Teruel GR, Leyva MMA, Borjas LR, Díaz TI

Language: Spanish
References: 10
Page:
PDF size: 145.96 Kb.

ABSTRACT

A 19-year-old patient carrier of a Poland’s syndrome in association with neurofibromatosis I and extended aortic tubular hypoplasia producing severe secondary high blood pressure. The physical examination showed hypoplasia of the left pectoral’s major muscle, including the breast, areola and nipple of the same side. The left arm was hypotrophy and with less length than the right one. Many café au lait spots, freckle in the axillae and typical neurofibromas were found in the skin. There was a severe asymptomatic high blood pressure with imperceptible femoral pulse and a grade II hypertensive retinopathy. The chest x-ray showed hypoplasia of the arch of the aorta and it was evident the concentric hypertrophy of the left ventricle by the echography. The both arms x-ray showed a hypotrophy of the left radius and ulna, and the left kidney was considered as not functional with absence of the contrast uptake in the urography. The multicut contrasted tomography determinated a sacral neurofibroma, stenosis of the left subclavian and renal arteries, and aortic hypoplasia in all of its extension with 9 mm. Ethyopathogenic considerations were made, the extraordinary rare of the tubular aortic hypoplasia and in the exceptional of the association of the three pathologic states.

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