Langerhans Cell Histiocytosis Causing Cervical Myelopathy in a Child

Abstract

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.

Primary Eosinophilic Granuloma of Adult Cervical Spine Presenting as a Radiculomyelopathy

“…This phenomenon is very rare in EG and it needs surgical management. Surgery should be performed for neurological deficits or severe mechanical pain2,9,15,20,21). As stated above, most of the reported cases underwent non-surgical treatment (close observation, immobilization) also have good results.…”

Thoracic spine Langerhans cell histiocytosis in a child with achondroplasia

“…Clinical and radiological features along with treatment algorithms of symptomatic spinal cord compression in paediatric LCH cases have been previously described in the literature 5–7…”

Paediatric spinal Langerhans cell histiocytosis requiring corpectomy and fusion at C7 and at Th8–Th9 levels

“…The differential diagnosis should include aneurysmal bone cyst, osteogenesis imperfecta, Gaucher's disease and neoplastic lesions 2 4–6. Some radiological patterns could assist the diagnosis of spinal LCH (symmetrical vertebral body collapse, maintenance of the disc spaces, absence of soft tissue masses1 6 7) but isolate spinal LCH may represent a real diagnostic challenge 4 8…”

“…Presently, radiation therapy is rarely advocated6 and chemotherapy is reserved for disseminated or aggressive diseases 4 6. Surgical treatment should be considered just in the uncommon cases of spinal cord compression2 4 5 8 9 asymmetric vertebral body breakdown with kyphosis or scoliosis3 6 8 12 and/or vertebral instability 7…”

“…However, in our experience,13 14 the modern versatile expandable cages and the new specifically designed plates usually permits optimal vertebral realignment and kyphosis correction. In general, in paediatric cases, autografts should be preferred3 8 10 but, if the patient is not too young, fusion/fixation at one/two levels may be easily tolerated, and metal instruments can be harmlessly and successfully used 2 3…”