus totalis. They were all from patients over sixty years of age and were diagnosed by postmortem examination. Every part of the heart and great vessels presented exact mirror images of the normal. However, the external muscle fibers of the ventricles ran clockwise from base to apex toward the center of the vortex, which had a striking resemblance to the normal rather than the mirror image pattern. Peeling off of the external layer revealed that the direction of fibers was first inverted and then changed into an approximate mirror image of the normal architecture within the deeper muscle layers, as illustrated by Taussig. The exception to this tendency was found at the posterior region of the morphologically left ventricle, in which there was no mirror imaging but a normal pattern throughout the depth of the wall. For the purpose of comparison, two hearts of corrected transposition (L-TGA) were examined. Their chambers were inverted but the external fibers followed the same clockwise course seen in the normal heart. Further dissection revealed that there was no mirror imaging of the architecture at any depth of the morphologically left ventricular wall. Thus there were regional differences and similarities in muscular architecture between these two kinds of ventricular inversion. It was concluded that the vortex layer was never reversely directed, even in situs inversus and L-loop anomaly, and that the deep layers within the sinus region of the left ventricle did not present a mirror image to the normal.

Adolescent↗

Case report on situs inversus totalis in two Sprague-Dawley rats.

We macro- and microscopically examined two cases of congenital visceral transposition (situs inversus totalis) in SD rats. We also investigated the possibility of situs inversus in association with immotile-cilia syndrome. The rats had grown normally with no clinical signs of disease. Although all organs including the vascular system were located opposite to the normal position and displayed a mirror image on macroscopic observation, no abnormality was found in any of the organs on microscopic examination. Electron-microscopic observation revealed in neither animal any structural abnormalities of the cilia and flagella, which are one of the diagnostic characterizations of immotile-cilia syndrome. Congenital transposition of the viscera is rare and there are few reports examining complications with situs inversus in rats. This report will be helpful in accumulating information on this condition.

Animals↗

Laparoscopic cholecystectomy in situs inversus totalis with "inferior" cystic artery: a case report.

A 76-year-old man with known situs inversus totalis presented with left-sided discomfort. Abdominal ultrasonography and CT scan confirmed the diagnosis of a gallstone, as well as, situs inversus; the liver and gallbladder on the left side and the spleen on the right. The biliary system was thought to be left-right reversal, mirror image in the view of drip infusion cholangiogram and MRI. Laparoscopic cholecystectomy was safely performed, despite of unexpected aberrant cystic artery running inferior to cystic duct of situs inversus. Laparoscopic surgeon should be careful for view of reversed relationships and also existence of other anomalies.

Aged↗