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The case of a successful orthotopic heart transplantation for complete situs inversus with double-inlet left ventricle and anomalies of the systemic venous return is reported. A piece of aortic homograft and a composite conduit made of the recipient right atrium and pericardium were used to connect, respectively, the left superior vena cava and the hepatic veins to the right-sided atrium of the donor heart.
A patient with congenital situs inversus totalis and a single coronary artery was referred for revascularization because of progressive disabling angina. Exercise thallium scintigraphy had disclosed ischaemia in the anterolateral, posterolateral, and inferior wall of the left ventricle. Percutaneous transluminal coronary angioplasty was performed successfully for stenoses in the right coronary artery.
We describe 2 cases of obstructive azoospermia associated with situs inversus and sinopulmonary infection due to ciliary defects. Electron microscopy of testicular sperm flagella demonstrated normal morphology with nine peripheral doublets surrounding a central pair and complete sets of inner and outer dynein arms. Electron microscopy of the nasal mucosa revealed partial defects of the dynein arms of cilia, although the "9+2" morphology was preserved. Our cases were considered unique variants of Young's syndrome but also had characteristic features of Kartagener syndrome, and thus support the hypothesis that Young's syndrome has a genetic etiology similar to that of Kartagener syndrome.
UNLABELLED: Hypoglossia is a rare malformation that is not fatal, unlike otocephaly. CASE REPORT: A neonate, born at 39 weeks of GA and dead at 5th day showed hypoglossia, dextrocardia, situs inversus and pituitary aplasia. Maternal hyperthermia was observed at 4 weeks gestation. CONCLUSION: This case is reminiscent of a community of syndrome with agnathia-holoprosencephaly and situs inversus. The responsibility of maternal hyperthermia is raised.
We present the first documented case of agnathia-holoprosencephaly (an uncommon form of craniofacial anomaly) associated with situs inversus. This case may represent the concordance of multiple field complex anomalies, but the possibility of a major midline malformation (situs inversus) caused by a timed insult (environmental or genetic) which affects multiple structures and occurs concurrently with a major field defect during early embryogenesis cannot be excluded.
A 71-year-old woman was admitted to our hospital by ambulance, because of right chest pain and hypotension. Chest X-ray and standard 12-lead ECG showed mirror-image dextrocardia with situs inversus totalis. ECG with right-left reversal of all leads showed acute inferior myocardial infarction. The patient underwent coronary bypass surgery due to postinfarction angina, and she is now doing well 2 years following the operation. Mirror-image dextrocardia with situs inversus totalis is very unusual. But the patients are believed to have normal longevity, and presumably have an incidence of atherosclerotic coronary artery disease similar to the general population. To our knowledge, this is the first case of coronary bypass surgery on a patient with mirror-image dextrocardia in Japan.
A nine-month-old golden retriever bitch was presented with exercise intolerance and recurrent nasal discharge. Based on clinical, radiographic and ultrasonographic examination, a diagnosis of rhinitis, situs inversus totalis and tricuspid valve insufficiency was established. The results of video- and electron microscopy studies of the respiratory epithelium were compatible with primary ciliary dyskinesia (PCD). However, no evidence of a primary ultrastructural defect of the cilia was found. The dog was euthanased because of the poor prognosis. At necropsy, a hydrocephalus internus and a subaortic stenosis were additionally diagnosed. PCD, in combination with situs inversus, has been previously reported in golden retrievers, but without a concomitant hydrocephalus internus. Furthermore, concomitant occurrence of internal cardiac malformation and PCD has not previously been reported in the dog.
A case of complete oesophageal duplication in association with dextrocardia and situs inversus is presented.
Mediastinoscopy has been widely used by thoracic surgeons to evaluate the superior mediastinum since 1959. Large series of mediastinoscopy have been reported with very low morbidity and no mortality. Proper attention to surgical techniques and mediastinal anatomy are essential to maintain the safety of the procedure. Situs inversus totalis is exceedingly rare, but variations in mediastinal anatomy in this group of patients can render the procedure challenging for the thoracic surgeon. A case of mediastinoscopy in a situs inversus patient is presented with emphasis on anatomical variations of the mediastinum and technical pitfalls of the procedure in this rare group.
Hypoglassia or aglossia is an uncommon anomaly, either of which may occur as an isolated finding or in association with other deformations, especially limb anomalies. Their genetic background is uncertain, and drug induced teratogen has not been clearly identified. We experienced a case of congenital aglossia with situs inversus in a female infant aged twelve days. Her initial complaints at admission were feeding difficulty and weight loss. In a review of literature, the association with situs inversus is very rare and only three cases have been reported until now.
We report here a premature female infant with agnathia, low-set but normally formed ears, a downward eye slant, choanal atresia and a cleft palate. She had severe respiratory distress and died despite maximum intervention at 5 days of age. Autopsy revealed situs inversus totalis; crossed fused renal ectopia; agnathia; normal thyroid, larynx, trachea, and bronchi; incomplete lobation of the lungs; immature pulmonary development with early hyaline membranes; and a normal central nervous system. This lack of significant central nervous system abnormalities distinguishes this infant from the majority of previously reported infants with agnathia and situs inversus.
We present a 24-year-old lady who reported at the radiology department, UBTH Benin City for a chest radiograph, as part of her pre-employment medical examination. She was asymptomatic, her past medical history was not significant and physical examination revealed an apex beat located on the 5th right intercoastal space. The chest radiograph confirmed dextrocardia and also revealed the gastric air bubble on the right, which was suggestive of situs inversus totalis. Barium meal examination confirmed the right-sided position of the stomach, in keeping with situs inversus. Ultrasonography revealed a left sided liver and gall bladder, with a right sided location of the spleen.
BACKGROUND: In adults suffering from Kartagener's syndrome-which is found in 50% of patients with primary ciliary dyskinesia (PCD)-bronchiectasis is still one of three typical clinical features. In this condition it is caused by chronic bacterial inflammation as a result of impaired mucociliary clearance in congenital ciliary dysfunction. Little information is available on the incidence, age-related development and prophylactic therapy of bronchiectasis in children suffering from PCD. CASE REPORT/RESULTS: We describe the case of a 2-year old boy with clinical features of Kartagener's syndrome who showed impaired ciliary motility and typical ultrastructural defects of PCD. Bronchiectasis was excluded by bronchography. CONCLUSIONS: The diagnosis of PCD implies disturbed ciliary motility and abnormal ultrastructure of the cilia. In 50% of cases PCD is associated with situs inversus and is then referred to as kartagener's syndrome, whereas situs inversus itself does not define Kartagener's syndrome. Bronchiectasis is not necessarily present in children with PCD. Physiotherapy, inhalations, vaccinations and early antibiotic treatment may be of prophylactic value in preventing bronchiectasis. Early start of the life-long treatment depends on early diagnosis which should be based on well-defined criteria.
Reconstruction was accomplished in a 2 heart-lung recipients with situs inversus resulting in a left-sided systemic venous atrium. We created a large common atrium that was closed on the left side, leaving an atrial cuff on the inferior right quadrant. To this we anastomosed the donor right atrium, which had been opened laterally between the cavae. This resulted in some clockwise rotation of the ventricles and anterior positioning of the apex. The right pulmonary veins passed superior to the atrial anastomosis and posterior to the donor right atrium. Cardiopulmonary function was excellent in both cases.
Transplantation of the heart was successfully performed in a patient with situs inversus of the viscera and atria. Anatomic constants common to all patients allowed the left atrium, pulmonary artery, and aorta to be joined as usual in the transplant operation. Systemic venous reconstruction was the crux of the operation. A composite superior vena cava was reconstructed on the right side from the recipient translocated superior vena cava, donor superior vena cava and innominate vein, and recipient in situ pericardium. The inferior vena cava was rerouted from the left side across the midline to the right through a composite conduit consisting of the recipient night atrium and in situ pericardium over the diaphragm. These reconstructed venous passageways have remained patent and unobstructed for 1 1/2 years after the operation.
We are studying the development of handedness, in particular the relationships between handed structures with bilateral symmetry, for example the limbs, and those with lateral asymmetry, such as the heart, lungs and gut. Asymmetric (unilateral) developmental limb abnormalities can be induced by chemical treatment of mouse embryos, either in utero by acetazolamide, or in culture by misonidazole. We have examined these effects in mice homozygous for the iv gene. The development of bilateral symmetry in iv/iv mice is normal, but the control of asymmetry appears to be random, that is 50% develop normally (situs solitus), 50% with laterally inverted viscera (situs inversus). We find that the handedness of induced asymmetric limb defects is highly correlated with embryonic visceral situs. Right limb defects are induced in situs solitus embryos, left-sided defects in situs inversus. This suggests that the mechanism of induction of asymmetric defects is not related to any intrinsic difference between the development of left and right limbs, but is connected to visceral asymmetry. In addition, the high correlation of limb defects with situs was observed in culture as well as in utero suggesting that the maternal environment plays no role in the development of asymmetry.
Although total gastrectomy with distal pancreatectomy has been the standard surgical treatment for advanced gastric cancer, this procedure is frequently complicated by leakage of pancreatic juice and postoperative diabetes mellitus. We present the case of a 74-year-old woman with proximal gastric cancer who underwent a laparoscopic modification of an open pancreas-preserving procedure first described by Maruyama et al. in 1995. With this novel technique, there is no pancreatic leakage, and at 12-month follow-up our patient remains free of diabetes. Herein we give the details of our new method and offer some caveats for its performance.
The good results reported for liver transplantation have encouraged a much wider application of the procedure, broadening the list of indications and increasing the number of candidates. The shortage of organs for transplantation is a main problem that limits hepatic replacement in the potential recipients. Consequently, the number of contraindications for donor selection has been reduced over the last years. Some factors that were previously thought to preclude successful transplantation have now been relegated to relative contraindications, while others are no longer included. This has frequently led to the use of livers under suboptimal conditions or with anatomical anomalies. This is the case of donors with abdominal situs inversus. In this article, we report an orthotopic liver transplantation using a donor with abdominal situs inversus. Immunosuppressive protocol following surgery was composed of a classic three-drug therapy (cyclosporine, azathioprine, and prednisolone). The modified piggyback technique was performed over the right suprahepatic vein with orthotopic position of the graft. The graft showed good long-term function in the recipient, with a normal hepatic biopsy 5 months after the transplantation. There was no patient readmission or other medical problem after a 2 1/2-year follow-up.