External validation of the Lawton brainstem cavernous malformation grading system in a cohort of 277 microsurgical patients

OBJECTIVE The brainstem cavernous malformation (BSCM) grading system predicts neurological outcomes associated with microsurgical resection and assists neurosurgeons in selecting patients for treatment. The predictive accuracy of the BSCM grading system should be validated in a large cohort from high-volume centers to generalize its use. METHODS An external validation cohort comprised patients with a BSCM resected by the senior author (M.T.L.) since the publication of the BSCM grading system and those resected by another neurosurgeon (R.F.S.) over a 16-year period. Size, crossing the axial midpoint, the presence of a developmental venous anomaly, patient age, and timing of last hemorrhage were used to assign BSCM grades from 0 to VII. Poor neurological outcomes were recorded as modified Rankin Scale scores > 2 at last follow-up examination. RESULTS A total of 277 patients were included in the study. The average BSCM grade was 3.9, and the majority of BSCMs (181 patients, 65%) were intermediate grade (grades III–V). Outcomes were predicted by BSCM grade, with good outcomes observed in 47 of 54 patients (87%) with low-grade BSCMs, in 135 of 181 patients (75%) with intermediate-grade BSCMs, and in 21 of 42 patients (50%) with high-grade BSCMs. Conversely, proportions of patients with neurological deterioration increased with increasing BSCM grade, with worsening observed in 2 of 54 patients (4%) with low-grade BSCMs, in 29 of 181 patients (16%) with intermediate-grade BSCMs, and in 17 of 42 patients (40%) with high-grade BSCMs. In the chi-square analysis, high-grade BSCMs were associated with increased odds of neurological worsening compared to low- and intermediate-grade BSCMs (OR 5.0, 95% CI 2.4–10.4; p < 0.001). The receiver operating characteristic analysis demonstrated acceptable discrimination for predicting unfavorable functional outcomes (modified Rankin Scale score > 2) with an area under the curve of 0.74 (95% CI 0.68–0.80; p < 0.001). CONCLUSIONS This study validates the BSCM grading system in a large cohort of patients from two high-volume surgeons. BSCM grade predicted neurological outcomes with accuracy comparable to that of other grading systems in widespread use. The BSCM grading system establishes categories of low-, intermediate-, and high-grade BSCMs and a boundary or cutoff for surgery at BSCM grade V. BSCM grading guides the analysis of a particular patient’s condition, but treatment recommendations must be individualized, and neurosurgeons must calibrate BSCM grading to their own outcome results, unique abilities, and practices.

Retrosigmoid transhorizontal fissure approach to lateral pontine cavernous malformation: comparison to transpetrosal presigmoid retrolabyrinthine approach

OBJECTIVE The retrosigmoid (RS) approach is a classic route used to access deep-seated brainstem cavernous malformation (CM). The angle of access is limited, so alternatives such as the transpetrosal presigmoid retrolabyrinthine (TPPR) approach have been used to overcome this limitation. Here, the authors evaluated a modification to the RS approach, horizontal fissure dissection by using the RS transhorizontal (RSTH) approach. METHODS Relevant clinical parameters were evaluated in 9 patients who underwent resection of lateral pontine CM. Cadaveric dissection was performed to compare the TPPR approach and the RSTH approach. RESULTS Five patients underwent the TPPR approach, and 4 underwent the RSTH approach. Dissection of the horizontal fissure allowed for access to the infratrigeminal safe entry zone, with a direct trajectory to the middle cerebellar peduncle similar to that used in TPPR exposure. Operative time was longer in the TPPR group. All patients had a modified Rankin Scale score ≤ 2 at the last follow-up. Cadaveric dissection confirmed increased anteroposterior working angle and middle cerebellar peduncle exposure with the addition of horizontal fissure dissection. CONCLUSIONS The RSTH approach leads to a direct lateral path to lateral pontine CM, with similar efficacy and shorter operative time compared with more extensive skull base exposure. The RSTH approach could be considered a valid alternative for resection of selected pontine CM.

Crossed brainstem syndrome revealing bleeding brainstem cavernous malformation: an illustrative case

Background Since the nineteenth century, a great variety of crossed brainstem syndromes (CBS) have been described in the medical literature. A CBS typically combines ipsilateral cranial nerves deficits to contralateral long tracts involvement such as hemiparesis or hemianesthesia. Classical CBS seem in fact not to be so clear-cut entities with up to 20% of patients showing different or unnamed combinations of crossed symptoms. In terms of etiologies, acute brainstem infarction predominates but CBS secondary to hemorrhage, neoplasm, abscess, and demyelination have been described. The aim of this study was to assess the proportion of CBS caused by a bleeding episode arising from a brainstem cavernous malformation (BCM) reported in the literature. Case presentation We present the case of a typical Foville syndrome in a 65-year-old man that was caused by a pontine BCM with extralesional bleeding. Following the first bleeding episode, a conservative management was decided but the patient had eventually to be operated on soon after the second bleeding event. Discussion A literature review was conducted focusing on the five most common CBS (Benedikt, Weber, Foville, Millard-Gubler, Wallenberg) on Medline database from inception to 2020. According to the literature, hemorrhagic BCM account for approximately 7 % of CBS. Microsurgical excision may be indicated after the second bleeding episode but needs to be carefully weighted up against the risks of the surgical procedure and openly discussed with the patient. Conclusions In the setting of a CBS, neuroimaging work-up may not infrequently reveal a BCM requiring complex multidisciplinary team management including neurosurgical advice.

Cheiro-oral-pedal syndrome as the presenting symptom of brainstem cavernous malformation: a case report

ABSTRACT The rare cheiro-oral-pedal syndrome (COPS) is characterized by sensory disturbances around the corner of the mouth, and in the hand and foot of the same side. The causative lesion is located in the thalamocortical projections, thalamus or brainstem and is usually due to ischemic or hemorrhagic stroke. We report a case of a patient with brain stem cavernous malformations presented as pure COPS with additional sensory disturbance in the thorax. We report this case to raise awareness of these very rare syndromes and demonstrate that mildly presenting symptoms can be caused by an underlying devastating condition.