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vol.28 issue1MULTIPLE THROMBOSIS WITH UNUSUAL PRESENTATION. CASE REPORTPULMONARY METASTATIC PARAGANGLIOMA RESPONDING TO PAZOPANIB. CASE REPORT AND LITERATURE REVIEW author indexsubject indexarticles search
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Revista Médica La Paz

On-line version ISSN 1726-8958

Abstract

URQUIETA MALDONADO, Miguel Ángel et al. EAGLE - BARRET SYNDROME: A PURPOSE OF A CASE. Rev. Méd. La Paz [online]. 2022, vol.28, n.1, pp.47-54.  Epub June 30, 2022. ISSN 1726-8958.

INTRODUCTION.

The syndrome of the triad of the abdomen in “Prune Plum” or Prune Belly is an infrequent congenital alteration, characterized by the absence of the muscles of the abdominal wall, abnormalities of the urinary tract and bilateral cryptorchidism.

CLINICAL CASE.

A 4-month-old infant with a prenatal ultrasound that reported a mega bladder, underwent vesico-amniotic bypass (first stage) at week 22 of gestation with evacuation of the bladder and a catheter implanted in the uterus. It was obtained by emergency cesarean section as the installed catheter was obstructed. At birth, the bladder-cutaneous diversion was performed (second stage) and diagnostic cystoscopy (third stage). A multidisciplinary approach was carried out until discharge from hospital and death of the patient at the fifth month of life at home.

DISCUSSION.

Corresponds to the true Prune Belly variety due to abdominal wall failure, urinary tract involvement, persistence of the urachus and cryptorchidism confirmed by ultrasonography. It was not possible to classify it fully according to the probability of mortality that Bedron adopted. The diagnostic suspicion at birth was clinical. Surgical treatment depends on the severity, prenatal diagnosis and intrauterine derivative surgery can be performed in order to decompress the bladder and avoid oligohydramnios, thus reducing kidney damage and its consequences. The prognosis depends on the severity and involvement of the renal system at the time of diagnosis. They are susceptible to recurrent urinary infections.

Knowing embryogenesis, prenatal diagnosis, classification and therapeutic alternatives according to renal function contribute to an adequate multidisciplinary management.

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