THYROID DYSGENESIS AND THE DYSPLASIA HYPOTHESIS IN TUBEROUS SCLEROSIS

Thyroid dysfunction is rare in tuberous sclerosis, although papillary adenomas (hamartomas) of the thyroid gland have been reported in a few autopsy cases. We describe a child with tuberous sclerosis and primar y congenital hypothyroidism secondary to a dysgenetic thyroid gland. T o our knowledge, this association has not been reported previously. Al though the association of these two disorders in one patient may be me rely coincidence, we speculate that the dysgenetic thyroid gland in th is patient may represent a ''hamartia'' as a consequence of the tubero us sclerosis gene. (C) 1993 Wiley-Liss, Inc.