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December 1980

The Association of 'Prune Belly' With Turner's Syndrome

Author Affiliations

Clinical Genetics Center Childrens Memorial Hospital Omaha, NE 68105; Department of Pediatrics Portage Clinic Portage, WI 53901; Department of Obstetrics and Gynecology State University of New York Stony Brook, NY 11794

Am J Dis Child. 1980;134(12):1171-1172. doi:10.1001/archpedi.1980.02130240051014

It has been postulated that fetal abdominal distention causes abdominal muscle deficiency, or "prune belly." Although urethral obstruction was proposed as the most common mechanism, other possible pathways were suggested, including fetal ascites secondary to impaired lymphatic development in cases of 45,X Turner's syndrome.1 We describe two patients with Turner's syndrome and prune belly and call attention to additional cases in the European literature that further delineate this association.

Report of Cases.—Case 1.—This female infant was 2.8 kg at birth after the uncomplicated term pregnancy, labor, and delivery of her gravida 2, para 3, 23-year-old mother. The father was 25 years old. Turner's syndrome was diagnosed neonatally. Long leg casts were required for genu recurvatum from the ages of 2 to 8 months. She has a mild to moderate conductive hearing loss. Her development is grossly normal. She has had one urinary tract infection.

The mother reported