Integrated Endoscopic Craniosynostosis Surgery and Cranial Orthosis Program.

INTRODUCTION: Craniosynostosis results from premature fusion of the cranial sutures. There has been a continuous evolution of surgical correction techniques from an extensive open procedure to the minimally invasive technique. AIMS AND OBJECTIVES: This novel surgical technique has never been previously done in the UAE and this article documents the experience of the first team to endoscopically correct craniosynostosis with planning, surgical techniques, and customized helmet therapy. METHODS: A retrospective case series of 16 nonsyndromic craniosynostosis patients with unicoronal (6), sagittal (5), bicoronal (1), frontosphenoidal (2), and metopic craniosynostoses (2) was corrected endoscopically and followed up with customized cranial helmet. Preoperative and postoperative photography, along with anthropometric measurements were taken. RESULTS: The mean age of all patients at the time of surgery was 101 (90-117) days, mean duration of surgery was 70 minutes (38-129), mean blood loss was 120 mL (100-150), and mean duration of hospital stay 4 days (4-5). No complications and mortality were reported. Mean number of helmets was 2 (1-3). Cranial index improved in all patients with sagittal and bicoronal craniosynostosis. Close cooperation with the orthotist ensured timely helmet therapy and accurate laser scanning documentation in head molding. CONCLUSIONS: This case series has demonstrated that endoscopically craniosynostosis correction is safe, effective, and reliable and is the first reported series from the Middle East. The authors believe that every patient with nonsyndromic single­suture craniosynostosis should be offered endoscopic correction before the age of 6 months. The orthotist forms an important part of the Multi-disciplinary team, enabling successful outcomes.

Initial experience with novel CGRP-receptor inhibitor therapy in Migraine in the United Arab Emirates: a retrospective observational study.

BACKGROUND: Erenumab is a calcitonin gene-related peptide (CGRP)-receptor antibody inhibiting CGRP function. CGRP is prominently involved in the pathophysiology of migraine through nociceptive modulation in the trigeminovascular system. This study aims to explore the treatment effect of erenumab in a real-life setting. METHODS: In this retrospective observational study, we analyzed the data of 91 patients with migraine receiving at least three consecutive monthly injections of erenumab and followed up for 3-12 months. The primary objective was to describe the reduction in monthly migraine days throughout the follow-up period. To identify patients who responded to treatment, we analyzed the association between different patient characteristics and their treatment outcomes. RESULTS: Seventy-three patients (80.2%) responded to erenumab treatment, defined as ≥50% reduction of migraine days per month, across all migraine types. It was noted that ethnicity (p-value = 0.015) and older age (p-value = 0.035) were associated with clinically relevant improvement of symptoms. Middle Eastern ethnicity was related to less improvement of symptoms while Europeans were more likely to benefit from erenumab therapy (odds ratio: 12.788, p = 0.037). Patients aged from 31 to 40 and 41-65 years benefited most from erenumab treatment with a response rate of 77.8 and 89.9%, respectively, also confirmed by logistic regression (p = 0.047). Neither gender nor dose increase of erenumab showed association with the reported clinically relevant improvement of the symptoms. An association between clinically relevant improvement of headaches and the type of migraine was also noted. Around 87.9% of patients with episodic migraine responded to treatment, followed by 84.1% of chronic migraine patients and 50% of medication overuse headache patients. Medication overuse headache showed a lower probability of therapy success with erenumab (odds ratio: 0.126, p = 0.039). An improvement of headaches was eminent in patients who received 140 mg erenumab monthly (2 × 70 mg injections) and patients who had one injection every two weeks. CONCLUSIONS: Erenumab is a novel preventive treatment for all migraine types. Clinically relevant improvement of headaches and reduction of monthly migraine days were demonstrated in patients that continued the treatment course. In real-life, a substantial number of patients suspended therapy early, reasons for which need further investigation.

Endovascular Modalities for the Treatment of Cavernous Sinus Arteriovenous Fistulas: A Single-Center Experience.

BACKGROUND: Cavernous sinus (CS) fistulas are classified into traumatic and spontaneous. Traumatic carotid-cavernous fistulas (CCFs) are usually direct internal carotid artery (ICA) high-flow fistulas; whereas spontaneous CCFs are usually dural, low-flow fistulas and generally possess less severe symptoms than direct carotid-cavernous fistulas. METHODS: This study involved 34 patients who were classified into 2 groups: Group A included 26 patients with direct carotid-cavernous fistula; and Group B included 8 patients with indirect dural cavernous fistula. All patients had ocular manifestations. One patient had subarachnoid hemorrhage. Coils were used alone in 19 cases of direct fistula and in 1 case of dural fistulas. Coils and Onyx (Covidien, Mansfield, MA, USA) were used in 7 cases of direct fistula and in 2 cases of dural fistulas. Onyx alone was used to treat 5 cases with dural fistulas but none of the cases with direct fistulas. Covered stents and coils were used in 2 cases of direct fistulas. RESULTS: All patients in both groups showed full recovery of their clinical signs and symptoms. Only 1 procedure-related complication was observed (3%) in which a patient had an embolic event and trigeminal dysesthesia as a result of Onyx reflux through external carotid artery-ICA anastomosis. CONCLUSION: Coils are superior solid embolic agents used for the treatment of direct high-flow fistulas, while Onyx is more valuable in dural low-flow CCF. Onyx shortens the procedure time and decreases procedure cost. Onyx injection inside the CS proper through the transarterial or transvenous route may be safer than Onyx injected inside dural arteries supplying the CS. However, more cases are needed to determine this.

Carotid artery occlusion for the treatment of symptomatic giant carotid aneurysms: a proposal of classification and surgical protocol.

Giant intracranial aneurysms are rare disorders that represent only 5% of all intracranial aneurysms; they have a wide variety of presentations including rupture, embolic effects, and mass effect symptoms that can mislead the diagnosis to tumors rather than aneurysms. Their treatment is difficult and carries higher morbidity and mortality than usual aneurysms due to their complex nature. This study involved retrospective analysis of data of 28 patients, managed between 2006 and 2012, suffering from giant internal carotid artery (ICA) aneurysms with various presenting symptoms, none of which was hemorrhage. They were all evaluated by BOT prior to any intervention; they were subjected to various treatment strategies including selective coiling, parent artery occlusion with or without bypass, aneurysm trapping with or without bypass, and patients were followed for a period ranging from 6 months to 5 years. Out of 26 patients with giant aneurysms with mass effects, 16 patients showed full recovery (61.5 %), 5 showed partial improvement (19.2 %), and 5 showed no change in mass effect symptoms (19.2 %). One patient died (3.5 %). Symptoms such as TIA or epistaxis showed complete recovery. This study shows that a well-designed protocol aiming at parent artery sacrifice will yield good to excellent results in managing ICA giant aneurysms, and it also shows that parent artery sacrifice is superior to other forms of treatment of these lesions regarding recurrence rates, morbidity, and mortality.

Cerebral abscess in Down syndrome: a systematic review on treatment and prognosis. Illustrative case.

BACKGROUND: No universal protocol exists for treating cerebral abscesses in Down syndrome. An illustrative case supplemented with a systematic literature review on brain abscesses in Down syndrome is presented, comprising a total of 16 cases. Preoperative infectious disease workups, cardiac examinations including echocardiography, as well as reported surgical and antibiotic treatments were correlated in the reported cohorts. OBSERVATIONS: Overall, 18.8% of cases (n = 3) had no reported cardiac evaluation. The majority of cases were treated surgically (n = 8), with aspiration (n = 3), drainage (n = 2), or other operations (n = 3); 25% (n = 4) were treated with antibiotics only. Strikingly, 25% of cases (n = 4) reported neither surgical nor antibiotic therapy, a significantly higher rate compared to 0%-3% of patients with brain abscess in other reported cohorts. Half of the patients (n = 8) who died either lacked a cardiac evaluation or had existing heart conditions. This mortality rate was about 4 times higher than the rates observed in other studies. LESSONS: Down syndrome patients with cerebral abscess have a high morbidity rate, mainly due to cardiac disease. Therefore, early diagnostic workup, including echocardiography, allows proactive management with an improved outcome. https://thejns.org/doi/10.3171/CASE23394.

Surpass Evolve Flow Diverter for the Treatment of Intracranial Aneurysm: A Systematic Review.

PURPOSE: This systematic review aims to summarize the evidence investigating the effectiveness and safety of the Surpass Evolve-Flow Diverter (SE-FD) to treat brain aneurysms. METHOD: We searched MEDLINE, EMBASE, CINAHL, Web of Science, and Cochrane Library from January 2019 to 29 March 2022. Terms related to the "intracranial aneurysm" and "surpass evolve flow diverter" concepts were used to search the databases; Medical Subject Headings (MeSH) and reference hand search were also utilized. RESULTS: The searches primarily identified 1586 documents. A total of five studies (four case series and one cohort) were included in this review. In the included studies, 192 (74 male and 118 females) patients with 198 aneurysms were involved. In total, 153 SE-FDs were used to treat 145 aneurysms. Complete occlusion was achieved in 69/145 (48%) cases and near-complete occlusion in 24/145 (17%) cases from aneurysms treated with SE-FD. Reported postoperative complications included stent thrombosis (n = 4 patients), hemorrhage (n = 5 patients), ischemia (n = 9 patients), and neurological complications (n = 12 patients). In total, four deaths were reported with only one related to the SE-FD procedure. CONCLUSION: The results of this review are based on observational data, due to the absence of clinical trials. The findings of the included studies suggest that the effectiveness of the SE-FD procedure is lower than previous FDs but the safety is similar. The included studies also suggested that SE-FD has navigability and resistance to twisting, which makes the procedure an easier method to treat aneurysms that are proximal and distal to the circle of Willis deployment. This review highlights the urgency to conduct clinical trials to confirm these suggestions.

Parietal Arteriovenous Malformation Mimicking Carotid-cavernous Fistula in Context of Sinus Thrombosis: Bidirectional or Unidirectional Relationship? Literature Review.

Cortically located arteriovenous malformations (AVMs) constitute majority of brain AVMs. A common drainage is through respective cortical veins into superior sagittal or transverse sinuses. Through a case report and literature review, we discuss three issues: first, the anomalous drainage of a cortical AVM into an anterior orbital venous drainage system; second, the impact of this drainage on the clinical picture; and third, importantly, the bidirectional versus unidirectional relationship of AVM and old venous sinus thrombosis.

Prevalence and characteristics of sellar masses in the city of Al Ain, United Arab Emirates: 2010 to 2016.

BACKGROUND: The prevalence of sellar masses (SMs) is reported in Europe and North America but only limited data are available from the Middle East and North Africa (MENA) region. OBJECTIVES: Assess the prevalence and characteristics of SMs in Al Ain city, United Arab Emirates. DESIGN: Retrospective, descriptive multicenter study. SETTING: Three endocrine centers in Al Ain. PATIENTS AND METHODS: All patients diagnosed with SMs in the city of Al Ain, Emirate of Abu Dhabi, between 2011 and 2016 were evaluated. Cases were identified using ICD 9 and 10 codes and demographic and clinical data were collected. The prevalence rate was calculated for patients alive and residing in Al Ain city until 31 December 2016. MAIN OUTCOME MEASURES: Clinical presentations and prevalence rate. SAMPLE SIZE: 272. RESULTS: The mean (SD) age on presentation was 40.8 (14.3) years (range: 6-114 years, median: 40.0). The 170 (61.8%) females and 128 (46.5%) were native citizens of the United Arab Emirates. Two hundred and forty five (90%) patients had pituitary adenomas (PAs) while 27 (10%) had non-pituitary sellar lesions. The four most common SMs were prolactinoma (n=139, 51.1%), nonfunctioning adenoma (NFA) (n= 69, 25.4%), somatotroph adenoma (n=32, 11.8%) and craniopharyngioma (n=15, 5.5%). Patients with prolactinoma, corticotroph adenoma, and Rathke's cleft cyst had small sellar masses (<1 centimeter) while the majority of patients with other SMs had macroadenomas. Hypogonadism and growth hormone deficiency was present in 41.8% and 20.5% of the patients, respectively. Of 268 patients with available data, 82 patients underwent surgery while 25 patients received radiotherapy. At the end of 2016, 197 patients were residing in Al Ain city. The overall prevalence of SMs was 25.7/100 000 with PAs constituting most of these masses (n=177) for a prevalence of 23.1/100 000. CONCLUSIONS: This is the first study of SMs in the United Arab Emirates and the MENA region. Prolactinoma and NFA were the two most common SMs. Further studies are needed to explore the reasons for the lower prevalence of SMs in our region compared with other countries. LIMITATIONS: Retrospective design, relatively small sample size. CONFLICT OF INTEREST: None.

Predictive Factors of the Management of Spinal Cord Arteriovenous Fistulas.

AIM: Spinal cord arteriovenous fistulas are rare cause of progressive myelopathy. The predictive factors of their outcome after endovascular or surgical treatment are still controversial. MATERIAL AND METHODS: Twenty patients were diagnosed with spinal arteriovenous fistulas; 13 of them had dural and 7 had perimedullary fistulas (1 Dorsal and 6 Ventral). Surgery, endovascular or both treatments were used. RESULTS: Ten of the 13 patients in the dural group were surgically treated, while 2 were managed by embolization and one combined. Seven patients improved, while 6 patients showed stationary clinical course after intervention. Five patients in the perimedullary group were embolized with clinical improvement, 1 patient refused intervention, and 1 patient had surgery. Clinical improvement was significantly correlated to the duration of symptoms before intervention (p=0.012), and preoperative neurological condition (p=0.001). No significant correlation was found with age, anatomic level of the fistula, fistula type, and type of intervention. CONCLUSION: Microsurgery was preferred for dural and dorsally located perimedullary fistula, while embolization was preferred for ventrally located ones. Clinical improvement was significantly correlated with early intervention and preoperative neurological condition of the patient. Age of the patient, fistula type, fistula location, and type of intervention did not show significant relation to the outcome. Patients could benefit from intervention even if they present with poor neurological condition.

Management of spinal dural arterio-venous fistulas. Report of 12 cases and review of literature.

INTRODUCTION: Spinal cord arteriovenous malformations and fistulae are rare vascular lesions than can lead to myelopathy that is at many instances overlooked during diagnosing the cause of progressive myelopathy and weakness. Treatment options involve either endovascular embolization, surgical disconnection or a combination of both. This study aims to evaluate various treatment methods for sDAVFs and the outcome of these methods. METHODS: This study involved 12 patients suffering from symptoms attributed to spinal dural arteriovenous fistulas; 11 were male and one was a female patient, with ages ranging between 50 years and 71 years. All patients presented with progressive spastic paraparesis of varying grades, and 6 had sphincter disturbances prior to treatment. Patients were evaluated by Aminoff-Logue motor disability scale. RESULTS: Three were managed by endovascular embolization and 9 by surgical disconnection. Three patients showed full recovery after treatment, 7 patients showed no change in their neurological status following treatment, and 2 patients showed partial recovery after treatment. CONCLUSION: Spinal AVF is a rare curable cause of spinal myelopathy if managed promptly. Good angiographic studies prior to treatment decision are a must, in order to plan the best approach according to the angioarchitecture of the fistula whether it will allow endovascular embolization or will surgery be more feasible.