Introduction

Seldom do intramedullary cavernous malformations of the spine occur; they make up only 3–5% of cavernous malformations and 5–12% of all intraspinal vascular malformations [1]. The peak of the disease occurs in the fourth decade of life, which is comparable to the highest incidence of cerebral cavernomas [2]. Compared to men, women are impacted more frequently [3, 4].

The spaces inside spinal cavernomas are filled with blood and endothelium; they are surrounded by thickened, hyalinized walls devoid of smooth muscle and elastic fibers [5, 6]. Cavernous malformations of the spinal cord are less well studied than those of the brain, especially when it comes to treatment and management. Because of their lower tolerance to mass lesions in the spine, they typically have a more aggressive clinical course than lesions in the brain. These lesions are prone to recurrent bleeding and progressive growth. A mechanism for the underlying acute episodes of neurological deterioration has been proposed: bleeding episodes. Gliosis and microbleeds can both lead to progressive myelopathy.

Ogilvy et al. [7] have reported that the most common symptoms include pain, weakness, and paresthesia. The literature has identified four distinct patterns of clinical presentation: (1) isolated episodes of neurological decline with varying degrees of recovery between episodes; (2) gradual neurological decline progression; (3) acute onset of symptoms with quick resolution; and (4) acute onset of mild symptoms followed by gradual resolution over weeks to months. In this study, we report the rare case of a 37-year-old man who underwent successful resection of cervical intramedullary cavernoma of the C5–C6 level.

Case presentation

Patient information

A 37-year-old man with a six-month history of progressively worsening neck pain and numbness in his right hand was admitted to our department because the pain became persistently unbearable and did not respond to standard pain medications. The patient is receiving medication for type 2 diabetes mellitus. He denied any alcohol or tobacco use, and there was no history of trauma.

Clinical findings

A neurological examination revealed no motor deficit in any extremities. He had decreased light touch and proprioceptive sensitivity in the right upper extremity. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities. A bilateral Babinski and Lhermitte sign was positive.

Diagnosis assessment

Magnetic resonance imaging (MRI) scan of the spine revealed a 15-mm-sized intramedullary lesion at the C5–C6 level with mixed signal on T1WI and T2WI sequences. A popcorn-like lesion was characterized by a hyperintense core surrounded by a hypointense rim on the sagittal T1WI and axial T2WI suggesting the diagnosis of intramedullary cavernoma (Fig. 1).

Fig. 1
figure 1

Magnetic resonance image of the cervical spine, T2-weighted, A sagittal, B axial show cavernous malformation at the C5–C6 level (arrows)

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Therapeutic intervention

The patient was operated on. The head was placed in the Mayfield head holder, while the patient was in the prone position and under general anesthesia. To protect peripheral nerves and reduce pressure on the chest and abdomen, all pressure points have been adequately padded. The posterior approach was used to perform laminectomies of C4, C5, and C6. Evoked somatosensory potentials are tracked. A baseline test was performed before dural opening to allow an informative interpretation of the intraoperative recordings. The dura mater was opened, a midline myelotomy was performed, and a well-circumscribed dark blue lesion was visualized spreading the posterior columns laterally to the right. The lesion was then completely removed “en bloc,” with the hemosiderin rim serving as a guide for the cleavage plane of the lesion. After the removal of the lesion, a thorough examination of the cavity revealed no evidence of residual deformity. Neither motor-evoked potentials nor somatosensory perception changed during the procedure. The histopathological findings were consistent with a cavernous angioma (Fig. 2).

Fig. 2
figure 2

A–C Benign tumor proliferation consisting of dilated vascular structures lined with regular endothelial cells, 40X

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Follow-up

Postoperatively, even though the severe neck pain has subsided, the patient experienced a weakness in the right upper extremity, which improved over eight months of physiotherapy. A month follow-up cervical spine magnetic resonance imaging demonstrated no residual lesion.

Methods

A systematic review of all the previously reported intramedullary cavernomas of the cervical spinal cord was performed according to the PRISMA guidelines (Preferred Reporting Items for Systematic Reviews and Meta-Analysis). Two authors (Y.C.H.D. and S.E.M.) independently performed a search from 1980 to 2023 using the following mesh terms “cervical AND intramedullary AND hemangioma, cavernous” on PubMed (245 results).

According to PRISMA guidelines, the research order consisted of screening titles and abstracts first, and then the full-text screening (Fig. 3) (Table 1). Finally, the references of the articles included were screened. No automation software was used.

Fig. 3
figure 3

PRISMA flowchart summarizing the selection of the studies

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Table 1 General information about the included articles
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We included case reports and case series reporting patients affected by intramedullary cavernoma of the cervical spinal cord with definitive histopathological analysis. Only articles were published in the English language. All articles (i) not dealing with intramedullary cavernomas of the spinal cord in the cervical region, (ii) without full text or with no disaggregated data, (iii) written in a language other than English, about a retrospective study (iv) and duplicates (v) were included.

A database based on the previously selected case reports or case series was created. Descriptive statistical analyses were conducted by Jamovi 2.3.18 [3] (Fig. 4).

Fig. 4
figure 4

A database based on the previously selected case reports or case series

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Results

A total of 29 studies reporting 423 patients with a mean age of 40.5 ± 6.3 years were included in this study. The overall male-to-female ratio was 1:2.5 with a median follow-up duration of 46 months [18.5–63.9]. Laminectomy and subsequent complete resection of the cavernomas were reported in 82.8% (n = 24) of the included papers. Only one article (3.4%) reported complete resection of the lesion by an anterolateral partial vertebrectomy, although this information was not available in the remaining four 13.8% of papers. The outcome was favorable in 82.8% (n = 24).

Discussion

It was previously believed that spinal cavernous malformations (SCM) were uncommon spinal cord lesions. However, over the past few decades, the prevalence of SCM has dramatically increased due to the widespread use of contemporary imaging techniques. The management of incidental and symptomatic findings is thus becoming increasingly important. However, experience with treatment and follow-up is minimal [8]. The same author reported only 7.1% of the cavernous malformations treated in their department over a decade were of spinal location. But surprisingly, 34.5% of these lesions were at the cervical spine. However, the authors do not provide any further details as to whether the location was extradural, intradural-extramedullary or intramedullary. Although the spine has been described as a rare site of cavernous malformations, the cervical spine may be the most common. However, the site of development showed an even distribution of cervical and thoracic locations, in contrast to previous reports documenting a thoracic predilection [7, 9]. Nevertheless, further studies on this topic are required to clarify precisely the intramedullary aspect, as is the case in our reported case.

Because of the difficulty in diagnosing these lesions before the advent of advanced imaging techniques, the actual incidence of asymptomatic spinal cavernomas is unknown. In their series, Badhiwala et al. [4] reported the distribution of symptoms as 60% motor, 58% sensory, 34% pain, and 24% disturbance of bladder and/or bowel function. The symptom presentation of patients with CMs may be acute or slowly progressive. Acute neurological deterioration occurs in cases with significant bleeding within the spinal cord, and slow progressive deterioration is related to multiple repeated microhemorrhages, subsequent thrombosis, hyalinization, and possible enlargement of the malformation [10]. The preferred imaging technique for identifying cavernous malformations is magnetic resonance imaging. Because cavernous malformations are angiographically occult and challenging to diagnose with traditional tomography and myelography, the majority of cases were documented following the introduction of MRI. A common observation is rounded areas of heterogeneous signal intensity on the T1- and T2-weighted images, which are caused by different age blood products and have a “popcorn appearance.” This is composed of a central lesion core that exhibits mixed heterogeneous signal intensity on both T1- and T2-weighted images, with minimal contrast enhancement, and a periphery with a hypointense ring of hemosiderin [11,12,13].

Most patients described in the literature were treated either surgically or conservatively. In their single-center serial analysis of intramedullary cavernous malformation of the spinal cord, Badhiwala et al. [4] reported that 90% of patients underwent resection and only 10% underwent conservative treatment. Only the symptomatic patients benefited from complete resection of the lesion. The result was that in the surgical group, 30% of patients improved, 58% were neurologically stable, and 11% worsened their neurological status. Meanwhile, outcomes after five years of follow-up were reported to be less favorable for those who chose conservative treatment. These results have been confirmed by many other authors [3, 14,15,16,17,18,19]. Nevertheless, according to our systematic review, the surgical outcome was positive, and good results were reported in 82.8% (n = 24) of all patients with totally resected cervical cavernoma.

Conclusion

Intramedullary cavernomas of the cervical spinal cord tend to be clinically progressive. Total macroscopic resection of the lesion is the gold standard treatment that should be performed whenever possible and is the better indication that guarantees a favorable outcome.