Successful surgical management of a superior cerebellar artery aneurysm in a patient with Marfan syndrome: illustrative case

Fangjun Liu Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China

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Mengqing Hu Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China

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Daling Ruan University of Milan, Milan, Italy

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Xiaoling Ruan University of Pavia, Pavia, Italy; and

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Ting Lei Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
Department of Neurosurgery, Affiliated Fuxing Hospital, Capital Medical University, Beijing, China

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Xiang’en Shi Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China
Department of Neurosurgery, Affiliated Fuxing Hospital, Capital Medical University, Beijing, China

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BACKGROUND

Marfan syndrome, a connective tissue disorder, poses unique challenges in neurosurgery, given the fragility of vascular structures. Superior cerebellar artery (SCA) aneurysms in patients with the syndrome are rare and present distinct surgical difficulties, necessitating innovative approaches.

OBSERVATIONS

A 29-year-old male with Marfan syndrome presented with a subarachnoid hemorrhage from a ruptured SCA aneurysm. Given the lack of a defined aneurysm neck and the small diameter of the SCA, standard clipping and endovascular therapies were unsuitable. A microsurgical approach using microsutures was successfully employed, effectively managing the aneurysm while preserving the parent artery.

LESSONS

This case underscores the efficacy of the microsuture technique in complex neurosurgical scenarios, particularly in patients with connective tissue disorders such as Marfan syndrome. The adaptability of surgical strategies, as demonstrated in this case, is crucial for achieving successful outcomes in patients with unique anatomical challenges.

ABBREVIATIONS

CT = computed tomography; SAH = subarachnoid hemorrhage; SCA = superior cerebellar artery

BACKGROUND

Marfan syndrome, a connective tissue disorder, poses unique challenges in neurosurgery, given the fragility of vascular structures. Superior cerebellar artery (SCA) aneurysms in patients with the syndrome are rare and present distinct surgical difficulties, necessitating innovative approaches.

OBSERVATIONS

A 29-year-old male with Marfan syndrome presented with a subarachnoid hemorrhage from a ruptured SCA aneurysm. Given the lack of a defined aneurysm neck and the small diameter of the SCA, standard clipping and endovascular therapies were unsuitable. A microsurgical approach using microsutures was successfully employed, effectively managing the aneurysm while preserving the parent artery.

LESSONS

This case underscores the efficacy of the microsuture technique in complex neurosurgical scenarios, particularly in patients with connective tissue disorders such as Marfan syndrome. The adaptability of surgical strategies, as demonstrated in this case, is crucial for achieving successful outcomes in patients with unique anatomical challenges.

ABBREVIATIONS

CT = computed tomography; SAH = subarachnoid hemorrhage; SCA = superior cerebellar artery

Marfan syndrome, a heritable connective tissue disorder, has a range of cardiovascular, ocular, and skeletal manifestations.1 Although its association with aortic root dilation and lens dislocation is well established, neurological complications, though less common, pose significant clinical challenges. Among these, the occurrence of cerebral aneurysms, particularly in the superior cerebellar artery (SCA), remains rare but critically important. This case report highlights the complexity and therapeutic nuances in managing an SCA aneurysm in a patient with Marfan syndrome, a scenario infrequently encountered in neurosurgical practice. Through this case, we aim to shed light on the unique challenges in and effective strategies for addressing cerebral aneurysms in this patient population, emphasizing the importance of tailored neurosurgical interventions.

Illustrative Case

A 29-year-old male with a known diagnosis of Marfan syndrome experienced the sudden onset of moderate headache and vomiting after walking, persisting for 40 days. The patient was promptly taken to the local emergency room, where a computed tomography (CT) scan revealed a subarachnoid hemorrhage (SAH). Subsequent cerebral angiography confirmed the presence of a fusiform aneurysm in the SCA, along with tortuous intracranial arteries due to abnormal fibrillin production (Figs. 1 and 2). Because of the aneurysm features unfavorable for endovascular therapy, the patient was referred to our hospital for microsurgical treatment. Four years prior to this presentation, the patient had undergone cardiac valve replacement and stent treatment for acute aortic dissection, with a successful recovery.

FIG. 1
FIG. 1

Preoperative left vertebral artery angiograms, anteroposterior (A) and oblique (B) views, revealed a dissecting aneurysm (arrowheads) in the SCA. Multiple abnormal turns and significant tortuosity were observed in the left vertebral artery.

FIG. 2
FIG. 2

Preoperative CT angiogram of the bilateral carotid and vertebral arteries revealed multiple abnormal turns and significant tortuosity (arrowheads).

With the patient under general anesthesia, a left subtemporal craniotomy was performed to access the ambient cistern for surgical management of the SCA aneurysm. Intraoperatively, we discovered a ruptured aneurysm in the SCA, containing sticky blood clots (Fig. 3A) and exhibiting fragile, thin-walled characteristics. The aneurysm was meticulously approached by dissecting the structures in the peduncular fossa, along the superior basilar trunk, and under the proximal oculomotor nerve. To secure the aneurysm, 2 temporary minor clips were applied to trap the SCA, followed by 3 microsuture stitches using 9-0 Prolene, which were placed on the SCA wall close to the aneurysm wall margin (Fig. 3B). During the 3-month postoperative period, follow-up CT angiography showed a patent left SCA without the aneurysm (Fig. 4), and the patient made a complete recovery.

FIG. 3
FIG. 3

Intraoperative photographs showing a ruptured aneurysm in the peduncular fossa (A) and the sutured aneurysm (B). N. = nerve; PCA = posterior cerebral artery; V. = vein.

FIG. 4
FIG. 4

Postoperative 3-dimensional CT angiography showing obliteration of aneurysms and patent SCA (arrowhead).

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Dissecting aneurysms in patients with Marfan syndrome, although uncommon, are significant clinical concerns. These aneurysms often involve hemorrhagic complications outside the vascular lumen, primarily due to pathological transintimal extravasation of blood from the true lumen into the vessel wall. This process can lead to a dissection between the internal elastic membrane and the intima, narrowing the true lumen, or can extend into the subadventitial plane, causing an adventitial outpouching. Occasionally, such dissections result in vessel wall rupture, producing an SAH.2 Marfan syndrome, a genetically determined condition, is associated with intracranial aneurysms and SAH, although some studies have not found a significant correlation.3

Observations

Surgical or endovascular interventions are often necessary for ruptured dissecting aneurysms, given their high risk of rebleeding. In this case, the absence of a distinct aneurysm neck rendered direct clipping unfeasible. Applying direct clipping risks sacrificing a portion of the parent artery, potentially leading to significant brainstem symptoms due to a reduced arterial diameter. Endovascular treatment is equally challenging, complicated by the small diameter of the SCA and arterial tortuosity characteristic of Marfan syndrome.4 Conversely, the direct suturing technique, typically a salvage strategy for intraoperative vessel ruptures,5 has shown promise. It has been successfully applied in cases of blister-like aneurysms,6 resulting in less arterial stenosis and potentially more favorable outcomes. In dealing with dissecting aneurysms, it is crucial to be prepared with microsuture tools for both open surgery and endovascular treatments. In our case, the microsuture technique was key in addressing the management of the underlying dissecting aneurysm. With skilled application, direct microsuture can provide a definitive and durable solution, excluding the aneurysm more effectively than bypass surgery or the flow replacement strategies typically used when coiling or stenting is not a viable option for specific clinical scenarios.

The microsuture technique requires only a brief period of temporary occlusion of the parent artery, minimizing the risk of ischemic complications. Typically, 9-0 or 8-0 nylon sutures are used in an interrupted fashion, applied perpendicular to the long axis of the artery. This is done after excising the less elastic parts of the aneurysm wall, including the tunica media and internal elastic lamina. The sutures are placed at a point twice the vessel wall’s thickness from the artery–lesion junction. This method ensures complete obliteration of the aneurysm opening, usually with 2–4 stitches within 30 minutes, preserving the normal arterial wall. However, this technique is not suitable if the vascular wall is extensively damaged or if the vessel loses its tubular structure after aneurysm wall tailoring. Care must be taken to avoid overly tight sutures, which could cause arterial stenosis and lead to cerebral ischemia. Although the microsuture technique is often a secondary option when other methods fail, its success in our case demonstrates its value as a primary surgical approach, marking a significant advancement in neurosurgical techniques and offering insights for complex cases.

Lessons

Although direct clipping surgery and endovascular therapy pose challenges in treating dissecting aneurysms in Marfan syndrome, the judicious use of the microsuture technique offers a viable alternative. This approach not only preserves the parent artery but also effectively eliminates the dissecting lesion, facilitating significant patient recovery. This case underscores the microsuture technique’s potential as a valuable tool in the neurosurgical treatment of dissecting aneurysms, particularly in complex cases such as those associated with Marfan disease.

Acknowledgments

This work was supported by Natural Science Foundation of Beijing Municipality (Beijing Natural Science Foundation).

Author Contributions

Conception and design: Shi. Acquisition of data: Shi, Liu, Hu, D Ruan, Lei. Analysis and interpretation of data: Shi. Drafting the article: Shi, D Ruan, X Ruan. Critically revising the article: Shi, X Ruan. Reviewed submitted version of manuscript: Shi, Hu, X Ruan. Approved the final version of the manuscript on behalf of all authors: Shi. Statistical analysis: D Ruan. Administrative/technical/material support: Hu. Study supervision: Shi, Hu.

References

  • 1

    Spinardi L, Vornetti G, De Martino S, et al. Intracranial arterial tortuosity in Marfan syndrome and Loeys-Dietz syndrome: tortuosity index evaluation is useful in the differential diagnosis. AJNR Am J Neuroradiol. 2020;41(10):19161922.

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    • Search Google Scholar
    • Export Citation
  • 2

    Dean JCS Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet. 2007;15(7):724733.

  • 3

    Rinkel GJ Intracranial aneurysm screening: indications and advice for practice. Lancet Neurol. 2005;4(2):122128.

  • 4

    Domingo RA, Perez-Vega C, Tripathi S, et al. Intracranial aneurysms in patients with Marfan syndrome: a multicenter propensity-matched analysis. World Neurosurg. 2021;155:e345e352.

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    • Search Google Scholar
    • Export Citation
  • 5

    Liu C, Shi X, Zhou Z, et al. Microsuturing technique for the treatment of blood blister aneurysms: a series of 7 cases. World Neurosurg. 2020;135:e19e27.

  • 6

    Yanaka K, Meguro K, Nose T Repair of a tear at the base of a blister-like aneurysm with suturing and an encircling clip: technical note. Neurosurgery. 2002;50(1):218221.

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    • Search Google Scholar
    • Export Citation
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  • Expand
  • FIG. 1

    Preoperative left vertebral artery angiograms, anteroposterior (A) and oblique (B) views, revealed a dissecting aneurysm (arrowheads) in the SCA. Multiple abnormal turns and significant tortuosity were observed in the left vertebral artery.

  • FIG. 2

    Preoperative CT angiogram of the bilateral carotid and vertebral arteries revealed multiple abnormal turns and significant tortuosity (arrowheads).

  • FIG. 3

    Intraoperative photographs showing a ruptured aneurysm in the peduncular fossa (A) and the sutured aneurysm (B). N. = nerve; PCA = posterior cerebral artery; V. = vein.

  • FIG. 4

    Postoperative 3-dimensional CT angiography showing obliteration of aneurysms and patent SCA (arrowhead).

  • 1

    Spinardi L, Vornetti G, De Martino S, et al. Intracranial arterial tortuosity in Marfan syndrome and Loeys-Dietz syndrome: tortuosity index evaluation is useful in the differential diagnosis. AJNR Am J Neuroradiol. 2020;41(10):19161922.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Dean JCS Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet. 2007;15(7):724733.

  • 3

    Rinkel GJ Intracranial aneurysm screening: indications and advice for practice. Lancet Neurol. 2005;4(2):122128.

  • 4

    Domingo RA, Perez-Vega C, Tripathi S, et al. Intracranial aneurysms in patients with Marfan syndrome: a multicenter propensity-matched analysis. World Neurosurg. 2021;155:e345e352.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Liu C, Shi X, Zhou Z, et al. Microsuturing technique for the treatment of blood blister aneurysms: a series of 7 cases. World Neurosurg. 2020;135:e19e27.

  • 6

    Yanaka K, Meguro K, Nose T Repair of a tear at the base of a blister-like aneurysm with suturing and an encircling clip: technical note. Neurosurgery. 2002;50(1):218221.

    • PubMed
    • Search Google Scholar
    • Export Citation

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