THE ROLE OF LYMPHOSCINTIGRAPHY IN THE DIAGNOSIS OF LYMPHEDEMA IN TURNER SYNDROME

C Bellini, E Di Battista, F Boccardo, C Campisi, G Villa, G Taddei, C Traggiai, A Amisamo, P Polo Perucchin, CS Benfenati, E Bonioli, R Lorini

Abstract


Lymphedema can be present in patientsaffected by Turner syndrome (TS) with thedorsum of the hands and feet most commonlyaffected. This lymphedema results fromunderdevelopment of the lymphatic systembefore birth, and it usually decreases duringchildhood. The aim of our study was toevaluate the role of lymphoscintigraphy as adiagnostic tool in patients with TS to assesspossible impairments in the lymphatic system.Eighteen patients with TS were karyotyped toconfirm diagnosis and were evaluated bylymphoscintigraphy. Lymphatic dysfunctionwas demonstrated in 15/18 patients. Lymphoscintigraphicstudies showed: 1) lymphaticchannels, 2) collateral lymphatic channels,3) interrupted lymphatic structures, and 4)lymph nodes of the deep lymphatic system.Our data demonstrate that lymphoscintigraphyshould be mandatory not only in patientsaffected by Turner syndrome with signs oflymphatic dysplasia but also in those withminimal or absent signs of lymphatic impairmentin order to obtain a very early diagnosisand to provide substantial information forpossible medical or surgical treatment.

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