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

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.

A Transcriptomic Comparative Study of Cranial Vasculature.

In genetic studies of cerebrovascular diseases, the optimal vessels to use as controls remain unclear. Our goal is to compare the transcriptomic profiles among 3 different types of control vessels: superficial temporal artery (STA), middle cerebral arteries (MCA), and arteries from the circle of Willis obtained from autopsies (AU). We examined the transcriptomic profiles of STA, MCA, and AU using RNAseq. We also investigated the effects of using these control groups on the results of the comparisons between aneurysms and the control arteries. Our study showed that when comparing pathological cerebral arteries to control groups, all control groups presented similar responses in the activation of immunological processes, the regulation of intracellular signaling pathways, and extracellular matrix productions, despite their intrinsic biological differences. When compared to STA, AU exhibited upregulation of stress and apoptosis genes, whereas MCA showed upregulation of genes associated with tRNA/rRNA processing. Moreover, our results suggest that the matched case-control study design, which involves control STA samples collected from the same subjects of matched aneurysm samples in our study, can improve the identification of non-inherited disease-associated genes. Given the challenges associated with obtaining fresh intracranial arteries from healthy individuals, our study suggests that using MCA, AU, or paired STA samples as controls are feasible strategies for future large-scale studies investigating cerebral vasculopathies. However, the intrinsic differences of each type of control should be taken into consideration when interpreting the results. With the limitations of each control type, it may be most optimal to use multiple tissues as controls.

Long-term outcomes after a transcranial microsurgical approach to craniopharyngiomas: a 20-year clinical follow-up study.

Craniopharyngioma (CP) is a mostly benign tumor that is nonetheless one of most formidable skull base lesions. CP tends to recur, and scarce clinical results are available regarding its long-term outcomes. From February 1996 to April 2002, craniopharyngiomas primarily resected by open surgery in a single-center and single-surgeon practice were screened. Medical information regarding patients' baseline, tumor parameters, endocrinological results, complications, and quality of life in those patients with a follow-up longer than 20 years were reviewed. Nineteen out of 187 patients who met the inclusion criteria were eligible, and 78.9% of the patients were admitted due to visual deficits. The transcranial approach was mostly applied for the first attempt of opening resection, with 94.7% gross total resection. The size of the tumor ranged from 25 to 45 mm with a mean maximal diameter of 34.7 mm. Although 7 out of 19 patients received an extra procedure, 6 patients (31.5%) regained fertility, with 3 women bearing a total of 5 children and 3 men fathering a total of 4 children, during the 21.4-year follow-up (range: 20.0-23.3 years). The mean Karnofsky Performance Status (KPS) score was 97.9 (range: 80-100) at the last follow-up, and the physical and mental 36-Item Short Form Health Survey (SF-36) scores were 88.0 and 80.5, respectively. The tumor sizes of the patients who regained fertility were not significantly different from those of the patients who remained infertile (t = 1.29, P > 0.2). The time interval from prior surgery to tumor resection for recurrent cases ranged from 0.3 to 17.4 years (mean, 7.3 years). There was no significant difference in the time until tumor recurrence in the patients who underwent a second surgery, a third surgery, or a fourth surgery. The satisfactory results in the present study revealed favorable long-term outcomes following the transcranial management of CPs, with acceptable endocrinological function and tumor-free survival. A decisive policy of open surgery with the objective of radical tumor removal remains a valid method for the primary treatment of CPs, aiming to avoid retreatment after tumor recurrence involving vital hypothalamic structures or hydrocephalus.

Treatment of chronic internal carotid artery occlusion by ipsilateral external carotid endarterectomy.

Chronic complete occlusion of the internal carotid artery (ICA) is a common and important cause of ischemic cerebrovascular disease, which can be treated in many ways: antithrombotic and anticoagulant agents, direct or indirect procedure for revascularization. However, there is still no evidence to prove which method is the most effective. Here we reported a case showed the successful endarterectomy of the external carotid artery (ECA) for a chronic complete occlusion of ICA patient. A 50-year-old male patient presented with progressively aggravated left-sided limb weakness and limb cold feeling. Digital subtraction angiography (DSA) showed ICA occlusion and ipsilateral ECA stenosis. The right ECA compensated for blood supply to the middle cerebral artery (MCA) through the accessory meningeal artery and ophthalmic artery. The patient was treated by endarterectomy of ipsilateral ECA (ECEA). In the second day after operation, the symptom was improved significantly. Surprisingly, both ICA and ECA were recanalized and the metabolism of the affected brain tissue was also increased. In conclusion, patients with chronic complete occlusion, ECEA would also be an effective therapeutic method.

How I do it: internal maxillary artery to middle cerebral artery bypass to manage giant thrombosed internal carotid artery aneurysm.

BACKGROUND: Internal maxillary artery (IMA) bypass has become popularized due to its medium-to-high blood flow, short graft length, and well-matched arterial caliber between donor and recipient vessels. METHOD: We described an open surgery of a NEW "workhorse," the IMA bypass, to treat a giant, thrombosed cerebral aneurysm. The extracranial middle infratemporal fossa (EMITF) approach was used to unveil the pterygoid segment of the IMA for cerebral revascularization. CONCLUSION: Although this technique is technically challenging, the variations in IMA can be effectively identified and sufficiently exposed in this technique to achieve favorable clinical outcomes with a high bypass patency rate.

Somatic Variants in SVIL in Cerebral Aneurysms.

Background and Objectives: While somatic mutations have been well-studied in cancer, their roles in other complex traits are much less understood. Our goal is to identify somatic variants that may contribute to the formation of saccular cerebral aneurysms. Methods: We performed whole-exome sequencing on aneurysm tissues and paired peripheral blood. RNA sequencing and the CRISPR/Cas9 system were then used to perform functional validation of our results. Results: Somatic variants involved in supervillin (SVIL) or its regulation were found in 17% of aneurysm tissues. In the presence of a mutation in the SVIL gene, the expression level of SVIL was downregulated in the aneurysm tissue compared with normal control vessels. Downstream signaling pathways that were induced by knockdown of SVIL via the CRISPR/Cas9 system in vascular smooth muscle cells (vSMCs) were determined by evaluating changes in gene expression and protein kinase phosphorylation. We found that SVIL regulated the phenotypic modulation of vSMCs to the synthetic phenotype via Krüppel-like factor 4 and platelet-derived growth factor and affected cell migration of vSMCs via the RhoA/ROCK pathway. Discussion: We propose that somatic variants form a novel mechanism for the development of cerebral aneurysms. Specifically, somatic variants in SVIL result in the phenotypic modulation of vSMCs, which increases the susceptibility to aneurysm formation. This finding suggests a new avenue for the therapeutic intervention and prevention of cerebral aneurysms.

Computed Tomography Perfusion Imaging Study of Intracranial Complex Aneurysms Treated by Internal Maxillary Artery Bypass Grafting.

BACKGROUND: Cerebral revascularization strategies through extracranial to intracranial bypass have been adopted in the management of complex intracranial aneurysms. The internal maxillary artery used as a donor in a bypass is an effective method. At present, there are few quantitative analyses of cerebral blood flow perfusion. The main focus of this study was to evaluate the effectiveness of blood perfusion after bypass grafting. METHODS: From April 2015 to December 2017, 19 patients who underwent internal maxillary artery radial artery middle cerebral artery bypass surgery with unobstructed bypass vessels were selected. Cerebral blood flow perfusion before and after bypass surgery was quantitatively evaluated by computed tomography perfusion imaging. The cerebral blood perfusion in the region of interest was measured by computed tomography perfusion. RESULTS: The aneurysms were excised after trapping in 2 cases with mass effects and neural compression. Proximal occlusion of the parent artery was performed in 9 cases of fusiform or giant dissecting aneurysms. Trapping was performed after bypass surgery in 8 cases. Within 3 months after surgery, 17 patients had good outcomes. After the hypothesis test, there was a significant difference between the preoperative △cerebral blood volume and postoperative △cerebral blood volume in the anterior area of the semioval center cross section (P = 0.001 < 0.05). CONCLUSIONS: The internal maxillary artery as a bypass donor is an effective method that can provide sufficient intracranial blood perfusion, and there is usually no cerebral ischemia in the surrounding area.

Maximal Resection of Tumors Encasing the Internal Carotid Artery and Hindering Internal Carotid Artery Expansion Followed by Revascularization Surgery: A Series of Nine Cases at a Single Tertiary Center.

PURPOSE: Cerebral reconstruction appears to play a diminished role in managing complex skull base tumors involving vital neurovascular structures. MATERIALS AND METHODS: Patients with recurrent or progressive middle cranial fossa tumors treated by radical resection followed by extracranial-to-intracranial (EC-IC) bypass from 2014 to 2019 were included. Balloon test occlusion (BTO) was performed preoperatively. RESULTS: Overall, 9 patients (5 males, 4 females; mean age, 29.9 years) were enrolled. The lesions arose from the parasellar region (3), cavernous sinus (3), petroclival region (2), or orbital apex (1), and all encased the cavernous/petrous portion of the internal carotid artery. Before tumor resection, internal maxillary artery (IMA) bypass was performed for 7 patients, cervical EC-IC bypass was performed for 1 patient, and interposed superficial temporal artery (STA) bypass was performed for 1 patient. BTO failed in 8 patients and was tolerated by one patient. Intraoperative blood flow of the interposed graft was 79.7 ± 37.86 ml/min after IMA bypass, 190.6 ml/min following cervical EC-IC bypass and 75 ml/min after interposed STA bypass. All bypasses were patent on intraoperative indocyanine green angiography. Radical tumor resection was achieved in 5 patients (55.6%), and patency was confirmed postoperatively in 88.8% (8/9) of bypasses. Six patients showed favorable outcomes at discharge. At the 2-year follow-up, 7 patients (77.8%) had favorable outcomes (Karnofsky Performance Scale score>80). At the 1.5-year follow-up, one patient had died due to infarction; at the 3-year follow-up, another patient had developed tumor recurrence despite being asymptomatic. CONCLUSION: Cerebral bypass remains a vital tool for managing select middle cranial fossa tumors that invade or erode the surrounding neurovasculature or hinder carotid artery expansion and are difficult to resect.

Aggressive resection of craniopharyngioma achieved by drilling the tuberculum sellae/planum sphenoidale using a frontal basal interhemispheric approach: A review of 55 cases.

OBJECTIVE: The frontal basal interhemispheric approach (FBIA) is preferable for resection of craniopharyngioma (CP), achieving desirable total resection rates in early reports of lesions located in the suprasellar region to the third ventricle. For tumours that have created a larger obstruction of the tuberculum sellae and planum sphenoidale, aggressive resection in the intrasellar region and medial wall of the cavernous sinus is not feasible compared to improving tumour visualization by drilling the tuberculum sellae and planum sphenoidale. In a report of drilling the sellar tuberculum and sphenoid planum, drilling allowed the direct visualization of tumours invading the intrasellar region and medial wall of the cavernous sinus. Reconstructing the opening of the sellar-sphenoid cavity is achieved by microsuturing a piece of the pericranium/dura around the dural edge of the defective dura of the open sphenoid sinus and sellar cavity to prevent cerebrospinal fluid (CSF) leakage. PATIENTS AND METHODS: The FBIA with drilling of the tuberculum sellae and planum sphenoidale was performed to remove the tumours that invaded the intrasellar region and cavernous sinus in 55 patients from January 2014 to October 2019 at our institution. The pre- and postoperative pituitary hormone levels and vision were evaluated as effective standards after surgery and compared using paired t-tests. The different rates of CSF leakage between the packing and microsuture groups were compared by χ2 test, p < 0.05. RESULTS: In all patients with a mean 37-month follow-up (range, 3-2 months), 43 (78.2%) patients returned to their normal life or school independently, 7 (12.7%) patients were able to perform normal activities with minor complaints or effort, and 4 (7.3%) patients could care for themselves or only required occasional assistance. One (1.8%) death occurred, attributed to CSF leak-related meningitis at 5 months after surgery. Postoperative CSF leakage occurred in eight (19.0%) of 42 patients with packed bone wax or pieces of muscle to the sphenoid sinus. Of 13 patients with a piece of the periosteum/dura microsutured around the defective dura of the sellar region and open sphenoid sinus, one (7.7%) of 13 patients experienced CSF leakage in the perioperative period. With statistical analysis, there was a potential risk for postoperative CSF leakage in the bone wax and muscle piece in the open sphenoid sinus, whereas microsuture manoeuvres were effective for avoiding the risk of postoperative CSF leakage (χ2 = 8.865, p < 0.005). The microsutures closed the open sphenoid sinus such that it was water-tight. Postoperative visual acuity and the visual field were not affected by the increased intrasellar exposure or the open sphenoid sinus achieved by drilling the tuberculum sellae and planum sphenoidale. CONCLUSION: Tuberculum sellae/planum sphenoidale drilling via FBIA is feasible to enhance the direct visualization of CP resection, which expands the intrasellar region with a direct resection of recurrent tumours in the sellar cavity and adhering to the medial wall of the cavernous sinus. The potential risk of a CSF leakage seemed to be mitigated when using water-tight microsutures on a piece of the pericranium/dura around the edge of the defective dura in the sellar region and the open sphenoid sinus cavity.

Microsuturing Technique for the Treatment of Blood Blister Aneurysms: A Series of 7 Cases.

BACKGROUND: A blood blister aneurysm (BBA) is an abnormal bulge at the nonbranching point of a vessel. However, the optimal treatment strategy for this formidable disorder remains unknown. The aim of this study was to evaluate the safety and validity of using a direct microsurgical repair technique in BBAs. METHODS: Direct microsuturing of aneurysms was performed with nylon thread in 7 patients from 2014-2018. Postoperative angiography was used to confirm the obliteration of the aneurysms and the absence of stenosis of the parent artery. Neurologic function was assessed by the modified Rankin Scale. RESULTS: Two male and 5 female patients with a mean age of 43.7 years (range, 29-62 years) were assessed. Subarachnoid hemorrhage occurred in 6 patients, including 4 patients with Hunt-Hess grade II and 2 patients with grade III. BBAs of the internal carotid artery were observed in 3 patients, BBAs of the middle cerebral artery trunk were observed in 2 patients, and a BBA of the anterior communicating artery was observed in 1 patient. One BBA of the anterior communicating artery in 1 patient was detected incidentally during the resection of a craniopharyngioma. All BBAs were closed with blood-tight sutures via standard frontotemporal craniotomies. Postoperatively, all BBAs were completely eliminated from the circulation without stenosis of the sutured parent vessel. CONCLUSIONS: The proposed microsuture technique appears to be a safe, cost-effective, durable treatment for BBAs in the anterior circulation, and should be a part of the arsenal of neurosurgical practitioners who treat anterior circulation BBAs.

Cerebral glucose metabolism changes in chronic ischemia patients following subcranial-intracranial bypass.

The use of the internal maxillary artery (IMA) in intracranial artery bypass or subcranial-intracranial (SC-IC) bypass has recently been described as an alternative to traditional bypass. This study explores cerebral glucose metabolism characteristics of SC-IC bypass. Ten crescendo transient ischemic attack (TIA) patients with chronic occlusion of the middle cerebral artery (MCA) received bypass surgery of IMA with the radial artery graft (RAG) to the branch of MCA. The graft's flow volume (FV) was measured by operative intraoperative duplex ultrasonography. Positron emission tomography (PET)/computed tomography (CT) was used to calculate the preoperational and postoperational average of the standard uptake value (SUVavg) of the 18-fluoro-2-deoxy-D-glucose (18F-FDG) in the region of interest (ROI). The asymmetric index (AI) is recommended to reflect the SUVavg changes, and subsequently, cerebral glucose metabolism changes are supposedly clarified. Patent IMA-RAG-MCA bypass in ten chronic ischemia patients was confirmed by angiography after surgery. The intraoperative FV measurement value was 65.64 ± 10.52 (58.11-73.17) ml/min. Before the operation, the SUVavg of the ROI in the ischemic hemisphere (4.76 ± 2.35 (3.08-6.04)) clearly decreased compared to the one (5.99 ± 2.63 (4.11-7.87)) in the contralateral mirror region (P = 0.003). The result of AI of preoperation minus AI of postoperation was more than 10% (P = 0.031), which indicated suspicious significant changes in cerebral metabolism. All symptoms of study patients having crescendo ischemia were resolved in 1 month after the operation. In the cerebral hypoperfusion territory, uptake of 18F-FDG deceased. Improving the flow volume via SC-IC bypass makes available an elevated uptake of 18F-FDG.

Natural history and clinical outcomes in patients with complex intracranial aneurysms: a review of 115 bypass cases and 22 nonsurgical cases.

The purpose of this study was to evaluate the natural history of patients with these heterogeneous aneurysms to provide guidance for their treatment. This retrospective analysis was performed at a single institution and included 137 patients with complex intracranial aneurysms who underwent a natural history evaluation. Among the 115 patients who underwent bypass surgery, stroke (n = 39, 33.9%) was the most common clinical symptom followed by progressively severe headaches (n = 25, 21.7%). Of the 104 patients with follow-up information, 87 (83.7%) returned to a normal life within a mean follow-up of 4.17 ± 2.09 years. Ten deaths (9.6%) occurred after a mean of 1.3 ± 0.9 years. Among the 22 patients who selected nonsurgical treatment, mass effect (n = 9, 40.9%) was the most common clinical presentation, and 14 deaths (63.6%) occurred after a mean of 3.3 ± 2.5 years. The modified Rankin scale (mRS) scores of 5 survivors (5/22, 22.7%) progressed from 0-2 at initial presentation to 3-4. Bypass surgical treatment for these aneurysms appears to be effective and can achieve good clinical outcomes without additional limitations related to individual aneurysms despite the impact of recent endovascular techniques on vascular surgery.

Serpentine aneurysm of the posterior cerebral artery treated by internal maxillary artery bypass followed by parent artery occlusion: a case report and literature review.

Serpentine aneurysms of the posterior cerebral artery (PCA) treated by the internal maxillary artery (IMA) bypass are rare. Here, the authors report the case of a 34-year-old male patient who presented with a half-year history of gradual severe headache and right-sided limb monoparesis and paresthesia lasting for 1 week. Preoperative angiograms showed a serpentine aneurysm in the left distal PCA, which was treated with internal maxillary artery-radial artery-posterior cerebral artery (IMA-RA-PCA) bypass followed by parent artery occlusion (PAO). The postoperative course was uneventful; radiological images revealed that the aneurysm disappeared, and there was good graft patency and excellent perfusion of the distal PCA territories. To the authors' knowledge, this is the first and only case of distal PCA serpentine aneurysm to be treated by IMA-RA-PCA bypass followed by proximal PAO. These findings suggest that IMA bypass surgery is a good and feasible treatment option for serpentine aneurysms of the PCA that can preserve the parent artery. Moreover, the anatomic segments of the PCA and different treatment options available for PCA serpentine aneurysms are also discussed in this study.

Intraoperative Finding of an Anterior Communicating Artery Blister-Like Aneurysm During a Primary Craniopharyngioma Resection: Accidental or Incidental?

BACKGROUND: The presence of an anterior communicating artery blister-like aneurysm in the setting of a craniopharyngioma has never been reported to our knowledge. CASE DESCRIPTION: This patient was admitted to our service for an untreated craniopharyngioma resection. An anterior interhemispheric approach with right frontal craniotomy was performed and a blister-like aneurysm of the anterior communicating artery was found during the surgery. After the tumor was completely resected, a suturing technique was used for the aneurysm. In this article, we are going to present this rare case and discuss the potential pathogeny of the aneurysm. CONCLUSIONS: We hypothesized that cystic craniopharyngioma may rupture spontaneously. The cystic fluid accumulated in the lower subarachnoid space due to gravity, and it may lead to local vascular cytotoxic and inflammatory reactions which may result in the vascular wall remodeling and lead to the reconstructed vascular wall weakness.

Distal Outflow-Related Anterior Inferior Cerebellar Artery Aneurysm in Hemangioblastoma.

Anterior inferior cerebellar artery (AICA) aneurysm is a rare cerebral entity, and its presentation in association with a hypervascularized tumor is even more exceptional. In this study, we describe a patient with rare coexistent pathologic conditions in combination with a cerebellar hypervascularized tumor with an outflow-related AICA aneurysm. A 1-stage procedure to remove both lesions was carried out after meticulous discussion with patient. Postoperative imaging demonstrated no residual aneurysm and total resection of the tumor, which was pathologically diagnosed as hemangioblastoma (HB). She made an excellent neurologic outcome and was discharged in unremarkable condition. At her 3-month follow-up visit, her condition remained stable for vasculopathologic states and neurologic functions. To our knowledge, this is the first published clinical experience regarding cerebellar HB coexistent with an AICA aneurysm that involved the efferent segment of the tumor's feeding vessel. Patients with coexistent vascular pathologic conditions should be aggressively treated, not by the wait-and-see practice, in case of unexpected rupture in the future.