Pediatric Stroke from Bench to Bedside: A Single-Center Experience in Saudi Arabia.

Purpose: Stroke is a leading cause of severe long-term disability and death worldwide. This study aimed to determine the genetic background, causative factors, and diagnostic and outcome measures of pediatric stroke in an area endemic to sickle cell disease (SCD). Patients and Methods: This retrospective review analyzed pediatric patients with acute stroke who were admitted to King Fahd Hospital of the University, Eastern Province, Saudi Arabia, between January and June 2019. We assessed 49 cases based on computed tomography (CT) and magnetic resonance imaging (MRI) findings. Patients with incomplete records or unavailable radiological images were excluded. Results: A high likelihood of familial coexistence of stroke was detected in patients with affected siblings (33%). Among various central nervous system manifestations, motor weakness (28.6%) and headache (20.4%) were the most common symptoms/signs. Hypoxic-ischemic encephalopathy (HIE) (28.6%), SCD (22.5%), and moyamoya disease (14.3%) were the most prevalent underlying etiologies. CT without intravenous contrast was the most used initial imaging technique (92.5%). An arterial blockage was more prevalent (53.4%) than a venous infarct (46.6%) (p = 0.041), while arterial ischemic stroke was more prevalent (56.5%) than hemorrhagic stroke (43.5%). The middle cerebral artery (MCA) was most affected (63.5%), followed by the anterior cerebral artery (22.7%) and posterior cerebral artery (13.6%). Most patients were managed with medical treatment (86.1%). No mortalities occurred during the initial hospital stay. The mean length of hospital stay was 12 days. Conclusion: HIE was the most prevalent etiology of pediatric stroke. Motor weakness and headache were the most common initial manifestations. Arterial ischemic stroke was more prevalent than venous or hemorrhagic stroke. Considering the rarity of pediatric stroke, future studies should be performed with a aborative effort nationally and internationally.

Embolization Tactics of Spinal Epidural Arteriovenous Fistulas.

PURPOSE: Spinal epidural arteriovenous fistulas (SEDAVFs) show an epidural venous sac often with venous congestive myelopathy (VCM) due to intradural reflux at a remote level to which a transarterial approach would be difficult. We present 12 cases of SEDAVF with VCM and describe 3 main tactics for effective transarterial embolization. MATERIALS AND METHODS: Among 152 patients with spinal vascular malformations diagnosed in our tertiary hospital between 1993 and 2019, 12 SEDAVF patients with VCM were included. Three different transarterial embolization tactics were applied according to the vascular configuration and microcatheter accessibility. We evaluated treatment results and clinical outcomes before and after treatment. RESULTS: Transarterial embolization with glue (20-30%) was performed in all patients. The embolization tactics applied in 12 patients were preferential flow (n=2), plug-and-push (n=6), and filling of the venous sac (n=4). Total occlusion of the SEDAVF, including intradural reflux, was achieved in 11 (91.7%) of 12 patients, and partial occlusion was achieved in 1 patient. No periprocedural complications were reported. Spinal cord edema was improved in all patients for an average of 18 months after treatment. Clinical functional outcome in terms of the pain, sensory, motor, and sphincter scale and modified Rankin scores improved during a mean 25-month follow-up (6.3 vs. 3.3, P=0.002; 3.6 vs. 2.3, P=0.002, respectively). CONCLUSION: Endovascular treatment for 12 SEDAVF patients with VCM achieved a total occlusion rate of 91.7% without any periprocedural complication. The combined embolization tactics can block intradural reflux causing VCM, resulting in overall good clinical outcomes.

The Analysis of Morphoradiological Parameters in Predicting Risk of Basilar Artery Tip Aneurysm Rupture: A Retrospective Cohort Study.

PURPOSE: Basilar artery tip aneurysms can have fatal consequences and poor prognostic outcomes in case of rupture. We investigated the standard parameters used to evaluate aneurysmal morphology to predict aneurysm rupture. PATIENTS AND METHODS: We measured the differences between ruptured and unruptured basilar bifurcation aneurysms in terms of morphological features, including aneurysm size, size ratio, aneurysm height, perpendicular height, aneurysm width, bottleneck ratio, aspect ratio, and present daughter domes, using 3D angiography. RESULTS: Among 34 patients, 21 (61.8%) and 13 (38.2%) had ruptured and unruptured basilar tip aneurysms, respectively. In the ruptured group, the mean aneurysm size, and aspect, size, and bottleneck ratios were 6.8 ± 1.9 mm, 3.2 ± 1.0, 2.3 ± 0.5, and 2.2 ± 0.4, respectively, compared to 4.5 ± 1.5 mm, 4.4 ± 2.0, 1.6 ± 0.4, and 2.7 ± 0.7, respectively, in the non-ruptured group. Univariate analysis showed a larger aneurysmal cross-sectional diameter (odds ratio [OR], 0.50; 95% confidence interval [CI], 0.5-5.392; p < 0.05), a larger size ratio (OR, 21.375; 95% CI, 3.283-139.177; p < 0.05), and presence of a daughter dome (OR, 72.0; 95% CI, 6.7-776.5; p < 0.05) with ruptured basilar artery tip aneurysms. CONCLUSION: A larger aneurysm size and size ratio, and the presence of a daughter dome were significantly associated with increased rupture risk in basilar tip aneurysms.

Microcatheter Stabilization Technique Using Partially Inflated Balloon for Coil Embolization of Paraclinoid Aneurysms.

PURPOSE: Coil embolization of paraclinoid aneurysms should be simple, safe, and effective considering the benign nature of the aneurysm. Here, we present a microcatheter stabilization technique using a partially inflated balloon for the treatment of paraclinoid aneurysms. MATERIALS AND METHODS: This retrospective study included 58 patients who underwent balloon-assisted coiling (BAC) for unruptured paraclinoid aneurysms at a tertiary neuro-intervention center between January 2019 and March 2020. We applied a technique to stabilize the microcatheter's position using the modified BAC technique in paraclinoid aneurysms showing various projections around the ophthalmic curve of the internal carotid artery. The basic concept of the technique is to place a partially inflated balloon just distal to the aneurysm neck and support the distal curve of the microcatheter using the proximal bottom of the balloon. Immediate radiological outcomes were analyzed, and clinical outcomes were evaluated with modified Rankin Scale (mRS) scores. RESULTS: The BAC was successfully performed in 51 of 58 patients (88%). We treated the remaining 7 patients by switching to stent-assisted coiling. We obtained a 37% mean packing density resulting in favorable occlusion in all 58 aneurysms (complete occlusion in 35 and residual neck in 23). There were no intraprocedural thromboembolic or hemorrhagic events except one that revealed an asymptomatic infarction after the procedure (1.7%). Magnetic resonance angiography follow-up was performed in 37 patients at an average of 11.8 months, in which 11 minor recurrences (29.7%) were found. There was no major recurrence nor retreatment. The mRS score was 0 in all patients during a mean follow-up of 17.7 months (range, 12-25 months). CONCLUSION: The modified balloon-assisted coiling technique using a partially inflated balloon was safe and effective and could serve as an option for treating paraclinoid aneurysms.

Rare Neurovascular Diseases in Korea: Classification and Related Genetic Variants.

Rare neurovascular diseases (RNVDs) have not been well-recognized in Korea. They involve the central nervous system and greatly affect the patients' lives. However, these diseases are difficult to diagnose and treat due to their rarity and incurability. We established a list of RNVDs by referring to the previous literature and databases worldwide to better understand the diseases and their current management status. We categorized 68 RNVDs based on their pathophysiology and clinical manifestations and estimated the prevalence of each disease in Korea. Recent advances in genetic, molecular, and developmental research have enabled further understanding of these RNVDs. Herein, we review each disease, while considering its classification based on updated pathologic mechanisms, and discuss the management status of RNVD in Korea.

Posterior fossa extra-axial variations of medulloblastoma: a pictorial review as a primer for radiologists.

Manifestations of an atypical variant of medulloblastoma of the posterior fossa in extra-axial locations have been reported, and key questions concerning its interpretation have been raised previously. This review illustrated the clinico-radiological and histopathological features of the posterior fossa extra-axial medulloblastoma and described possible management strategies. We thoroughly reviewed all atypical anatomical locations of medulloblastoma reported within the posterior fossa and extra-axial spaces. The main characteristics of diagnostic imaging and histopathological results, primarily the distinctive radiopathological characteristics, were summarized to distinguish between intra- and extra-axial medulloblastoma, or pathologies mimicking this tumor. Most cases of posterior fossa extra-axial medulloblastoma have been reported in the cerebellopontine angle, followed by the tentorial and lateral cerebellar locations. The dural tail sign, which is commonly observed in meningioma, is rarely seen in intra- or extra-axial medulloblastoma and might be associated with other benign or malignant lesions. In addition to magnetic resonance imaging, the proposed new imaging techniques, including advances in modern neuroimaging modalities, were discussed, as potentially efficient modalities for characterizing extra-axial medulloblastoma. Radionuclide imaging and magnetic resonance perfusion imaging are practical alternatives to limit the number of differential diagnoses. We believe that medulloblastoma cases are likely under-reported because of publication bias and frequent tumors in unusual locations. Addressing these issues would help establish a more accurate understanding of this entity.

Staged Approach for Stent-Assisted Coiling of Cerebral Aneurysms after Failure of Initial Intra-Saccular Catheterization.

PURPOSE: Microcatheter navigation into an aneurysm sac can present difficulties through negative interactions between the deployed stent mesh and microcatheter. We hypothesized that endothelialization of the stent mesh would minimize these interactions. We aimed to assess the feasibility of staged coiling after stenting by reviewing our experiences with unavoidably staged embolization cases. MATERIALS AND METHODS: Between 2011 and 2019, 7 patients (mean age 57.2 years, range 49-76 years) including 5 females, experienced 9 unruptured aneurysms treated with staged stenting and coiling due to unstable microcatheter navigation into the aneurysm after stent placement. The aneurysms were in the paraclinoid internal carotid artery (ICA) (n=3), ophthalmic origin ICA (n=1), superior cerebellar artery origin (n=2), basilar tip (n=2), and the middle cerebral artery bifurcation (n=1). The stents used were the Neuroform Atlas (n=4), Neuroform EZ (n=2), and Low-profile Visualized Intraluminal Support Blue (n=1). RESULTS: The mean interval between stenting and coiling was 15 weeks (range, 12-21 weeks). The average navigation time between the first roadmap imaging and microcatheter insertion in the sac was 14 minutes (range, 8-20 minutes). One aneurysm was occluded without further coiling on follow-up. Staged coiling successfully treated the remaining aneurysms (n=8). No complications were identified. CONCLUSION: In cases of difficult intra-saccular catheterization, intentional staged coiling may be a feasible option for stent-assisted coiling of the cerebral aneurysms.

Spinal anastomosed remnant imprints of vertebral veins linking the transverse foramina: a case report of a novel anatomic variant of the cervical venous plexus.

PURPOSE: Despite various postulated classifications attempting to simplify the complex angioarchitecture of the cervical spine, the nomenclature of spinal variants and lesions remains inconsistent. Knowledge of variations in the anatomy of the vertebral veins will assist in avoiding complications during neck surgery and procedures such as vertebroplasty. In addition, venous variants may act as a route for the spread of infection, emboli, or metastases. Therefore, we report a novel variant encountered at our institution in this case report. METHODS: We coincidentally discovered an original anatomical variant of the cervical venous plexus linking the transverse foramina in a Saudi man. RESULTS: We termed the variant "spinal anastomosed remnant imprints" (SARI), guided by the second edition of Terminologia Embryologica, a project of the Federative International Programme for Anatomical Terminology. This variant anastomoses with the vertebral veins at the same level, forming segmented osseous impressions. It shares a topographical relationship with the embryonic anterior cardinal veins, which normally regress in the prenatal period. We hypothesize that these intersegmental anastomosing veins do not always regress and may persist into adulthood, with individualized variations of the venous circulation. CONCLUSION: This report highlights an important finding of interpersonal anatomical variation of veins in the cervical spine, discovered with the aid of advanced imaging to distinguish it from pathological conditions. This will be of assistance to radiologists, anatomists, and clinicians in decision-making and to surgeons in planning for neck surgery.

Multiple Dural Arteriovenous Fistulas Manifesting as Progressive Otalgia and Tinnitus and Treated Using a Single Session of Endovascular Embolization.

BACKGROUND: Multiple cranial dural arteriovenous fistulas (dAVFs) are considered to be an uncommon vascular condition. dAVFs usually manifest with vague symptoms, which are related to the site of occurrence of the lesion and present with complex angio-architectural features, which in turn can render treatment procedures difficult. Appropriate treatment that aims to reduce the risk of intracranial bleeding and manage the patient's symptoms requires a multidisciplinary approach. CASE PRESENTATION: The patient was a 62-year-old man who developed symptoms of progressive right-sided otalgia and tinnitus. Cross-sectional imaging revealed long-standing venous congestion with intracranial hemorrhage in the right parieto-occipital region. Moreover, cerebral angiography demonstrated multiple complex dAVFs at the right transverse sinus-sigmoid sinus (TS-SS) junction, right posterior condylar confluence (PCC), and torcula. Treatment consisted of one session of endovascular treatment, in which transarterial Onyx embolization was used to treat the dVAF at the right TS-SS junction, transvenous coil embolization for the dVAF at the right PCC, and particle embolization for both occipital arteries feeding the torcular dAVF. Postoperative improvement with favorable clinical outcomes was observed at the patient's last follow-up visit. CONCLUSION: Multiple cranial dAVFs are an uncommon vascular lesion, which can present with unusual symptoms such as otalgia. Moreover, a single session of endovascular therapy can treat this lesion, ie, it can improve the patient's symptoms and provide a beneficial long-term outcome.

A ruptured posterior communicating artery aneurysm presenting as tentorial and spinal isolated subdural hemorrhage: a case report and literature review.

BACKGROUND: Ruptured intracranial aneurysms are often associated with subarachnoid or intraparenchymal hemorrhage. However, the prevalence of subdural hemorrhage post aneurysmal rupture is low and rarely reported in scientific studies. Here, we report an unusual case of a ruptured posterior communicating artery aneurysm resulting in an isolated subdural hematoma located in the tentorial and spinal canal without subarachnoid or intraparenchymal hemorrhage. CASE PRESENTATION: In this case, a 34-year-old woman with no history of trauma or coagulopathy was diagnosed with a subdural hematoma in the tentorium cerebellum tracing to the subdural space of the spinal column. Computed tomography angiography was used to identify the source of the bleeding, which revealed a ruptured left-sided posterior communicating artery saccular aneurysm. The aneurysm was clipped, and the hematoma was evacuated. The patient recovered without any neurological complications. CONCLUSIONS: Our results suggest that a diagnosis of ruptured intracranial aneurysm should be considered in patients with nontraumatic subdural hematoma. Prompt diagnostic imaging and interventional diagnostic procedures are required to ensure proper management of these patients and to avoid unnecessary complications.

Primary pigmented meningeal melanocytoma originating in Meckel cave in a patient with carney complex: A case report.

RATIONALE: Primary melanin-producing tumors are rare extra-axial neoplasms OPEN of the central nervous system. In the literature, few case reports have discussed neoplasms involving the cavernous sinus; of these, only 4 have reported on neoplasms originating in Meckel cave. The diagnostic approach, including clinical and radiological analysis, is challenging, and cytopathological assessment with a molecular basis is the best approach to discriminate between these lesions. Herein, we discuss the pathophysiology, diagnostic approach, intraoperative features, and postoperative management in a unique case of primary pigmented meningeal melanocytoma originating in Meckel cave in a patient who was diagnosed with Carney complex (CCx) and sickle cell disease (SCD). PATIENT CONCERNS: A 23-year-old man diagnosed with SCD had also been diagnosed previously with CCx, without any familial history or neurocutaneous melanosis. He had experienced headaches accompanied by left facial pain and paresthesia for 2 months. DIAGNOSIS: The initial computed tomography scan and magnetic resonance imaging (MRI) revealed a mass arising from the left Meckel cave. On MRI, it followed the signal intensity of melanin. He underwent subtotal resection of the mass. Considering the patient's history of CCx, melanocytic schwannoma was the most relevant diagnosis. A postoperative histopathological examination was suggestive of benign pigmented meningeal melanocytoma. INTERVENTIONS: The patient underwent an uneventful subtotal resection of the mass through a left temporal linear incision. OUTCOMES: The patient showed progressive improvement of neurologic deficits, and after 2 years of follow-up, he did not present with any new complaints. LESSONS: To the best of our knowledge, this is the first report of the unusual presentation of both SCD, as well as of primary pigmented meningeal melanocytoma in a patient with CCx. Complete surgical resection can be curative in most cases of melanocytoma. The presence of CCx with SCD suggests potential shared genetic contributions that will require further exploration.

Successful Retrieval of the Distal Anti-embolic Device after Carotid Stenting Using the Balloon Bridge Technique.

Background: During carotid artery stenting (CAS), retrieval of the distal umbrella portion of the anti-embolic device (AED) could be difficult. Herein, we report a case of left CAS managed with balloon angioplasty and stent placement with successful retrieval of the umbrella portion of the AED using the balloon bridge technique after failure of retrieval with the standard technique. Case Presentation: After successful revascularization of the asymptomatic severe carotid bulb stenosis in a 62-year-old woman, we could not pass the re-sheathing catheter over the deployed stent because of the ledge effect between the guidewire of the AED and the stent mesh. However, using the balloon bridge technique, which helped minimize the ledge effect, we could advance the guiding catheter beyond the stented segment over the partially inflated balloon. The umbrella portion of the AED could be easily retrieved through the guiding catheter without complications. Conclusion: During CAS, the balloon bridge technique could be used to retrieve the AED after failure of retrieval with the standard techniques.

Magnetic resonance and retrograde endoscopic cholangiopancreatography-based identification of biliary tree variants: are there type-related variabilities among the Saudi population?

PURPOSE: This study investigated biliary tree patterns in a Saudi Arabian population to identify common anatomical variations to establish a common ground for improved surgical practice and to avoid unexpected complications. We consider this type of investigation clinically important because the findings are valuable for pre-surgical planning in a broad range of procedures, including laparoscopic cholecystectomy and liver transplantation. METHODS: We conducted an imaging-based retrospective cross-sectional study involving 150 patients who underwent endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography for different indications at King Fahd Hospital of the University between January 2011 and December 2014. RESULTS: Typical right hepatic duct (RHD) tributaries were observed in 56% of patients and typical left hepatic duct (LHD) anatomy was detected in 81.4% of patients. The typical anatomical pattern for the cystic duct was found in 72% of patients. CONCLUSIONS: Our findings showed that types A1 and A3b were the two most common variations in the RHD, whereas those in LHD were types B1 (segment IV duct opens to the LHD), and B2 (segment IV duct opens to the common hepatic duct separately). Although the angular type was the most prevalent among cystic duct variations, there were many differences in the types of variations observed. The findings somewhat correlated with those of other studies, suggesting that the normal biliary tree anatomy is similar among the Saudi population and in other ethnic groups.

Neuroschistosomiasis mimicking lower back pain: case report of a rare differential diagnosis in a pediatric patient.

BACKGROUND: Spinal myelitis is an infrequent manifestation of spinal cord infection. It is caused by the Schistosoma species, which are endemic in South America, part of the Middle East, and Africa. CASE PRESENTATION: We report the case of a 13-year-old male adolescent complaining of progressive lower back pain and weakness of the lower extremities for 3 days. Initial magnetic resonance imaging revealed typical transverse myelitis. Subsequently, parasite serology showed a markedly elevated level of Schistosoma antibody titers, and cerebrospinal fluid analysis yielded normal results. Because of our presumptive diagnosis of neuroschistosomiasis, the patient was prescribed an empirical regimen of an anti-parasitic agent, after which his neurological deficit promptly subsided. The patient was followed for 1 year and showed a complete long-term resolution of symptoms. CONCLUSIONS: This case highlights the increasing prevalence of neuroschistosomiasis in recent years, particularly in patients with a history of travel to endemic regions. Moreover, the study reports the clinicoradiological features of this enigmatic disorder. This rare occurrence potentiates further studies to address unanswered questions about neuroschistosomiasis.

A unique finding of cavum velum interpositum colloid-like cyst and literature review of a commonplace lesion in an uncommon place.

Colloid cysts typically reside within the area of the anterior third ventricle, in the proximity of the foramen of Monro. Although they are considered to commonly localize in various parts of the cerebrum, they are exceedingly rare outside the ventricular system and rarely occur within the velum interpositum. We have reported here a rare case of a velum interpositum colloid-like cyst in a 23-year-old man, who presented to our clinic with temporary binocular strabismus, which he had been experiencing for the previous year. In addition, we have briefly reviewed evidence regarding the generation, anatomy, and pathogenesis of colloid cysts, as well as the management options for such rare cases. The present report is only the third to describe a colloid-like cyst located within the velum interpositum, providing additional data that may aid in elucidating the pathogenesis of these neoplasms.

An Atypical Porencephalic Cyst Manifesting as a Simple Partial Seizure: A Case Report and Literature Review.

BACKGROUND: Porencephaly is an extremely rare neurological disease characterized by the presence of solitary or multiple degenerative cerebrospinal fluid (CSF) cavities within the brain parenchyma. CASE REPORT: We describe a case involving a 23-year-old male who presented with involuntary movements of the left upper limb of 6 months' duration. A diagnosis of porencephaly was confirmed by magnetic resonance imaging (MRI). CONCLUSION: The rarity of occurrence and atypical presentation of such a lesion present a challenge to clinicians. Little is known about the pathogenesis and appropriate management of porencephaly. Further studies of the implications of porencephaly for neurodevelopment and behavior are needed.