Current knowledge about the in utero and peripartum management of fetal goiter associated with maternal Graves' disease.

Maternal Graves' disease is the most common cause of fetal goiter. Fetal goiter can cause complications attributable either to the physical effects of the goiter itself or to thyroid dysfunction, which can be life-threatening and cause neurological impairment. Determining whether a goiter is caused by fetal hyperthyroidism or hypothyroidism is the main clinical problem, and in utero evaluations and management are essential. Ultrasonography combined with color Doppler and magnetic resonance imaging are helpful for the initial diagnosis and monitoring, but these imaging techniques have a limited ability to discriminate between fetal hyperthyroidism and hypothyroidism. To determine the fetal thyroid status, fetal blood sampling using cordocentesis is reliable but hazardous, and the indications must be considered carefully. Amniocentesis is an easier and safer alternative, but the correlations between the amniotic fluid and fetal serum thyroid hormone levels remain unclear. If a fetal goiter is accompanied by hypothyroidism, administering thyroid hormone intra-amniotically may be effective and relatively safe. However, the wide variety of approaches to treatment exemplifies the lack of guidelines, and no systematic studies have been conducted to date. Therefore, intrauterine treatment should be reserved for selected patients at a high risk of complications. Moreover, when intrauterine treatment fails and a fetal goiter can cause airway obstruction, intrapartum management, such as ex utero intrapartum treatment, may be required; however, reports describing the use of this procedure for fetal goiter are limited. This review summarizes the current knowledge about fetal goiter associated with maternal Graves' disease and evaluates the most significant new findings regarding its in utero and peripartum management.