A baby girl with prenatal diagnosis of complex cardiac anomalies and diaphragmatic hernia was born at 36 weeks of gestation. At 4 hr of life, the baby developed respiratory distress and was intubated. She was found to have right hetetrotaxy with total anomalous pulmonary venous drainage into the portal vein, five hepatic veins draining the liver and intrathoracic herniation of the stomach. The child also developed abdominal distension on the second day of life with passage of scanty meconium. The diagnosis of Hirschsprung's disease (HD) was confirmed by histology. HD in association with right heterotaxy has not been reported earlier. The association of heterotaxy with HD in our patient raises a possible genetic link between the two anomalies that needs further research.
This is a case report of an unusual case of a patient with dextrocardia and "situs inversus totalis" who presented with unstable angina. Coronary angiography revealed severe main stem and severe triple vessel coronary artery disease. The patient later underwent successful emergency coronary artery bypass graft surgery. To the authors' knowledge this is the first reported case in Malaysia and also, the first ever report in the literature of multiple vessel coronary artery grafting, including the use of the right internal mammary artery.
Situs inversus totalis is the complete transpositioning of thoracoabdominal viscera into a mirror image of the normal configuration. Choledochal cyst is the congenital cystic dilation of the biliary tract. Both these conditions coexisting in a patient is extremely rare. We hereby present a case of type IC choledochal cyst in a patient with situs inversus totalis presenting with biliary sepsis secondary to choledocholithiasis. Also detailed are the management and operative strategies employed to deal with this rare entity.
Matched MeSH terms: Situs Inversus/diagnosis*; Situs Inversus/pathology
1. Three cases of situs inversus are described and the clinical and aetiological factors are discussed briefly.
2. This abnormality occurring in father and son whose family history indicates a great degree of consaguinity seems to support the view held by Cockayne that it is a recessive inheritance.
3. Situs inversus when complete seems to cause no disability. When incomplete as in examples of isolated dextrocardia, congenital heart diseases are commonly seen in association with it. Other congenital malformations may also co-exist. Recently the syndrome of hypertrophic rhinitis, nasal polyposis and sinusitis and bronchiectasis have been encountered in association with transposition of the viscera.
Sudden deaths because of congenital heart diseases are infrequently referred to the forensic pathologist for autopsy. Many of such deaths, if already diagnosed are released directly from the hospital without autopsy. Even forensic pathologists face a few difficulties in performing the autopsy on such infrequent cases, as they are not always updated with the anatomy of anomalies. While dealing with such cases, the concerned forensic pathologist is compelled to refer literature and textbooks again to understand the nature of developmental defects. This is especially so when dealing with cases of situs inversus accompanied by transposition of great arteries and other congenital cardiac abnormalities and variants such as single ventricle, double outlet right ventricle, Taussig-Bing variety etc. In the present case also, situs inversus with transposition of great vessels and other anomalies have been noted and studied.