Paradoxical symptomatic cerebral blood flow decreases after combined revascularization surgery for patients with pediatric moyamoya disease: illustrative case

BACKGROUND Transient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD). The authors report a rare pediatric MMD case with extensive decreased cerebral blood flow (CBF) and prolonged TNDs after combined revascularization. OBSERVATIONS A 9-year-old boy presented with transient left upper limb weakness, and MMD was diagnosed. A right-sided combined surgery was performed. Two years after the surgery, frequent but transient facial (right-sided) and upper limb weakness appeared. The left internal carotid artery terminal stenosis had progressed. Therefore, a left combined revascularization was performed. The patient’s motor aphasia and right upper limb weakness persisted for approximately 10 days after surgery. Magnetic resonance angiography showed that the direct bypass was patent, but extensive decreases in left CBF were observed using single photon emission tomography. With adequate fluid therapy and blood pressure control, the neurological symptoms eventually disappeared, and CBF improved. LESSONS The environment of cerebral hemodynamics is heterogeneous after cerebral revascularization for MMD, and the exact mechanism of CBF decreases was not identified. TNDs are significantly associated with the onset of stroke during the early postoperative period. Therefore, appropriate treatment is desired after determining complex cerebral hemodynamics using CBF studies.

Imaging Challenges in the Diagnosis of Anatomical Variations of the Supra-Aortic Vessels: A Case Report and Review of Literature

A 73-year-old woman was referred to our Cardiology Department due to recurrent headaches and dizziness. She had a history of hypertension of 10 years. In the territorial hospital, left internal carotid artery significant stenosis was suspected. Neurological examination and laboratory tests were normal. A neck vascular ultrasound was performed, showing a low bifurcation of the left common carotid artery (CCA) and a hypoplastic left internal carotid artery (ICA) with a sinuous path at the cervical level. Therefore, a computed tomographic (CT) angiography examination of the head and neck vessels was performed. The images confirmed the presence of a hypoplastic left ICA, anatomic variation in the left CCA, and also showed that the left vertebral artery (VA) was stemming directly from the aortic arch, exhibiting a kinking trajectory.

Anesthetic Management for Spine Surgery in a Patient with Left Internal Carotid Artery Occlusion and Penetrating Aortic Ulcer: A Case Report

A key challenge of anesthesia is to provide patients with safe and optimized anesthetic management to improve prognosis and minimize mortality and morbidity. To this end, the anesthesiologist should comprehensively understand the patient’s physical status through pre-anesthetic assessment and carefully monitor the patient during surgery. Several types of novel patient-monitoring devices may be useful to achieve this purpose. We report a case of anesthetic management in a patient with left internal carotid artery occlusion and penetrating aortic ulcer.

Cerebral Revascularization for the Management of Symptomatic Pure Arterial Malformations

Background: Pure arterial malformations (PAMs) are extremely rare abnormalities defined as dilated, overlapping, and tortuous arteries with a coil-like appearance in the absence of venous components. Over the last half century, only seven published reports have described cases of patients with PAMs who received treatment.Methods: Here, we report two cases of women with PAMs who received surgical treatment, and we present a systematic review of the literature. We searched the PubMed, Embase, Web of Science, and Medline databases (up until October 1, 2021) for relevant publications. We performed independent-sample t-tests and Fisher's exact tests to compare continuous and categorical characteristics among the available cases.Results: Our first patient was a 43-year-old woman with PAM of the left internal carotid artery (ICA), who received an ICA-radial artery (RA)-M2 bypass. Post-operative digital subtraction angiography (DSA) revealed the disappearance of the left ICA PAM without ischemic events during follow-up. The second patient was a 53-year-old woman with PAMs of the right ICA and posterior cerebral artery. The P1 lesion was treated by proximal occlusion combined with a superficial temporal artery-P2 bypass. During the 12-month follow-up period, the size of the PAMs decreased significantly as indicated by the post-operative DSA showing the absence of hemorrhages. Our systematic review, which includes 56 PAMs, shows that the reported PAMs were more common in the anterior circulation (33/56, 58.9%) than in the posterior circulation (11/56, 19.7%). Bilateral PAMs were more likely to affect bilateral anterior cerebral arteries (ACA) (ACAbilateral vs. ACAunilateral: 63.6 vs. 26.2%, p = 0.02). In addition, PAMs involving the anterior circulation were likely to affect multiple arteries (anteriormulti vs. posteriormulti: 30.3 vs. 0%, p = 0.038).Conclusion: We found very few reports on treated PAMs; further studies with large sample sizes and long follow-up periods are required to explore the appropriate treatment strategy for PAMs.

Young Stroke Secondary To Fibromuscular Dysplasia Causing Carotid Artery Dissection

Background: Fibromuscular dysplasia (FMD) is a rare, medium-sized arteriopathy affecting less than 7% of the population. It affects predominantly young women of childbearing age. Stroke caused by fibromuscular dysplasia of the carotid artery is uncommon and reported to range between 0.6% by angiography and 1.1% by autopsy. Case Presentation: A 35-year-old mother of two children, previously well, was admitted to our hospital with right-sided spastic hemiparesis with upper motor neuron type facial nerve palsy. Non-contrast CT (NCCT) brain on admission revealed left-sided middle cerebral arterial territory infarction with possible hemorrhagic transformation with midline shift. She had progressive neurological features where NCCT brain in 24 hours showed an increase in the size of the hemorrhage. Magnetic resonance imaging (MRI) of the brain, including angiogram of neck vessels, revealed acute dissection of the left internal carotid artery with acute thrombosis, left frontoparietal hemorrhagic infarction, and the constellation of MRI showed features suggestive of left cervico-cranial fibromuscular dysplasia complicated by acute internal carotid artery dissection. She was started on low-dose aspirin, and 40 mg of atorvastatin and anticoagulants were not started because of the bleeding risk as she had a hemorrhagic transformation infarction. She was discharged and referred for inward physiotherapy and planned to repeat the MRI brain with the neck vessel angiogram in three weeks and arranged digital subtraction angiography in three months. Unfortunately, she was lost for follow-up.Conclusion: Detailed evaluation of the young patients with ischaemic stroke is helpful to diagnose rare diseases such as FMD involving carotid arteries leading to a better treatment choice between anticoagulation vs. antiplatelet therapy.

A rare case of macroprolactinoma in a patient with Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome

Summary A 17-year-old lady presented with primary amenorrhoea, headache, nausea and lethargy. She had delayed pubertal development that also includes under-developed breast (Tanner Stage 2). Hormonal investigations showed a high serum prolactin level of 1 680 000 mIU/L (normal value: 45–375 mIU/L), with low oestradiol, progesterone, follicular-stimulating hormone and luteinizing hormone. Early morning cortisol level was 206 nmol/L (normal value: >450 nmol/L), thyroxine was 7.5 pmol/L (normal value: 9.0–24.0 pmol/L) with TSH 5.091 mIU/L (normal value: 0.4–4.5 mlU/L). A pituitary MRI showed a 2.7 (AP) × 3.7 (W) × 4.6 cm (CC) macroadenoma, with invasion into the left cavernous sinus and encasement of cavernous portion of the left internal carotid artery. MRI pelvis showed absent uterus, cervix and 2/3 upper vagina confirming Mullerian hypoplasia. Cytogenetics showed 46XX. These findings were suggestive of Mayer–Rokitansky–Kauser–Hauser (MRKH) syndrome with the presence of a pituitary macroprolactinoma and panhypopituitarism. She was treated with hydrocortisone, levothyroxine and cabergoline. Repeated MRI showed a reduction in tumour size by approximately 50%. This case illustrated a rare coexistence of these two conditions, being only the third reported case in the world. In addition, this would be the first case of a functioning pituitary adenoma in a patient with MRKH syndrome. Learning points Comprehensive hormonal and radiological investigations are important in the management of a young patient with primary amenorrhoea. Coexistence pathology of two separate pathologies should be considered in patient presenting with primary amenorrhoea. Early diagnosis of MRKH or any disorders of sex development should be treated early, providing pharmacological, surgical, psychological and emotional support to the patient and reducing risk of associated complications. Abnormal pituitary hormones, particularly panhypopituitarism, would impose greater impact not only psychologically but also metabolically leading to cardiovascular, morbidity and mortality risks in this patient if not treated early. A multidisciplinary approach is necessary for patients presenting with MRKH to ensure appropriate treatments and follow-up across the lifespan of the patient.

Syncope caused by lymphomatous encasement of the internal carotid artery

We present a case of a 77-year-old man who reported 5 months of syncopal episodes. He was found to have diffuse large B-cell lymphoma encasing the left internal carotid artery but not impeding blood flow. The syncopal episodes resolved after his first cycle of chemotherapy. Recurrent syncope in non-cardiac lymphomas and other head and neck masses is exceedingly rare and may be due to reflex syncope prompted by carotid baroreceptor activation. There are 11 previously described cases of recurrent syncope associated with non-cardiac lymphoma. In all cases, lymphadenopathy abutting the carotid artery was present and the syncopal episodes resolved with treatment. Our case illustrates that malignancy should be considered in patients with unexplained recurrent syncope.

Vorapaxar as an Alternative for Ticagrelor Resistance in Neuroendovascular Intervention

BACKGROUND Perioperative dual‐antiplatelet therapy for flow diversion limits thromboembolic complications. However, resistance to dual‐antiplatelet therapy medications remains a concern for neuroendovascular intervention. To date, there is no standardized approach for resistance to ADP receptor antagonists. METHODS We report a case of ticagrelor resistance for flow diversion of an intracranial aneurysm treated with vorapaxar, as well as a narrative review of the literature for previous cases of ticagrelor resistance. RESULTS Flow diversion with the Pipeline embolization device was deployed for a left internal carotid artery blister aneurysm and bilateral internal carotid artery dissecting pseudoaneurysms. The patient had 3 thromboembolic complications while on dual‐antiplatelet therapy with ticagrelor or prasugrel, leading to transition of antiplatelet therapy to vorapaxar. At 84 days follow‐up, the patient was fully recovered with complete occlusion of the aneurysms. CONCLUSION Our case suggests that vorapaxar is a promising alternative for patients with ticagrelor resistance in flow diversion–treated intracranial aneurysms. High‐quality randomized controlled trials are needed to elucidate the safety and efficacy of vorapaxar in neuroendovascular procedures.

A Novel Thromboplastin-Based Rat Model of Ischemic Stroke

The thromboembolic ischemia model is one of the most applicable for studying ischemic stroke in humans. The aim of this study was to develop a novel thromboembolic stroke model, allowing, by affordable tools, to reproduce cerebral infarction in rats. In the experimental group, the left common carotid artery, external carotid artery, and pterygopalatine branch of maxillary artery were ligated. A blood clot that was previously formed (during a 20 min period, in a catheter and syringe, by mixing with a thromboplastin solution and CaCl2) was injected into the left internal carotid artery. After 10 min, the catheter was removed, and the incision was sutured. The neurological status of the animals was evaluated using a 20-point scale. Histological examination of brain tissue was performed 6, 24, 72 h, and 6 days post-stroke. All groups showed motor and behavioral disturbances 24 h after surgery, which persisted throughout the study period. A histological examination revealed necrotic foci of varying severity in the cortex and subcortical regions of the ipsilateral hemisphere, for all experimental groups. A decrease in the density of hippocampal pyramidal neurons was revealed. Compared with existing models, the proposed ischemic stroke model significantly reduces surgical time, does not require an expensive operating microscope, and consistently reproduces brain infarction in the area of the middle cerebral artery supply.