F. Baulieu

Lymphœdème de membre: Critères prédictifs de l'efficacité du traitement physique combiné

BACKGROUND Limb lymphedema results from incompetence of the lymphatic system, and treatment of both primary and secondary forms involves manual lymphatic drainage and support. The effectiveness of treatment varies from patient to patient and can be unpredictable. OBJECTIVE To investigate clinical and paraclinical criteria able to predict responses to combined physical treatment (lymphatic drainage and multilayered support) during hospitalization. PATIENTS AND METHODS A retrospective study was performed in 45 patients admitted for one weeks intensive treatment of limb lymphedema in the Lymphology Unit of the Department of Dermatology of the University Hospital of Tours. Lymphoscintigraphy was performed for all patients on admission. Loss of volume in affected limbs was studied to evaluate the effectiveness of treatment. RESULTS Two criteria were predictive of resistance to combined treatment, i.e. venous insufficiency and continuing lymph node evidence of scintigraphic activity four hours after lymphoscintigraphy. The two factors were significantly related. CONCLUSION Failure of combined physical treatment for lymphedema appears to be related to venous insufficiency. It is therefore essential to investigate and treat venous insufficiency, particularly in patients with continuing evidence of scintigraphic activity in lymph nodes.Resume Introduction Le lymphœdeme de membre resulte d’une incompetence du systeme lymphatique. Qu’il soit primitif ou acquis, la prise en charge est le drainage lymphatique manuel et la contention. L’efficacite de ce traitement varie beaucoup selon les malades, sans caractere previsible. Buts de l’etude Chercher des criteres cliniques ou paracliniques permettant de prevoir la reponse au traitement physique combine (drainage lymphatique et bandage multicouches) pratique en hospitalisation. Malades et methodes L’etude retrospective a porte sur 45 malades, tous hospitalises une semaine dans l’unite de lymphologie du service de Dermatologie du CHU de Tours pour traitement intensif d’un lymphœdeme de membre. Tous ces malades avaient eu une lymphoscintigraphie du membre lymphœdemateux en debut d’hospitalisation. Pour evaluer l’efficacite des drainages, nous avons etudie la perte de volume du membre atteint. Resultats Deux criteres se sont averes predictifs d’une resistance au traitement combine : l’existence d’une insuffisance veineuse et une activite ganglionnaire a la lymphoscintigraphie a 4 heures. Ces deux criteres etaient lies entre eux de facon tres significative. Conclusion L’insuffisance veineuse semble etre un facteur de resistance au traitement physique combine. Pour optimiser les resultats de ce traitement, il convient donc de la depister et de la traiter, en particulier chez tous les patients ayant une activite ganglionnaire sur les cliches tardifs de lymphoscintigraphie.

Generalized lymphedema associated with neurologic signs (GLANS) syndrome: A new entity?

BACKGROUND Primary lymphedema in children, especially generalized disease with facial involvement, is rare. OBJECTIVE We sought to report 3 childhood cases of lymphedema with associated neurologic findings and to provide a pathophysiologic explanation for this association. METHODS Clinical observations, electroencephalography, and neuroimaging studies were evaluated. Microcomparative genomic hybridization was performed in 1 case. RESULTS The 3 children had primary lymphedema of all 4 limbs and the face. This was confirmed by lymphoscintigraphy, which showed hypoplasia of vessels and hypofixation of lymph nodes. They had nonspecific neurologic disorders and electroencephalography abnormalities, without intellectual deficit. Neuroimaging revealed normal findings. Microcomparative genomic hybridization in 1 patient revealed no cytogenetic anomaly. The outcome was fatal in 1 case with development of visceral lymphedema and coma. LIMITATIONS Genetic studies were performed in only 1 case. CONCLUSION These observations suggest that neurologic assessment and electroencephalography are indicated for patients with lymphedema of the limbs and face to identify this syndrome.