Abstract
Introduction: Carpal tunnel syndrome is frequent in children with mucopolysaccharidosis. Diagnosis is difficult according to the slow progression of compression of the medial nerve and treatment is controversial.
Material & Methods: Twelve children were studied: 8 Hurler’s, 2 Hunter’s and 2 Maroteaux-Lamy’s diseases. All had clinical and EMG evaluation. Eight of them were operated, both sides.
Results: All the children had progressive and severe hypoesthesia before surgery. Nerve conduction velocity was very slow compared to normal values. After the open surgical release (16 cases) and synovectomy of flexor tendons (13 of 16 cases), all the patients were improved. The histology of the synovitis showed less glycosaminoglycans in patients who had a bone marrow transplantation. Surgical treatment must be an open release of the anterior ligament associated with a synovectomy of flexor tendons and a ventral epineurotomy
Discussion: According to the literature, carpal tunnel syndrome is observed in two third of patients of type I, II and VI mucopolysaccharidosis. Diagnosis is often difficult when cervical compression of the spinal cord is an associated factor. The diagnosis is made with clinical and EMG evaluation. Treatment must be early. MRI is an alternative to evaluate the morphology of the nerve: its compression below the carpal ligament and its bulky aspect just proximal to the carpal tunnel are clearly shown.
The abstracts were prepared by Editorial Secretary, Mr Robin W Paton. Correspondence should be addressed to BSCOS at the Royal College of Surgeons, 35– 43 Lincoln’s Inn Fields, London WC2A 3PN
