Details

Autor(en) / Beteiligte

• Saltzman, Charles L
• Herzenberg, John E
• Phillips, William A
• Hensinger, Robert N
• Hopwood, Nancy J

Titel

Thick Lips, Bumpy Tongue, and Slipped Capital Femoral Epiphysis—A Deadly Combination

Ist Teil von

• Journal of pediatric orthopaedics, 1988-03, Vol.8 (2), p.219-222

Ort / Verlag

Hagerstown, MD: Lippincott-Raven Publishers

Erscheinungsjahr

1988

Quelle

MEDLINE

Beschreibungen/Notizen

• Multiple endocrine neoplasia type 2b (MEN 2b) is a rare genetic disorder. Affected individuals have malignant thyroid tumors, pheochromocytoma, and ganglioneuromatosis. The musculoskeletal manifestations of MEN 2b include a Marfanoid habitus, pes cavus, scoliosis, slipped capital femoral epiphysis, joint laxity, poor muscle development, and delayed maturation. The initial clinical presentation of MEN 2b frequently involves the musculoskeletal system. The characteristic ganglioneuromatosis of the lips and tongue, however, should alert the orthopedic surgeon to the underlying disorder. Effective treatment of the malignant neoplasms hinges on early diagnosis. The risk of perioperative hypertensive crisis is significant; it may be prevented by appropriate treatment of the pheochromocytoma.