We report a 12-year-old girl with long-standing severe hypothyroidism due to autoimmune thyroiditis. She presented with congestive heart failure as a result of dilated cardiomyopathy, multiple-valve regurgitation, and pericardial effusion. She also had severe short stature (Ϫ5 standard deviation) and profound retarded bone age (3 years). Despite severe growth retardation, she had breast development without galactorrhea. She had a prepubertal response to luteinizing hormone-releasing hormone. Multicystic ovaries were noted on pelvic sonography. Magnetic resonance imaging of the brain revealed pituitary hyperplasia. Treatment with L-thyroxine and digoxin was promptly initiated with a subsequent clinical improvement and near-normal thyroid function tests. Unfortunately, she developed digitalis intoxication at 6 weeks, and eventually expired. Thus, titrating the dose of digoxin and frequent monitoring of serum digoxin levels are essential to prevent digitalis intoxication in the hypothyroid patient.A lthough common signs and symptoms of childhood hypothyroidism are well-recognized, there are rare clinical features associated with long-standing primary hypothyroidism including isosexual precocity with multicystic ovaries, the Van Wyk-Grumbach syndrome. 1-4 Apart from the endocrine complications, cardiac involvement is also reported periodically in adults but rarely in children. [5][6][7][8][9][10] We report a case of long-standing profound hypothyroidism due to autoimmune thyroiditis presenting with congestive heart failure (CHF) as a result of dilated cardiomyopathy and pericardial effusion with pseudopuberty associated with multiple small ovarian cysts.
CASE REPORTA 12-year-old Thai girl was referred to the Department of Pediatrics at Ramathibodi Hospital, Mahidol University. She had a history of CHF for 1 month. She also had extreme short stature and mental retardation. She was born at term to an uncomplicated pregnancy with a birth weight of 1.9 kg. Her weight was 20 kg or -3.5 standard deviation (SD) and she was 102 cm tall or -5.0 SD. Signs of CHF included tachypnea, orthopnea, crepitant rales, and pretibial pitting edema were noted. She had a puffy face, coarse dry hair, carotenemic skin, and abundant lanugo hair. The thyroid gland was not enlarged. Neurologic examination revealed slightly pale optic discs and a slow relaxation phase of the deep tendon reflexes. There were no other neurologic deficits. Pubertal staging was Tanner stage III for breast development and Tanner stage I for pubic hair. There was no galactorrhea. Chest x-ray revealed marked cardiomegaly (Fig. 1). Electrocardiography showed generalized low voltage and a heart rate of 100 bpm. Echocardiography findings showed impaired cardiac contraction (left ventricular ejection fraction of 0.26), dilatation of all chambers with moderate regurgitation of all valves, mildly thickened mitral and aortic valves, and a pericardial effusion of 12 mm thickness (Fig. 2). The cardiac enzymes, including creatine kinase-MB, lactic dehydrogenase, and aspartate am...