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Iatrogenic Spinal Intradural Hemorrhage in a Patient with Dural Ectasia in Marfan Syndrome

Published online by Cambridge University Press:  05 October 2023

Giulio Vara Open the ORCID record for Giulio Vara [Opens in a new window] ,
Gianfranco Vornetti  and
Luca Spinardi
Giulio Vara*
Affiliation:
University of Bologna, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy Diagnostic and Interventional Radiology, Ospedale Civile Umberto I, Lugo (RA), Emilia Romagna, Italy
Gianfranco Vornetti
Affiliation:
Dipartimento di Scienze Biomediche e Neuromotorie, University of Bologna, Bologna, Italy
Luca Spinardi
Affiliation:
University of Bologna, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
*
Corresponding author: G. Vara; Email: [email protected]
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Abstract

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Keywords

intradural hemorrhagedural ectasiamarfan syndromeMRISPINE
Type
Neuroimaging Highlight
Information
Canadian Journal of Neurological Sciences , First View , pp. 1 - 2
Copyright
© The Author(s), 2023. Published by Cambridge University Press on behalf of Canadian Neurological Sciences Federation

Marfan Syndrome (MFS) is a connective tissue disorder that affects multiple organ systems, characterized by abnormal skeletal and cardiovascular features. Reference Loeys, Dietz and Braverman1

A 46-year-old woman affected by MFS developed acute lower limb weakness and sensory deficit after cardiac surgery with cerebrospinal fluid pressure monitoring with a lumbar catheter.

The patient underwent lumbar MRI with T1w (A), T2w(B), and post-contrast T1w with fat saturation (C) sequences (Fig. 1). MRI revealed the presence of inhomogeneous content within the dural sac due to different phases of hemoglobin catabolism. A collection with a fluid level was evident posteriorly to L2–L3, with focal contrast enhancement related to active bleeding (“spot-sign”). This was indicative of the patient undergoing active bleeding post-surgery. Dural ectasia, a major diagnostic criterion of MFS, was observed in the patient, defined as dilatation of the dural sac and the subsequent expansion of the spinal canal. The patient underwent decompressive laminectomy, with partial recovery of the symptoms.

Figure 1: The lumbar MRI was performed with T1w ( a ), T2w ( b ), and post-contrast T1w with fat saturation ( c ) sequences. The dural sac is filled with inhomogeneous content due to different phases of hemoglobin catabolism. A collection with a fluid level is evident posteriorly to L2–L3, with focal contrast enhancement related to active bleeding (“spot-sign”). Dural ectasia, according to the Ghent nosology, represents a major diagnostic criterion of Marfan syndrome; it is defined as a dilatation of the dural sac (and the subsequent expansion of the spinal canal), and is very evident in this patient.

This case report highlights the possibility of an increased risk of intradural hemorrhage in patients with Marfan syndrome and dural ectasia following a lumbar puncture and the importance of prompt recognition and treatment. Dural ectasia is already an established anesthesiological risk factor, impairing the efficacy of spinal anesthesia. Reference Lacassie, Millar and Leithe2 The risk of spinal hematoma following a lumbar puncture is low in the general population, while spontaneous occurrence in Marfan patients is reported only anecdotally. Reference Wityk, Zanferrari and Oppenheimer3,Reference Bodilsen, Mariager and Vestergaard4 To minimize the risk of complication, patients with genetic conditions related to dural ectasia should undergo an imaging screening of the lumbar spine or an opportunistic post hoc evaluation of previous imaging studies that include it in the field of view. Further studies are needed to better understand the underlying mechanisms and risk factors for this complication in this patient population. If dural ectasia proves to be a risk factor for intradural hemorrhage, alternative surgical monitoring methods should be researched.

Funding

None.

Competing interests

None.

Ethical standard

The patient has provided informed consent.

Statement of authorship

Writing, original draft preparation: GVa, GVo; conceptualization, review, and editing: LS.

References

Loeys, BL, Dietz, HC, Braverman, AC, et al. The revised ghent nosology for the marfan syndrome. J Med Genet. 2010;47:476–85.CrossRefGoogle ScholarPubMed
Lacassie, HJ, Millar, S, Leithe, LG, et al. Dural ectasia: a likely cause of inadequate spinal anaesthesia in two parturients with marfan’s syndrome. Br J Anaesth. 2005;94:500–4.CrossRefGoogle ScholarPubMed
Wityk, RJ, Zanferrari, C, Oppenheimer, S. Neurovascular complications of marfan syndrome: a retrospective, hospital-based study. Stroke. 2002;33:680–4.CrossRefGoogle ScholarPubMed
Bodilsen, J, Mariager, T, Vestergaard, HH, et al. Association of lumbar puncture with spinal hematoma in patients with and without coagulopathy. JAMA. 2020;324:1419–28.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1: The lumbar MRI was performed with T1w (a), T2w (b), and post-contrast T1w with fat saturation (c) sequences. The dural sac is filled with inhomogeneous content due to different phases of hemoglobin catabolism. A collection with a fluid level is evident posteriorly to L2–L3, with focal contrast enhancement related to active bleeding (“spot-sign”). Dural ectasia, according to the Ghent nosology, represents a major diagnostic criterion of Marfan syndrome; it is defined as a dilatation of the dural sac (and the subsequent expansion of the spinal canal), and is very evident in this patient.