Pediatric median mononeuropathies: A clinical and electromyographic study

Deymeer, Feza; Hr, Jones

doi:10.1002/mus.880170709

Cited by 41 publications

(19 citation statements)

References 45 publications

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“…Occasional idiopathic or familial cases have been reported, but the usual causes are anomalous anatomy, trauma, repetitive activity, bleeding disorders, and, most frequently, connective-tissue disorders. [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.…”

Section: Discussionmentioning

confidence: 99%

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%

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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“…A proximal PMM was identified in 10 children, including 8 with trauma, 1 with an osteoid osteoma and 1 with juvenile cutaneous mucinosis. Five children (3 with CTS and 1 each with mucolipidosis III and juvenile cutaneous mucinosis) had bilateral disease [19]. In our case, no other disease was present except type 1 diabetes mellitus.…”

Section: Discussionmentioning

confidence: 86%

“…Musharbash [18]reported a 28-month-old male child, known to have Maroteaux-Lamy Syndrome (mucopolysaccharidosis type VI), who presented with CTS. Deymer and Jones [19]reported 17 children, 6 girls and 11 boys, aged 5–17 years with pediatric median mononeuropathies (PMMs). EMG documented the PMM to be at the wrist in 7 children, including 3 children with idiopathic CTS, 1 whose symptoms were accentuated by skiing and 2 with a systemic illness (mucolipidosis III and scleroderma), and in 1 child each, the distal PMM was secondary to a cast or laceration.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Carpal Tunnel Syndrome with Type 1 Diabetes mellitus in Childhood

Kayali¹,

Kahraman²,

Şirin³

et al. 2003

Pediatr Neurosurg

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The differences between diabetic mono- or polyneuropathy and entrapment neuropathy are most important with respect to choosing treatment alternatives in pediatric patients. A 7-year-old girl with type 1 diabetes mellitus was admitted to our clinic with a complaint of bilateral weakness in her hands. Her clinical findings and electromyography study revealed an entrapment neuropathy of the median nerve at the wrist. She underwent operation by open carpal tunnel release. All symptoms resolved within 6 months after the operation. Carpal tunnel syndrome, especially bilateral, is very rare in childhood and it can be treated surgically.

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“…CTS is a common condition in adult life occurring principally in middleaged women, but is rarely seen during the growth period [6]: only six cases of idiopathic CTS have been reported in prepubertal patients [2,7]. In childhood, most cases of CTS occur in mucopolysaccharidoses and related disorders [9], but may occasionally also be associated with trauma, sports, or strenuous use of the hands, for instance in young carpet weavers [2,8].…”

Section: Discussionmentioning

confidence: 99%