Causes of death in congenital hypothyroidism. An autopsy study

Children with congenital hypothyroidism are prone to die unexpectedly. In order to test this hypothesis, the primary and contributing causes of death were studied in a case series of sixteen consecutive children coming to autopsy. Four patients with absent thyroid died undiagnosed and untreated. The remaining twelve cases had documented hypothyroidism with low T3 and T4 levels. Diagnosis was established after the age of two months. Nine of the sixteen cases died unexpectedly, three while in the hospital and six at home. Autopsy findings suggested bronchoaspiration in five and heart failure in four. The remaining seven cases died under predictable circumstances with serious infections. Children with congenital hypothyroidism with delayed treatment may die unexpectedly as a result of the organic dysfunction caused by their primary disease.