[Situs inversus viscerum].
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A patient presenting the association of anomalous cerebral dominance for language, as revealed by crossed aphasia, and partial visceral situs inversus is described. Although it cannot be excluded that the two abnormalities coincided by chance, it is suggested that they both resulted from a single aberration of development.
We report a case of mitro-aortic atresia with situs inversus. The patient was studied with two-dimensional echocardiography and the diagnosis was confirmed in the necropsy. Our newborn infant is the second case reported with this abnormality and the first in our country.
A case of successful surgical correction of double-outlet right ventricle with concordant atrioventricular relation, pulmonic stenosis, and situs inversus is described. Transposition of the great vessels was not present. Intracardiac repair with a Teflon tunnel conduit gave an excellent result. Proper resection of the subpulmonic obstructing fibromuscular band required no patch reconstruction of the outflow tract.
The patient was a 79-year-old male who complained a sudden back pain. He had dextrocardia and situs inversus. An aortogram showed the dilatation of the ascending and aortic arch which reached 5.6 cm in diameter. A coronary angiogram also showed the 75 percent stenosis of segment 3 in the right coronary artery. Coronary artery bypass grafting to the right coronary artery was performed using a saphenous vein graft during cooling stage of the extracorporeal circulation. After that total arch replacement was carried out using a graft with four side branches with an aid of selective cerebral perfusion. He complicated with respiratory failure postoperatively and needed tracheostomy but recovered gradually. A postoperative computed tomogram showed good patency of three side grafts for cerebral perfusion and no abnormality of graft anastomosis.
A 69-year-old man and a 54-year-old woman with situs inversus totalis were admitted to our hospital for surgery for gastric malignant disease. The diagnosis was made radiologically and successful total gastrectomies were performed.
A case of congenital horizontal gaze paralysis in association with facial hemiatrophy and situs inversus of the optic discs is described. Except for the presence of deafness on the side of the atrophic face no other neurologic abnormality was present. To our knowledge, no similar case has been reported.
A healthy, non-consanguineous couple had a son with complete situs inversus viscerum (including dextrocardia but without other cardiac defects), broad thumbs and big toes, postaxial polydactyly, average intelligence and length proportion of the extremities, and a normal face. The common cause of these defects may have a role in the origin of sidedness and symmetry in morphogenesis.
The authors report a case of a forty-one-year-old white woman with dextrocardia with situs inversus who presented episodes of prolonged sinus arrest and syncopal episodes secondary to possible idiopathic degeneration of the conduction system, managed successfully with a permanent bicameral pacemaker. In their literature review they found that this case represents a very rare association.
A 23-year-old man with situs inversus and bronchiectasis was investigated because of azoospermia. Serum levels of testosterone and pituitary gonadotropins were normal. Scrotal exploration revealed no abnormalities of the testes, epididymides or vasa deferentia. Electron microscopy of a testicular biopsy specimen revealed a normal sperm flagellum structure, including the presence of dynein arms but 2 separate biopsies of the nasal mucosa revealed complete ciliary aplasia. This case, which bears a striking resemblance to Young's syndrome (bronchiectasis and azoospermia), represents a unique variant of the immotile cilia syndrome.
Organ anomalies and organ system transposition may cause diagnostic and therapeutic difficulties. We report a patient with situs inversus totalis and symptomatic cholelithiasis successfully treated via laparoscopic cholecystectomy. We present a laparoscopic and MR cholangiographic pictures of our patient with gallbladder agenesis.
The authors report a case of symptomatic cholelithiasis in a patient with situs inversus totalis, in which the surgical treatment was performed by laparoscopy. They discuss the etiopathogenesis of this anomaly and malformations often associated, moreover related clinical and surgical problems. Finally, they describe operative technique stressing the real possibility to perform the operation by laparoscopy on condition that the surgeons have sufficiently experience of biliary and laparoscopic surgery.
Diagnosis of congenitally corrected transposition of the great arteries (L-TGA) with situs inversus totalis in two adult patients was made by magnetic resonance imaging (MRI). Visualization of the complete anatomy and quantification of ventricular function was possible. Relevant concomitant disease such as perimembraneous ventricular septal defect, atrial secundum septal defect, tricuspid regurgitation, valvular pulmonic stenosis, and pulmonary artery dilatation were clearly depicted by MRI using standard spin-echo and gradient-echo techniques. Findings were confirmed by cardiac catheterization in both patients. In this rare and complex congenital cardiac anomaly, MRI is an excellent imaging modality as echocardiography may be difficult to interpret due to restricted imaging windows. MRI may help in the decision about the necessity to undergo further invasive evaluation and may help to make cardiac catheterization a straightforward procedure.
An adult case of partial anomalous pulmonary venous drainage of left pulmonary veins with dextrocardia, situs inversus and azygos continuation of inferior vena cava is reported. Surgical repair was accomplished by constructing a baffle to direct the blood flow from left pulmonary veins to the left atrium through the created atrial septal defect. This case is a rare type of PAPVD.
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.
An autopsy case of bilateral renal dysplasia with other congenital malformations is reported. Malformations included severe pancreatic fibrosis with meconium ileus, situs inversus totalis, cardiovascular anomalies, and others. The only syndrome of multiple congenital malformations involving renal dysplasia which is similar to the present case is Ivemark's syndrome, though the present case lacked hepatic lesions. Apart from the pancreatic lesion, there were no abnormalities compatible with cystic fibrosis (mucoviscidosis). It was considered that meconium ileus in the present case was caused by pancreatic achylia secondary to severe pancreatic fibrosis, unrelated to cystic fibrosis, but closely allied to renal dysplasia.
We describe a case of successful high-speed rotational atherectomy and stenting of a long calcified lesion in a patient with dextrocardia and situs inversus.