[Generalized lymphatic dysplasia in a 6-month-old nursing infant].
G.L.D. is a rare syndrome characterized by chylothorax, chylous ascites and lymphedema associated to minor symptoms following from lymphangiectasis or to lymphangiomatosis. This syndrome is caused by congenital dysplasia of lymphatic vessels and has, generally, an extremely severe prognosis. In the present papers the authors describe one case of G.L.D. in a six months old infant with chylous ascites, chylothorax, lymphedema, hemangioma and a history of fetal ascites. The preservative (dietetic and evacuative) therapy was not resolutive, therefore an exploratory laparotomy was necessary, but no surgical solution was possible because of the absence of chylous vessels in the anatomical region of the principal lymphatic route. However, laparatomy led to a progressive recovery of ascites and chylothorax, probably due to thrombosis of dysplasic lymph vessels and to neoformation of collateral circulation.
Massimo
L
L
I Divisione di Pediatria, Ospedale Santobono, USL 40 Napoli, Italia.
Di Iorio
G
G
Martone
A
A
Sottile
R
R
Retolatto
A
A
Loiodice
F
F
Campa
A
A
Angelone
S
S
D002916:Chylothorax / Q000175:diagnosis / Q000628:therapy; D002915:Chylous Ascites / Q000175:diagnosis / Q000628:therapy; D003131:Combined Modality Therapy / Q000379:methods; D006801:Humans; D007223:Infant; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000628:therapy; D008209:Lymphedema / Q000175:diagnosis / Q000628:therapy; D008297:Male; D013577:Syndrome
Displasia linfatica generalizzata in un lattante di sei mesi.