A case of prune belly syndrome.
The prune belly syndrome was first described in 1839 by Frolich. Till now about 300 cases have been reported in literature. The complete form of the syndrome is presented by a classic triad that is seen only in boys and is incompatible with life. The etiology of the syndrome is uncertain. Genetic factors, intrauterine infections, adverse mechanical factors have been implicated. The prenatal diagnosis relies on the ultrasonographic findings of oligohydramnion, renal anomalies, and non-immune fetal hydropsy. The present case concerns a newborn of male sex suffering from the most severe and rare form of the syndrome. The child died 5 minutes after his birth. The pathological examination found the patient devoid of striated muscles anterior abdominal wall, urethral atresia, hyperthrophy of the urinary bladder, bilateral hydroureter and hydronephrosis and coinciding cryptorchidism. The case indicates the PBS as one of the possible reasons for non-immune fetal hydropsy.
Peshev
Z V
ZV
Departement of General and Clinical Pathology, Higher Medical Institute, Plovdiv, Bulgaria.
Krusteva
M B
MB
Danev
V H
VH
D000009:Abdominal Muscles / Q000473:pathology; D006801:Humans; D006869:Hydronephrosis / Q000150:complications; D006984:Hypertrophy; D007231:Infant, Newborn; D018482:Muscle, Skeletal / Q000473:pathology; D011535:Prune Belly Syndrome / Q000150:complications* / Q000473:pathology*; D014515:Ureteral Diseases / Q000150:complications; D014521:Urethra / Q000002:abnormalities; D001743:Urinary Bladder / Q000473:pathology