Abstract Ref Number = APCP880
Recurrent Pneumonia in Patient with Prune Belly Syndrome
EvaJeumpa Soelaeman,Budi Purnomo,Ariani Dewi Widodo
Pediatric Gastrohepatology Working Group Department of Pediatrics RSAB Harapan Kita
Background : Prune-belly syndrome (PBS) is a rare congenital malformation characterized by the lack of abdominal muscles, urinary tract malformation, and cryptorchidism. We report a patient with PBS who has recurrent lower respiratory tract infection.
Case Presentation Summary : A 4-month-old baby boy born with PBS was admitted for the fifth time for severe pneumonia, two of which involved hospitalization at the ICU. Five days prior to admission, patient started to have cough, and starting from 3 days prior to admission dyspnea was observed and progressed rapidly. On physical exam HR: 124 bpm, RR: 35 rpm, T: 36.7’C, weight: 5773 grams, anemic conjunctiva, nasal flaring present, retraction on the chest wall, crackles +/+, prune-like wrinkled skin abdomen wall on inspection. His laboratories are haemoglobin 8.6, leukocyte: 9360, platelet: 243000. Blood gas analysis indicated respiratory acidosis. Renal function showed blood urea 14 mg/dL and creatinine 0.2 mg/dL with GFR 261.6 mL/min/1.73m2. Routine urinalysis: albumin +2, occult blood +3, leukocyte +2. Chest X-ray examination indicates inhomogenous consolidation in the mid of right lung, and hyperinflation of both lungs. Urinary tract sonography indicated hydronephrosis grade I on right kidney. Bronchoscopy examination indicated laryngomalacia type 3, mucous plug at the upper right lobe. Patient was given intravenous antibiotics, inhalation, cough combination powder, nasogastric intubation, and fasting.
Learning Points/Discussion : Recurrent respiratory tract infection is a common complication in PBS due to ineffective valsava and poor expectoration. This is one of the most common reasons for readmission of children with PBS, and should be a focus of follow-up.
Keywords: Prune belly syndrome recurrent pneumonia abdominal muscular defect respiratory tract infection