Polysplenia and jejunal atresia with agenesis of the dorsal mesentery

Jejunal atresia presenting as meconium cyst with polysplenia in a newborn: an extremely rare association

“…Jejunal atresia has been associated with a number of other congenital malformations such as cystic fibrosis, malformation, congenital heart disease, biliary atresia, total colonic aganglionosis etc. 4,5 We reported presence of polysplenia. There was two spleen of equal size with separate hilum at their normal location.…”

Genetics of gastrointestinal atresias

“…With regard to the association with CF, it has been reported that newborns with JIA have an increased frequency, 8e11%, of CF (Roberts et al, 1998;Siersma et al, 2012). JIA has also been associated with biliary atresia, total colonic aganglionosis, intestinal neuronal dysplasia and polysplenia (Asabe et al, 2005;Barber et al, 1989;Grosfeld, 2006). Some compounds, especially vasoconstrictor drugs, have been found to increase the risk for the fetus of developing JIA.…”