Vertebral artery medullary compression syndrome (VAMCS) is a clinical diagnosis that is a combination of both imaging and symptoms. Vertebral artery ectasia and dolichoectasia resulting in medullary compression are often benign findings on imaging.^{Reference Cierpiol, Schäfer and Gossner1,Reference Tsutsumi, Nonaka, Ono and Ishii2} However, they can present as VAMCS when symptoms such as gait imbalance, weakness, quadriparesis, imbalance and a range of sensory disturbances arise in conjunction with imaging findings.^{Reference Lombarski, Kunert, Skawiński, Prokopienko, Lewandowski and Marchel3,Reference Ghannam, Berns, Salari, Moore and Brown4} Treatment of VAMCS is widely debated, and there has been a long-standing lack of consensus toward standard protocols of care because of the unique symptomology and anatomy of compression.^{Reference Savitz, Ronthal and Caplan5,Reference Maitas, Bob-Manuel, Price, Noor, Obi, Okoh, Garikapati, Kim, Jahan and Jenkins6} Most common surgical interventions include microvascular decompression, although conservative care may also be effective.

In this letter, we describe the sole four documented patients who presented to the senior author from 2013 to 2023 with VAMCS that significantly affected their quality of life. We outline their initial presenting symptoms, the surgical procedure they underwent and their outcomes. Each patient elected for microvascular decompression, and each resulted in improved gait ataxia, vertigo and subjectively improved quality of life.

The first patient was a 64-year-old female who presented with a one-year history of vertigo and light-headedness. Her relevant medical history included a moderately dilated ascending aorta, paroxysmal tachycardia and hypertension. She did not endorse any visual symptoms and had no history of falls. She had no truncal ataxia when sitting up and no appendicular ataxia on finger-to-nose or heel-to-shin testing. She maintained normal fine motor movements. She exhibited a wide and ataxic gait, was unable to do a tandem walk and elicited instability when transitioning from sitting to standing. Her MRI showed ectatic medial deviation of the bilateral vertebral arteries at the V4 segment that appeared to be causing moderate lateral compression of the medulla oblongata, without other abnormalities (Figure 1A1 and A2).

Figure 1. Axial T2-weighted MRI images for Patients 1 through 4, demonstrating vertebral artery compression of the medulla. For each patient, an inferior slice (A1, B1, C1, D1) and a corresponding superior slice through the level of maximal compression (A2, B2, C2, D2) are presented.

Our second patient was a 64-year-old female who presented with a complex two-year history of significant neurological symptoms including vertigo, reliance on a four-wheel walker due to imbalance, unsteady gait, a weak voice, left pulsatile tinnitus and the inability to perform heel-to-toe walking. Diagnostic MRI evaluations revealed pronounced left pontomedullary compression, primarily due to ectatic vertebral arteries assumed to be exerting compressive forces on the medulla and pons, with greater severity on the left side (Figure 1B1 and B2).

The third patient was a 41-year-old female patient who presented with a two-year history of intermittent episodic dizziness and vertigo, with gait ataxia and varying symptom severity. Additionally, she reported a constant bruit on the left side of her head, accompanied by a dull, persistent headache. Her MRI demonstrated an ectatic left vertebral artery compressing the anterolateral medulla (Figure 1C1 and C2).

Our final patient was a 60-year-old male patient who presented with a four-year history of disequilibrium, involuntary tremulous movements in his upper limbs and gait ataxia. He had a complex medical history, including a concussion in 2017, transient global amnesia episodes and a moderately sized left frontal hemorrhage. Initial evaluations included an MRI, which showed a left frontal hemorrhage and a dolichoectatic vertebral basilar system compressing the medulla (Figure 1D1 and D2). The patient underwent a cerebral angiogram that confirmed the vascular anatomy without additional findings.

Each of the four patients underwent similar variations of a far lateral or retrosigmoid craniotomy. A C-shaped or lazy-S incision was used; the dura was opened, and CSF was drained to relax the cerebellum. The vertebral artery was carefully exposed, and an arachnoid dissection along the length of the artery was performed for appropriate access (Figure 2B). Teflon pledgets were then used to separate the offending vessel from the medulla, and then a small amount of fibrin glue was injected over the pledget (Figure 2C). A reference for surgical anatomy can be seen in Figure 1. Patient 1 underwent bilateral decompression procedures 11 months apart. Patient 3 received two pledgets, as well as additional exposure around the seventh and eighth cranial nerve complex to rule out any additional compressive vessels due to a history of pulsatile tinnitus, but no such vessels were found. Subsequently, each patient’s dura was closed primarily and reinforced with muscle. The bone was reconstructed with MRI-compatible screws; the muscle, fascia and galea were closed; and the skin was closed with staples. All patients experienced minimal blood loss and experienced no complications intraoperatively or postoperatively.

Figure 2. Illustrative depiction of (A) normal vertebral artery anatomy as visualized during a far lateral approach craniotomy. (B) Tortuous dolichoectatic vertebral artery anatomy compressing the medulla oblongata. (C) Postsurgical placement of Teflon pledgets to relieve pressure of the vessels on the medulla.

At follow-up, the first patient stated that she had improvement in her symptoms, primarily vertigo and gait ataxia. She continues to use a cane and has a moderately imbalanced gait. She was able to resume driving and a modified work program; overall, she described her quality of life as significantly improved. Our second patient had profound improvements in her neurological function. Notably, her gait ataxia and vertigo improved, and she no longer required a walker or cane for mobility, voice strength returned and gait normalized, and she regained the ability to perform heel-to-toe walking. The third patient was able to begin attending activities of daily living and noted significant progress in her balance and ability to walk steadily but continued to have persistent ringing in her left ear. Finally, our last patient’s involuntary movements resolved, his disequilibrium improved and he regained the ability to walk steadily without the need for a cane. At subsequent follow-up visits, the patient reported continued well-being, with no recurrence of his preoperative symptoms.

VAMCS presents with a range of symptoms, which can vary greatly between patients. This letter suggests that microvascular decompression surgery can subjectively improve the quality of life for patients suffering from symptoms related to VAMCS. The persistence of some symptoms post-surgery suggests that while surgical intervention can be effective, it is not always curative. This emphasizes the continuing need for setting expectations regarding surgical outcomes, and that certain symptoms like gait ataxia and vertigo may be more likely to respond than others such as tinnitus and headaches. The absence of a consensus on treatment protocols for VAMCS is a significant challenge in clinical practice. Our outcomes support the notion that surgical management may lead to improvement in the quality of life in patients with VAMCS.