Article
Carpal tunnel syndrom in children suffering from Mucopolysacharidosis
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Authors
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Gerlinde Weigel
- Orthopädisches Spital Speising, 1. Abteilung, Team Rekonstr. Nerven-/ Hand-/ Mikrochirurgie, Wien, Österreich
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Werner Girsch
- Orthopädisches Spital Speising, 1. Abteilung, Team Rekonstr. Nerven-/ Hand-/ Mikrochirurgie, Wien, Österreich
| Published: | August 16, 2017 |
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Outline
Text
Carpal tunnel syndrome (CTS) is rarely seen in children. In CTS secondary to Mucopolysaccharidosis (MPS), typical clinical CTS signs seen in adults are atypical or minimal or absent, especially in those children with intellectual deficiencies secondary to MPS. If untreated, carpal tunnel syndrome can cause significant, permanent deficiencies in hand function.
We present findings in 4 children (mean age at surgery 10 years) suffering from MPS (3x MPS II, 1x MPS I) and suspected median neuropathies at wrist and carpal tunnel level.
They underwent motor and sensory nerve conduction studies of bilateral median and ulnar nerves together with 18mHz ultrasound studies for these nerves. In 1 child the diagnistic had to be done under general anaethesia due to lacking compliance.
In 3 children we performed a Semmes Weinstein monofilament exam additionally.
All diagnostic tools together with the clinical situation lead to the diagnosis of severe carpal tunnel syndrome.
All cases underwent wide open carpal tunnel release in both hands with resection of all connective tissue adjacent to nerve and flexor tendons and epineuriotomia of the median nerve. In one child suffering from loss of opposition of the thumb we perfomed an opponensplasty by FDS IV transfer.
Despite the severe signs of compression of the nerves during surgery all nerves recovered, proofed clinically and by Semmes Weinstein testing. Neurolysis and FDS IV opponensplasty effectively normalized hand function for ADL activities in all children.
CTS in children suffering from MPS often shows an unusual presentation, with modest or no complaints, as the children are often too young to communicate their problem.
Every child with even mild symptoms and changes in use of their hand must be thoroughly examined and a family history must be taken. Children with storage diseases will benefit from early clinical and electrophysiological screening before they develop obvious clinical signs.
We recommend screening for median neuropathies at the wrist from early age (3 years) for children with MPS, particularly types I, II and IV, regardless of whether they are symptomatic or asymptomatic of carpal tunnel syndrome.
