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Congenital and Juvenile Hypothyroidism Due to Thyroid Dysgenesis

San Paulo, Brazil

ABSTRACT

Thirteen cases of ectopic thyroid tissue were followed for two years, divided into four groups:

Group one: (4 cases) presented a complete clinical picture of congenital hypothyroidism, with marked mental retardation.

Group two: (5 cases) developed hypothyroidism at different ages between 1–5 years; usually these patients exhibit a less severe clinical picture and variable mental retardation.

Group three: (2 cases) the hypothyroidism began at adolescence with normal sexual differentiation and intelligence, but a stunted growth.

Group four: (2 cases) euthyroid adults with a palpable mass at sublingual region and short stature.

No neurological signs of deaf-mutism were found among these patients. Familiar goiters or inbreeding were not found. Two cases, however, were found in the same family. Considerable delay in the bone age, and four cases of epiphyseal dysgenesis was observed in groups one and two. Radioactive scanning showed in all cases, a small, usually round mass of thyroid tissue in the upper middle cervical area. An attempt was made to correlate the size and weight of the mass with the clinical picture, giving negative results.

Two and six hours after a TSH stimulation test, a small increase in the PBI in groups one and two was noted. No response was observed in groups three and four. This may suggest a different level of endogenous TSH in those groups.

In eight patients and their respective mothers, agar diffusion and tanned red cell agglutinations tests were negative. These findings give little support for an autoimmune background for these patients. It is suggested that perhaps an intra-uterine factor may affect the normal glandular development. In nine cases the thyroid remnant was able to provide sufficient hormone for normal intra-uterine and variable postnatal development.

FOOTNOTES

¹ Fellow in Radiological Research, James Picker Foundation.

² From the First Medical Clinics, Hospital das Clinicas da Universidade de Sao Paulo and Instituto de Energia Atomica, Sao Paulo, Brazil.