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Situs inversus totalis.

The etiology of situs inversus totalis remains uncertain. However, the literature establishes that isolated situs inversus totalis is usually asymptomatic in the neonate. This case study illustrates the importance of physical assessment skills in identifying situs inversus totalis in the neonate. Current research may reveal the etiology of this rare but fascinating abnormality.

Female↗

Coronary artery bypass grafting in dextrocardia with situs inversus totalis.

Dextrocardia with situs inversus totalis is a rare congenital abnormality of development involving a left-handed malrotation of the visceral organs. The incidence of coronary artery disease in this condition is probably similar to that in the general population. We report herein the case of a 62-year-old man with dextrocardia and situs inversus totalis who underwent myocardial revascularization by the use of right internal mammary artery and radial artery.

Coronary Angiography↗

Synchronous double cancer of the stomach and rectum with situs inversus totalis and polysplenia syndrome.

Situs solitus refers to the normal arrangement of body organs. Situs inversus totalis is a complete mirror image or reverse isomeric form of the thoracic and abdominal viscera. Any arrangement of organs between these two extremes is designated by heterotaxia. Several patterns of vascular and visceral abnormalities are associated with heterotaxia, and two loosely defined syndromes of splenic anomalies (asplenia and polysplenia) are most common. We present the case of a 71-year-old woman with situs inversus totalis and polysplenia syndrome who developed synchronous double cancer originating from the stomach and rectum. Abdominal manifestations were situs inversus totalis combined with multiple lobulated spleen, azygous continuation of the interrupted inferior vena cava, direct drainage of hepatic vein to left atrium, preduodenal portal vein, short pancreas, incomplete rotation of the colon, and malrotation of the intestine. Histologically, gastric cancer was diagnosed as papillary adenocarcinoma and rectal cancer, as moderately differentiated adenocarcinoma. The patient was successfully treated with total gastrectomy for gastric cancer and low-anterior resection of the rectum for rectal cancer.

Adenocarcinoma↗

Abdominal situs inversus in a Holstein calf.

Situs inversus is a rare congenital anomaly wherein the normal, left-right organ location is inverted. The anatomical inversion (heterotaxy) is usually detected in routine radiography or other medical interventions. A 5-month-old Holstein heifer calf was identified as a suspected situs inversus totalis during abdominal surgery. Following surgery the calf did not gain weight. On admission to the Ontario Veterinary Hospital, it was given a routine clinical examination and a detailed cardiovascular, Doppler ultrasound examination, which appeared normal. Because of declining health and chronic bloating in the heifer, euthanasia was performed. A detailed post-mortem dissection was carried out. No heart or lung anomalies were detected. Other thoracic organs were normal, except for a helical coil of the esophagus as it traversed the diaphragm. The rumen was located entirely on the right side of the abdomen and had a large area of adhesion to the parietal peritoneal wall. The left kidney was abnormally placed: retroperitoneal and cranial to the right. There were two spleens. The caudal vena cava was distended and twisted to the left. Thus the calf was situs inversus abdominalis. This suggests that the development of laterality in the early embryo may include both timing and positional regulation.

Abdomen↗

Laparoscopic cholecystectomy in a patient with situs inversus totalis and previous abdominal surgery.

Situs inversus totalis is a rare congenital defect that can present difficulties during laparoscopic surgery due to the mirror-image anatomy. We report a patient with symptomatic cholelithiasis and previous abdominal surgery in whom a chest X-ray revealed a right-sided heart, whereas abdominal ultrasound revealed that his gallbladder was located in the left hypochondrium. At surgery, the surgeon and the camera assistant were standing on the right-hand side of the patient, and the first assistant was standing on the left. The camera was introduced through an umbilical incision, and laparoscopy confirmed the situs inversus. The other 10-mm trocar was placed in the midline left of the falciform ligament and two 5-mm trocars were placed in the left subcostal midclavicular line and anterior axillary line, respectively. After dissection of multiple adhesions caused by previous abdominal surgery, a standard laparoscopic cholecystectomy was performed successfully. This report suggests that situs inversus is not a contraindication for laparoscopic surgery. However, the procedure is more difficult and potentially hazardous due to the mirror-image anatomy (particularly the transposition of biliary ducts) causing difficulties in orientation, so that extreme care is required to avoid iatrogenic injuries. Despite these factors, laparoscopic cholecystectomy can be performed safely in patients with situs inversus totalis.

Abdomen↗

Situs inversus of donor or recipient in liver transplantation.

Situs inversus is a rare anatomical abnormality that is often associated with multiple, complex malformations. In the past, patients with situs inversus were considered unsuitable candidates for transplantation or organ donation because associated visceral, and especially vascular, anomalies pose special technical difficulties. Recently, several cases of successful liver transplantation in recipients with situs inversus have been published using modified surgical techniques. This report reviews the literature and describes our own experience, including two liver graft recipients with complete and incomplete situs inversus, and one patient who underwent successful transplantation using a liver from a donor with situs inversus.

Child↗

Off-pump quintuple coronary artery bypass grafting for situs inversus totalis.

Dextrocardia associated with situs inversus totalis is a rare condition. A 49-year-old man with dextrocardia and situs inversus totalis underwent off-pump quintuple coronary artery bypass grafting using bilateral internal thoracic arteries and bilateral radial arteries. The operative technique was similar to that of off-pump coronary artery bypass grafting for situs solitus. His postoperative recovery was uneventful.

Coronary Artery Bypass↗

Laparoscopic appendectomy in a female patient with situs inversus: case report and literature review.

BACKGROUND: Situs inversus is an uncommon condition caused by a single autosomal recessive gene of incomplete penetration. A potential diagnostic dilemma can occur in the young female patient with a history of situs inversus who presents with pelvic pain. METHODS: A 32-year-old multiparous patient with a known history of situs inversus presented with complaints of pelvic pain. A medical history and full physical examination were indicative of possible endometriosis. RESULTS: The patient underwent an operative laparoscopy, which revealed stage II pelvic endometriosis based on the American Fertility Society Revised Classification for Endometriosis (R-AFS), with appendicular and periappendicular adhesions involving the cecum. Ablation of endometriosis and an appendectomy were performed. CONCLUSION: The authors believe the laparoscopic approach to an appendectomy is ideal in a patient with situs inversus and should be performed at the time of laparoscopy performed for another reason.

Adult↗

Renal cell carcinoma with situs inversus totalis.

A case of renal cell carcinoma in a patient with situs inversus totalis is reported. Only 8 cases of malignant neoplasms have been reported in situs inversus totalis, and our case represents the second case report of renal cell carcinoma in a patient with situs inversus totalis. The frequency of situs inversus totalis is between 1 in 8,000 and 1 in 20,000. Cardiac and pulmonary anomalies are common in patients with situs inversus totalis. Renal anomalies, including agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney, have been reported. Because of the association between situs inversus and cardiac, pulmonary, renal, and vascular anomalies, management of the patient with situs inversus and urologic disease requires careful preoperative evaluation.

Aged↗

Human situs inversus of the thoracoabdominal structures.

Situs inversus totalis is well known, but its comprehensive description has been rare, especially on the internal view of the heart. For discussions based on recent results in developmental biology, the present study demonstrates a region- or part-specific manner of the inverted morphology found in a male donated cadaver and discusses the pathogenetic mechanisms of situs inversus in the human. Therein, clearly inverted morphologies existed in the coronary vessels, the apex position (dextracardia), connections between the heart and great vessels, the internal view of the atrium, the aortic arch with the three major branches, lung and liver segments and abdominal gastrointestinal tract. However, the ventricular internal view suggested incomplete laterality, such as tricuspid atrioventricular valves for both ventricles. The cardiac conductive system appeared not to be inverted but abnormal. The thoracic aorta and pulmonary artery took an L-spiral position with modifications. The inferior vena cava was located on the right side of the abdominal aorta. However, the left-sided kidney was located superior to the right-sided kidney. Similarly, the testicular vessels did not exhibit a clearly inverted morphology, but were almost normal. Therefore, the posterior mediastinal and retroperitoneal structures appeared to exhibit neutral laterality, incomplete inverted morphology or even normal morphology. According to the personal history and present histology, this specimen was unlikely to correspond to Kartagener's syndrome. The present observations seem to be consistent with recent findings in mutant models of laterality disturbances, in which a single gene or molecule is responsible for the changes in a region-specific manner.

Aged↗

Aortic atresia with visceral situs inversus with mirror-image dextrocardia.

A newborn infant with situs inversus totalis (visceral situs inversus with mirror-image dextrocardia) with aortic atresia is presented. No reports of this anatomical variant have previously been reported in the literature. A brief discussion of the various types of congenital heart defects and their incidence in association with situs inversus totalis is presented.

Abnormalities, Multiple↗

Conducting tissues in congenitally corrected transposition with situs inversus.

Three cases of congenitally corrected transposition in situs inversus individuals were characterised by visceroatrial situs inversus, atrioventricular discordance, and ventiruloarterial discordance: one case was studied clinically, and the other 2 were necropsy specimens. The disposition of the atrioventricular conducting tissues was established in each case, in the living patient by intraoperative mapping, and in the necropsy specimens by histopathological investigation. In all, the connecting atrioventricular bundle arose from a normally situated posterior atrioventricular node, normally related to the landmarks of the atrial septum. Though anterior nodes were identified in the necropsy specimens, as reported in congenitally corrected transposition in situs individuals, they differed from those in the latter situation in that they made no atrioventricular connection. These findings have obvious surgical importance. It is suggested that the posterior connection is related to the good septal alignment in these cases which lacked significant septal defects. Posterior connections are not necessarily present in all situs inversus individuals with corrected transposition, particularly when there are malalignment ventricular septal defects: further studies are required in such cases.

Aorta, Thoracic↗

Split cord malformation and situs inversus totalis: case report and review of the literature.

INTRODUCTION: Situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and type II split cord malformation. This patient had no complaints and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has led to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.

Child↗

Split cord malformation and situs inversus totalis: case report and review of the literature.

INTRODUCTION: Situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and Type II split cord malformation. The patient had no symptoms and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has lead to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.

Child↗

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.

Biliary Atresia↗

Prolonged survival with isolated levocardia and situs inversus.

Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.

Abnormalities, Multiple↗

Situs inversus and coronary artery disease.

Situs inversus is a rare condition and there are few reports of myocardial revascularization in such patients. A 56-year-old woman with situs inversus totalis and coronary artery disease underwent successful anastomosis of the right internal mammary artery to the anterior descending coronary artery, and a saphenous vein graft to the right coronary artery.

Coronary Artery Bypass↗

Situs inversus in homozygous mice without immotile cilia.

Mice homozygous for the mutation situs inversus, iv, are characterized by situs inversus in 50 percent of the individuals. We have investigated the ultrastructure and motility of tracheal cilia and sperm tails in homozygous individuals with and without situs inversus. We found no abnormalities of ciliary structure. Therefore, ciliary immotility is not an essential correlate of situs inversus.

Animals↗