Direct versus indirect revascularization in the treatment of moyamoya disease

OBJECTIVE For patients with moyamoya disease (MMD), surgical intervention is usually required because of progressive occlusion of the internal carotid artery. The indirect bypass method has been widely accepted as the treatment of choice in pediatric patients. However, in adult patients with MMD, the most effective treatment method remains a matter of debate. Here, the authors compared the clinical outcomes from MMD patients treated with either extracranial-intracranial arterial bypass (EIAB; 43 hemispheres) or modified encephaloduroarteriosynangiosis (mEDAS; 75 hemispheres) to investigate whether mEDAS is an effective surgical method for treating adults with symptomatic MMD. METHODS A comparative analysis was performed in patients treated using either mEDAS or EIAB. Collateral grading, collateral vein counting, and symptom analysis were used to assess the outcome of surgery. RESULTS Seventy-seven percent (58/75) of mEDAS cases and 83.7% (36/43) of EIAB cases in the analysis experienced improvement in their symptoms after surgery. Furthermore, patients in 98.7% (74/75) of mEDAS cases and those in 95.3% (41/43) of EIAB cases exhibited improved collateral grades. Increases in regions of perfusion were seen after both procedures. CONCLUSIONS Modified EDAS and EIAB both result in positive outcomes for symptomatic adults with MMD. However, when considering the benefit of both surgeries, the authors propose mEDAS, a simpler and less strenuous surgery with a lower risk of complications, as a sufficient and safe treatment option for symptomatic adults with MMD.restrictio

Neuroendoscopy: Current and Future Perspectives

Neuroendoscopic surgery is performed because it causes minimal damage to normal structures, carries a lower rate of complications, and achieves excellent outcomes. Surgeons using an endoscope and related instruments can perform complex operations through very small incisions, which is especially useful for minimally invasive procedures for the brain and spine. Neuroendoscopic surgery is now performed in cases of obstructive hydrocephalus, various intraventricular lesions, hypothalamic hamartomas, craniosynostosis, skull base tumors, and spinal lesions. This review discusses the brief history of neuroendoscopy and the current state and future perspectives of endoscopic surgery.ope

How would we deal with hypothalamic hamartomas?

Hypothalamic hamartoma (HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neurosurgical approaches. Treatment options for intractable gelastic seizure in HH patients include direct open surgery with craniotomy, endoscopic surgery, radiosurgery with gamma knife and stereotactic radiofrequency thermocoagulation. Selection of treatment modalities depends on type and size of the HH and the surgeon’s preference. Two surgical techniques, resection and disconnection, had been described with favorable outcomes. Pretreatment evaluation, patient selection, surgical techniques, complications, and possible selection of treatment are discussed.ope

Multiple Spontaneous Intracerebral Hematoma without Presenting Risk Factors

The incidence of intracerebral hemorrhage in those aged 45-84 years is 0.3-0.5%. In people over 80 years of age, this incidence increases 25-fold compared with that of the total population. The most common causes of spontaneous intracerebral hemorrhage in the younger population are vascular malformation, aneurysm, and overuse of drugs. In contrast, common causes in the elderly include hypertension, tumors, and coagulation disorders. Here, we present a case involving a 72-year-old male patient who, without any of these predisposing conditions, was admitted to the hospital with spontaneous intracerebral hemorrhage and showed signs of multifocal intracerebral hemorrhage during his stay. We conclude that spontaneous intracerebral hemorrhage can occur without any predisposing factors, and can lead to a patient's death. Therefore, the possibility of recurrent spontaneous intracerebral hemorrhage must be considered in patients with primary spontaneous intracerebral hemorrhage.ope

Orbital Space-occupying Congenital Naso-orbital Encephalocele

Purpose A case of a huge ethmoid sinus and orbital cyst causing ocular deviation initially diagnosed as a mucocele-like cyst and after orbitotomy eventually discovered to be a naso-orbital encephalocele is described. Case summary A 5-year-old male with no previous medical history presented with a palpable mass in the medial canthus area with left exodeviation of the left eye. His best-corrected visual acuity was 20/25 in the right eye and 6/12 in the left eye with limitation of motion of -4 at adduction in the left eye. Brain magnetic resonance imaging scans revealed a 3.0 × 2.5 × 2.5 cm-sized cystic mass suspected as a mucocele or nasolacrimal duct cyst invading the orbital space. The cyst was removed using a Lynch and caruncle incision, and intracystic fluid was aspirated. During the excision, a small defect of the skull base was detected with cerebrospinal fluid (CSF) leakage and leaking point of dura was sealed. His pathologic result was disorganized glial and fibrous tissue, consistent with encephalocele. Three days later, CSF leakage recurred. On postoperative day 8, duroplasty was performed. A one month later, his best-corrected visual acuity was 6/12 in the left eye with orthotropic eye position. Limitation of motion was improved to -1 at adduction with enophthalmos. No other complications were detected. Conclusions Congenital naso-orbital encephalocele invading the orbit is rare. However, a cystic mass in the sinuses involving the orbit should be considered, rendering thorough physical and radiologic examinations including computed tomography scans necessary to look for bone defects.ope

The Unnecessity of Positron Emission Tomography Computed Tomography in the Etiologic Evaluation of Neurodevelopmental Delay in Craniosynostosis Patients

BACKGROUND: In evaluation of craniosynostosis patients in terms of neurodevelopmental delay, positron emission tomography computed tomography (PET-CT) scan can be used to assess brain abnormalities through glucose metabolism. We aimed to determine the unnecessity of PET-CT in this study. METHODS: Thirty-eight patients diagnosed with craniosynostosis who underwent distraction osteogenesis from October, 2010 to November, 2013 were reviewed. Magnetic resonance imaging (MRI) and PET-CT scan were carried out for evaluation of the brain structure and function, whereas X-ray and CT scan were taken for evaluation of the skull. RESULTS: Nine patients reported abnormal MRI findings which were not significant, and five patients showed local problem on brain on PET-CT scan. No correlation was found among them. CONCLUSION: PET-CT evaluation of possible abnormal brain findings do not affect surgical planning or require additional therapy. Preoperative PET-CT scan is not the essential study to get any etiologic information of the disease consequences or to establish the treatment plan.ope

Cognitive Outcome of Pediatric Moyamoya Disease

Quality of life is the current trend and issue for the most of human diseases. In moyamoya disease (MMD), surgical revascularization has been recognized as the possible assistance to reduce the neurological insult. However, the progressive nature of the disease has been invincible so far. To improve the quality of life of MMD patients not only the protection from the neurological insult but also the maintenance or improvement of cognitive function is inevitable. For pediatric MMD patients, younger age or longer duration of disease is the key factor among the prognostic factors for bad neurological outcomes. Hence, 'the earlier, the better' is the most precious rule for treatment. Protection from neurological insult is very critical and foremost important to improve cognitive outcome. Clinicians need to know the neuropsychological profile of MMD patients for the care of whole person and make an effort to protect the patients from neurological insults to maintain or improve it.ope

Hemispherotomy and Functional Hemispherectomy: Indications and Outcomes

Hemispherectomy constitutes an established surgical method in the management of patients with medically intractable epilepsy secondary to severe unilateral hemisphere damage. As opposed to focal resections, in hemispherotomies, the entire hemisphere is disconnected from the remaining nervous system, including the functional regions and fibers. Despite this, hemispherotomy is recommended for certain indications, and has good functional and epilepsy outcomes. Here we describe the indications, epilepsy outcomes, and surgical techniques for several hemispheric surgeries.ope

Neurodevelopmental Problems in Non-Syndromic Craniosynostosis.

Craniosynostosis is the premature fusion of calvarial sutures, resulting in deformed craniofacial appearance. Hence, for a long time, it has been considered an aesthetic disorder. Fused sutures restrict growth adjacent to the suture, but compensatory skull growth occurs to accommodate the growing brain. The primary goal for the management of this craniofacial deformity has been to release the constricted skull and reform the distorted shape of the skull vault. However, the intellectual and behavioral prognosis of affected children has also been taken into consideration since the beginning of the modern era of surgical management of craniosynostosis. A growing body of literature indicates that extensive surgery, such as the whole-vault cranioplasty approach, would result in better outcomes. In addition, the age at treatment is becoming a major concern for optimal outcome in terms of cosmetic results as well as neurodevelopment. This review will discuss major concerns regarding neurodevelopmental issues and related factors.ope

Preoperative cephalhematoma size measured with computed tomography predicts intraoperative bleeding in pediatric patients undergoing cranioplasty

Background: Cranioplasty for the treatment of cephalhematomas in small infants with limited blood volume is challenging because of massive bleeding. This study aimed to elucidate the correlation between cephalhematoma size and intraoperative blood loss and identify criteria that can predict large intraoperative blood loss. Methods: We reviewed the medical records of 120 pediatric patients aged less than 24 months who underwent cranioplasty for treatment of a cephalhematoma. The cephalhematoma sizes in preoperative brain computed tomography (CT) were measured using ImageJ. Results: Pearson correlation showed that the cephalhematoma size in the pre-operative brain CT was weakly correlated with intraoperative blood loss (Pearson coefficient = 0.192, P = 0.037). In a multivariable logistic regression analysis, a cephalhematoma size greater than 113.5 cm3 was found to be a risk factor for large blood loss. The area under the curve in the receiver operating characteristic plot of the multivariable model was 0.714 (0.619-0.809). Conclusions: A cephalhematoma size cutoff value of 113.5 cm3, as measured in the preoperative CT imaging, can predict intraoperative blood loss exceeding 30% of the total body blood volume. The establishment of a transfusion strategy prior to surgery based on cephalhematoma size could be useful in pediatric cranioplasty.ope