Hunterʼs Syndrome as a Cause of Childhood Carpal Tunnel Syndrome

Norman-Taylor, Fabian; Fixsen, J. A.; Chir, M.; Sharrard, W. J. W.

doi:10.1097/01202412-199504010-00018

Cited by 23 publications

(13 citation statements)

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“…Norman-Taylor et al 10 reported definite symptoms in three children with MPS II. Subtle clinical changes include alterations in grasp or playing pattern, increasing difficulty with fine motor tasks and manual clumsiness.…”

Section: Discussionmentioning

confidence: 99%

“…[13][14][15][16][17] The relationship to the mucopolysaccharidoses is recognised and it has been reported in MPS I, MPS II, MPS VI, ML II and ML III. [1][2][3][4][5][6][7][8][9][10] The carpal tunnel syndrome may be the first presenting feature of MPS or ML; 8,9 this was true of one of our patients with ML III. In 1974, Fisher, Horner and Wood 18 described dysfunction of the upper limbs in children with mucopolysaccharide disorders.…”

Section: Discussionmentioning

confidence: 99%

“…Progressive disability of the upper limbs is recognised in both MPS and ML, and the carpal tunnel syndrome has been reported in association with MPS I, MPS II, MPS VI, ML II and ML III. [1][2][3][4][5][6][7][8][9][10] Many authors have advised decompression, but there are few objective data on the precise cause, natural history, neurophysiology and functional outcome after operation in these children. We have reviewed 48 children with mucopolysaccharidoses and mucolipidoses and have developed a standard system for assessment, operation and postoperative review.…”

mentioning

confidence: 99%

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Carpal Tunnel Syndrome in the Mucopolysaccharidoses and Mucolipidoses

Haddad

Jones

Vellodi

et al. 1997

The Journal of Bone and Joint Surgery. British volume

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Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium.Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement.We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Carpal Tunnel Syndrome in the Mucopolysaccharidoses and Mucolipidoses

Haddad

Jones

Vellodi

et al. 1997

The Journal of Bone and Joint Surgery. British volume

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“…15 Radiographic examination reveals abnormal thickness of all bones, and irregular epiphyseal ossification in the joints of the hand, shoulder, and elbow. 15 The hands are reported to take on a claw-like appearance, 42 and, in combination with carpal tunnel syndrome, 45 loss of hand function can result. 46 The ribs are thickened and have an unusual shape (Fig 3), and clavicles can be increased in bulk.…”

Section: General Appearance and Skeletal Abnormalitiesmentioning

confidence: 97%

“…However, in children with MPS disorders, it is common and underrecognized. 45 In 1 study of 7 consecutive patients with Hunter syndrome, all patients Ͼ2 years of age had signs of carpal tunnel compression with rapid progression of clinical signs and symptoms. 71 Tunnel decompression surgery results in clinical improvement in most patients.…”

Section: Neurologic Involvementmentioning

confidence: 99%

Recognition and Diagnosis of Mucopolysaccharidosis II (Hunter Syndrome)

Beck²,

et al. 2008

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Mucopolysaccharidosis II, also known as Hunter syndrome, is a rare, X-linked disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase, which catalyzes a step in the catabolism of glycosaminoglycans. In patients with mucopolysaccharidosis II, glycosaminoglycans accumulate within tissues and organs, contributing to the signs and symptoms of the disease. Mucopolysaccharidosis II affects multiple organs and physiologic systems and has a variable age of onset and variable rate of progression. Common presenting features include excess urinary glycosaminoglycan excretion, facial dysmorphism, organomegaly, joint stiffness and contractures, pulmonary dysfunction, myocardial enlargement and valvular dysfunction, and neurologic involvement. In patients with neurologic involvement, intelligence is impaired, and death usually occurs in the second decade of life, whereas those patients with minimal or no neurologic involvement may survive into adulthood with normal intellectual development. Enzyme replacement therapy has emerged as a new treatment for mucopolysaccharidosis disorders, including Hunter syndrome. The purpose of this report is to provide a concise review of mucopolysaccharidosis II for practitioners with the hope that such information will help identify affected boys earlier in the course of their disease.

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