Consideraciones quirúrgicas del bocio amiloide / Surgical considerations about amyloid goiter

La amiloidosis es un síndrome poco frecuente, constituido por un conjunto de trastornos que tienen en común el depósito extracelular de proteínas fibrilares. De ello resultan alteraciones funcionales y estructurales de los órganos afectados, dependientes de la localización e intensidad del depósito.La infiltración amiloide del tiroides puede verse en el 50% de la amiloidosis primaria y hasta en el 80% de la forma secundaria. El bocio amiloide (BA) es una verdadera rareza, habitualmente asociado a la forma secundaria. El BA puede tener indicación quirúrgica, generalmente justificada por problemas compresivosComunicamos el caso de un paciente con un gran BA en el curso de una amiloidosis secundaria asociada a una artritis idiopática juvenil de inicio poliarticular, al que se le practicó una tiroidectomía total. Revisamos la literatura, intentando aportar pautas de actuación y algunas consideraciones quirúrgicas sobre esta rara afección (AU)

Amyloidosis is an uncommon syndrome consisting of a number of disorders having in common an extracellular deposit of fibrillary proteins. This results in functional and structural changes in the affected organs, depending on deposit location and severity.Amyloid infiltration of the thyroid gland may occur in 50% and up to 80% of patients with primary and secondary amyloidosis respectively. Amyloid goiter (AG) is a true rarity, usually found associated to secondary amyloidosis. AG may require surgical excision, usually because of compressive symptoms.We report the case of a patient with a big AG occurring in the course of a secondary amyloidosis associated to polyarticular onset juvenile idiopathic arthritis who underwent total thyroidectomy. Current literature is reviewed, an attempt is made to provide action guidelines, and some surgical considerations on this rare condition are given (AU)

Surgical considerations about amyloid goiter.

Amyloidosis is an uncommon syndrome consisting of a number of disorders having in common an extracellular deposit of fibrillary proteins. This results in functional and structural changes in the affected organs, depending on deposit location and severity. Amyloid infiltration of the thyroid gland may occur in 50% and up to 80% of patients with primary and secondary amyloidosis respectively. Amyloid goiter (AG) is a true rarity, usually found associated to secondary amyloidosis. AG may require surgical excision, usually because of compressive symptoms. We report the case of a patient with a big AG occurring in the course of a secondary amyloidosis associated to polyarticular onset juvenile idiopathic arthritis who underwent total thyroidectomy. Current literature is reviewed, an attempt is made to provide action guidelines, and some surgical considerations on this rare condition are given.