RT Journal Article
T1 Microcystic Lymphatic Malformation Successfully Treated With Topical Rapamycin
A1 Garcia-Montero, Pablo
A1 del Boz, Javier
A1 Sanchez-Martinez, Miguel
A1 Escudero Santos, Isabel Maria
A1 Baselga, Eulalia
K1 Pulsed dye-laser
K1 Tuberous-sclerosis
K1 Double-blind
K1 Sirolimus
K1 Lymphangiomatosis
K1 Management
K1 Lymphangioleiomyomatosis
K1 Angiofibromas
K1 Trial
AB Microcystic lymphatic malformations (MLM) are low-flow vascular malformations composed of multiple small cysts. MLM usually affect deep lying structures, which makes their treatment even more difficult and complex. A novel and interesting treatment is rapamycin, a mammalian target of rapamycin inhibitor that when orally administrated has offered favorable results. However, until recently, topical rapamycin had not been used in the treatment of MLM. Case 1 is a girl aged 13 years with extensive MLM affecting the muscles in the right buttock. The patient had received frequent cycles of cryotherapy, but they had failed to control the associated symptoms. In the previous 12 months, the patient had reported greater discomfort, swelling, exudate, and superinfection of the affected region. Because no specific treatment has yet been approved for MLM, and as a step before the use of aggressive systemic or intralesional treatments, it was decided to initiate treatment with 1% rapamycin ointment. After 4 months of treatment, the patient presented a marked improvement, with a significant reduction of associated complications and no major side effects. Case 2 is a boy aged 5 years who underwent surgery for an intergluteal lipoblastoma at 3 weeks of life and developed a MLM on the scar 6 months afterward. The lesion showed slow growth and continuous exudation with frequent episodes of superinfection. Treatments with laser multiplex and intralesionalbleomycin were performed unsuccessfully. In the previous 4 months, the patient had been treated with 1% rapamycin ointment with significant improvement and no side effects.
PB Amer acad pediatrics
SN 0031-4005
YR 2017
FD 2017-05-01
LK http://hdl.handle.net/10668/19188
UL http://hdl.handle.net/10668/19188
LA en
DS RISalud
RD Apr 6, 2025