Resumo

MONTIEL ALFONSO, Miguel Angel; ACCHIARDI PAEZ, Rossana Marietta; KLASSEN DE BURGOS, Marisa Beatriz  e  CAUSARANO ZARZA, Hugo. Cavernous transformation of the portal vein associated with occult essential thrombocythemia. Rev. virtual Soc. Parag. Med. Int. [online]. 2021, vol.8, n.2, pp.114-119. ISSN 2312-3893.  https://doi.org/10.18004/rvspmi/2312-3893/2021.08.02.114.

Cavernous transformation of the portal vein is a condition characterized by the formation of a network of dilated collateral veins along a previously thrombosed portal vein. It is considered a low-frequency entity and occurs more commonly in the pediatric population.

We present the case of an adult woman with a diagnosis of cavernous transformation of the portal vein, originated as a consequence of occult essential thrombocythemia.

As a therapeutic measure for portal hypertension, endoscopic ligation of gastroesophageal varices was performed on multiple occasions without positive results. Portosystemic bypass was not performed due to the presence of abundant collaterals. Finally, a splenectomy was performed, after which essential thrombocythemia was evidenced.

The patient evolved with multiple medical-surgical complications, which led to multisystem failure and subsequent death.

There are no regional prevalence data, nor have there been reports of cavernous transformation of the portal vein associated with essential thrombocythemia, for which reason we consider of great importance to make this case known, in order to help establish the diagnosis and treatment of this rare entity with greater precision and speed.

Palavras-chave : essential thrombocythemia; portal hypertension; gastroesophageal varices; portal vein..

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