CONGENITAL ADRENAL APLASIA

CONGENITAL aplasia of the adrenal glands has been noted previously to occur in cases of cranial anomaly, such as an encephalus or hydrocephalus, or other central-nervous-system malformations extensive enough to involve the pituitary gland. There have been cases of Addison's disease in adults in which at autopsy the adrenals could not be found.¹ Present knowledge of adrenal function would seem to indicate that the condition of these adrenals was acquired rather than present at birth. Selye states that it is doubtful that complete aplasia ever occurs and that it would be incompatible with postnatal life.² Acute or chronic adrenal insufficiency during the first year of life with the clinical features of either bronze pigmentation of the skin, gastrointestinal symptoms, or hydrolability, together with autopsy findings of adrenal pathology or successful adrenal replacement therapy, has been reported infrequently. There are seven such cases on record, with the earliest onset