Internal Medicine

0521779407-15 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:43

1046 Neurofibromatosis

➣Do occur in other genetic diseases

Other Conditions with CALMs

McCune-Albright’s syndrome

Polyostotic fibrous dysplasia

Russell-Silver

Bloom

Noonan

Watson

LEOPARD/multiple lentigines

Sotos

Proteus

management

N/A

specific therapy

n/a

follow-up

■Check child’s blood pressure regularly.

■Evaluate neurodevelopmental progress – e.g., learning disabilities.

■Growth velocity increase or decrease may indicate intracranial pro-

cess.

■Evaluate for optic pathway tumors, esp. if precocious puberty and

tall stature.

■Evaluate for skeletal changes (scoliosis).

■Support group (National NF Foundation)

complications and prognosis

Complications

■Response to nondepolarizing anesthetics

➣Old data that are incorrect

➣Recent data show no problem

■Psychological

➣Stigma of the “Elephant Man”

➣Proteus syndrome, not NF-1

■Malignancy

➣Neurofibrosarcomas

5–10% degeneration rate

➣Focal neurologic signs

➣Rapid growth of plexiform NF

➣Persistent pain

➣Host of other neuroid malignancies reported