Wilson and colleagues proposed that an abnormality in body folding in the cephalic region of the fetus results in an epigastric omphalocele, while a caudal folding abnormality causes a low or hypogastric omphalocele often in association with bladder or cloacal exstrophy
, and anomalous lateral body wall folding results in midabdominal defects .
Abdominal wall defects (AWDs) are a complex group of anomalies, and include the common AWDs (gastroschisis and omphalocele) as well as the complex AWDs (body stalk anomaly, abdominoschisis, pentalogy of Cantrell, bladder exstrophy, and cloacal exstrophy
All children with cloacal exstrophy
have some degree of pubic symphysis diastasis and may have a spinal malformation, most commonly myelocystocele.
An increased rate of sex change from the female to the male sex role has been seen in children and adolescents with genital malformation (agenesis of the penis, cloacal exstrophy
) who grow up as girls and had a normal level of male hormones at birth (34).
These diverse groups of conditions include posterior urethral valve (PUV), bladder and cloacal exstrophy
, and epispadias and myelomeningocele [3-5].
Abdominal wall defects like gastroschisis, omphalocele, patent vitellointestinal duct, exstrophy bladder, and cloacal exstrophy
accounted for 8.15% of the anomalies.
Although double IVC is known to be associated with a range of genitourinary abnormalities including horseshoe kidney, crossed fused ectopia, cloacal exstrophy
, and retroaortic renal vein, none of these anomalies were seen in our patient .
Lastly, other syndromes and conditions with genital abnormalities, such as cloacal exstrophy
of the bladder (multi-organ pelvic defect resulting in severe inadequacy or absence of genitalia), perineoscrotal hypospadias, micropenis, penile agenesis (aphallia), vaginal agenesis, and penile ablation (loss of penis from traumatic injury), are blanketed by the phrase DSD.
Omphalocoele is frequently seen in the context of congenital syndromes, with multiple associated anomalies, such as Beckwith Wiedemann syndrome, cloacal exstrophy
, OEIS complex (Omphalocoele, exstrophy, and imperforated anus, spinal), Cantrells pentalogy and trisomies.
Associated conditions of relevance to the abdominal procurement surgeon include anomalies of the renal veins (circum aortic renal collar, and retro aortic renal vein) (1), right retro caval ureter (3), horseshoe kidney, congenital right renal agenesis (4) and cloacal exstrophy