Carpal tunnel in children research from Belgium

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From Acta Orthop Belg. 2003 Oct; 69(5): 387-95.

Carpal tunnel syndrome in children.

Van Meir N, De Smet L.

University Hospital Pellenberg, Leuven, Belgium.

Carpal tunnel syndrome (CTS) is rarely seen in children. A literature

search in 1989 revealed 52 published cases. The authors review 163

additional cases that were published since that date. The majority of

these cases were related with a genetic condition. The most common

aetiology was lysosomal storage disease: mucopolysaccharidoses (MPS) in

95 and mucolipidoses (ML) in 22. In CTS secondary to MPS, clinical signs

typical of adult CTS are rarely seen, and difficulty with fine motor

tasks is the most frequent finding. CTS in MPS does not seem to be

prevented by bone marrow transplantation, the usual treatment for the

condition. CTS is probably due to a combination of excessive lysosomal

storage in the connective tissue of the flexor retinaculum and a

distorted anatomy because of underlying bone dysplasia. Mucolipidoses

come next in the aetiology, with essentially similar symptoms. The

authors found in the literature 11 cases of primary familial CTS, a

condition which presents as an inheritable disorder of connective tissue

mediated by an autosomal dominant gene; the symptoms may be more typical

in some cases, but are more similar to MPS in others. A case with

self-mutilation has been reported. Hereditary neuropathy with liability

to pressure palsies (HNPP) is a rare autosomal dominant condition

characterised by episodes of decreased sensation or palsies after slight

traction or pressure on peripheral nerves; it may also give symptoms of

CTS.

Schwartz-Jampel syndrome (SJS), another genetic disorder with autosomal

recessive skeletal dysplasia, is characterised by varying degrees of

myotonia and chondrodysplasia; it has also been noted associated with

CTS in a child. Melorrheostosis and Leri's syndrome have also been noted

in children with CTS, as well as Dejerine-Sottas syndrome and

Weill-Marchesani syndrome. Among non-genetic causes

of CTS in children, idiopathic cases with children onset have been

reported, usually but not always related with thickening of the

transverse carpal ligament. Intensive sports practice has been reported

as an aetiological factor in several cases of childhood CTS. Nerve

territory oriented macrodactily, a benign localised form of gigantism,

is another

unusual cause of CTS in children, as are fibrolipomas of the median

nerve or intraneural perineuroma or haemangioma of the median nerve.

Acute cases have been reported in children with haemophilia, secondary

to local bleeding. Another local cause is a musculotendinous

malformation of the palmaris longus, the flexor digitorum superficialis,

the flexor carpi radialis brevis (a supernumerary muscle), the first

lumbricalis or the palmaris brevis. Isolated cases of childhood CTS have

also been reported in Klippel-Trenaunay syndrome, in Poland's syndrome

and in scleroderma. Finally, several cases have been noted following

Trauma, most often related with epiphysiolysis of the distal radius.

Immediate reduction has cleared the problem in most cases, but

exploration of the median nerve should be considered otherwise, and also

in cases with delayed occurrence of symptoms. Overall 145 of the 163

reviewed cases have undergone open carpal tunnel release. Childhood CTS

often has an unusual presentation, with modest complaints and children

are often too young to communicate their problem. In CTS with specific

aetiologies such as storage disease, the symptoms may be masked by the

skeletal dysplasia and joint stiffness. Every child with even mild

symptoms must be thoroughly examined and a family history must be taken.

Children with storage disease may benefit from early clinical and

electrophysiological screening before they develop obvious clinical

signs.