Coma mixedematoso e trombo de veia cava superior. Relato de caso e revisão da literatura / Myxedema coma and superior vena cava thrombus: case report and literature review

O coma mixedematoso é uma emergência endocrinológica rara e consiste na máxima expressão do hipotireoidismo, com alta mortalidade por suas complicações hemodinâmicas e ventilatórias, podendo ser agravadas por distúrbios da coagulação. Relatamos o caso de uma paciente diagnosticada com coma mixedematoso e trombo de veia cava superior. Buscamos salientar os distúrbios de coagulação frequentes no hipotireoidismo grave, que contribuem para o aumento da mortalidade deste grupo de pacientes. O diagnóstico e o tratamento precoce do coma mixedematoso, aliados à instituição imediata da terapia para o fenômeno trombótico encontrado, permitiram a evolução favorável do quadro. O relato, juntamente da bibliografia pesquisada, orientou o raciocínio sobre a relação dos distúrbios de coagulação, que ocorrem no hipotireoidismo descompensado. Apesar de poucos relatos, estes distúrbios podem ser frequentes e devem ser pesquisados, pois contribuem com o aumento da mortalidade.(AU)

Myxedema coma is a rare endocrinological emergency, consisting of the highest expression of hypothyroidism with high mortality due to hemodynamic and ventilatory complications, which may be aggravated by coagulation disorders. We report the case of a patient diagnosed with myxedema coma and superior vena cava thrombus. We sought to emphasize the frequent coagulation disorders in severe hypothyroidism, which contribute to increased mortality in this group of patients. The diagnosis and early treatment of myxedema coma, together with the immediate institution of therapy for the thrombotic phenomenon found, allowed the favorable evolution of the condition. The report, together with the literature, has guided the rationale for the influence of coagulation disorders that occur in decompensated hypothyroidism. Despite the few number of reports, these disorders can be frequent and should be investigated because they contribute to the increase in mortality.(AU)

Coma mixedematoso / Myxedema coma

El hipotiroidismo es una enfermedad frecuente, de diagnóstico y tratamiento simples. Si no es detectada a tiempo puede progresar a la forma más grave conocida como coma mixedematoso. El término "coma mixedematoso" es considerado generalmente engañoso, ya que la mayoría de los pacientes no se presenta inicialmente en estado de coma. La progresión típica es la letargia, evolucionando al estupor y eventualmente al coma, con insuficiencia respiratoria e hipotermia. Es relativamente infrecuente, afecta fundamentalmente a mujeres ancianas, y a menudo ocurre en invierno. Esta entidad debe ser considerada una forma de hipotiroidismo descompensado, desencadenada a partir de una variedad de enfermedades o condiciones no tiroideas que provocan un compromiso sistémico generalizado de extrema gravedad, con desenlace fatal de no mediar un diagnóstico precoz y un tratamiento intensivo.

Hypothyroidism is a frequently diagnosed and simply treated disease. If not recognised, however, in time it may develop into the most severe manifestation of hypothyroidism known as myxedema coma. The term "myxedema coma" is generally seen as misleading since most patients do not initially present in a coma. The typical progression is lethargy evolving into stupor and, eventually, into coma with respiratory failure and hypothermia. It mainly affects elderly women, often occurring in winter and is relatively rare. It can be considered a form of decompensated hypothyroidism often triggered by a variety of non-thyroid conditions or diseases provoking an extremely severe condition of multiple system failure with lethal consequences unless an early diagnosis is made and an aggressive treatment is administered.

Coma mixedematoso en hombre de 74 años: reporte de caso con revisión narrativa / Myxedema coma in a 74 year old man: case report and narrative review

Las células foliculares de la glándula tiroides producen las hormonas tiroxina y triyodotironina, reguladas por la hormona tirotrópica de la hipófisis anterior o tiroestimulante. El coma mixedematoso es definido como un hipotiroidismo profundo que se caracteriza por alteraciones de la conciencia que van desde letargia, estupor y coma al cual se asocia hipotermia, hipoglucemia, convulsiones, hipotensión arterial y manifestaciones de un hipotiroidismo descompensado. La causa o factor desencadenante puede prevenirse si existe diagnóstico previo de hipotiroidismo primario con tratamiento sustitutivo de hormonas tiroideas bien establecido o bien, si en ausencia de diagnóstico éste se sospecha y se trata adecuadamente. Constituye una urgencia médica de mortalidad elevada, donde los factores medioambientales como el frío, infecciones severas e intoxicaciones medicamentosas son importantes factores desencadenantes. Se presenta el caso de un adulto mayor de 74 años, de sexo masculino, sin antecedentes de enfermedad tiroidea que ingresó en Sala de Medicina Interna del Hospital Enrique Cabrera, Habana, Cuba, en el mes de diciembre de 2011, con signología clínica y de laboratorio propias de la hipofunción tiroidea, siendo confirmado coma mixedematoso y con evolución desfavorable.

The follicular cells of the thyroid gland produce thyroxine and triiodothyronine hormones, as regulated by the thyrotropic hormone of the anterior pituitary, also called thyroid stimulating hormone. Myxedema coma is defined as profound hypothyroidism characterized by impairment of consciousness ranging from lethargy to stupor and coma, associated with hypothermia, hypoglycemia, seizures, hypotension, and manifestations of uncompensated hypothyroidism. The condition can be prevented if diagnosis of primary hypothyroidism is established and thyroid hormone replacement therapy is instituted. It is considered a medical emergency with high mortality rates, where environmental factors such as cold, severe infections and drug poisoning are important triggering factors. We report the case of a 74 year old male adult with no history of thyroid disease who was admitted to the Internal Medicine Ward of the “Enrique Cabrera” Hospital, Havana, Cuba, in December 2011, with clinical and laboratory signs of thyroid hypofunction. Myxedema coma was confirmed and patient course was untoward.

A Case of Myxedema Coma Presenting as a Brain Stem Infarct in a 74-Year-Old Korean Woman

Myxedema coma is the extreme form of untreated hypothyroidism. In reality, few patients present comatose with severe myxedema. We describe a patient with myxedema coma which was initially misdiagnosed as a brain stem infarct. She presented to the hospital with alteration of the mental status, generalized edema, hypothermia, hypoventilation, and hypotension. Initially her brain stem reflexes were absent. After respiratory and circulatory support, her neurologic status was not improved soon. The diagnosis of myxedema coma was often missed or delayed due to various clinical findings and concomitant medical condition and precipitating factors. It is more difficult to diagnose when a patient has no medical history of hypothyroidism. A high index of clinical suspicion can make a timely diagnosis and initiate appropriate treatment. We report this case to alert clinicians considering diagnosis of myxedema coma in patients with severe decompensated metabolic state including mental change.

Elephantiasic pretibial myxedema

Grave's disease is an autoimmune disease of thyroid gland and pretibial myxedema occurs in 4% of these patients. We present a 50-year-old man with Grave's disease who was referred with nonpitting, edematous, erythematous plaques on legs and feet that became elephantiasic. Biopsy of skin revealed granular material deposition in dermis with splitting of collagen fibers. This is a rare presentation of pretibial myxedema that presented in this case

Coma mixedematoso / Myxedematous coma

É relatado o caso de um paciente de 76 anos, masculino, em coma mixedematoso, que evoluiu para óbito após 27 dia de tratamento intensivo. Discutem-se os métodos diagnóstico e a conduta terapêutica no coma do hipotireoidismo.

This case report of myxedema coma in a male adult patient (76 years old), who died after 27 days in the Intensive Care Unit (ICU). The diagnosis and treatment of myxedema coma is discussed.

Ascites due to hypothyroidism in a patient with alcoholic cirrhosis

Myxedema is the cause of ascites in less than 1 per cent of new-onset ascites cases, where as only 4 per cent of patients with hypothyroidism present ascites. When ascites is the first manifestation of thyroid insufficiency, there is usually a delay in diagnosis. We report here a case of myxedema ascites occuring in a patient with alcoholic cirrhosis, that was first thought to be the cause of the ascites, and review the features of 48 cases previously reported. Some clinic and analytical findings that have been commonly reported, are the prompt response (with resolution of ascites) to thyroid replacement treatment, a high total protein concentration in ascitec fluid, white moderate white blood cell counts and a lymphocyte predominance. Serum-ascites albumin gradient has been postulated to be high in myxedema ascites, but we believe this has been studied in too few cases thus far, to be conclusive.

Mucinosis cutáneas / Cutaneous mucinosis

La mucina está constituida por ácido hialurónico, que es el principal mucopolisacárido ácido de la dermis en condiciones fisiológicas. Su apariencia es la de un gel transparent y viscoso, y a la microscopia óptica se evidencia con tinciones especiales (azul de alcian a pH 2,5, hierro coloidal y mucicarmín). El aumento de depósito de mucina dérmica se denomina mucinosis cutánea, y si este depósito es el principal elemento observado en la histopatología constituye una mucinosis primaria o idiopática, con características clínicas e histopatológicas propias. Es importante diferenciarlas de aquellas dermatosis en que secundariamente se observa un depósito de mucina (dermatofibroma, granuloma anular, tumores cutáneos, etc.). Las mucinosis cutáneas primarias pueden clasificarse en focales o difusas, según sea la distribución de la mucina, y según sean sus características histopatológicas pueden constituir entidades clínicas bien diferenciadas

Dermatoses that Present with Cutaneous Sclerosis

The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.