Prune belly syndrome with penile and urethral agenesis: Report of a case

• Published in: Journal of pediatric surgery Vol. 33; no. 12; pp. 1825 - 1828
• Main Authors: Kuga, Takayuki, Esato, Kensuke, Sase, Masakatu, Nakata, Masahiko, Kaneko, Junko, Inoue, Tamotsu
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01-12-1998; Elsevier
• Subjects: Biological and medical sciences; Fetal Diseases - surgery; Humans; Hydronephrosis - complications; Hydronephrosis - etiology; Hydronephrosis - surgery; Infant, Newborn; Male; Medical sciences; Penis - abnormalities; Prune Belly Syndrome - etiology; Prune Belly Syndrome - surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the urinary system; Urethra - abnormalities; penile agenesis; Prune belly syndrome; urethral agenesis; fetal treatment; Urethral disease; Pregnancy disorders; Catheter; Male; Obstruction; Prenatal; Fetal diseases; Histopathology; Urethra; Fetus; Diagnosis; Fetal therapy; Male genital diseases; Sonography; Human; Agenesis; Urinary system disease; Urinary tract disease; Congenital disease; Case study; Pathology; Striated muscle disease; Newborn; Malformation; Penis; Abdominal disease; Echography; Abdominal wall; Prune Belly syndrome
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/S0022-3468(98)90297-4

The authors report the case of an infant born with prune belly syndrome associated with penile and urethral agenesis. At 15 weeks' gestation, antenatal ultrasonography showed a fetal giant bladder, congenital hydronephrosis, and oligohydramnios, and at 17 weeks' gestation, a fetal vesicoamniotic shunt operation was performed. A boy was born at 33 weeks' gestation with prune belly syndrome, an anocutaneous fistula, and penile agenesis. A cystostomy and cut-back operation were performed immediately, showing urethral agenesis, no urethral opening, and left renal hypoplasia. Thereafter, his renal system began functioning normally, and a urinary tract infection resolved. The authors speculate that the prune belly syndrome in this patient was caused by penile and urethral agenesis.