Surgical management of female congenital adrenal hyperplasia (adrenogenital syndrome)

1977 ◽  
Vol 60 (1) ◽  
pp. 142
Author(s):  
Wilbur D. Latham
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Surgical Management ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome

PSYCHOLOGIC STUDIES BEFORE AND AFTER CLITORIDECTOMY IN FEMALE PSEUDOHERMAPHRODITISM CAUSED BY CONGENITAL VIRILIZING ADRENAL HYPERPLASIA

PEDIATRICS ◽  
1958 ◽  
Vol 21 (5) ◽  
pp. 832-839
Author(s):  
Alan K. Rosenwald ◽  
Joseph H. Handlon ◽  
Ira M. Rosenthal ◽  
John S. Hyde ◽  
I. Pat Bronstein
Keyword(s):  
Adverse Effects ◽  
Congenital Adrenal Hyperplasia ◽  
Diagnostic Error ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
General Change ◽  
Female Pseudohermaphroditism ◽  
Sex Assignment ◽  
Before And After ◽  
Special Circumstances

Psychobogic tests were performed with five female pseudohermaphrodites with congenital adrenal hyperplasia both before and some time after clitoridectomy. Of these children, four had been reared as girls. The child who had been reared as a boy underwent change of sex assignment at the age of 6 years, after performance of the initial psychologic tests. None of the children showed evidence of significant psychopathology before clitoridectomy. None showed evidence of deleterious psychologic effects after the operation. The child whose sex was changed at the age of 6 years apparently made a good adjustment and showed no adverse effects as evaluated by psychologic tests. If there is no significant psychopathology, clitoridectomy may be performed without fear of deleterious psychologic effects in female pseudohermaphrodites who have been reared as girls. While, in general, change of sex assignment should not be done in any hermaphrodite beyond the infantile age period, female pseudohermaphrodites with adrenogenital syndrome who have been reared as boys, because of an original diagnostic error, should be individually evaluated with regard to possible change of sex assignment. Despite the apparent success in the case reported here with its unusual special circumstances, the psychologic hazards involved will make reassignment of sex inadvisable in most of these cases.

scholarly journals Nerve Sparing Reduction Clitoroplasty in a Case of Congenital Adrenal Hyperplasia

2021 ◽  
Vol 6 (2) ◽  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Psychological Stress ◽  
Neurovascular Bundle ◽  
Sexual Response ◽  
Tactile Sensation ◽  
Nerve Sparing ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
Young Girls

Adrenogenital syndrome, or Congenital Adrenal Hyperplasia, is caused by a congenital insufficiency of the enzyme 21-hydroxylase, which is responsible for converting cortisol into cholesterol. Because of virilizing effect of androgens and its over- production, girls develop clitoral hypertrophy or Clitoromegaly. Clitoromegaly is an embarrassing condition, causing psychological stress to young girls and, hence requiring intervention. The goals of clitoroplasty are to achieve normal genital anatomy and to preserve tactile sensation with a satisfactory sexual response. We present a case of Adrenogenital syndrome with Clitoromegaly managed by reduction Clitoroplasty, preserving the dorsal neurovascular bundle and extensive network of nerves around the corpora to the glans and there by preserving the tactile sensation of the clitoris.

Panel Discussion

PEDIATRICS ◽  
1949 ◽  
Vol 3 (4) ◽  
pp. 515-548
Author(s):  
A. WILMOT JACOBSEN ◽  
GEORGE F. KOEPF ◽  
NATHAN B. TALBOT ◽  
LAWSON WILKINS
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Panel Discussion ◽  
Cushing's Syndrome ◽  
Urogenital Sinus ◽  
Adrenal Tumors ◽  
Postnatal Life ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
Acute And Chronic Stress

The role of the adrenal cortex in resistance to stress and strain has been reviewed. Certain experimental data indicating increased secretion of the adrenal cortical hormone in acute and chronic stress has been presented and discussed in terms of Sayers' homeostatic theory of the acute stress mechanism and in terms of Selye's "Adaptation Syndrome." In addition, studies made on a patient with acute rheumatic fever were discussed. Changes in uric acid metabolism compatible with those that might be seen in an alarm reaction were noted in this patient. [SEE THE TABLE I IN SOURCE PDF]. In the adrenogenital syndrome there is increased protein anabolism due to excessive androgen, in Cushing's syndrome inhibition of protein anabolism due to excessive gluconeogenetic hormones. The manifestations of the adrenogenital syndrome differ according to the patient's sex and the age of onset. Congenital adrenal hyperplasia causes pseudohermaphroditism in the female. This can be differentiated from various types of genetic intersexuality by the evidences of excessive androgen from infancy on and by a fairly uniform type of heterosexual development with a persistent urogenital sinus. In the male congenital adrenal hyperplasia causes macrogenitosomia precox which is sometimes accompanied by Addisonian-like symptoms. This can be differentiated from other types of male precocity by the excessive excretion of androgen and lack of maturation of the testes. In postnatal life androgenic adrenal tumors or hyperplasia cause virilization in both sexes unaccompanied by embryonic abnormalities. Occasionally gynecomastia occurs in males. Most of the symptoms of Cushing's syndrome are probably caused by excess of the gluconeogenetic hormones. However, there is also usually an increased secretion of androgen and in some cases evidences of increased electrolyte-controlling hormones.

DIFFERENCES IN CORTICOSTEROID EXCRETION PATTERN BETWEEN INFANTS AND CHILDREN WITH THE ADRENOGENITAL SYNDROME

1960 ◽  
Vol XXXIV (IV) ◽  
pp. 508-512 ◽  
Author(s):  
C. G. Bergstrand ◽  
G. Birke ◽  
L.-O. Plantin
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Infants And Children ◽  
Adrenal Hyperplasia ◽  
Older Children ◽  
Adrenogenital Syndrome ◽  
Excretion Pattern

ABSTRACT In a previous study of infants and children with congenital adrenal hyperplasia two types of corticosteroid excretion pattern were demonstrated. The dominating steroid excreted by the older children was found to be pregnane-3α,17α,20-triol whereas the infants, who were »salt-losers« or »potential salt-losers«, excreted fifty to sixty per cent of the corticosteroids as pregnane-3α,17α,20-triol-11-one. Two of these infants were reexamined at the age of 2½ years and it was found that the pattern of the non-ketonic steroids had changed and was similar to that demonstrated by older children. The implications of these findings are discussed and it is concluded that the change is most probably related to age. Why the adrenal capacity to oxidize at C-11 decreases with age is obscure.

Sign in / Sign up

Export Citation Format

Share Document