Myxedema or severe hypothyroidism is clinical condition where the thyroid gland does not produce any thyroid hormone. In some cases, myxedema condition is also being referred to as underactive thyroid. Myxedema typically present with a distinct facial appearance, hoarse slow speech, and dryness of the skin.
Presentation
The symptomatology of myxedema varies depending on the severity of the hormonal deficiency. In general, the hormones progress in severity with age and starts to worsen with each passing year. Mild symptoms of myxedema like weight gain and fatigue hallmarks the beginning of the progression of the disease. The chronic decrease in body metabolism gives rise to more obvious symptomatology like:
- Constipation
- Chronic fatigue
- Increased sensitivity to cold
- Unexplained weight gain
- Edematous face
- Skin dryness
- Hoarseness of voice
- Weakness of the muscles
- Hypercholesterolemia
- Myositis
- Swelling of joints
- Bradycardia
- Hair thinning
- Irregular menstruation
- Impaired memory
- Depression
Myxedema in children happens when the infant is born without a thyroid gland. Infants with hypothyroid will present with jaundice, protruding tongue, frequent choking, puffy face, poor muscle tone, constipation, and excessive sleepiness.
Workup
Patients that have signs and symptoms of myxedema should submit for blood test for thyroid hormone quantitative analysis. Blood levels of Thyroxine (T4) usually appears low due to thyroid gland dysfunction or aplasia (absence). The levels of thyroid stimulating hormone (TSH) from the pituitary gland is usually increased as a compensatory reaction to the decreasing thyroid hormone levels in an attempt to increase its level by stimulating the thyroid gland to produce more [8]. Women who are pregnant and those who intend to be pregnant with previous history thyroid dysfunction must submit for this thyroid test because of the risk of recurrence [9].
Treatment
In general, thyroid deficiency in myxedema is adequately replaced by synthetic thyroid hormones like levothyroxine. The daily maintenance of oral synthetic hormones reverses the signs and symptoms of myxedema among patients [10]. Fatigue symptoms gradually decreases within 1 to 2 weeks from the start of hormonal replacement. Treatment with oral synthetic thyroid hormones are usually given for life as maintenance.
Prognosis
The thyroid deficiency in myxedema is easily treated with synthetic thyroid hormones which are taken for life. Myxedema that complicates to coma and cardiovascular crisis carries a high mortality in up to 50% among those with late therapeutic interventions. Even if these myxedema complications are diagnosed and addressed promptly, mortality remains high at 25% [6]. Factors like old age, concomitant cardiac dysfunction, sepsis, persistent hypothermia, and hypotension worsens the prognosis of myxedema crisis [7].
Etiology
Myxedema as clinical condition that produce deficient thyroid hormones can result from a number of medical condition or event. These include:
- Autoimmune disorders
- Post thyroid gland surgery
- Treatment for hypothyroidism
- Radiation therapy
- Medications (i.e. Lithium)
- Tyrosine Kinase inhibitors [1]
- Congenital hypothyroidism [2]
- Pituitary dysfunction
- Iodine deficiency
- Pregnancy
- Bexarotene [3]
Epidemiology
In the United States, myxedema is a common disorder of the elderly [4]. Myxedema or severe hypothyroid is seen in 8% of women and 2% of men beyond the age of 50 years old. In countries worldwide with no iodine food supply insufficiency, almost 8% of women above the age of 50 are suffering from myxedema.
The most common causes of myxedema internationally are autoimmune disorder and post-operation thyroid ablation. In regions with insufficient iodine supply however, iodine deficiency is the leading cause of the hypothyroid disorder. Neonatal myxedema is observed among patients with severe iodine deficiency clinically expressed as cretinism. This clinical condition abounds in the mountainous regions of Africa, Asia and South America. There are no racial predilection to the disease with women more prone in up to 4 times more than men.
Pathophysiology
The thyroid hormone is a vital hormone in the human body needed for proper cell growth or maturity, modulates other hormones, and energy production. The low energy states leads to chronic inactivity that leads to obesity if hypothyroid states in myxedema is left unabated. The decreasing rate of drug metabolism can make the patient prone to over dosage from common medications like hypnotics, sedatives, anesthetics, and morphine. Neurologic symptoms in myxedema is due to the decreased thyroid hormones triiodothyronine (T3) and thyroxine (T4) which reduces the cerebral oxygen and glucose consumption. Cardiac function is significantly depressed due to the decreased contractility of the myocytes due to the low T3 hormones that decreases available supply of adenosine triphosphate (ATP) for the heart cells [5].
The decrease in cardiac output and the increase in the vascular resistance may lead to signs of cardiac failure like pulmonary effusion and pericardial effusion with edema. The fluid accumulation in the lungs impairs the oxygen exchange function of the organ. Long standing low cardiac output will lead to renal insufficiency and compromises the kidney. The low serum thyroid hormonal levels decreases intestinal motility that advertently leads to megacolon, gastric atony, and paralytic ileus.
Prevention
Myxedema with congenital causes are not preventable. Patients scheduled for surgical intervention for goiter and other thyroid masses should submit for close monitoring to prevent complications of hypothyroidism from ensuing. Proper dosage of thyroid drugs should always be observed to prevent untoward complications like myxedema. Mild infections of the thyroid gland like thyroiditis must ardently be treated to prevent dysfunction.
Summary
Myxedema or underactive thyroid is a medical condition where there is not enough thyroid hormone in the circulation. Myxedema is common among women beyond the age of 60 years old causing a physiologic imbalance to the body systems. Untreated myxedema leads to obesity, infertility, heart diseases, and arthritis. The therapeutic goal in the treatment of myxedema is the replacement of thyroid hormone with synthetic thyroid hormones which corrects the hormonal imbalance.
Patient Information
- Definition: Myxedema or underactive thyroid is a medical condition where there is not enough thyroid hormone produced from the thyroid gland available in the circulation.
- Cause: Myxedema is caused by autoimmune disorders, post-surgical complications, medications, infections, pregnancy, and radiation therapy.
- Symptoms: Hypothyroidism signs of myxedema includes fatigue and unexplained weight gain. Progressive signs like constipation, dry skin, hoarseness of voice, arthritis, and bradycardia.
- Diagnosis: Patients with myxedema are subjected to blood test to determine the levels of thyroxine and TSH levels.
- Treatment and follow-up: The treatment of myxedema is basically by the replacement of the deficient thyroid hormone with oral synthetic thyroid hormone which is taken in for life.
References
- Wolter P, Dumez H, Schöffski P. Sunitinib and hypothyroidism. N Engl J Med. Apr 12 2007; 356(15):1580; author reply 1580-1.
- Vono-Toniolo J, Rivolta CM, Targovnik HM, Medeiros-Neto G, Kopp P. Naturally occurring mutations in the thyroglobulin gene. Thyroid. Sep 2005; 15(9):1021-33.
- Smit JW, Stokkel MP, Pereira AM, Romijn JA, Visser TJ. Bexarotene-induced hypothyroidism: bexarotene stimulates the peripheral metabolism of thyroid hormones. J Clin Endocrinol Metab. Jul 2007; 92(7):2496-9.
- Rehman SU, Cope DW, Senseney AD, et al. Thyroid disorders in elderly patients. South Med J. May 2005; 98(5):543-9.
- Diekman MJ, Harms MP, Endert E, et al. Endocrine factors related to changes in total peripheral vascular resistance after treatment of thyrotoxic and hypothyroid patients. Eur J Endocrinol. Apr 2001; 144(4):339-46.
- Mathew V, Misgar RA, Ghosh S, Mukhopadhyay P, Roychowdhury P, Pandit K, et al. Myxedema coma: a new look into an old crisis. J Thyroid Res. 2011; 2011:493462.
- Rodríguez I, Fluiters E, Pérez-Méndez LF, et al. Factors associated with mortality of patients with myxoedema coma: prospective study in 11 cases treated in a single institution. J Endocrinol. Feb 2004; 180(2):347-50.
- Aoki Y, Belin RM, Clickner R, Jeffries R, Phillips L, Mahaffey KR. Serum TSH and total T4 in the United States population and their association with participant characteristics: National Health and Nutrition Examination Survey (NHANES 1999-2002). Thyroid. Dec 2007; 17(12):1211-23.
- Stuckey BG, Kent GN, Ward LC, Brown SJ, Walsh JP. Postpartum thyroid dysfunction and the long-term risk of hypothyroidism: results from a 12-year follow-up study of women with and without postpartum thyroid dysfunction. Clin Endocrinol (Oxf). Sep 2010; 73(3):389-95.
- McDermott MT. Does combination T4 and T3 therapy make sense? Endocr Pract. Sep-Oct 2012; 18(5):750-7.