1 / 27

Cauza rara de noduli pulmonari multipli

Cauza rara de noduli pulmonari multipli. Andreea Plesita Institutul Pneumologie Marius Nasta Bucuresti. DATE GENERALE. Varsta 44 ani Sex: feminin Nefumatoare Mediu urban Fara expunere profesionala la noxe respiratorii. Motivele internarii.

moya
Download Presentation

Cauza rara de noduli pulmonari multipli

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Cauzarara de nodulipulmonarimultipli Andreea Plesita Institutul Pneumologie Marius Nasta Bucuresti

  2. DATE GENERALE • Varsta 44 ani • Sex: feminin • Nefumatoare • Mediu urban • Fara expunere profesionala la noxe respiratorii

  3. Motiveleinternarii • Investigarea etiologiei unui nodul pulmonar stg (Rx) • Durere toracica latero-bazala stanga cca 2 saptamani

  4. istoric • APP: • Dislipidemie mixta • Leziune ischemica cerebeloasa dreapta si leziuni degenerative periventriculare (RMN 2010) • Fibromatoza uterina (2009) • Chist ovarian drept (2007) • Mastoza fibrochistica (2005) • Poliglobulie de cauza incerta • Boala Rendu – Osler (2003 ) • AHC : tatal boala Rendu – Osler

  5. Examen clinic • Telangiectazii marginea limbii bilateral, fara sangerare spontana / la atingere • San: formatiuni nodulare , cadran supero-extern stang , fara adenopatii periferice • Sa O2= 98% in AA - clinostatism, ortostatism • Restul examenului obiectiv - limite normale

  6. Investigatii - biologic • Poliglobulie ( Hb=16,9 g/dl, E=5 500 000/mm3, Ht=50,9%) • Proteina C reactiva > 6 • Functie renala, hepatica in limite normale

  7. Rx ToRACICA

  8. INVESTIGATII • EKG: RS, AV=70/min, ax QRS = +60.fara modificari de repolarizare • Spirometrie: valori normale • Test de mers 6 min: fara desaturare la efort (SaO2 =98% initial, Sa O2=97% final)

  9. investigatii • Fibrobronhoscopia: fara modificari notabile in zonele explorabile, inclusiv in subsegmentarele de ordinul I ale lingulei. • LBA – lingula ,100 ml SF. • LBA: - citologie diferentiala normala - fara celule tumorale - coloratie Ziehl- Neelsen negativa.

  10. Diagnostic diferential • Neoplasm primitiv pulmonar • Metastaza de la un neoplasm extratoracic • Tumora pulmonara benigna • Tuberculom • Malformatie vasculara in cadrul bolii Rendu-Osler • Chist hidatic

  11. CT torace

  12. diagnostic • Malformatii arterio venoase in cadrul bolii Rendu Osler • Poliglobulie in observatie etiologica • Dislipidemie mixta • Mastoza fibrochistica • Chist ovarian drept • Fibromatoza uterina

  13. Tratament • Embolizare: DE URGENTA : hemoptizie • Embolizare: MAV ≥ 3 mm • Rezultateexcelente (93%) • Profilaxieantibiotica • ↓ risculabcesului cerebral • Complicatii rare: - emboliegazoasa (5%) - pleurezie (30%) - perforatiaMAVp - AIT, infarct cerebral • Rezectietoracoscopica, pneumectomie

  14. EVOLUTIE,prognostic,COMPLICATII • Prognostic favorabil : MAV – screening, fara complicatii • Complicatii: - hemoptizii, hemotorax, HTP - abces cerebral, AVC - embolie septica • Mortalitate ↑ in lipsa tratamentului, in caz de complicatii

  15. Screening Pulmonar: - pulsoximetrie - Ecocardiografie substanta de contrast - angio CT SNC: - RMN

  16. BoalaRendu-Osler-Weber • SAU: Telangiectazie hemoragica ereditara • Transmitere autosomal dominanta • Cauza majora : MAV la nivel pulmonar, cerebral, gastrointestinal, hepatic + hemoragii. • Incidenta: 1-2 cazuri/100.000 locuitori • Prevalenta 1-2/10 000

  17. PATOGENIE

  18. Fiziopatologie • Vascularizatie anormala: telangiectazii, MAV, anevrisme • Defect genetic: ENG – TEH tip I receptori ALK1- TEH tip II TGF beta • SMAD 4 • 1-2% TEH • 10% TEH suspecta, teste genetice negative

  19. Manifestariclinice I • Mucoasa nazala: • Epistaxis (90%) • Tract GI: • Hemoragia GI recurenta - nedureroasa / dureri abdominale • Pulmonar: • Platipnee, ortodeoxie • Sunt dreapta-stanga : cianoza, hipoxemie, policitemie secundara • Embolie septica • Hemoptizie • Migrene 15-30% (cauza neclara)

  20. Manifestariclinice II • SNC: • cefalee, convulsii, paraplegie, paralizie • AVC, abcese cerebrale • Hepatic: • Fibroza hepatica,insuficienta hepatica • Ocular: - tulburari de vedere - lacrimi hemoragice

  21. Diagnostic pozitiv • Epistaxis spontan / recurent • Telangiectazii cutaneomucose: buze, cavitate orala, degete, nazal • MAV viscerale: pulmonar, cerebral, hepatic, spinal, gastrointestinal • Istoric familial pozitiv Diagnostic: Certitudine: 3-4 criterii Posibil/suspect: 2 citerii Improbabil :< 2 criterii • Teste genetice pentru ENG si ALK1 • SMAD4

  22. Diganosticdiferential • Boala Von Willebrand • CREST • Ataxie telangiectazie • Telangiectazie ereditara benigna: telangiectazii diseminate (fata, gat, membre superioare, trunchi) • Dermatomiozita • Sclerodermie • Boli hepatice cronice: stelute vasculare pe fata torace, periombilical

  23. Tratament • Epistaxis: tamponament nazal, terapie cu laser, cauterizare electrica/chimica in sedinte repetate; termoplastie septala; embolizare. • Telangiectazii tegumentare : terapie laser • MAV GI: tansfuzii de sange, tratamente endoscopice, fotocoagulare, rezectie chirurgicala • MAV cerebrale /pulmonare: embolizare • MAV hepatice: transplant hepatic

  24. DISCUTII • Criterii de diag. poz .de certitudine • MAV pulmonara depistata tardiv • MAV p – la nivel LS, LI • Fara platipnee/ortodeoxie • Poliglobulie (?)

  25. CONCLUZII • In diagnosticul diferential al unui nodul pulmonar solitar trebuie avuta in vedere si aceasta entitate rara MAV p In cazul pacientilor cu boala Rendu Osler cunoscuta aceasta asociere este notorie, insa la pacientii la care boala nu e inca diagnosticata ( manifestari fruste, istoric familial insuficient explorat), aceasta etiologie a nodulului este mult mai dificil de avut in vedere in diagnosticul diferential.

  26. Vamultumesc!

More Related