| 제목 |
UNUSUAL PRESENTATION OF CHIARI TYPE 1 MALFORMATION WITH SYRINGOMYELIA |
| 소속 |
Korea University Ansan Hospital, Department of Rehabilitation Medicine1 |
| 저자 |
Jin Young Im1*, Seok Jun Lee1, Ki Hoon Kim1, Byung Kyu Park1, Dong Hwee Kim1† |
Introduction: A Chiari malformation is a congenital deformity of the brain stem and cerebellum that are characterized by caudal displacement of the hindbrain with or without meningomyelocele. Chiari type 1 malformation is characterized by cerebellar tonsils below the foramen magnum. The clinical manifestations of Chiari malformation are variable. A Chiari type 1 patient may be asymptomatic for a long time. This type has also been called the adult type due to the rarity of clinical manifestation in children. The most common symptoms are pain, weakness, numbness. In this case report we describe a child with unusual presentation of Chiari type 1 malformation before and after surgical management.
Case Report: A 4-year-old girl with right shoulder elevation noticed by the mother was visited. The spine x-ray showed thoraco-lumbar scoliosis. Conservative management including spinal orthosis and physical therapy was done. After 1 year, her mother observed that the right hand was larger than the left (Fig. 1A & B; Table 1). Whole spine magnetic resonance imaging (MRI) study showed Chiari malformation type 1 with severe syringomyelia of whole spinal cord, especially cervical cord (Fig. 2). Resection of cerebellar vermis was performed. After surgery, the size of the right hand was not changed, and loss of pain and itching senses, the delay of wound healing, and skin dryness on the right hand (Fig. 1C) were developed. Muscle strength of both upper and lower extremities was normal. Sensation to pinprick and temperature on the right side but the proprioception was normal, bilaterally. Follow-up MRI study demonstrated that the size of syringomyelia was much improved (Fig. 2C).
Conclusion: Localized hypertrophy of a hand (chiromegaly) is a rare but pathognonomic trophic disturbance in syringomyelia, which might be related with sympathetic dysfunction. Chiromegaly might be the presenting manifestation of Chiari type 1 malformation with syringomyelia.
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Morphometric data before surgery
Mild hypertrophy of right hand before sugery (A and B) and trophic change and delay of wound healing on right hand after surgery (C)
Fig. 2. T2-weighted sagittal magnetic resonance image (MRI) showed a downward displacement of the cerebellar tonsils through the foramen magnum (A). T2-weighted sagittal MRI of whole spine demonstrated large syrinx along the entire length of the spinal co
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