Meetings
- 10/31/2009
Gainesville, FL 9:00AM ~ 1:00PM
News
SUGGESTED SCREENING* GUIDELINES FOR Von Hippel-Lindau Syndrome (VHL)
(*Clinical evaluations of individuals at risk for VHL. Also see: Lonser RR, Glenn GM, Walther ML, et al: The Lancet Vol 361, June 14, 2003; and Choyke, Glenn, Walther, et al: Radiology. 194:629-642, 1995.)
Any Age: Families are informed they may choose a Genetic Counselor if they wish testing for a mutation causing VHL in their family members, e.g., by contacting the Molecular Genetics Laboratory, Children’s Hospital of Philadelphia, Pennsylvania, or another CLIA approved Genetic Testing Laboratory.
Before any type of surgery or childbirth labor or delivery, rule out pheochromocytoma, a tumor most often arising in adrenal glands
From Conception: Inform Obstetrician of VHL and need to rule out pheochromocytoma
From Birth: Inform Pediatrician of family history of VHL. Eye exams yearly by Pediatric Ophthalmologist informed about VHL
Ages 2-8: Yearly: Eye/retinal examination by Ophthal-mologist informed about VHL. Physical with neurologic examination, and blood pressure measurements by Pediatrician informed about VHL. Measure plasma or 24-hour urinary catecholamines & metanephrines If clinically indicated, abdominal ultrasound or MRI, or craniospinal axis MRI.
Ages 8-18: Every 6 months: Eye/retinal examination by Ophthalmologist informed about VHL. Yearly: Physical with neurologic examination, blood pressure measurements and review of systems by Physician informed about VHL Measure plasma or 24-hour urinary catecholamines & metanephrines Ultrasound of abdomen (kidneys, pancreas, adrenals); if indicated abdominal MRI.
From Age 11: MRI of craniospinal axis pre- and post-contrast
Age 18 & older: Yearly: Eye/retinal examination by Ophthalmologist informed about VHL. Physical with neurologic examination, blood pressure measurements and review of systems by Physician informed about VHL. CT of abdomen & pelvis (kidneys/pancreas/adrenals) pre- & post-i.v. contrast. Measure plasma or 24-hour urinary catecholamines & metanephrines MRI of craniospinal axis pre- and post-contrast
NOTE:
- Slight modifications of screening schedules may sometimes by made by personal physicians familiar with individual patients and with their VHL family history.
- After age 60 for individual at risk for VHL who have no tumors to be monitored, imaging may be every two years for CT and every 3 years for MRI.
- All head/brain MRIs should be examined by Neuroradiologist for any suggestion of ELST (endolymphatic sac tumor). If suspicious, or at first sign or symptom of hearing loss, tinnitus or vertigo, MRI and CT of internal auditory canal and audiometry are indicated.