Thirteen patients (10.5%) had other associated congenital anomalies, such as cardiac anomalies in nine patients, intestinal malrotation in three, situs inversus in two, and polysplenia
in one patient.
(3) A female preponderance has been described and associated cardiac and skeletal anomalies, biliary atresia, polysplenia
, and hepatic tumors often coexist.
In our patient, left isomerism resulted in the presence of two left symmetrical bilobed lungs, polysplenia
, a transverse liver (centrally positioned) and a left-sided stomach bubble (Figs 2-4).
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
Heterotaxia in a fetus with campomelia, cervical lymphocele, polysplenia
, and multicystic dysplastic kidneys: Expanding the phenotype of Cumming syndrome.
The abdomen and pelvic ultrasounds were done, which showed situs inversus and polysplenia
. The body of the stomach and the spleen were found in the right upper quadrant.
(2) Zic3 (spina bifida with abdominal situs inversus, complex cardiac defects, asplenia, and polysplenia
(4) described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia
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.