PubMed · 14101472
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Y UEKI, H NEMOTO. 1963. [CONGENITAL HYDROCEPHALUS].. https://pubmed.ncbi.nlm.nih.gov/14101472/
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Myelocystoceles are rare lesions. Rarer still are thoracic myelocystoceles. Two patients with thoracic myelocystoceles are being reported. The first patient presented with swelling in the thoracic region with paraplegia and incontinence. MR with MR myelography revealed the malformation to be a thoracic myelocystocele. The second patient presented with a midline cutaneous mass lesion without neurological deficit. MRI revealed a Type I split cord malformation, a CSF containing sac in the thoracic region that communicated through a stalk-like structure to the hydromyelic hemicord. Surgery in both these patients did not produce any change in the neurological status. The importance of recognizing thoracic myelocystoceles, their clinical presentation, embryology, value of MR myelography in the diagnosis and role of surgery are discussed. The differences between terminal myelocystoceles and thoracic myelocystoceles are also highlighted.
INTRODUCTION: The authors report a case of three brothers. Two of these siblings presented with congenital growth hormone deficiency and Chiari I malformation. The third and younger brother has been found not to have growth hormone deficiency or the Chiari I malformation. RESULTS: Sparse cases of these two clinical occurrences have been reported. Further, the posterior cranial fossa has been determined to be altered in patients with Chiari I malformation and growth hormone deficiency. CONCLUSION: Our current case reports strengthen the association between these two pathological entities and are, to our knowledge, the first description of both defects in siblings.
BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.