NEONATAL GRAVES' DISEASE

 

NEONATAL GRAVES' DISEASE

 

DEFINITION:

A disorder caused by the transplacental passage of maternal Thyroid-stimulating Immunoglobulin (TSI) to the fetus resulting in thyrotoxicosis.

EPIDEMIOLOGY:

  • incidence: 1% of infants born to mothers with Graves' Disease
  • age of onset:
    • neonate
  • risk factors:
    • mother with Graves' Disease, rarely with lymphocytic thryoiditis
    • M = F

PATHOGENESIS:

1. Background

  • maternal levels of TSI must be at least 5x normal to produce neonatal thyrotoxicosis thus only 1% of infants born to mothers with Graves' Disease will be affected
  • may occur in the absence of clinical maternal hyperthyroidism
  • clinical onset, severity, and course may be affected by the concomitant transplacental passage of TSH-inhibiting antibodies (TBIAb) and antithyroid medications (both tend to delay the onset of thyrotoxicosis)

CLINICAL FEATURES:

1. In Utero

  • fetal tachycardia in 3rd trimester (>160 beats/min)
  • many are premature

2. Neonatal

  • thyrotoxicosis usually evident within hours of birth although may be delayed for 4-6 weeks

    1. Thyrotoxicosis

    • exophthalmos, flushing, hyperactive, irritable, tremor
    • tachycardia, tachypnea, hypertension, elevated temperature
    • arrhythmias, congestive heart failure
    • vomiting and diarrhea with weight loss despite a voracious appetite
    • hepatosplenomegaly, jaundice
    • thyroid - goiter +/- airway obstruction

INVESTIGATIONS:

1. Serum

  • increased T3, total and free T4; decreased TSH
  • increased maternal TSI levels
  • hyperbilirubinemia, prolonged PT, thrombocytopenia

2. Imaging Studies

1. Skeletal X-Rays

  • advanced bone age
  • craniosynostosis
  • frontal bossing

MANAGEMENT:

1. Medical

1. Propylthiouracil

  • titrate to keep heart rate <160 beats/min
  • 5-15 mg/day po tid

2. Lugol Solution

  • 1 drop po q8h

3. For Cardiovascular Compromise

  • IV fluids
  • digitalization
  • propranolol
    • 1-2 mg/kg/day po qid

2. Natural History

  • usually remits spontaneously within 6-12 weeks but may rarely persist for longer or years depending on the level of TSI
  • 16% mortality rate

 

 

Pediatric Database - NEONATAL GRAVES' DISEASE

Pediatric Organization - Pedbase [at] Gmail.com