Surgical Outcomes of Symptomatic Intramedullary Spinal Cord Cavernous Malformations: Analysis of Consecutive Cases in a Single Center

Abstract

Objective: Intramedullary spinal cavernous malformations (ISCMs) are rare vascular lesions of the spinal cord with unclear natural history and controversy over treatment. This study aimed to report a series of symptomatic ISCMs underwent microsurgical management to illustrate the natural history, clinical presentation, and surgical outcomes and to evaluate factors associated with hemorrhage events and neurological prognosis.

Methods: This single-center retrospective study included 29 consecutive patients with whose demographic, symptomology, imaging, neurological, and surgical data were collected. The risk for hemorrhage events and factors affecting surgical outcomes were retrospectively analyzed.

Results: There were 12 female (41.4%) and 17 male patients (58.6%), with an average age of 45.2 years (range, 17-69 years). The mean size of the lesion was 9.7 mm (range, 3-20 mm). Most patients had a bowel or/and bladder dysfunction symptom (n = 11, 37.9%), followed by sensory deficits (n = 5, 17.2%), gait disturbance (n = 5, 17.2%), pain (n = 4, 13.8%), and weakness (n = 4, 13.8%), most (n = 15, 51.7%) with a chronic onset. All patients received total resection without rehemorrhages after surgical resection in follow-up. Sixty-five point five percent patients (n = 19) improved, 13.8% (n = 4) remained stable, 20.7% (n = 6) got worsen. The overall annual hemorrhage risk was 2.1% per patient-year. A total of 27 hemorrhages occurred in the 18 patients, of which rehemorrhage rate increased to 50.0% (n = 9) with a previous history of hemorrhage. Patients with smaller lesion sizes were more likely to have hemorrhage or rehemorrhage events (p = 0.008). Recurrent hemorrhage of the lesions was a risk factor for neurological outcomes (p = 0.016).

Conclusion: The risk of rehemorrhage was significantly increased in symptomatic ISCM patients with a previous history of hemorrhage. Rehemorrhage was a risk factor for neurological outcomes. Patients can benefit from microsurgical treatment to avoid rehemorrhage and further neurological deterioration.

Keywords: Cavernous malformations; Intramedullary tumors; Spinal cord cavernous malformations; Spinal cord malformations.

Fig. 1.

(A) A 52-year-old male patient with 10 years of both lower-limbs paresthesias was admitted. Preoperative magnetic resonance imaging (MRI) demonstrated a dorsally located T6 spinal cavernous malformation. (B) Upon opening the dura, the cavernous malformation was visualized at the spinal cord's dorsal surface with surrounding hemosiderin-stained gliotic tissue. (C) A middle-line myelotomy was performed directly over the lesion, and the lesion was removed via en bloc. (D) Careful cavity inspection demonstrates complete removal without residual cavernous malformation. (E) Grossly, it was a well-circumscribed lesion with a mulberry-like appearance. (F, G) Postoperative MRI shows complete removal, and the patient remained neurologically stable.

Fig. 2.

A 52-year-old female with 10 months of left-arm paresthesia was admitted. (A, B) Magnetic resonance imaging (MRI) demonstrated a C3–5 dorsolaterally located cavernous malformation. The patient underwent C3–6 laminoplasty. (C, D) The cavernous malformation was revealed after incising the posterior lateral sulcus of the spinal cord. (E) The lesion was resected via microsurgical techniques. (F) The cavity was explored and found free of the residual lesion. (G) Pathological images showed many vessels lined with a single layer of flattened endothelial cells with no intervening parenchymal tissue (hematoxylin and eosin, ×100). (H) Three months postoperative MRI demonstrates complete removal. The patient made a full neurologic recovery.

Fig. 3.

Flowchart showing patient selection. ISCM, intramedullary spinal cavernous malformation.

Fig. 4.

Hemorrhage events of intramedullary spinal cavernous malformation in the cervical spinal cord. The patient was admitted to the hospital with a sudden onset of limb hemiparesis. (A-C) Magnetic resonance imaging (MRI) revealed an intramedullary occupancy of the C2 cervical intramedullary spinal cord, with noticeable hemorrhagic changes and large edema in the right cervical medulla. After 2 weeks of conservative treatment with dehydration and hormones, most of the hemiplegic symptoms improved, then the limb movement function was fully restored at 3 months. (D, E) The repeat MRI showed that most hematoma and edema were absorbed, and the tumor border was formed. With an elective procedure, the tumor was well visualized and resected. (F, G) A hemosiderin-stained cavity can be observed. Three months after the operation, the MRI cavernous hemangioma was completely removed, and the patient recovered well and could work normally. (H, I) MRI 3 months later showed a GTR of the lesion, and the patient recovered well and can normally work.