C hiari malformation, sometimes also referred to as hindbrain descent or cerebellar ectopia, reportedly was first surgically treated by van Houweninge Graftdijk (1932) 35 and Penfield and Coburn (1938). 30 The condition is being diagnosed more frequently in adult patients due to the widespread use of MRI, particularly in the management of patients with headache. The association of syringomyelia with cerebellar tonsillar descent was proposed by Lichtenstein in 1949. 22 Surgical therapy for these entities has evolved over the years, but there are
Ulrich Batzdorf, David L. McArthur and John R. Bentson
Langston T. Holly and Ulrich Batzdorf
Intradural arachnoid cysts are relatively uncommon pouches of cerebrospinal fluid (CSF) found within the subarachnoid space. The authors present a series of eight symptomatic patients in whom syrinx cavities were associated with arachnoid cysts, and they discuss treatment strategies for this entity.
The population comprised eight men whose mean age was 50 years (range 35–81 years). All patients experienced gait difficulty, and it was the chief complaint in seven; arm pain was the primary complaint in one. No patient had a history of spinal trauma, meningitis, or previous spinal surgery at the level of the syrinx cavity or arachnoid cyst. In each patient imaging revealed a syrinx cavity affecting two to 10 vertebral levels. Posterior thoracic arachnoid cysts were found in proximity to the syrinx cavity in each case. There was no evidence of cavity enhancement, Chiari malformation, tethered cord, or hydrocephalus.
All patients underwent thoracic laminectomy and resection of the arachnoid cyst wall, and postoperative neurological improvement was documented in each case. The mean follow-up duration was 19 months (range 4–37 months). Follow-up magnetic resonance imaging demonstrated the disappearance of the arachnoid cyst and a significant decrease in syrinx cavity size in each patient.
Spinal arachnoid cysts can be associated with syringomyelia, likely due to alterations in normal CSF dynamics. In symptomatic patients these cysts should be resected and the normal CSF flow restored. The results of the present series indicate that neurological improvement and reduction in syrinx cavity size can be achieved in patients with syringomyelia associated with intradural arachnoid cysts.
Russell R. Lonser, John D. Heiss and Edward H. Oldfield
Syringomyelia can be associated with Chiari malformation, spinal neoplasms, trauma, arachnoiditis, and other structural spinal lesions. 1 Our patient had two potential underlying causes for a cervicothoracic syrinx: acquired Chiari I malformation and a spinal neoplasm. This 18-year-old woman with von Hippel—Lindau (VHL) disease suffered from increasingly intense occipital headaches. Magnetic resonance (MR) imaging revealed two contrast-enhancing lesions in the right cerebellar hemisphere ( Fig. 1A ), herniation of the cerebellar tonsils to C-1 ( Fig. 1B ), and
Masaya Nakamura, Kazuhiro Chiba, Takashi Nishizawa, Hirofumi Maruiwa, Morio Matsumoto and Yoshiaki Toyama
W ith the widespread use of MR imaging in recent years, syringomyelia is being diagnosed in an increasing number of patients. The authors of recent reports have suggested that early diagnosis and the establishment of surgical techniques such as foramen magnum decompression and creation of a syringosubarachnoid shunt have made it possible to reduce the size of the syrinx. 4, 5 Improvements in syringomyelia-related symptoms, however, do not always correspond to reductions in the size of the syrinx, and this may pose treatment difficulties. 2, 12 Pain is one of
Ali I. Raja and Badih Adada
S yringomyelia is known to be associated with caudally located cerebellar tonsils (CM-I), 6 , 7 foramen magnum arachnoiditis, 1 , 15 , 16 spinal cord 21 or posterior fossa 5 , 17 tumors, craniovertebral anomalies such as basilar invagination, 8 , 13 cervical spondylosis, 14 and trauma. 9 , 19 , 20 In some cases, no hindbrain herniation 12 or other underlying pathological entity can be found. 2 The finding of syringomyelia in association with tonsillar herniation and posterior fossa tumors is not common. 3 , 5 , 22 A decrease in the size of the
Ken Sakushima, Kazutoshi Hida, Ichiro Yabe, Satoshi Tsuboi, Ritei Uehara and Hidenao Sasaki
S yringomyelia is a rare disease characterized by abnormal fluid-filled cavities or cysts within the spinal cord. Syringomyelia is caused by various pathological conditions, the most common of which is a Chiari I malformation, which can disturb the flow of CSF between the posterior cranial fossa and the spinal canal. Other causes include trauma, arachnoiditis, Chiari II malformation, and idiopathic origin. The essential treatment for syringomyelia is surgery to repair the structural abnormality associated with impaired CSF flow and syrinx. 14 Until the
Giuseppe Cinalli, Laurent Vinikoff, Michel Zerah, Dominique Renier and Alain Pierre-Kahn
months after cystoperitoneal shunt placement, showing herniation of the cyst down to the C-2 level and the formation of a cervical syringomyelic cavity. C: At the age of 7 years, showing a large syringomyelic cavity, in the presence of a working shunt. D: Three months after suboccipital decompression, C-1 laminectomy, and duraplasty, showing resolution of the syrinx. The association between DWM and syringomyelia has rarely been described, either as isolated case reports 1, 5 or as an occasional finding in large autopsy or clinical series. 2–4 Milhorat, et al
Jian Tu, Jinxin Liao, Marcus A. Stoodley and Anne M. Cunningham
S yringomyelia develops in 1 of 4 patients following spinal cord injury. 30 Posttraumatic syringomyelia causes progressive neurological deficits in patients who often already have disabilities, and surgical treatment is often unsatisfactory. Endogenous stem cells could theoretically replace lost tissue and repair deficits caused by syringes. 48 Neural progenitor cells are derived from the nervous system and have the capacity to divide, self-renew, and differentiate into neurons and glial cells. The differentiation of progenitor cells to neurons and glia
Kazutoshi Hida and Yoshinobu Iwasaki
The authors describe the surgical procedures for placing syringosubarachnoid shunts and the results of surgery, as well as the prevention of shunt malfunction.
The series consisted of 59 patients with syringomyelia associated with Chiari I malformation in whom syringosubarachnoid shunts were placed. Their ages ranged from 4 to 62 years (median 28 years). The follow-up period ranged from 13 to 219 months. The authors principally implanted the shunts in patients with large-sized syringes. Neurological improvement was satisfactory, and postoperative magnetic resonance imaging demonstrated that the syringes had resolved or decreased in size in all patients. Reoperation was necessary in 10 patients who were treated before 1993.
To prevent shunt malfunction, both dorsal root entry zone myelotomy and placement of the syringo-subarachnoid shunt tube into the ventral subarachnoid space are useful.
Yingsong Wang, Jingming Xie, Zhi Zhao, Ying Zhang, Tao Li and Yongyu Si
C hiari malformation Type I is defined as cerebellar tonsillar descent below the foramen magnum and into the spinal canal. It is the leading cause of syringomyelia, which is characterized by a fluid-filled cavity within the substance of the spinal cord. Syringomyelia associated with CM-I is often limited to the cervical region of the spinal cord, although it may extend to the thoracic portion. The exact pathogenesis of syringomyelia in patients with CM-I has not been clarified. Disturbed or obstructed CSF flow around the foramen magnum was assumed to be