2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Congenital Anomalies 740-759 > Chromosomal Anomalies 758.* > 2006 ICD-9-CM Diagnosis 758.6 Gonadal dysgenesis View the most recent version of ICD-9-CM 758.6 A syndrome in which the affected patients have only 45 chromosomes, the loss of one of the X chromosomes producing an XO chromosome constitution. The syndrome occurs in 1/2500 female births and nearly 99% of XO fetuses are aborted. Gonadal agenesis and short stature are the main features in the surviving infants. Associated anomalies may included webbed neck, cubitus valgus, shield chest, short stature, lymphedema, coarctation of the aorta, pigmented nevi, and various renal, skeletal, dermatologic, neoplastic, and autoimmune complications. Mental retardation is attributed to ring chromosome X. The phenotype varies and not all abnormalities occur in all patients. A syndrome of defective gonadal development in phenotypic women with a karyotype of sex chromosome monosomy (45,X or 45,XO), associated with the loss of a sex chromosome X or Y. Patients generally are of short stature with undifferentiated (streak) gonads, sexual infantilism (HYPOGONADISM), webbing of the neck, cubitus valgus, elevated GONADOTROPINS (LH and FOLLICLE STIMULATING HORMONE) and decreased ESTRADIOL level in blood. Studies of Turner Syndrome and its variants have contributed significantly to the understanding of SEX DIFFERENTIATION. NOONAN SYNDROME bears similarity to this disorder; however, it also occurs in males, has normal karyotype, and is inherited as an autosomal dominant. This syndrome that was originally observed by Ullrich, and designated as identical to TURNER SYNDROME, related the webbing of the neck, loose skin and other anomalies of the syndrome to accumulation of fluid in the embryo starting at the head and dispersing to the extremities (as observed by Bonnevie in mice). Commonly observed at birth in Turner Syndrome and NOONAN SYNDROME; EDEMA of the extremities usually recedes by one year and is an early sign of Turner syndrome, especially in female neonates. 758.6 is a specific code that can be used to specify a diagnosis 758.6 contains 22 index entries View the ICD-9-CM Volume 1 758.* hierarchy Alternate Terminology Ovarian dysgenesis Turner's syndrome XO syndrome 758.6 Excludes pure gonadal dysgenesis (752.7)  Index entries containing 758.6: Absence (organ or part) (complete or partial) gonadal, congenital NEC 758.6 Agenesis - see also Absence, by site, congenital gonadal 758.6 Anomaly, anomalous (congenital) (unspecified type) 759.9 chromosomes, chromosomal 758.9 sex 758.81 complement, XO 758.6 gonadal dysgenesis 758.6 Turner's 758.6 Bonnevie-Ullrich syndrome 758.6 Dwarf, dwarfism 259.4 ovarian 758.6 Dysgenesis gonadal (due to chromosomal anomaly) 758.6 pure 752.7 ovarian 758.6 seminiferous tubules 758.6 OX syndrome 758.6 Syndrome - see also Disease Bonnevie-Ullrich 758.6 OX 758.6 pterygolymphangiectasia 758.6 Turner's 758.6 Turner-Varny 758.6 Ullrich (-Bonnevie) (-Turner) 758.6 XO 758.6 Turner's syndrome 758.6 Turner-Varny syndrome 758.6 Ullrich (-Bonnevie) (-Turner) syndrome 758.6 XO syndrome 758.6