ADRENO-GENITAL SYNDROME 343 



tion is absent. In male children, there is also hypertrichiasis, 

 a deep voice, enlargement of the penis, and external genitalia, 

 but the testes remain immature and show no spermatogenesis. 

 There is an unusual muscular development, more marked 

 particularly in the male, so that the child appears as an "infant 

 Hercules." The patients appear matured and have an adult 

 appearance. 



A typical case in a male child as described by Gordon and 

 Browder 232 follows: In their patient, at nine months of age, 

 an abnormal development of the external genitalia was appar- 

 ent. Pubic hair appeared at 12 months. On admission to 

 the hospital, at the age of three, the patient appeared like an 

 "adult dwarf with the musculature and general development 

 of a young Hercules." The voice was deep and gruff. "The 

 scalp was covered with thick hair extending well down over 

 the forehead and posteriorly down on the neck. The eye- 

 brows were heavy, shaggy and continuous across the bridge of 

 the nose." There was a marked growth of hair on the face, 

 upper Up, chin, back, pubic, and perineal regions. The skele- 

 ton was markedly enlarged except for the humeri, which were 

 the length expected in a three-year-old child. Dentition and 

 osseous development were that of a child of seven to ten years 

 of age. At no time had there been any priapism, nor evidence 

 of nocturnal emissions or masturbation. 



Autopsy of the above described case revealed numerous 

 metastatic tumors in the lung which had been the immediate 

 cause of death. In the region of the left adrenal and extending 

 from the dome of the left diaphragm downward to the upper 

 pole of the right kidney and medially to the midline, was pres- 

 ent an irregular nodular tumor lying entirely retroperitoneally. 

 The right adrenal was absent. That part of the adrenal gland 

 not affected by the tumor was normal on gross examination. 



An important feature of the juvenile form of the adreno- 

 genital syndrome is the absence of true precocious puberty. 

 Menstruation does not occur in the female at an early age nor 



