Belly syndrome prune-Barre Eagle. Definition

Medical Definition: belly syndrome prune-Barre Eagle

Syndrome characterized by absence, deficiency or hypoplasia of the abdominal muscles, accompanied by filing a large bladder, hypotonic and dilated and tortuous ureter with bilateral cryptorchidism. Its frequency is 1 in 35000-50000 live births and its cause is unknown. In 50% of cases are accompanied by some degree of renal dysplasia and hydronephrosis are not uncommon. The ureter has an overall decrease in the number of muscle fibers. Vesicoureteral reflux affects 85% of patients. The bladder muscle is diminished and replaced by connective tissue. The compliance bladder is normal but the capacity is significantly decreased bladder contraction, so that only 50% of patients void spontaneously, although with obvious post-void residual. The prostatic urethra is dilated due to prostatic hypoplasia. Although the urethra and penis are normal in most patients, sometimes associated with megalourethra syndrome, both in variety and in its variety scaphoid fusiforme. Regarding bilateral cryptorchidism is a permanent fact, in most cases, both tests are intraabdominal. The reason is unknown, although it suggests an intrinsic testicular abnormality that explains the absence of spermatogonia in the testes of the majority of patients. Although erections and orgasm are apparently normal, is not documented any cases of paternity. The potential fertility is compromised not only by testicular abnormalities, but also by alterations of the epididymis, seminal vesicles, absence of prostatic epithelium and bladder neck open. In relation to the abdominal wall defect, typically the abdominal muscles are missing in the middle and lower abdominal wall, although the internal oblique muscles rectosuperior and are usually developed. Deficiency ranges from a small hypoplasia and complete absence of muscles. The poor support of the lower chest wall makes an effective cough mechanism and contribute to respiratory infections proliferate. The most frequent pulmonary complications include the tendency to atelectasis and lobar pneumonia. Regarding the clinical presentation, note that the considerable variability in the severity of the changes, makes the syndrome is classified into three categories: Category I includes infants with severe renal dysplasia or lung, the survival of these patients is limited , category II, these patients usually survive the neonatal period should be treated in well equipped medical centers, category III includes those patients with incomplete or not very marked for the syndrome.

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