Article Text

TREATMENT AND EVOLUTION PECULIARITIES IN AN INFANT WITH EAGLE-BARRETT SYNDROME
  1. S I Iurian1,
  2. B Neamtu1,
  3. E Costea Begu2,
  4. H Jidveianu3,
  5. C Berghea Neamtu3,
  6. G Gradinariu1
  1. 1Pediatric Clinic, Pediatric Hospital, Sibiu, Romania
  2. 2Ambulatory Department, Pediatric Hospital, Sibiu, Romania
  3. 3Surgery Pediatric Clinic, Pediatric Hospital, Sibiu, Romania

Abstract

Objectives To emphasize the diagnosis, treatment and clinical evolution particularities in an infant diagnosed with Eagle-Barrett (prune-belly) syndrome.

Methods The authors present a 3 month-old male infant admitted for high fever. Regarding the medical history, we noted a surgical intervention (cutaneous ureterostomy to increase the urine passage) at the age of 2 months justified by a persistent urinary tract infection (UTI) complicated with Enterococcus septicaemia. The clinical exam in the emergency room revealed pallor skin, growth impairment, retroglossoptosis, mandibular hypoplasia, feeding problem, loss of appetite, systolic murmur, tachycardia, severe abdominal distention with wrinkled appearance, bowel loops visible, constipation, and cryptorchidism; hand pressure on the abdomen is followed by elimination of cloudy urine through the ureterostomy stoma.

Results The laboratory investigations have shown anemia, positive inflammatory markers and an urine exam suggestive for UTI. The imagistic findings have revealed atrial septal defect, dextrocardia and hydronephrosis. In evolution, the infant developed frequent episodes of UTI with a high frequency admission rate. The positive diagnosis was UTI in an infant with prune-belly syndrome, cardiac anomalies and Pierre-Robin sequence.

Conclusions Although the ureterostomy was performed because of a previous complicated UTI, the risk of UTI has remained high, so we can consider the ureterostomy as an additional risk factor for developing recurrent UTI(s). The case prognosis could be appreciated as unfavorable. As a particularity, the cardiac malformations could be considered associated features in both Pierre-Robin sequence and Eagle-Barrett syndrome.

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