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Review Question - QID 533

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QID 533 (Type "533" in App Search)
Children with lysosomal storage diseases have increased rates of which of the following disorders?

Syringomyelia

16%

397/2525

Pectus excavatum

5%

123/2525

Macrognathism

11%

279/2525

Carpal tunnel syndrome

58%

1460/2525

Thoracic diastomyelia

10%

250/2525

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Children with lysosomal storage diseases have increased rates of carpal tunnel syndrome. Named lysosomal storage syndromes include: Hurler, Hunter, Schele, and Pseudo-Hurler. These patients have increased tissue nodularity, girth, and thickened carpal ligaments. Symptoms include paresthesias, nocturnal symptoms, and clumsiness of the hands. Operative release of the transverse carpal ligament is the treatment of choice. The reviews by Lamberti and MacDougal describe the pathophysiology of CTS in children with and without lysosomal storage disease, and inaccuracy of common clinical tests like Phalen's and Tinel's testing for the diagnosis in children. The treatment is the same, regardless of the pathophysiology: surgical release of the transverse carpal ligament.

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