Heterotaxy syndrome or situs ambiguous is a rare congenital disease in which the pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures is not arranged in normal position. Patients with heterotaxy syndrome represent a wide range of anatomical variations including thoraco-abdominal structures. Here we report a rare case of asymptomatic heterotaxy syndrome in...

et al. Mucocele apendicular: reporte de un caso clı́nico. Cuad Cir (Valdivia). 2004;18:43–7. 6. Hernández E, Reguero J, Aguilar J, Fragela A. Mucocele del apéndice: formas de presentación. AMC. 2004;8. 1025-0255. 7. Soueı̈-Mhiri M. Mucocele of the appendix: retrospective study of 10 cases. J Radiol. 2001;82:463–8. 8. Stocchi L, Bruce G, Dirk R, Jeff R. Surgical treatment of appendiceal mucocele. ...

Obscure gastrointestinal bleeding is an important dilemma. Brunner's gland hamartoma is an extremely rare tumor generally localized in the duodenal bulb. We present a 34-year-old woman who had suffered from several episodes of melena for the past three years. Endoscopic examinations were normal. Computed tomography showed a large target lesion over the right abdomen and an image representing in...

McKusick-Kaufman syndrome is a rare syndrome inherited in an autosomal recessive pattern with a phenotypic triad comprising hydrometrocolpos, postaxial polydactyly and congenital cardiac disease. The syndrome is caused by mutations in the MKKS gene mapped onto chromosome 20p12. Diagnosis of this syndrome is based on clinical and imaging findings. In our paper, we have presented the first McKusi...

We report 37 patients ranging in age from 1 month to 14 years treated for intestinal malrotation during a five year period. The main presenting features consisted of intermittent attacks of vomiting (15 patients), failure to thrive (seven), and recurrent colicky abdominal pain (seven). The diagnosis was confirmed by gastrointestinal contrast studies in all but three patients. A standard Ladd's ...

Neonatal intestinal volvulus in the absence of malrotation is a rare occurrence and rarer still is the intestinal volvulus in absence of any other predisposing factors. Primary segmental volvulus in neonates is very rare entity, which can have catastrophic outcome if not intervened at appropriate time. We report two such cases, which were preoperatively diagnosed as ileal atresia and intraopera...

Ileal atresia associated with malrotation is rare, but the additional diagnosis of Hirschsprung’s disease (HD) in the same patient has not been described in the literature. This case report presents a newborn with a bowel obstruction requiring surgery for ileal atresia and malrotation. The post-operative course was complicated by a distal bowel obstruction. Evaluation of the resected bowel from...

Intestinal malrotation complicated by the development of midgut volvulus presents a difficult management dilemma because of the risk of short bowel syndrome. Here, we present our experience with a case of severe intestinal ischemia after derotation in a newborn successfully managed using systemic tissue plasminogen activator. The present report supports the usefulness of thrombolysis as a thera...

Midgut malrotation is a congenital anomaly referring to either lack of or incomplete rotation of the fetal intestines around the axis of the superior mesenteric artery during fetal development. It is rare in adulthood and the true incidence is difficult to estimate because most patients are asymptomatic. The diagnosis is usually performed with several radiological and surgical methods. We repor...