Usefulness of lymphoscintigraphy in demonstrating lymphedema in patients with Noonan syndrome
Journal
Clinical Nuclear Medicine
Journal Volume
33
Journal Issue
3
Pages
226-227
Date Issued
2008
Author(s)
Abstract
Lymphatic dysplasia/hypoplasia is found in 15% to 20% of patients with Noonan syndrome. We report a 16-year-old boy with a classic phenotype of Noonan syndrome but a normal karyotype in chromosomal study. During the last 5 years, he had progressive bilateral lower limb edema. The microfilaria study, duplex sonography, and MRI of the lower extremities were all unremarkable. But lymphoscintigraphy showed stocking-like dermal backflow in both legs, delayed lymphatic flow to the inguinal nodes, and dilated lymphatic channels in the abdomen and thorax. These findings suggest that lymphoscintigraphy may be useful in providing vital information on the lymphatic drainage for patients with Noonan syndrome. ? 2008 Lippincott Williams & Wilkins, Inc.
SDGs
Other Subjects
technetium sulfur colloid tc 99m; adolescent; article; case report; chromosome; congenital heart disease; disease duration; echography; face dysmorphia; human; inguinal lymph node; karyotype; leg; lymphadenopathy; lymphangiectasis; lymphedema; lymphoscintigraphy; male; Microfilaria; Noonan syndrome; nuclear magnetic resonance imaging; peripheral edema; phenotype; short stature; thorax disease; Adolescent; Humans; Lymphedema; Male; Noonan Syndrome
Type
journal article