2009 convegno malattie rare foeldi jung [23 01]
DESCRIPTION
Linfedema primarioTRANSCRIPT
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Linfedema primario
Dott.ssa K. Jung
Földiklinik, clinica specialistica di Linfologia,
79856 Hinterzarten/Schwarzwald Germany
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4,6%
15,3%
10,3%
16,7%
Linfed. multicausale
Lipolinfedema
Linfedema cronico
linfedema sec. gamba
linfed. sec.bracchio
Frequenza nella clinica
20,7%
3,0%
4,2%
2,6%
4,0%
2,9%
15,7%Malformazione venosa
flebolinfedema
linfed. maligno
linf. prim. bambini
linf. prim.gamba/gen
linfed. prim. gamba
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Linfedema primario : diversi quadri clinici
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Linfedema primario con linfedema del genitale
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Prima e dopo operazione
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Vasi linfatici iniziali
Linfedema primario: diversi quadri clinici
Fibrosi dei linfonodi inguinali
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Linfedema primario in combinazione con linfangiodisplasia intestinale
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Linfangiodisplasia con ascite
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Alexej Lobanov
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Il linfedema primario necessità di
differenti indagini e complessa
terapia !terapia !
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Dal punto di vista medico dobbiamo porci queste domande:
- localizzazione della malattia
- età del paziente
- multimorbidità
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Vorrei sottolineare che vi sono inoltre diversi
esami e metodi per :
� esaminare la permeabilitá dei capillari� esaminare la permeabilitá dei capillari
� esaminare la formazione della linfa
� esaminare il trasporto della linfa
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Ulteriori terapie in altre disciplineRiabilitazione
ambulatoriale
Day hospital
Riabilitazione stazionaria
Terapia ospedaliera
Policlinico universitario di Friburgo
Centro specialistico di linfologia Hinterzarten-Friburgo
Servizio sanitario
nazionale e privato
Medico di famiglia
Sevizio medico
Terapia ambulatoriale riabilitatione Fisioterapia
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Bimba,età di 6 anni:
Angiomi a livello del dorso
Linfedema del genitale
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Mascio, età di 3 anni:
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Paziente, eta`20 anni,linfangioma a livello della guancia
• Terapia:
operazione , Neurolisi del Nervo Neurolisi del Nervo facialie, Facelift
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prima dopo
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Esami diagnostici supplementari:
� nel caso di un linfedema dei genitali:
� diagnostica ginecologica con il sospetto di un linfangioma a livello del piccolo bacino linfangioma a livello del piccolo bacino
� „cream test“ per esaminare un flusso di chilo
� MRI
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Phase I :Decongestione Phase II: conservazione e miglioramento
DCP
Drenaggio linfatico manuale; terapia compressiva; cura della pelle, ginnastica
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Terapia complessa di Decongestione
igiene della pelle LM compressione
Mobilizzazione
Tetrade
Terapia di 2-fasi
fase 1: - fase di Decongestione (quotidiano) fase 2: - fase di conservazione
Compressione durante fase 1: bendaggio
Compressione durante fase 2 : calze su misure
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Phase I :Decongestione Phase II: conservazione e miglioramento
DCP
Drenaggio linfatico manuale; terapia compressiva; cura della pelle, ginnastica
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Controindicazioni assolute:
- Insufficienza cardiaca congestizia
- infiamazione acute da agenti - infiamazione acute da agenti patogeni
- trombosi
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Linfedema primario si manifesta in combinazioni con diversi malattie
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Linfedema in
combina-combina-zione con obesità
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Linfedema in combinazione con insufficienza venosa + poliartroisi
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Elefantiasi
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Troppo per un fisioterapista: elefantiasi
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Terapia decongestionate
medico
fisioterapist
fisioterapista
paziente
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Cura e igiene cutanea
• Disinfettante ipoallergenico
• Pomata che non irriti la pelle
• Antistaminici• Antistaminici
• Antimicotici
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Terapia compressiva
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„effetto di massagio“ della compressione
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Terapia compressiva: riduzione della pressione!
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Effetto secondario della terapia
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Effetto secondario della terapia
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Regole generali del trattamento compressivo in linfologia
Bendaggio compressivo
� Nessun bendaggio rigido e permanente
I bendaggi compressivi � I bendaggi compressivi permettono:
- l‘ uso di materiale per imbottitura
- l‘ adattamento quotidiano alle alterazioni della circonferenza e della consistenza
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bambini
età: 2-6 anni: Classe I
età: 6-12 anni: Classe II
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Conclusione:
Terapia adeguata, controlli ogni anno
rendono sicuro una vità abastanza normale, rendono sicuro una vità abastanza normale,
educazione e professione dipendente alla
capacità.
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Conclusion:
Interventi chirurgici sono indicati solo nel
caso di malformazioni combinati come:
Shunt arterio-venosi, linfangioma, etc.
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Stadio Denominazione Sintomi Diagnosi
0
Stadio subclinico
di latenza
Latenza
Linfangiopatia con vasi linfatici sufficienti
∅∅∅∅Linfoscintigrafia
patologica
1 Stadio Reversibile Segno della Diagnostica di
Stadi clinici del linfedema
1 Stadio Reversibile Regredisce
spontaneamente
Segno della
Fovea
Diagnostica di base
2 Stadio Irreversibile Fenomeni sclerotici tissutali
Diagnostica di base
3 Stadio Elefantiasi L‘arto perde la sua normale morfologia
Diagnostica di base
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Therapeutical options:
1) CDP
2) Additive lympholgical surgery
3) Reconstructive surgery3) Reconstructive surgery
4) Laser therapy
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StadiStadi del del linfedemalinfedema
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Paziente , 50 anni
Dopo 3 settimane
Dopo 8 settimane
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Paziente i, età 56 anni
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Grazie per la Vostra gentile attenzione