Guilin Li

Clinical and Pathological Features of Intradural Retroclival Chordoma

OBJECTIVE To investigate the clinical and pathologic characteristics of primary intradural retroclival chordoma and improve the understanding of this rare disease. METHODS A retrospective study was conducted on six cases patients withof an intradural chordoma in the retroclival region who underwent surgery and were confirmed by pathology and imaging. Expression of brachyury, galectin-3, and Ki-67 in paraffin-embedded sections of the specimens was detected by streptavidin-peroxidase immunohistochemistry. RESULTS Lesions were located in the subdural prepontine cistern. Computed tomography scan showed that the bone of the skull base was not destroyed, and findings on magnetic resonance imaging varied, resulting in a misdiagnosis of 50% of the cases in the preoperative imaging. All six cases were classified by their pathology into the classical subtype. They presented a strong positive staining for brachyury and galectin-3, and the Ki-67 labeling index was between 2.5% and 8.2%. Three cases presented no signs of recurrence or regrowth, whereas in the other three patients recurrence or regrowth occurred at 7 ∼ 14 months after initial surgery. Two patients died of this disease. CONCLUSIONS Our study suggests that a positive staining for brachyury, galectin-3, and Ki-67 would be helpful for differential diagnosis, discriminating intradural retroclival chordoma from ecchordosis physaliphora and chordoid meningioma. Our study also shows that within intradural retroclival chordoma, there are significant prognostic differences. Tumors with an abundant blood supply, flake-like cellular arrangement, and a Ki-67 labeling index greater than 5% belong to a rapid-growth type and are prone to short-term recurrence and poorer prognosis.

Primary Adult Infradiaphragmatic Craniopharyngiomas: Clinical Features, Management, and Outcomes in One Chinese Institution

OBJECTIVE This study was designed to evaluate the clinical, radiologic, histologic, and surgical outcome characteristics of this disease treated in a single institution. METHODS Sixteen adult patients underwent transsphenoidal microsurgery from October 2005 to December 2010 at Neurosurgical Center of Beijing Tiantan Hospital. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. RESULTS Pituitary dysfunction was presented in 12 patients, visual acuity and/or field deterioration in 11 patients, and headache in 8 patients. Hyperprolactinemia was presented in 7 of 9 female patients. All lesions were resected by transsphenoidal microsurgery as the primary procedure. A gross total resection was achieved in 3 of 16 patients, a radical subtotal resection in the remaining 13 patients. Nine cases were histologically classified as adamantinous subtype. After a mean follow-up of 50 months, 2 patients experienced recurrence. All female patients who had hyperprolactinemia experienced a gain of function postoperatively. Six patients experienced new diabetes insipidus. Visual field improved or normalized in 8 of 9 patients. Visual acuity improved in 1 case, and worsened in 1 patient. CONCLUSIONS Primary adult infradiaphragmatic craniopharyngiomas are relatively rare lesions occurring in young adults. Pituitary dysfunction, visual acuity and/or field deterioration, and headache were the most common chief symptoms. Transsphenoidal surgery, including tearing the cyst walls off the diaphragma sellae and protecting normal pituitary tissue as much as possible, is recommended. Although at the risk of impairing the function of anterior pituitary, transsphenoidal surgery results in a high rate of both visual field and hyperprolactinemia improvement with a low associated risk of recurrence. In terms of pathological aspects, the adamantinous subtype was more common.

Concomitant Lumbosacral Perimedullary Arteriovenous Fistula and Spinal Dural Arteriovenous Fistula

BACKGROUND Although multifocal spinal arteriovenous malformations (AVMs) have been reported before, the present case is the first case of 2 different types, including 1 perimedullary arteriovenous fistula and 2 spinal dural arteriovenous fistulas of lumbosacral AVMs, coexisting in 1 patient. We also report the use of hybrid techniques in treatment of concomitant lumbosacral spinal AVMs. CASE DESCRIPTION A 65-year-old man presented with a 4-year history of progressive sensory, motor, and sphincter dysfunction. Spinal magnetic resonance imaging and digital subtraction angiography showed 2 spinal dural arteriovenous fistulas (fed by the right L2 lumbar artery and the right lateral sacral artery, respectively) and 1 perimedullary arteriovenous fistula (fed by the filum terminale artery from the left L2 lumbar artery [i.e., filum terminale arteriovenous fistulas]. A hybrid technique was used to perform embolization of the right L2 spinal dural arteriovenous fistula and microsurgery of the L5 level filum terminale vein. The patient was asymptomatic 1 year later. CONCLUSIONS Multifocal spinal vascular malformations may coexist in 1 case, and standardized spinal digital subtraction angiography, including the bilateral internal iliac arteries and median sacral artery, should be performed to avoid a missed diagnosis. The concomitant phenomenon indicates that venous hypertension may be a risk factor for the development of arteriovenous fistulas. Hybrid techniques are effective in treatment of multifocal and complex spinal AVMs.

Dural Arteriovenous Fistulas at the Petrous Apex

OBJECTIVE Dural arteriovenous fistulas (DAVFs) at the petrous apex are rare but may cause subarachnoid hemorrhage (SAH) or severe brainstem edema. This study aimed to summarize their clinical features and discuss the classification. METHODS During a 15-year period, 64 consecutive patients with DAVF at the petrous apex were reviewed. According to their angioarchitecture, these cases were classified as follows: type I, no venous ectasia (48.4%); type II, venous ectasia but with normal vein proximal to the fistula (29.7%); and type III, venous ectasia at the site of the fistula (21.9%). RESULTS There were 53 men and 11 women included. Presented symptoms were SAH in 8 patients (12.5%), nonhemorrhagic neurologic defects (NHNDs) in 53 patients (82.8%), and no symptoms in 3 patients (4.7%). There were 49 patients who received transarterial embolization, 8 patients who received microsurgery, and 7 patients who received embolization and microsurgery. Complications occurred in 9 patients (14.1%), including transient cranial nerve palsy (4.7%), rebleeding (6.3%), and respiratory failure (3.1%). Of the type I patients, 96.77% presented with NHNDs and 77.42% presented with infratentorial drainage. However, SAH occurred more often in type II (21.05%)/type III cases (28.57%), and most patients carried a supratentorial drainage (63.16% and 85.71%, respectively). In different types of DAVFs, the necessity for embolization combined with microsurgery (6.45% in type I, 10.53% in type II, 21.43% in type III) and the occurrence of rebleeding complications (0% in type I, 10.53% in type II, and 14.29% in type III) were varied. CONCLUSIONS Petrous apex DAVFs carried a high risk of embolization-related complications. Based on the vascular architecture, this classification may reflect their clinical features and provide some advice on the treatment of DAVFs at the petrous apex.

Dural Arteriovenous Fistulas at the Petrous Apex with Pial Arterial Supplies

OBJECTIVE Dural arteriovenous fistula (DAVF) at the petrous apex with pial arterial supplies possesses complicated angioarchitecture, associates with aggressive neurologic behaviors, and associates with high risk of complications. This study aimed to summarize our experience with this type of DAVF in the last 15 years. METHODS This retrospective study reviewed 26 consecutive patients diagnosed with DAVFs of the petrous apex with pial arterial supplies from February 2002 to June 2017. Clinical information was extracted from medical records, radiographic data, intraoperative images, and complications and follow-up records. RESULTS The 26 patients included 24 men and 2 women, with a mean age of 46.54 ± 12.49 years (range, 28-73 years). Manifestations included nonhemorrhagic neurologic defects (19/26, 73.1%), subarachnoid hemorrhage (SAH) (5/26, 19.2%), and no symptoms (2/26, 7.7%). Supratentorial draining direction was detected in 89.47% of the lesions, and 80% of the patients with venous ectasia had SAH. Complete occlusion was achieved in 21 patients (80.77%) through transarterial embolization and 5 patients (19.2%) through preoperative embolization combined with microsurgery. Complications included transient cranial nerve palsy in 1 patient (3.8%) and rebleeding in 2 patients (7.7%). The Barthel Index scores ranged from 10 to 100 (average, 66.73) and improved significantly (average, 96.62) in the follow-up period (10-175 months; median, 85.6 months). CONCLUSIONS Supratentorial draining direction may be a risk factor for venous ectasia and SAH in petrous apex DAVFs with pial arterial supplies. The feeding pial arteries appeared to be at risk of embolization-related hemorrhagic complication and are therefore suggested to be embolized first.

Endovascular patch embolization for blood blister–like aneurysms in the dorsal segment of the internal carotid artery

OBJECTIVE Blood blister-like aneurysms (BBAs) in the dorsal segment of the internal carotid artery are fragile and difficult to treat, and the optimal treatment for BBAs is still controversial. We report clinical and angiographic results with procedural details for the treatment of BBA by using the endovascular patch embolization method. METHODS We retrospectively reviewed patients who presented with subarachnoid hemorrhage caused by internal carotid artery-BBAs and were treated with the endovascular patch embolization method in our center from October 2011 to March 2015. Clinical records, angiographic findings, procedural details, and follow-up results are reported in this study. RESULTS Eight patients were enrolled in this study. All patients were treated with the endovascular patch embolization method. The key points of this method are step-by-step stent deployment and swaying of the microcatheter to coil the aneurysm sac and the wedge-shaped space between the stent and parent artery and, thereby, in the aneurysm sac and parent artery around the aneurysm neck. When the stent is completely deployed, an endovascular patch is formed and anchored around the neck of the BBA. The procedure was successful in all cases. No acute complications developed in any case. No rerupture or recurrence of the BBA occurred during follow-up. One patient with Hunt-Hess V subarachnoid hemorrhage died of multiple organ failure 4 months post treatment. Another patient died of intracranial infection related to the ventricle-peritoneal shunt. The remaining 6 patients had good clinical outcomes (modified Rankin Scale score of zero). CONCLUSION Endovascular patch embolization is an improvement on stent-assisted coil embolization, which could be successfully performed only with extensive skill and patience. Endovascular patch embolization could be an effective method in BBA treatment. However, its efficacy and safety should be verified in a larger patient cohort and long-term follow-up study.

Transient Cortical Blindness Associated with Endovascular Procedures for Intracranial Aneurysms

BACKGROUND We presented 3 cases of transient cortical blindness secondary to contrast medium toxicity after endovascular procedures for intracranial aneurysms. We also reviewed the literature and found 12 cases of contrast-induced cortical blindness after endovascular procedures for intracranial aneurysms. CASE DESCRIPTION Two patients (cases 1 and 2) noted bilateral blindness 5 and 6 hours, respectively, after awakening from general anesthesia following aneurysm treatment. The third patient (case 3) noted bilateral blindness during vertebral angiography under local anesthesia. Immediate angiography was performed in case 1 and showed no arterial occlusion. Computed tomography was performed in case 2 and showed brain edema. Magnetic resonance imaging was performed in all 3 cases, and cases 2 and 3 showed abnormal presentation on fluid attenuated inversion recovery sequences. With the use of corticosteroid and intravenous hydration, cortical blindness resolved within 1 week in 2 patients (Cases 1 and 2). The remaining patient (case 3) had incomplete quadrantanopia 3 months after blindness onset. CONCLUSIONS Based on our experience and the literature reports, we advocate corticosteroid and intravenous hydration for patients with contrast-induced cortical blindness after endovascular procedures for intracranial aneurysms.

Hybrid Operation of a Ruptured Aneurysm Associated with a Developmental Venous Anomaly

BACKGROUND As a normal variation of the cerebral venous angioarchitecture, developmental venous anomalies (DVAs) represent a rare cause of intraparenchymal hemorrhage. Different from mixed vascular lesions, such as capillary malformations, arteriovenous malformations, arteriovenous fistulas, and thrombosis, here we describe the first case of a ruptured hemodynamic aneurysm associated with a DVA and introduce the first use of hybrid operation for the treatment of a hemorrhagic DVA. METHODS A 14-year-old girl suffered from sudden onset of headache, aphasia, and left hemiplegia. On the way of her transfer to our center, she suddenly fell into a coma. Computed tomography scan demonstrated an enlarged intraparenchymal hemorrhage in the left temporal lobe. Angiography revealed a large frontal DVA with an associated hemodynamic aneurysm. Superselective angiography of left middle cerebral artery confirmed that the aneurysm was located on the turning site of arteriovenous transition. RESULTS Considering the large hematoma and the possible occlusion of surrounding collecting veins, we attempted transarterial embolization but were unsuccessful. Hematoma evacuation and aneurysm isolation were performed in a hybrid operation room. Intraoperative angiography was used to confirm the location of the aneurysm and to recheck the result. The patient woke up 1 day later and the symptoms were relieved entirely 1 year later. CONCLUSIONS Associated aneurysm may be a cause of intraparenchymal hemorrhage in DVAs and routine imaging monitoring is needed. Hybrid operation is a possible treatment for such complicated mixed lesions in DVA, which proved to be safe and effective in this patient.

Clinical outcomes and prognostic factors in patients with spinal dural arteriovenous fistulas : a prospective cohort study in two Chinese centres

Background The short-term outcomes and prognostic factors of patients with spinal dural arteriovenous fistulas (SDAVFs) have not been defined in large cohorts. Objective To define the short-term clinical outcomes and prognostic factors in patients with SDAVFs. Methods A prospective cohort of 112 patients with SDAVFs were included consecutively in this study. The patients were serially evaluated with the modified Aminoff and Logue’s Scale (mALS) one day before surgery and at 3 months, 6 months and 12 months after treatment. Univariate and multivariate analyses were performed to identify demographic, clinical and procedural factors related to favourable outcome. Results A total of 94 patients (mean age 53.5 years, 78 were men) met the criteria and are included in the final analyses. Duration of symptom ranged from 0.5 to 66 months (average time period of 12.7 months). The location of SDAVFs was as follows: 31.6% above T7 level, 48.4% between T7 and T12 level (including T7 and T12) and 20.0% below T12 level. A total of 81 patients (86.2%) underwent neurosurgical treatment, 10 patients (10.6%) underwent endovascular treatment, and 3 patients (3.2%) underwent neurosurgical treatment after unsuccessful embolisation. A total of 78 patients demonstrated an improvement in mALS score of one point or greater at 12 months. Preoperative mALS score was associated with clinical improvement after adjusting for age, gender, duration of symptoms, location of fistula and treatment modality using unconditional logistic regression analysis (p<0.05). Conclusion Approximately four fifths of the patients experienced clinical improvement at 12 months and preoperative mALS was the strongest predictor of clinical improvement in the cohort.