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Year : 2017  |  Volume : 12  |  Issue : 4  |  Page : 393-394

Chari 1 malformation with scoliosis and holocord syringomyelia: A rare association

Department of Neurosurgery, Government Medical College Trivandrum, Trivandrum, Kerala, India

Date of Web Publication26-Mar-2018

Correspondence Address:
Dr. Raja K Kutty
Associate Professor, Department of Neurosurgery, Government Medical College, Trivandrum, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JPN.JPN_85_17

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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.

Keywords: Chiari 1 malformation, holocord syrinx, scoliosis

How to cite this article:
Kutty RK, SunilKumar B S, Peethambaran A. Chari 1 malformation with scoliosis and holocord syringomyelia: A rare association. J Pediatr Neurosci 2017;12:393-4

How to cite this URL:
Kutty RK, SunilKumar B S, Peethambaran A. Chari 1 malformation with scoliosis and holocord syringomyelia: A rare association. J Pediatr Neurosci [serial online] 2017 [cited 2023 Jun 3];12:393-4. Available from: https://www.pediatricneurosciences.com/text.asp?2017/12/4/393/227987

   Case Presentation Top

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

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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.[4],[5] 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.[5],[9] 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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There are no conflicts of interest.

   References Top

Ilizarov G, Chowdhury N, Kalva S. poster64. Post-traumatic syringomyelia with holocord involvement: a case report. PM&R 2015;7(9):S112-3. https://doi.org/10.1016/j.pmrj.2015.06.105.  Back to cited text no. 1
Khalid M, Khalid S, Mittal S, Ahmad U. Intramedullary tubercular abscess with syrinx formation. J Pediatr Neurosci 2012;7:61-3.  Back to cited text no. 2
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Su C-F, Chen J-C, Hsu Y-H, Yang H-J, Lee C-C. Hemangioblastoma of the spinal cord associated with holocord syringomyelia and syringobulbia. Tzu Chi Med J 2001;13:187-92.  Back to cited text no. 3
Segal D, Heary RF, Sabharwal S, Barry MT, Ming X. Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome. J Neurosurg Pediatr 2016;18:79-82.  Back to cited text no. 4
Feldstein NA, Choudhri TF. Management of Chiari I malformations with holocord syringohydromyelia. Pediatr Neurosurg 1999;31:143-9.  Back to cited text no. 5
Awai L, Curt A. Preserved sensory-motor function despite large-scale morphological alterations in a series of patients with holocord syringomyelia. J Neurotrauma 2015;32:403-10.  Back to cited text no. 6
Panda AK, Kaur M. Rapidly progressive foot drop: an uncommon and underappreciated cause of Chiari I malformation and holocord syrinx. BMJ Case Rep 2013;2013:2-5.  Back to cited text no. 7
Krieger MD, Falkinstein Y, Bowen IE, Tolo VT, McComb JG. Scoliosis and Chiari malformation Type I in children. J Neurosurg Pediatr 2011;7:25-9.  Back to cited text no. 8
Sengupta DK, Dorgan J, Findlay GF. Can hindbrain decompression for syringomyelia lead to regression of scoliosis? Eur Spine J 2000;9:198-201.  Back to cited text no. 9
Xie J, Wang Y, Zhao Z, Zhang Y, Si Y, Yang Z, et al. One-stage and posterior approach for correction of moderate to severe scoliosis in adolescents associated with Chiari I malformation: is a prior suboccipital decompression always necessary? Eur Spine J 2011;20:1106-13.  Back to cited text no. 10


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