Brainstem cavernous malformation (BSCM)

Symptomatic brainstem cavernous malformations carry a high risk of permanent neurological deficit related to recurrent hemorrhage, which justifies aggressive management. Detailed knowledge of the microscopic and surface anatomy is important for understanding the clinical presentation, predicting possible surgical complications, and formulating an adequate surgical plan 1).

The natural history of brainstem cavernous malformation is particularly complex. Currently, there is a wide range of reported annual rates of hemorrhage.

A systematic review and meta-analysis of 25 studies show that the incidence of symptomatic hemorrhage or rehemorrhage is higher in brainstem lesions. First symptomatic hemorrhage increases the chance of symptomatic rehemorrhage, which decreases after 2 years 2). But reported variance in the literature may also be due to study limitations along with selection, patient and disease-specific; follow-up; and recall bias. An accurate assessment of hemorrhagic risk along with evaluation of patient and lesion-specific characteristics is critical in the decision-making process for potential intervention, as microsurgical intervention can significantly decrease the risk of future hemorrhage, but may be associated with significant complications 3).

Medulla Oblongata cavernous malformation.

Pontine cavernous malformation….

Rather than developing a grading system for all cerebral cavernous malformations that is weak with BSCMs, Garcia et al. propose a system for the patients who need it most. The BSCM grading system differentiates patients who might expect favorable surgical outcomes and offers guidance to neurosurgeons forced to select these patients 4).

Brainstem cavernous malformation diagnosis.

see Brainstem cavernous malformation treatment.

Brainstem cavernous malformations are associated with a considerable risk of hemorrhage and subsequent morbidity, with significant focal impairment caused by hemorrhages leading to facial nerves damage 5).

Favorable surgical outcomes can be predicted in brainstem CM patients with early age at presentation, pontine cavernous malformationlocation of the cavernoma, favorable preoperative mRS and those undergoing early surgery. The outcomes at long-term follow-up were associated with location of the CM in the brainstem, size of the CM and the preoperative mRS 6).

Patients who had undergone surgery of symptomatic BSCMs were evaluated pre- and postoperatively both neurologically and neuroradiologically supplemented by telephone interviews. Additionally, patients were scored according to the Scandinavian Stroke Scale. Multiple uni- and multivariate analyses of possible clinical and radiological prognostic factors were conducted. The study population comprised 35 patients. Mean age at operation was 39.3 ± 13.0 years with microsurgical resection of a total of 37 different BSCMs between 2002 and 2011. Median clinical follow-up was 44.0 months (range 8-116 months). Postoperative MRI showed eventually complete resection of all BSCMs. Postoperative overall outcome revealed complete resolution of neurological symptoms for 5/35 patients, 14/35 improved and 9/35 remained unchanged. 7/35 suffered from a postoperative new and permanent neurological deficit, mostly affecting the facial nerve or hemipareses with mild impairment. Pre- and postoperative Scandinavian Stroke Scale scores were 11.0 ± 2.4 and 11.4 ± 2.2 (p = 0.55). None of the analyzed factors were found to significantly correlate with patients' clinical outcome. Complete resection of brainstem cavernous malformations can be achieved with an acceptable risk for long-term morbidity and surgery-related new deficits (~20 %). Neurological outcome is mainly determined within the first 6 months after surgery 7).

Brainstem Cavernous Malformation Case Series.

A case of a 42-year-old man with a brainstem cavernous hemangioma presenting with fever of unknown origin and mild headache without meningismus. The patient underwent a midline suboccipital craniectomy and removal of a ruptured brainstem cavernous hemangioma and the surrounding thrombus. Postoperatively, the patient developed left facial nerve palsy, left abducens nerve palsy, and xerostomia. Abducens palsy and xerostomia resolved spontaneously days after the operation. At the 6-month follow-up, the patient showed stable improvement with resolution of his neurological deficits. CONCLUSIONS To our knowledge, there is no reported case of a patient with a ruptured brainstem cavernoma presenting with fever of unknown origin as the main symptom. We assume that the minimal intraventricular hemorrhage triggered the hypothalamic thermoregulating mechanism. Thus, it would be useful for physicians to raise the suspicion of a ruptured brainstem cavernous malformation with further imaging evaluation when investigating fever of unknown origin 8).

Giliberto G, Lanzino DJ, Diehn FE, Factor D, Flemming KD, Lanzino G. Brainstem cavernous malformations: anatomical, clinical, and surgical considerations. Neurosurg Focus. 2010 Sep;29(3):E9. doi: 10.3171/2010.6.FOCUS10133. Review. PubMed PMID: 20809767.
Taslimi S, Modabbernia A, Amin-Hanjani S, Barker FG 2nd, Macdonald RL. Natural history of cavernous malformation: Systematic review and meta-analysis of 25 studies. Neurology. 2016 Apr 22. pii: 10.1212/WNL.0000000000002701. [Epub ahead of print] PubMed PMID: 27164680.
Starke RM. Do brainstem cavernous malformations have a higher rate of hemorrhage? Expert Rev Neurother. 2015 Jul 18:1-3. [Epub ahead of print] PubMed PMID: 26189553.
Garcia RM, Ivan ME, Lawton MT. Brainstem cavernous malformations: surgical results in 104 patients and a proposed grading system to predict neurological outcomes. Neurosurgery. 2015 Mar;76(3):265-78. doi: 10.1227/NEU.0000000000000602.PubMed PMID: 25599205.
Sindou M, Yada J, Salord F. Functional results after microsurgical resection of brainstem cavernous malformations (retrospective study of a 12 patient series and review of the recent literature). Acta Neurochir (Wien). 2000;142(8):843-52; discussion 852-3.
Chotai S, Qi S, Xu S. Prediction of outcomes for brainstem cavernous malformation. Clin Neurol Neurosurg. 2013 Oct;115(10):2117-23. doi: 10.1016/j.clineuro.2013.07.033. Epub 2013 Aug 6. PubMed PMID: 23962756.
Schwartz C, Grillhösl A, Schichor C, Suchorska B, Romagna A, Tonn JC, Zausinger S. Symptomatic cavernous malformations of the brainstem: functional outcome after microsurgical resection. J Neurol. 2013 Nov;260(11):2815-22. doi: 10.1007/s00415-013-7071-3. Epub 2013 Aug 22. PubMed PMID: 23974645.
Fermeli DD, Theofanopoulos A, Papadakos D, Boulieris S, Constantoyannis C. Hemorrhage of a Cavernous Hemangioma of the Brainstem Presenting with Fever of Unknown Origin: A Case Report. Am J Case Rep. 2021 May 25;22:e930437. doi: 10.12659/AJCR.930437. PMID: 34031354.
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