Introduction. Carpal tunnel syndrome (CTS) has usually a different clinical presentation in children compared to adults. On top of this, its rarity in childhood makes it difficult to diagnose. It has also different etiologies at this age, the majority of cases occur in children with lysosomal storage diseases (mucopolysaccharidosis and mucolipidosis) which may be the first manifestation of this disease in some cases. Clinical case. In this article we describe 3 cases of children aged between 4 and 10 years, 2 with idiopathic CTS and 1 with primary familial origin. The symptoms of the former were intermittent paresthesias and handling difficulty, while the familial case presented with a more typical paresthesias and pain. The neurophysiological study confirmed the diagnosis. Concerning the patient with a history of CTS in the family, MRI showed a compression of the nerve due to the thickening of the flexor retinaculum. In all cases, lysosomal storage disease was ruled out and a conservative treatment was initiated. Evolution was favorable in the idiopathic cases, one of them with an almost complete remission of symptoms, while the patient with a familial CTS follows a progressive course and is waiting for the surgical assessment. Discussion. The CTS in pediatric age presents milder and more unspecific symptoms than in adults, and the results of the exploration and provocation tests are often unclear. Surgical treatment is almost always necessary, although some idiopathic cases, particularly if they are not associated to the thickening of the flexor retinaculum, may improve with a conservative treatment.
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