left eyebrow 16 years from a distant rural area, with height above 2,500 m above sea level, in the Department of Nariño-Colombia, with clinical picture of 3 months of evolution consistent with the appearance of abdominal mass.
The patient had never had contact with a health service before.
The patient was admitted to the hospital with dyspnea on mild exertion, perioral cleft, hyperchromic conjunctiva and holosystolic murmur in all foci grade IV/VI, predominantly tricuspid.
In the abdomen, a non-mobile mass with defined borders, with a diameter of 14 x 15 cm, painless, located on the left dorsum, reaching hypogastrium.
Limbs in tambor pin and legs with grade I edema.
It was decided to hospitalize for study of abdominal mass, diagnosis and compensation of cardiovascular disease.
The admission blood count showed a marked increase in red blood cells (9,320,000/mm3), hemoglobin 19.9 g/dL, hematocrit 66%, white blood cells 3,500/mm3, platelets 48,000/mm3, acid matrix.
Echocardiography showed severe dilation of the right cavities, tricuspid regurgitation and severe pulmonary hypertension (PH) at the suprasystemic level (pulmonary artery systolic pressure - PASP: 115 mmHg).
Abdominal computed tomography (CT) was performed with intravenous contrast, which showed large bilateral intrarenal tumor mass, larger size and exophytic growth to left and with marked thinning of both kidneys.
The examination also showed multiple retroperitoneal lymph nodes and osteolytic vertebral lesions.
Chest CT showed no abnormalities in the lung parenchyma.
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Due to suspicion of severe PH with clinical signs of chronic hypoxia, sildenafil and oxygen were administered as pulmonary vasodilators, furosemide for management of fluid overload and allopurinol as a treatment of hyperuricemia.
Bone marrow biopsy reported erythroid hyperplasia with no evidence of malignancy, which ruled out lymphoma.
Due to anesthetic risk, secondary to PH, renal biopsy with Trucut guided by ultrasound was performed, whose pathology report confirmed the diagnosis of EMH.
Follow-up was performed for 8 months, with no significant changes in outcome.
