Chari 1 Malformation with Scoliosis and Holocord Syringomyelia: A Rare Association.

Syringomyelia and scoliosis are not uncommon phenomena in the spectrum of Chiari malformations. However, the presence of holocord syrinx in association with Chiari 1 malformation is quite rare.

Case Presentation

A 15-year-old female presented with back pain and progressive scoliosis over the thoracolumbar region. She was admitted to the orthopedics department for correction of spinal deformity. There was no history of any motor or sensory weakness. She was referred to us when her magnetic resonance imaging (MRI) revealed a holocord syrinx. When we interrogated her, we learned that she had a history of burns in the right palm that she suffered while cooking. On examination, there was dissociated sensory loss over her limbs and thorax which she was not aware of. The MRI image showed a descend of the tonsils below the foramen magnum. She is posted for foramen magnum decompression [Figure 1].

Figure 1

(A) MRI brain TIW sagittal section shows descend of the cerebellar tonsils below the level of the foramen magnum. Upper end of the syrinx can be visualized in the region of C2. (B) MRI spine T1W coronal image showing left-sided scoliosis. (C) MRI spine T2W sagittal image showing well-defined holocord syrinx extending from C2 to D11 spine

Holocord syrinx has been described in various pathological states involving the spinal cord ranging from subarachnoid hemorrhage, trauma,[1] intramedullary abscess,[2] tumors,[3] and congenital abnormalities.[45] The manifestations of this abnormality can vary in different individuals. This can range in the form of relatively asymptomatic states,[6] hemihydrosis,[7] foot drop to frank sensory abnormalities, and scoliosis.[5] Scoliosis as a presenting symptom of Chiari 1 malformations has been previously reported in the literature. In a retrospective series of 10 years, Krieger et al.[8] found 79 patients who were evaluated for scoliosis and found to have Chiari 1 malformations. Management of such patients is difficult. A majority of surgeons will perform foramen magnum decompression with or without duroplasty and look for resolution of the syrinx before attempting to correct the spinal deformity. This has been shown to be an effective procedure for the resolution of holocord syrinxes and to stabilize the progression of deformity.[59] There have also been suggestions regarding management of deformity first without addressing the foramen magnum with promising results.[10] This approach is along the line of thought that significant deformity with larger curves in association with syrinxes cannot be resolved following FMD alone. Although syrinxes are common in the cervical region when associated with Chiari malformations, holocord syrinxes are rare.

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Conflicts of interest

There are no conflicts of interest.