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PubMed1982

Incidental discovery of asplenia syndrome, with situs inversus and a normal heart by radionuclide biliary imaging. A case report.

Tc-99m biliary imaging was performed on a 34-year-old woman who was being investigated for suspected cholelithiasis. A left-sided liver was detected. On subsequent radionuclide imaging, partial visceral situs inversus and asplenia were demonstrated. Extensive roentgenographic investigation, ECG, echocardiogram, and laparotomy confirmed the radionuclide findings. Asplenia syndrome may exist without cardiovascular abnormalities and thus be undiscovered. Radionuclide imaging is important in the diagnosis of this syndrome.

Adult↗
PubMed1978

Successful surgical repair of double outlet right ventricle with situs inversus, l-loop, l-malposition and subaortic VSD in a 16-month-old patient.

The case of a 16-mth-old patient who had double outlet right ventricle, l-loop and l-malposition in the presence of dextrocardia and situs inversus is presented. A successful repair was performed using an internal conduit to divert the blood from the left ventricle to the aorta. The outflow tract of the right ventricle was then enlarged with a dacron patch. The anatomic variations of double outlet right ventricle surgically corrected are reviewed.

Aorta↗
PubMed2001

Right varicocele associated with inferior vena cava malformation in situs inversus: percutaneous treatment with retrograde sclerotherapy.

Isolated right varicocele is a rare condition. It could be secondary to a retroperitoneal neoplastic mass involving the right internal spermatic vein, but sometimes, an anatomic variant must be considered. We present a case of a young man with situs inversus and right varicocele, a mirror image of the normal condition, associated with inferior vena cava malformation, who was treated successfully with retrograde selective percutaneous sclerotherapy of the right internal spermatic vein. Selective sclerotherapy of the internal spermatic vein at the time of venography has proved to be a valuable therapeutic option in right-sided varicocele associated with anatomic abnormalities.

Abnormalities, Multiple↗
PubMed1998

[Right varicocele as first manifestation of situs inversus].

Traditionally, right varicoceles have been considered a rare entity nearly always secondary to a neoplastic or retroperitoneal disease. There are other possible etiologies for right varicoceles such as the venous disease of the large veins and visceral malposition syndromes. This paper presents one case report of right varicocele as the only clinical sign of situs inversus. Revision of the literature and nomenclature of these syndromes.

Adult↗
PubMed2003

[Haematemesis and dysphagia in a 20-year-old woman with congenital spine malformation and situs inversus partialis].

A 20-year-old woman with a four-week history of dysphagia, weight loss of four kilograms and unspecific abdominal pain was admitted because of sudden haematemesis. The physical examination showed a patient with a prominent kyphoskoliosis. The patient reported of having a situs inversus abdominalis and a tethered cord syndrome. Bladder function disorders were present since childhood. Upper endoscopy demonstrated a 4 cm large, exophytically growing necrotic tumour of the oesophagus. The CT scan showed a space occupying tumour of the oesophagus and metastases in a size of 1.5 cm in both lungs. Further imaging revealed a UICC-Stadium IVB (T2NxMIb ). Histology of the tumour biopsies showed a poor differentiated squamous cell carcinoma. Staging after the 6 th dose cisplatin (100 mg/m2/die) and 5-fluorouracil (5 x 1000 mg/m2/die) showed a mild reduction of the tumour and the metastases. The patient died ten months later of multiorgan failure after severe progress of tumour and metastatic growth. The manifestation of squamous cell carcinoma of the oesophagus is unusual in people at the age of twenty. Genetic and chromosomal analysis of the patient gave no evidence for a hereditary disorder. Drug history revealed that the patient had been treated with the alpha-receptor blocking drug phenoxybenzamine over at least 12 years for bladder dysfunction. Animal experiments of rats with exposition of phenoxybenzamine over 24 months produced gastrointestinal malignomas. By the German admission board phenoxybenzamine is only recommended for short term therapy. It seems to be likely that even in humans phenoxybenzamine acts as a mutagenic substance and should be carefully used in long-term treatment.

Adrenergic alpha-Antagonists↗
PubMed2003

A new variant of esophageal atresia with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus.

Esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is the most common anatomic pattern within congenital anomalies of the esophagus. Also, more than 50% of the infants with esophageal atresia have other congenital malformations. To our knowledge, this is the first case report of EA with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus (SI).

Abnormalities, Multiple↗
PubMed2005

Radiofrequency catheter ablation of a coronary sinus-ventricular accessory connection in dextrocardia with complete situs inversus and an anomalous inferior vena cava.

An anomalous inferior vena cava (IVC) is often associated with patients with dextrocardia. However, radiofrequency catheter ablation in such a case with that combination has not been reported. We encountered a case of Wolff-Parkinson-White syndrome with dextrocardia associated with complete situs inversus and an azygos continuation of the IVC. A steep angulation at the junction of the azygos vein and superior vena cava precluded the precise mapping of the anatomical right cavity with a femoral vein approach. Successful catheter ablation of an accessory connection between the coronary sinus and left ventricle could be achieved with a right cubital vein approach.

Catheter Ablation↗
PubMed1992

Combined mustard and Rastelli operations. An alternative approach for repair of associated anomalies in congenitally corrected transposition in situs inversus [I,D,D].

We report on two patients with congenitally corrected transposition of the great arteries in situs inversus who underwent successful anatomic repair of associated cardiac anomalies--ventricular septal defect and pulmonary outflow tract obstruction. Surgical intervention was influenced by the recently introduced technique of Ilbawi and colleagues in cases of congenitally corrected transposition in situs solitus. Principles of the correction are as follows: (1) patch redirection of venous flows at the atrial level through an incision in the left-sided right atrium; (2) patch closure of the ventricular septal defect through a right ventriculotomy, baffling the left ventricle to the aorta; and (3) valved conduit interposition between the right ventricle and the pulmonary artery. If the ventricular septal defect is restrictive, it can be safely enlarged by extensive resection of the anterosuperior border, because of the posteroinferior location of the bundle of His in this anomaly. With this technique, the left ventricle and the native mitral and aortic valves are recruited for systemic work, with intrinsic long-term advantages. It is, however, a complex procedure involving substantial use of prosthetic material. More experience is necessary to establish whether this approach is superior to currently available physiologic repairs.

Abnormalities, Multiple↗
PubMedDate not supplied

[Post traumatic diaphragmatic hernia with situs inversus in a student 11 years old].

A case is reported of generalized acute abdominal pain in a school boy 11 years old with history of abdominal trauma produced by the introduction of a steel rod into the right hypochondrium. Laparatomy and prophylactic appendectomy were practiced and the diagnosis of situs inversus was made in his homestead. Six months later, the patient complained of intermittent abdominal pain, vomiting and absence of stools. With this picture, he was reoperated at this hospital where a right perforation of the diaphragm was found, through which the stomach, the epiploon and part of the ascending colon protruded into the chest. Said viscera were returned to the abdominal cavity and the diaphragmatic defect was sutured. The evolution was uneventful and the patient was discharged 10 days after surgery.

Child↗
PubMed1992

Prenatal diagnosis of short rib (polydactyly) syndrome with situs inversus.

We present a case of short rib (polydactyly) syndrome in which the diagnosis was made prenatally by ultrasound examination. The more specific diagnosis of short rib (polydactyly) syndrome type III was made on the basis of findings on radiographs obtained at birth. The sonographic and radiographic features are discussed. The patient had complete situs inversus and hypospadias. The former was reported in one other case of type III and the later has not been previously reported in this entity.

Adult↗
PubMed1986

Pancreatic fibrosis calcification and situs inversus in a Liberian female; a case report.

We report the first case of the Pancreatic fibrosis calcification syndrome (PFCS) in a Liberian. The patient presented with the classical features of the syndrome - a history of recurrent abdominal pain, diabetes mellitus, malabsorption and pancreatic calcification on plain abdominal X-ray. The patient also has situs inversus; we believe that this combination is a casual one. Since describing this case, five more cases have been diagnosed; we believe that this condition is not rare in Liberia.

Adolescent↗
PubMed2002

Mustard procedure in atrial situs inversus: three-dimensional ablation of intra-atrial reentry tachycardia on a surgically created inversus cavo-tricuspid isthmus.

It has been demonstrated that the Biosense Carto system can improve the success rate of ablation in case of an intra-atrial reentry tachycardia in patients submitted to the Mustard repair. This system was used to map an intra-atrial reentry tachycardia in a young patient who had been submitted to the Mustard procedure for atrial situs inversus. A line of block was created connecting the right sided tricuspid valve to the left sided inferior vena cava. This terminated the arrhythmia and prevented its re-initiation. This case confirmed the notion that the cavo-tricuspid isthmus is often critical to the maintenance of an intra-atrial reentry tachycardia after the Mustard procedure even if its location is in the inversus side.

Adolescent↗
PubMed2000

Neonatal arterial switch operation for transposition of the great arteries in a patient with mirror image dextrocardia and situs inversus totalis.

The neonatal arterial switch operation has become the standard therapy for D-transposition of the great arteries in the absence of left ventricular outflow tract obstruction. We describe our experience of successful arterial switch operation after balloon atrial septostomy in a 5-day-old infant girl who had atrial and visceral situs inversus totalis, mirror image dextrocardia, and D-transposition of the great arteries. To our knowledge, ours is the first report of this operation in a patient with this anatomy.

Dextrocardia↗
PubMed1993

Evidence that spontaneous situs inversus in cultured neural plate staged rat embryos is additive with and not mediated through adrenergic mechanisms.

Approximately 50% of untreated presomite rat embryos in culture have demonstrated inversions of cardiac looping (laeval instead of dextral) or tail flexure (left-sided instead of right-sided), or both. This spontaneous situs inversus (SI) was not accompanied by growth inhibition or any other observable defects. The incidence of SI was directly related to the stage at dissection, and all heart defects and most flexure defects were eliminated by delaying explantation to the early somite stage. The incidence of SI was not lowered significantly either by removal of endogenous catecholamines from the culture serum by dialysis or by inclusion of alpha- or beta-adrenergic antagonists in the medium. However, the alpha-adrenergic agonist L-phenylephrine (50 micrograms/ml) increased the incidence of SI to 73%. These findings appear to rule out adrenergic mechanisms as a cause of spontaneous SI in cultured, neural plate-staged rat embryos but suggest a mechanism, yet unknown, that is additive with SI induced by alpha-adrenergic agonists. The low incidence of non-SI-related defects suggests that the high incidence of SI is not an artifact of suboptimal culture conditions. The virtual absence of SI in embryos cultured in bovine serum, a medium in which overall embryonic growth and development were retarded, provides further evidence against nonspecific artifacts.

Adrenergic Agonists↗
PubMed1992

Heart-lung transplantation in situs inversus. A case report in a patient with Kartagener's syndrome.

After a long history of recurrent chronic pulmonary infections in a 25-year-old woman with Kartagener's syndrome, a heart-lung transplantation was performed. A modified surgical procedure was needed to perform transplantation because of the presence of a situs inversus, which is usually associated with bronchiectasis and sinusitis in this congenital syndrome. A large single atrium was created with both the right and left recipient atria used to facilitate anastomosis with the donor's right atrium. The patient was discharged after resolution of early ventilatory complications and is in good condition 8 months after transplantation.

Adult↗
PubMed2001

Marfan's syndrome, dextrocardia and situs inversus associated with discrete subaortic stenosis and aortic insufficiency in an adult female: case report.

Marfan's syndrome is an inherited connective tissue defect that affects many organs, especially of the musculoskeletal, ophthalmic and cardiovascular systems, and may be associated with some rare conditions. Here, we report the first known case of Marfan's syndrome, combined with situs inversus totalis with dextrocardia and discrete subaortic stenosis and aortic insufficiency in a 22-year-old woman.

Adult↗