Zeferino Demartini

Diffuse idiopathic skeletal hyperostosis: A review.

Background: Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic noninflammatory disease characterized by ossification of the entheses. Methods: This paper reviews the etiopathogenesis, epidemiology, clinical features, differential diagnosis, and treatment of DISH, based on current available literature. Results: Exact prevalence and incidence of DISH remains undetermined. Many external and genetic factors have been reported as being contributors to the pathogenesis of DISH. Current theories focus on the pathologic calcification of the anterior longitudinal ligament of the spine as the main physiopathological mechanism of disease. Clinical features are variable from monoarticular sinovitis to airway obstruction, and can be associated to systemic conditions. Comorbidities include obesity, hypertension, diabetes mellitus, hyperinsulinemia, dyslipidemia, and hyperuricemia according to a number of reports. Conclusions: DISH is a disease which involves the calcification of the anterior longitudinal ligament of the spine and can be associated with numerous clinical presentations and comorbidities.

SURGICAL TREATMENT OF MOYAMOYA DISEASE IN CHILDREN

MD, Head of the Unit of Endovascular Neurosurgery at Hospital de Base de Sao Jose do Rio Preto, SP, Brazil. Received 25 September 2007, received in final form 14 January 2008. Accepted 25 February 2008.Dr. Zeferino Demartini Jr – Hospital de Base / Unidade de Neurocirurgia Endovascular - Avenida Brig. Faria Lima 5544 - 15090-000 Sao Jose do Rio Preto SP - Brasil. E-mail: demartini9@yahoo.com

Facial palsy after embolization of dural arteriovenous fistula: A case report and literature review

Background: Dural arteriovenous fistulas (DAVF) are unusual intracranial vascular malformations consisting of anomalous connections between meningeal arteries and dural sinuses or the veins that pass through them. They have variable clinical presentation and prognosis, which depend on their location and venous hemodynamics. Treatment is based on the closure of the abnormal connections, which is usually conducted via arterial and/or transvenous endovascular techniques. Case Description: We present a male patient who complained of headaches and left-sided pulsatile tinnitus due to DAVF from the external carotid artery branches draining directly into the ipsilateral sigmoid sinus. Embolization with Onyx® was successful, obtaining angiographic occlusion and symptom remission. However, on postoperative day 4, the patient presented with left facial palsy and spontaneous regression. Conclusion: Although embolization is an effective and safe procedure, complications may occur. Reflux of the embolic agent to the vasa nervorum of the cranial nerve may lead to ischemic neuropathy. Here, we reported a case of embolized DAVF presenting with a postoperative peripheral facial palsy where the two embolized pedicles were branches of the middle meningeal and occipital arteries involved in the vascularization of the extratemporal segment of the facial nerve. We discuss the etiopathogenic, anatomical, and pathophysiological aspects of this complication.

Sinus Thrombosis after Endovascular Treatment of Vein of Galen Aneurysmal Malformation

A full-term small for gestational age Caucasian male was born by Cesarean delivery with a healthy twin brother. In his first week of life, he presented 2 rapid pallid episodes followed by perioral cyanosis, and was hospitalized for investigation of his condition. A chest radiograph showed an enlarged cardiac silhouette, and echocardiogram revealed hypertrophic cardiomyopathy with tricuspid valve failure. Cardiac failure was diagnosed and medicated with digoxin, furosemide, and captopril, obtaining a satisfactory clinical response. The patient evolved with growth delay and at 6 months he presented with vomiting, sleepiness, prominent cranial veins, and tense anterior fontanelle; he had a 43-cm head circumference (0.6 SD), weighed 5.1 kg (–1.9 SD) and measured 63.5 cm in length (–3.9 SD). A cranial CT revealed supratentorial hydrocephalus and an isodense median lesion, with homogenous enhancement by contrast. A ventricle-peritoneal shunt was performed with a good response. Two weeks later he started having seizures that were confirmed by EEG, and which were controlled with phenobarbital. The Bicêtre neonatal score was 19 (4 cardiac, 2 cerebral, 5 respiratory, 4 hepatic, 4 renal). The patient was submitted to digital subtraction angiography (DSA; Fig. 1 ), which revealed choroidal type VGAM, with a high flow fistula produced by ectatic pericallosal and choroidal arteries. Thus, at the same time, embolization was performed through a combined simultaneous approach (transarterial and transvenous route) with Axium TM platinum coils (Ev3, Irvine, CA, USA), interrupted due to the amount of contrast. Another complementary transarterial session using coils and n-butyl-2-cyanoacrylate (Histoacryl ® , B. Braun, Melsungen, Germany) was performed 2 days Introduction

Single-session percutaneous embolization with onyx and coils of sinus pericranii

Background: Sinus pericranii (SP) is a rare vascular malformation consisting of an abnormal communication between the extra- and the intracranial venous system. It occurs due to the adhesion of vessels without a muscular layer or a hemangioma on the outer surface of the skull through diploic vessels, communicating with an intracranial venous sinus. Case Description: A 10-month-old female presented with a pulsatile mass on the posterior parietal region. Investigation with brain vascular examinations showed a venous malformation communicating with the superior sagittal sinus under the scalp, without arterial feeder or nidus. An endovascular embolization with coils and a percutaneous embolization with Onyx ® were performed. The final venography showed complete exclusion of the lesion, which was gradually being absorbed. Conclusion: This is the first case of an SP successfully treated in a single session by embolization with coils and onyx.

Posterior Inferior Cerebellar Artery Origin over the C2 Posterior Arch

a Department of Neurosurgery, Hospital de Clinicas UFPR, Curitiba, Brazil; b Department of Neurosurgery, Hospital Pequeno Principe and Cajuru University Hospital, Pontifical Catholic University of Parana – PUCPR, Curitiba, Brazil; c Department of Neurology, Hospital Pequeno Principe, Curitiba, Brazil; d Department of Neurosurgery, Hospital Vita Curitiba, Curitiba, Brazil Received: March 5, 2018 Accepted after revision: April 10, 2018 Published online: July 26, 2018

Atypical Presentation of PHACE Syndrome: Hidden Facial Hemangioma

PHACE(S) syndrome is a neurocutaneous syndrome with a wide array of presentations. The most known and present trait is facial hemangioma > 5 cm. The name is an acronym for Posterior fossa malformations, infantile Hemangiomas, Arterial anomalies, aortic Coarctation, Eye abnormalities, and middle-line malformations of the Sternum. The exact etiopathogenic mechanism of this syndrome is not fully understood, and its treatment depends on detailed and individualized assessment. The aim of this paper is to describe a child with a throat hemangioma, vascular malformations, cognitive delay, and other anomalies to illustrate the neuroimaging found in this syndrome.

Is the Mayfield Head Holder Obligatory for Intracranial Aneurysm Clipping

Intracranial aneurysm surgery is commonly performed using pinned head holders, which pose a higher risk for the pediatric population. Several authors recommend avoiding the use of this device when it is not strictly necessary, and this is currently possible considering advances in anesthesiology and monitoring. As the literature on microsurgery without skull clamp use is scant, we report the case of a 15-year-old boy presenting with a subarachnoid hemorrhage after rupture of a middle cerebral artery aneurysm. Surgical treatment was performed with the head resting on a gel cushion horseshoe; aneurysm clipping was achieved without wakefulness or awareness and the patient had a good recovery.

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

ABSTRACT Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

Carotid artery occlusion in Kabuki syndrome: Case report and literature review

Background: Kabuki syndrome is a rare multiple congenital anomaly syndrome whose main diagnostic findings are craniofacial phenotypic changes and mental retardation. Organic structural lesions in the central nervous system are rare, although have been described already. Systemic vascular changes have also been reported rarely. Case Description: We report the case of a young patient with Kabuki syndrome who had a transient ischemic attack due to dissection of the internal carotid artery and a likely gliosis area on the white matter. Conclusion: Association of cervical arterial disease with this syndrome has never been described, and its pathophysiology is not yet established; however, it can direct future research and maybe treatment.