J Korean Child Neurol Soc.  2017 Sep;25(3):187-190. 10.26815/jkcns.2017.25.3.187.

Idiopathic Cervicosyringomyelia in 2 year-old Boy

• Cho YH
• Kim H
• Lim BC
• Hwang H
• Chae JH
• Kim KJ
• Choi JE
• Shin SM

• KMID: 2392558
• DOI: http://doi.org/10.26815/jkcns.2017.25.3.187

Abstract

Syringomyelia is a disorder in which a cavity has formed within the spinal cord. Idiopathic syringomyelia is not associated with identifiable causes such as Chari type 1 malformation, spinal cord tumor, vascular malformation, tethered cord, arachnoiditis, hydrocephalus, or previous spinal surgery. The main neurologic symptoms of idiopathic syringomyelia are toe-walking, constipation, incontinence, abnormal reflexes, and lower extremity weakness. Patients may present with various symptoms such as scoliosis, cutaneous markers, pain in the lower extremities or back, or may be asymptomatic. Herein, we report a young child with idiopathic syringomyelia presenting with subtle neck pain. A 23-month-old boy visited the neurologic clinic after 3 months of right occipital area neck pain. He had no history of trauma or central nervous system infection, and neurologic examination results were normal except for right posterior neck hyperesthesia. Brain and spinal magnetic resonance imaging showed an ovoid intramedullary cystic lesion (9.7×5.0×4.7 mm) at C6/7 of the spinal cord. There was no evidence of Chiari malformation or other lesions that can be primary pathologies of syringomyelia. Electromyogram/nerve conduction velocity results were normal. The subject was diagnosed as idiopathic syringomyelia. His symptoms and neurologic/radiologic indications showed no change at a 1-year follow-up. Idiopathic syringomyelia symptoms are varied and may be overlooked by physicians. Pediatricians may consider syringomyelia if patients complain about persistent sensory abnormality. All patients who present with syringomyelia should undergo detailed neuroimaging of the entire neuraxis to elucidate the proximate cause of the lesion.

Keyword

Syringomyelia

MeSH Terms

Arachnoid
Arachnoiditis
Brain
Central Nervous System Infections
Child
Constipation
Follow-Up Studies
Humans
Hydrocephalus
Hyperesthesia
Infant
Lower Extremity
Magnetic Resonance Imaging
Male*
Neck
Neck Pain
Neuroimaging
Neurologic Examination
Neurologic Manifestations
Pathology
Reflex, Abnormal
Scoliosis
Spinal Cord
Spinal Cord Neoplasms
Syringomyelia
Vascular Malformations