Omphalocele and epigastric heteropagus: implications and treatment

In heteropagus twins, the parasitic twin is incompletely formed which is attached to the autosite. We report a case of epigastric heteropagus twins with omphalocele. The parasite had two lower limbs, a rudimentary upper limb, genitalia with developed phallus and scrotum but absent testis. An omphalocele was present just below the attachment of the parasitic twin. A single kidney with ureter and a bladder filled with urine seen during exploration. The parasite bowel was attached to a sleeve of liver tissue from the autosite within the omphalocele sac and this connection was also the main source of its blood supply. Less than 20 cases of omphalocele with EH have been reported previously. The thin sac with underlying adherent bowel led us to go ahead with surgery in the neonatal period for both, excision of epigastric heteropagus and omphalocele repair. The rectus sheath around the omphalocele sac helped in the final wound closure.