Cervical intramedullary spinal cavernoma in setting of unresolved myelopathy: A case report

Background: Spinal cavernous malformations are rare, accounting for approximately 5–12% of all spinal cord vascular lesions. Fortunately, improvements in imaging technologies have made it easier to establish the diagnosis of intramedullary spinal cavernomas (ISCs). Case Description: Here, we report the case of a 63-year-old male with an >11-year history of left-sided radiculopathy, ataxia, and quadriparesis. Initially, radiographic findings were interpreted as consistent with spondylotic myelopathy with cord signal changes from the C3-C7 levels. The patient underwent a C3-C7 laminectomy/foraminotomy with instrumentation. It was only after several symptomatic recurrences and repeated magnetic resonance images (MRI) that the diagnosis of a ventrally-located intramedullary lesion, concerning for a cavernoma, at the level C6 was established. Conclusion: Early and repeated enhanced MR studies may be required to correctly establish the diagnosis and determine the optimal surgical management of ISCs.

Spinal Cavernoma in an Infant: A Rare Pathological Entity with an Atypical Presentation

<b><i>Introduction:</i></b> Cavernous malformation (CM) of the pediatric spine is uncommon, and its presentation especially in infancy is extremely rare. We report an unusual case of thoracolumbar intramedullary CM with hemorrhage in a 5-month-old male child. <b><i>Case Presentation:</i></b> The child presented with the predominant symptom of urinary retention, and the underlying neurological cause was initially overlooked. However, magnetic resonance imaging obtained after the onset of progressive limb weakness revealed a D11-L1 intramedullary lesion with features of intralesional bleed. Intraoperatively, the lesion showed evidence of hemorrhage and was completely excised. The final histopathology confirmed a cavernoma. <b><i>Conclusion:</i></b> Although rare, spinal CM can present with bleed in very young children. It is imperative to identify the subtle clinico-radiological findings and suspect such lesions, as an early treatment portends a good outcome.

“Radiologically Isolated” Spinal Cavernoma Associated with Familial Cerebral Cavernomatosis

We describe a 45 year-old woman with a randomly discovered cavernous malformation of the cervical cord following investigation for neck injury. She had multiple brain cavernomas increasing in number over the following two years. She was finally diagnosed with familial cavernomatosis due to a PDCD10 mutation (CCM3).

Intramedullary spinal cavernoma: clinical presentation, microsurgical approach, and long-term outcome in a cohort of 48 patients

OBJECT Intramedullary spinal cavernoma (ISC) is a rare entity and accounts for approximately 5%−12% of all spinal vascular pathologies. The purpose of the present study was to examine the influence of clinical presentation, localization, and different surgical approaches on long-term outcome in patients treated for ISC. METHODS The authors performed a retrospective single-center study of 48 cases of ISC treated microsurgically over the past 28 years. Analyzed factors included preoperative clinical history, microsurgical strategies, neurological outcome (American Spinal Injury Association [ASIA] grade, Epstein and Cooper grade), and the occurrence of postoperative spinal ataxia. Univariate analysis was performed to identify factors influencing long-term outcome. RESULTS Preoperatively, 18.8% of all patients experienced a slow, progressive decline in neurological function and 33.3% suffered repetitive episodes of acute neurological deterioration over a time frame of months to years. Moreover, 16.7% noted the sudden onset of a severe neurological deficit, whereas 25% experienced the sudden onset of symptoms with a subsequent gradually progressive decline in neurological function. On long-term follow-up after treatment (mean ± SD, 79.3 ± 35.2 months), 70.8% of patients showed no change in neurological function, 6.3% suffered from a decline, and 22.9% improved neurologically. Thoracolumbar localization (p = 0.043), low preoperative Epstein and Cooper grade for the lower extremities (p < 0.001), and a low preoperative ASIA grade (p < 0.001) were identified as factors associated with an unfavorable outcome (ASIA Grade A-C). The rate of spinal ataxia related to surgical approach was 16.7%. CONCLUSION Postoperative neurological function in ISC patients is determined by the preoperative neurological status. On long-term follow-up after microsurgical treatment, 93.7% of patients presented with a stable or improved condition (ASIA grade); thus, definite microsurgical treatment should be considered as long as patients present with only mild symptoms after the diagnosis of symptomatic ISC.

Outcome after microsurgery in 14 patients with spinal cavernomas and review of the literature

Object Spinal cavernomas are rare, but can cause significant neurological deficits due to mass effect and extralesional hemorrhage. The authors present their results of microsurgical treatment of 14 consecutive patients with spinal cavernoma, and review the literature. Methods Of the 376 patients with cavernomas of the CNS treated at Helsinki University Central Hospital (a catchment area close to 2 million inhabitants) between January 1980 and June 2009, 14 (4%) had a spinal cavernoma. The authors reexamined and analyzed the patient files and images retrospectively. Median patient age at presentation was 45 years (range 20–57 years). The female/male ratio was equal. Median duration of symptoms before admission to the department was 12 months (range 0.1–168 months). Patients suffered from sensorimotor paresis, radicular pain, or neurogenic micturition disorders in different combinations or separately. Hemorrhage had occurred in 7 patients (50%) before surgery. In 9 patients (64%) the cavernoma was intramedullary, in 4 (29%) extradural, and in 1 intradural extramedullary. On MR imaging, 6 patients (43%) had a cavernoma in the cervical region, 7 (50%) in the thoracic region, and 1 (7%) in the lumbar region. Results Postoperatively, patients were followed up for a median of 3 years (range 1–10 years). At follow-up, 13 patients (93%) experienced significant improvement in motor ability after surgery, and all patients were able to walk with or without aid. Ten of the 11 patients with pain syndrome (91%) showed significant pain relief without recurrence. Micturition disorder was noted in 6 patients (43%) at follow-up, but in 5 the condition had existed before surgery. No patient improved in bladder function after surgery, and 1 patient developed micturition dysfunction postoperatively. Conclusions Microsurgical removal of spinal cavernomas alleviates sensorimotor deficits and pain caused by mass effect and hemorrhage. However, bladder dysfunction remains unchanged after surgery.

Cervical intradural extramedullary cavernoma presenting with isolated intramedullary hemorrhage

✓Among cavernomas of the central nervous system, spinal ones are rare. The true incidence of spinal cavernomas is unclear, but with widespread use of magnetic resonance imaging the number of cases is increasing. Furthermore, cav-ernomas represent only 5–12% of all vascular anomalies of the spinal cord, with a mere 3% reported to be intradural and intramedullary in location. Cervical spine intradural extramedullary cavernomas are very seldom seen, and only 4 cases have been reported in world literature previously. In this report, a unique case of an intradural extramedullary spinal cavernoma was surgically treated in a patient who presented only with an intramedullary hemorrhage.