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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 6  |  Page : 967-969

Hemorrhage within a syrinx: An unusual presentation of Chiari malformation type I

Department of Neurosurgery, West Hospital, Sichuan University, Sichuan, China

Date of Web Publication20-Nov-2015

Correspondence Address:
Siqing Huang
Department of Neurosurgery, West Hospital, Sichuan University, Sichuan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.170105

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How to cite this article:
Hu Y, Liu J, Jiang S, Huang S. Hemorrhage within a syrinx: An unusual presentation of Chiari malformation type I. Neurol India 2015;63:967-9

How to cite this URL:
Hu Y, Liu J, Jiang S, Huang S. Hemorrhage within a syrinx: An unusual presentation of Chiari malformation type I. Neurol India [serial online] 2015 [cited 2022 Sep 25];63:967-9. Available from: https://www.neurologyindia.com/text.asp?2015/63/6/967/170105


A 40-year-old previously healthy man with progressive pain in the neck and left upper limb was found to be having  Chiari malformation More Details type I (CM-I) and syringomyelia [Figure 1]. He underwent a foramen magnum decompression, cerebellar tonsillectomy, and a wide duraplasty. The postoperative clinical course was uneventful except for the presence of slight headache and mild fever. Two months later, he was readmitted to the hospital as he started having progressive neck pain and stiffness, and numbness and paralysis of the left arm. The symptoms were acute in onset. There was no history of trauma, excessive coughing, or heavy labor. The lumbar puncture revealed xanthochromia in the cerebrospinal fluid, with increased mononuclear cells, elevated proteins, and normal glucose. All culture specimens failed to identify an organism. Spinal magnetic resonance imaging (MRI) demonstrated loculated fluid-filled cysts at the C1–C2 vertebral level, a heterogeneous intramedullary lesion from the C2–C6 levels, and meningeal gadolinium enhancement [Figure 2]. A diagnosis of hematomyelia and aseptic meningitis was made, and the patient's clinical picture gradually improved after 2 weeks of intravenous methylprednisolone. A repeat cervical MRI revealed the partial absorption of the hemorrhagic products and also that the enhancing region had become almost undetectable [Figure 2]. The patient regained a near-normal neurological status at follow up after 2-years.
Figure  1:   (a and b) Sagittal T1-  and T2-weighted images show herniation of the cerebellar tonsils, and a syrinx cavity at the C1–C6 vertebral levels

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Figure  2: (a) Sagittal T1-weighted image reveals loculated cysts with cerebrospinal fluid signal intensity and a hyper-  or hypo-intense lesion within the central cervical cord; (b) the lesion is hypointense with parenchymal edema on sagittal T2-weighted image, and a fluid–fluid level is identified in the lowest cyst  (left arrow); (c) sagittal and axial  (inset) contrast-enhanced T1-weighted images show meningeal enhancement and a peripheral ring enhancing lesion  (right arrow); and, (d) this lesion almost disappeared in the postcontrast T1-weighted image taken after 2  weeks of medical treatment

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The onset of intrasyringeal hemorrhage (ISH) in CM-I patients is an extremely rare condition, and only 6 cases (with clinical details available) have been reported in literature [Table 1].[1],[2],[3],[4],[5],[6] ISH may occur spontaneously, may be precipitated by trauma, or may result following the surgical treatment of CM-I. The age of onset of the ISH has been reported to be ranging from 8 to 43 (mean 30.5) years, without there being any gender predominance. ISH is usually characterized by an acute onset of symptoms and/or rapid neurological deterioration (even death). It is usually seen after a variable period during which the patient has symptoms/signs linked to the presence of CM-I with syringomyelia.[1],[3],[4],[5],[6] Although an acute presentation of ISH has been commonly observed, a gradual neurological deterioration may also occur.[2]
Table  1: Summary of reported cases of ISH associated with CM-I

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The ideal treatment of ISH associated with CM-I is still unknown because of the rarity of the disease. The review of literature of the 6 available patients reveals that ISH had occurred in 5 patients (83.3%) prior to their undergoing posterior fossa decompression for their Chiari I malformation; and, was observed only in one patient following decompressive surgery, suggesting that surgically treated CM-I patients may have a lower propensity to develop ISH.[1],[2],[3],[4],[5],[6] It has been proposed that the neurological deficits caused by ISH would improve following a myelotomy with aspiration of the hematoma; however, solely carrying out a myelotomy without simultaneously performing a posterior fossa decompression would lead to the persistence, or even deterioration, of CM-I-associated deficits.[1],[4],[5] Performance of a myelotomy for an ISH has been associated with a favorable outcome in patients without CM-I, except in a patient suffering from hemophilia.[5] However, nearly 50% (2/4) of CM-I patients also needed an additional posterior fossa decompression for their CM-I after undergoing the urgent myelotomy due to worsening or lack of improvement of their neurological status precipitated by the tonsillar herniation and syringomyelia.[1],[2],[4],[5] Therefore, simultaneous hematoma evacuation and posterior fossa decompression may be a better approach to treat ISH associated with CM-I.[1],[4] Our patient is the first case of ISH associated with CM-I who was successfully treated without a myelotomy.[3] The patient experienced neurological improvement without surgical intervention possibly because : (1) The posterior fossa decompression prior to the development of ISH normalized the intrasyringal pressure as revealed by the collapse of the syrinx cavity; and, (2) only a small volume of hemorrhagic component persisted in the collapsed cavity that got gradually absorbed without surgery.

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 » References Top

Williams B, Turner E. Communicating syringomyelia presenting immediately after trauma. A case description and some theoretical concepts. Acta Neurochir (Wien) 1971;24:97-106.  Back to cited text no. 1
Bidzinski J, Kahl-Kunstetter J. Chronic bleeding into the syringomyelic cavity (Gower's syringal hemorrhage). Neurol Neurochir Pol 1975;9:415-7.  Back to cited text no. 2
Roig C, Lopez-Pousa S, Ferrer I. Bleeding in syringobulbia. A fatal complication. Eur Neurol 1982;21:189-93.  Back to cited text no. 3
Ayuzawa S, Tsukada A, Enomoto T, Yasuda S, Uemura K, Yamada T, et al. Intrasyringal hemorrhage of the cervical cord associated with Chiari type I malformation – Case report. Neurol Med Chir (Tokyo) 1995;35:243-6.  Back to cited text no. 4
Hamlat A, Adn M, Ben Yahia M, Morandi X, Brassier G, Guegan Y. Gowers intrasyringal hemorrhage. Case report and review of the literature. J Neurosurg Spine 2005;3:477-81.  Back to cited text no. 5
Mitsuhara T, Yamaguchi S, Takeda M, Kurisu K. Gowers' intrasyringeal hemorrhage associated with Chiari type I malformation in Noonan syndrome. Surg Neurol Int 2014;5:6.  Back to cited text no. 6
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  [Figure 1], [Figure 2]

  [Table 1]

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