Left ovarian cyst and right streak ovary in a neonate with a normal karyotype. Report of a case of neonatal Slotnick-Goldfarb syndrome or recessive gonadal dysgenesis.
A female neonate with a streak ovary on one side and huge ovarian cyst on the other side is presented. Her karyotype was 46,XX. Adnexectomy was mandatory, leaving the patient with a single streak gonad. Whether this infant has the recessive form of gonadal dysgenesis with one ovary, streak or hypoplastic, replaced by a cyst or the Slotnick-Goldfarb syndrome is impossible to elucidate at present. The potential implications of a streak gonad, either related to gonadal dysgenesis or as a possible source of neoplasia, are reviewed. Current approaches to an ovarian cyst and streak ovary in the neonatal period are discussed.
Freud
E
E
Department of Pediatric Surgery, Beilinson Medical Center, Petah Tiqva, Israel.
Zer
M
M
Merlob
P
P
D005260:Female; D005808:Genes, Recessive; D006059:Gonadal Dysgenesis / Q000235:genetics*; D006801:Humans; D007231:Infant, Newborn; D007621:Karyotyping; D010048:Ovarian Cysts / Q000150:complications / Q000235:genetics*; D010053:Ovary / Q000002:abnormalities*; D013577:Syndrome