Thyroid "vise" in an infant with neonatal Graves' disease

On the rare occasion when neonatal goiter is the cause of airway compromise, it typically presents with a palpable neck mass. In the setting of maternal Graves' disease (GD), fetal and neonatal goiters are most commonly caused by maternal treatment with antithyroid medication, and the goiter resolves within days of initiation of thyroxine replacement in the neonate. We describe an atypical presentation of a patient with severe neonatal GD born to a euthyroid mother at 35 weeks' gestational age with respiratory compromise, symptoms of hyperthyroidism, and a nonpalpable thyroid gland. The mother had a history of GD treated with radioactive iodine ablation; during the pregnancy she was treated with levothyroxine throughout and propylthiouracil beginning at 5 months' gestation, for fetal tachycardia. Laboratory testing after birth confirmed neonatal GD. The patient was treated with methimazole, Lugol's solution, and levothyroxine, and the patient remained euthyroid from day of life 10. After multiple extubation attempts failed, the patient was found on visualization studies to have a large, predominantly posterior, "vise-like" goiter encasing the larynx and upper trachea. The patient was successfully extubated, and all medications were discontinued on day of life 60. The patient remained euthyroid 1 month after discontinuation of treatment. The patient's atypical presentation illustrates the need for early neck imaging in patients with neonatal GD and respiratory distress, especially when the thyroid gland is not palpable. Treatment options for resolving a goiter due to neonatal GD are not clear.