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Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications

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Abstract

Chiari I malformation (CI) continues to raise great interest among physicians due to the larger and larger number of newly diagnosed cases. The clinical and radiological picture and the management options of such a chronic disease are well acknowledged as well as those of the associated syringomyelia. Little is known, on the other hand, about abrupt clinical onset following decompensation of CI/syringomyelia complex. This review on the sudden onset of these two conditions shows that this is a very rare phenomenon; only 41 cases are being reported in the last three decades. In all these cases, acute onset was referable to CI/syringomyelia and the clinical course quickly precipitated. Motor deficits (36.5 %), respiratory failure (29 %), cranial nerve palsy (17 %), and cardiac arrest (14.5 %) were the most common findings, thus confirming that abrupt onset may have severe and life-threatening consequences. Indeed, sudden or early mortality accounted for 19.5 % of cases. In spite of that, most of the surviving subjects had an excellent outcome following either surgical or medical/rehabilitation treatment. Physiopathology of abrupt onset is attributed to the acute compression of the brainstem/upper cervical spinal cord by ectopic tonsils and syringobulbia/syringomyelia, frequently precipitated by a minor injury, followed by impairment of medullary baroreceptors and midbrain reticular substance (cardiac arrest, syncope), medullary chemoreceptors and phrenic nerve nuclei (respiratory failure), lower cranial nerve nuclei (cardiac arrest, cranial nerve palsy), and pyramidal tracts (motor deficits). About 87 % of patients of this review were asymptomatic prior to their acute onset. The problem of the management of asymptomatic subjects is still open.

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Authors and Affiliations

  1. Institute of Neurosurgery—A. Gemelli Hospital, Largo A. Gemelli, 8, 00168, Rome, Italy

    Luca Massimi, Giuseppe M. Della Pepa, Massimo Caldarelli & Concezio Di Rocco

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  1. Luca Massimi
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  2. Giuseppe M. Della Pepa
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  3. Massimo Caldarelli
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  4. Concezio Di Rocco
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Correspondence to Luca Massimi.

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Comments

Jörg Klekamp, Quakenbrück, Germany

Massimi et al. raise the question of how to handle asymptomatic patients with Chiari I malformations. Should they undergo surgery? May they participate in sports activities? Should they undergo cesarean section to deliver their children? These are probably the questions most neurosurgeons will be asked if symptoms are either not present at all or so minor that the patient is reluctant to undergo a decompression. The authors point to the fact that sudden deteriorations are possible even to the extent of life-threatening situations. On the other hand, foramen magnum decompression is an operation with potentially life-threatening complications. Surgical mortality is in the order of 1 %. Therefore, every patient has to be informed about the risks and benefits of surgery as well as the natural course. This paper is a welcome adjunct for patient counseling. Fortunately, not all adverse things happen that one would expect given the compression of important central nervous structures at the foramen magnum: all adults presenting with this malformation had this anatomical constellation since early childhood. Yet, hardly any of them reports neurological incidents with sport activities. Almost no woman recalls such problems during labor either. In my series of 565 patients with Chiari I malformation, not a single incident such as those reported in this paper was encountered, unless there was an additional craniocervical instability, which needs to be ruled out in patients with additional basilar invagination. In my opinion, surgery should not be recommended to asymptomatic patients but offered if the patient wants to have the operation specifically before neurological symptoms have set in. Likewise, questions such as how to deliver a child or what sports activities are allowed should be discussed individually.

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Massimi, L., Della Pepa, G.M., Caldarelli, M. et al. Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications. Neurosurg Rev 35, 321–329 (2012). https://doi.org/10.1007/s10143-012-0391-4

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