Potter’s Syndrome associated with Pouch Colon Anomaly in Exomphalos

Abstract

Potter’s Syndrome is a rare congenital malformation which is an atypical physical appearance of the foetus or neonate due to Oligohydramnios experienced in the womb with distinctive facial characteristics. Oligohydramnios is the cause of the various deformities observed in Potter’s Sequence. It is characterised by bilateral renal agenesis, pulmonary hypoplasia, and skeletal defects of the neonate as a direct result of lack of amniotic fluid. Congenital pouch colon (CPC) is an extremely rare variant of anorectal malformation (ARM), in which varying lengths of the colon is replaced by a dilated pouch accompanied by a fistula communicating with the genitourinary tract. Complete Penoscrotal Transposition (CPST) is a rare and unusual malformation in which the scrotum is located cephalic to the penis. It is associated with major and often life threatening malformations. The present case has multiple malformations which include, potter’s syndrome, congenital pouch colon in omphalocele and complete penoscrotal transposition. The aim of this study was to determine the incidence, prevalence of these multiple malformations according to anatomical localization. This complex anomaly is very rare and interesting. Survival is extremely rare. In view of prognosis early diagnosis allows for earlier and less traumatic therapeutic abortion.