In addition, left-sided polysplenia
, abnormal symmetric bronchial branching pattern, and bilateral left atrial appendages were detected.
syndrome concomitant with situs inversus totalis and distal pancreatic hypoplasia are rare congenital anomalies.
Cardiac Malposition with specific emphasis on visceral heterotaxy (asplenia and polysplenia
4) described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia
1,3,5,6) Others include biliary atresia, polysplenia
, skeletal anomalies and renal tract anomalies.
Anomalies which are according to the literature associated with Pentalogy of Cantrell are: agenesis 1 of the corpus callosum, hydrocephalus, anencephaly, lip and palate cleft, fusion of the adrenal gland with liver, colon malrotation, clinodactily [9, 10], the absence of tibia and radius, polysplenia
, underdevelopment of the gallbladder .
IVC hypoplasia may also be associated with other congenital anomalies, such as polysplenia
, renal or pulmonary dysgeneses, dextrocardia or other congenital cardiac diseases.
Combination of partial situs inversus, polysplenia
and annular pancreas with duodenal obstruction and intestinal malrotation.
6) Patients are more likely to be symptomatic in the presence of other associated anomalies such as congenital heart disease, asplenia, polysplenia
or inversion of the bowel.
The first is the embryonic/fetal, "early," or syndromic form, accounting for 10% to 20% of cases, which is associated with a high frequency of additional congenital malformations (including asplenia, polysplenia
, cardiovascular defects, situs inversus, intestinal malrotation, small-intestinal atresia, anomalous choledochopancreatic ductal junction, and various positional abnormalities of the portal vein and hepatic artery), and is referred to as biliary atresia-splenic malformation (BASM) syndrome.
Agenesis of the dorsal pancreas with polysplenia
syndrome: CT features.
Congenital heart disease associated with polysplenia