Pathogenesis of the prune-belly anomalad

A Quantitative Study of Normal Nephrogenesis in the Human Fetus: Its Implication in the Natural History of Kidney Changes due to Low Obstructive Uropathies

“…The obstructive uropathy and the renal lesions could result from a common disor dered mesodermal development [31,32], In this hypothesis, a uniform morphological and clinical pattern should be expected in opposi tion with the variability of observed situa tions, and the proved benefit of in utero deri vation [33], Anatomical stenosis should be constant in contrast with reported observa tions [7][8][9][10] and as seen in cases 15 and 17. Some experimental data favored this theory: ureteral ligation in lambs [34], rabbits [35] or chicks [36] produced hydronephrosis without 380 Gasscr/Mauss/Ghnassia/Favre/Kohler/ Nephrogenetic Repercussions of Yu/Vonesch Obstructive Uropathies dysplasia.…”

“…It is cer tain that no functional benefit can be ob tained in this situation from a vesicoamniotic shunt. In case of either a normal or at least partial amount of nephrogenic blastema, one can expect recovery of nephrogenic capacity with an either normal or partial pattern (cases 1,2,3,7,8,12,15). However, many questions remain unanswered.…”

Prune belly syndrome: expanding the phenotype

“…It has been suggested that maldevelopment of the abdominal musculature and abdominal wall laxity are secondary phenomena, the primary event being marked distension of the fetal abdomen because of various causes, mostly frequently enlarged bladder secondary to urethral obstruction (King and Prescott, 1978). Significant abdominal visceromegaly, such as in Beckwith-Wiedemann syndrome, can also stretch the abdominal wall musculature and lead to prune belly appearance.…”

Double Shunt in a Case of Fetal Low-Level Obstructive Uropathy

“…Since obstruction interferes with fetal development, and since the presence of oligo hydramnios is associated with pulmonary hy poplasia and skeletal and facial deformities, an early intervention in utero can, in selected cases, restore amnionic fluid volume and pre vent pulmonary hypoplasia [7][8][9][10][11], There are several alternatives for intrauterine decom pression of an obstructed fetal urinary tract, but it is difficult to evaluate their efficacy [ 12].…”