Prune belly syndrome with penile and urethral agenesis: report of a case. 1998

T Kuga, and K Esato, and M Sase, and M Nakata, and J Kaneko, and T Inoue
First Department of Surgery, Yamaguchi University School of Medicine, Ube, Japan.

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.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D010413 Penis The external reproductive organ of males. It is composed of a mass of erectile tissue enclosed in three cylindrical fibrous compartments. Two of the three compartments, the corpus cavernosa, are placed side-by-side along the upper part of the organ. The third compartment below, the corpus spongiosum, houses the urethra. Glans Penis,Penis, Glans
D011535 Prune Belly Syndrome A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from its characteristic distended abdomen with wrinkled skin. Abdominal Muscle Deficiency Syndrome,Congenital Absence of the Abdominal Muscles,Eagle-Barrett Syndrome,Obrinsky Syndrome,Prune-Belly Syndrome,Eagle Barrett Syndrome,Prune Belly Syndromes,Prune-Belly Syndromes,Syndrome, Eagle-Barrett,Syndrome, Obrinsky,Syndrome, Prune Belly,Syndrome, Prune-Belly,Syndromes, Prune Belly,Syndromes, Prune-Belly
D005315 Fetal Diseases Pathophysiological conditions of the FETUS in the UTERUS. Some fetal diseases may be treated with FETAL THERAPIES. Embryopathies,Disease, Fetal,Diseases, Fetal,Embryopathy,Fetal Disease
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006869 Hydronephrosis Abnormal enlargement or swelling of a KIDNEY due to dilation of the KIDNEY CALICES and the KIDNEY PELVIS. It is often associated with obstruction of the URETER or chronic kidney diseases that prevents normal drainage of urine into the URINARY BLADDER. Hydronephroses
D014521 Urethra A tube that transports URINE from the URINARY BLADDER to the outside of the body in both the sexes. It also has a reproductive function in the male by providing a passage for SPERM. External Urethral Sphincter,External Urinary Sphincter,Internal Urethral Sphincter,Internal Urinary Sphincter,Internal Vesical Sphincter,Urethral Sphincters,External Urethral Sphincters,External Urinary Sphincters,Internal Urethral Sphincters,Internal Urinary Sphincters,Internal Vesical Sphincters,Sphincter, External Urethral,Sphincter, External Urinary,Sphincter, Internal Urethral,Sphincter, Internal Urinary,Sphincter, Internal Vesical,Sphincter, Urethral,Urethral Sphincter,Urethral Sphincter, External,Urethras,Urinary Sphincter, External,Urinary Sphincter, Internal,Vesical Sphincter, Internal

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