Vascular Ehlers-Danlos Syndrome: Literature review and surgical management of intracranial vascular complications.

Vascular Ehlers-Danlos (vEDS) is a rare form of the Ehlers-Danlos Syndrome (EDS) where arterial fragility results from mutations in the gene that encodes type III collagen. The disease can lead to major neurological complications including carotico-cavernous fistulae (CCF), aneurysms of the Circle of Willis and endovascular procedures have an increased risk profile due to the delicate vasculature. Management of intracranial disease in vEDS requires an intricate understanding of the syndrome but is still associated with significant complications that lead to morbidity and mortality. As well as providing an approach to the management of neurovascular complications in vEDS, the relevant literature regarding nosology, aetiology and genetics of the condition is summarised here. Particular emphasis is placed on the two most common intracranial complications, namely carotico-cavernous fistulas and and cerebral aneurysms. Pros and cons of surgical and endovascular interventions are discussed and a technical discussion is concentrated on the surgical aspects of management.

Cranio-spinal Rosai Dorfman disease: case series and literature review.

Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder. Radiologically cranio-spinal RDD often mimics commoner dural-based lesions like meningioma, with only subtle radiological differentiating findings on Magnetic Resonance Imaging (MRI). The histopathology of RDD is diagnostic. Surgical excision is preferred modality of treatment. However, adjuvant therapies like steroids and radiation may help controlling residual or recurrent disease. There are multiple sporadic reports and short case publications in the literature, often focusing on a particular aspect of RDD. In this study, authors aim to present five cases of craniospinal RDD, and comprehensive review of literature and highlight neurological complications of systemic RDD.

Management of Concomitant Moyamoya Disease, Arterial Venous Malformation, and Intracranial Aneurysm: Case Illustration, Literature Review, and Management Algorithm.

BACKGROUND: Moyamoya disease (MMD), arterial venous malformations (AVMs), and intracranial aneurysms are distinct cerebrovascular disease processes that most commonly occur in isolation. The literature on the treatment algorithm of each individual condition is well established. An association between MMD and intracranial aneurysms is also known, but MMD in association with AVM is rare. CASE DESCRIPTIONS: The authors discuss various dilemmas in the multimodality management of these conditions when they coexist with an illustrative case of a 46-year-old woman who presented following a stroke, with 1) left-sided MMD, 2) left-sided frontal AVM, and 3) an aneurysm arising from the A1 segment of the right anterior cerebral artery. These were managed respectively by 1) the left external carotid artery to M2 segment of middle cerebral artery bypass using an autologous radial artery graft and left indirect superior temporal artery-encephaloduroarteriosynangiosis, 2) stereotactic radiosurgery, and 3) endovascular coiling of the aneurysm. Three years following intervention, cerebral angiography showed a patent bypass, complete obliteration of the AVM, and no residual filling of the coiled aneurysm. CONCLUSIONS: Our strategy of surgical revascularization for MMD, radiosurgery for AVM, and endovascular coiling for aneurysm resulted in a positive long-term clinical outcome. In view of the rarity of the condition, the authors propose a management algorithm for such patients.

Neurosurgical referral service using smartphone client WhatsApp: preliminary study at a tertiary referral neurosurgical unit.

BACKGROUND: Smartphone-based clients are being increasingly used in a medical world for individual as well as group communications. The authors report the use of WhatsApp, a common social media smartphone client for neurosurgical referral service in their Institution. They extend the use of WhatsApp application from a purely intra-departmental informal communication to a formal tertiary referral service for Neurosurgical referrals. The pros and cons of deploying such system are discussed and important concerns are highlighted. METHODS: Over a period of one year, the authors record 1412 referrals to the Neurosurgical service at a tertiary referral centre using WhatsApp application. All the data gathered through WhatsApp referrals was subsequently validated and analysed for any errors. They identify eight incidences where the system did not accurately convey the intended information. They highlight the potential issues related to data accuracy and confidentiality encountered in using such a system. OBSERVATIONS: In this preliminary study, the authors consider WhatsApp application as an important tool for Neurosurgical referrals. It is safe, cheap, reliable and user-friendly application allowing seamless transfer of data including clinical videos and scan images to the on call neurosurgical team. Loss of data quality, concerns surrounding patient confidentiality, errors caused by inter-operator variability in capturing data and default prohibition of use of such social media applications in many institutions are important disadvantages in regularly using such service. CONCLUSIONS: Smartphone-based social media clients such as WhatsApp provide a promising future for faster, reliable communication of clinical and radiological data, which can be used for on-call neurosurgical referrals. Integration with PACs applications and facial recognition may facilitate increasing use of such applications in future addressing many of the concerns that currently prohibit their universal use.

Time to implement a national referral pathway for suspected cauda equina syndrome: review and outcome of 250 referrals.

INTRODUCTION: Cauda equina syndrome (CES) is a condition with significant implications and medico-legal profile. The literature still lacks large primary studies to provide strong evidence for a robust management pathway. Statements from Neurosurgical and Spinal societies support early diagnosis and imaging but this has not resulted in any noticeable shift in referral pattern. We strongly feel the need for a nationally agreed, evidence-based referral pathway in practice. We present our large series and in-depth analysis of the referral pathway to provide strong evidence for more robust referrals and management. METHODS: We reviewed 250 referrals of suspected CES (sCES) to the regional neurosurgical unit, evaluating the importance of clinical findings and the imaging pathway. RESULTS: After clinico-radiological evaluation only 32 (13%) had confirmed CES requiring urgent surgery. There was no significant difference in terms of clinical presentation between these true cases of CES (tCES) and false cases (fCES). Imaging was therefore the key rate-limiting step. MRI was the most common investigation used. 73 patients presented without imaging out of hours (OOH). In this group, investigation was delayed to the next day in 60/73 (82%) patients while only 13 (18%) patients underwent OOH MRI. Only 2 (3%) were able to have this at their local hospital. CONCLUSIONS:  As with previous studies we conclude that signs/symptoms are insufficient to identify tCES. Taking into consideration the improved outcome with early diagnosis, the importance of early scanning in diagnosing tCES, and the poor availability of OOH MRI scanning outside of neurosurgical units, we recommend a national policy of 24/7 MRI availability for cases of sCES at all hospitals with MRI scanners. This would remove the 87% of patients not requiring urgent surgery from an unnecessary and distracting referral process.

Air-embolism and cerebral ischaemia following epidural hydrogen peroxide irrigation in a closed lumbar cavity.

Authors report a case of air-embolism and subsequent ischaemic damage to the brain following intra-operative irrigation with hydrogen peroxide within a closed cavity of a spinal cold abscess of tuberculous origin. Copious amount of undiluted hydrogen peroxide irrigation was deployed under moderate pressure to clean-up the abscess cavity. Post-operatively, the patient developed seizures followed by clinical and radiological features of brain ischaemia ultimately resulting in a fatal outcome. The authors reflect on the events, review the relevant literature regarding intra-operative use of hydrogen peroxide in cranio-spinal procedures, outline mechanism of its actions, and highlight its disadvantages and potential complications.

Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report.

INTRODUCTION: The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed. CASE PRESENTATION: A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs. CONCLUSION: Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.

Central neurocytoma: a multi-disciplinary review.

Benign central neurocytoma (CN) is a rare neuronal tumour of the central nervous system recognised since the early eighties. More than 300 articles have been published in the literature, mostly comprising of case reports and short series from individual specialties. These tumours, though normally benign, are more often likely to recur after surgery than previously thought. A multi-modality team involvement, therefore, has become increasingly necessary for their optimum management. In this article, the authors from various neurosciences sub-specialties, with a specific interest and experience in managing CN, review the epidemiology, clinical features, pathological findings, radiological characteristics and surgical treatment, with an emphasis on the latest developments in their histology, molecular biology and adjuvant treatment modalities for recurrent or residual disease.

Facial herpes zoster infection precipitated by surgical manipulation of the trigeminal nerve during exploration of the posterior fossa: a case report.

INTRODUCTION: We present a case of herpes zoster infection (shingles) precipitated by surgical manipulation of the trigeminal nerve root during an attempted microvascular decompression procedure. The pathogenesis of this phenomenon, as well as the importance and role of prophylactic acyclovir in its management, are discussed. CASE PRESENTATION: A 54-year-old Caucasian man with a classical long-standing left-sided V2 and V3 division primary trigeminal neuralgia refractory to medical management, underwent posterior fossa exploration for microvascular decompression via a standard retromastoid craniectomy. The patient had immediate and complete relief from pain. Three days after the operation, he developed severely painful vesicles with V2 and V3 dermatomal distribution. Rather than the classical paroxysmal, lancinating type of trigeminal neuralgia, the pain experienced by the patient was of a constant burning nature. A clinical diagnosis of herpes zoster (shingles) was made after smear confirmation from microbiological testing. The patient was commenced on antiviral treatment with acyclovir. His vesicular rash and pain gradually subsided over the next two weeks. He remains asymptomatic one year later. CONCLUSIONS: Postoperative shingles precipitated by trigeminal nerve manipulation during surgery for trigeminal neuralgia can be a distressing and demoralizing experience for the patient. A careful preoperative history, early recognition, and prompt antiviral therapy is necessary.

Thomas Willis of the "circle of Willis".

Thomas Willis is best known for the circle of Willis. The life story of this 17th- century medical genius, who remains an inspiration for all neuroscientists 300 years later, is summarized in this article. We outline his academic achievements, including his description of the famous basal arterial circle, and we attempt to obtain insight into his visionary thought process through this historical review.

Aneurysm at the fenestrated anterior cerebral artery: surgical anatomy and management.

The authors report a rare case of an aneurysm arising at fenestrated anterior cerebral artery. The embryogenesis of this anatomical variation is discussed along with the review of relevant literature. Management of such lesions depends upon the location and morphology of the aneurysms and intracranial vascular anatomy. The authors discuss various management options and highlight possible technical difficulties that can be encountered in the surgical management of this rare sub-group of aneurysms.

Broncho-pleural fistula due to trans-diaphragmatic migration of the distal end of ventriculo-peritoneal shunt.

We report two cases of broncho-pleural fistula resulting from trans-diaphragmatic migration of the distal catheter of a ventriculo-peritoneal shunt. Relevant literature on thoracic complications of a ventriculo-peritoneal shunt is reviewed. The clinical presentation, diagnosis and management of V-P shunt-related broncho-pleural fistulae are discussed.

An overview of head lice infestation in neurosurgical patients.

Head lice or pediculosis is recognized as an increasing problem in medical practice (Down et al, 1999). Secondary bacterial infections can occur in patients with infestation. In neurosurgical patients, head lice infestation may be a potential risk factor for peri-operative complications. Secondary infection could further complicate neurosurgical wounds with subsequent complications. The authors discuss epidemiology, pathogenesis of potential peri-operative complications resulting from pediculosis and methodology of treatment of this common condition. The importance of early recognition and prompt treatment in patients with neurological diseases is highlighted. A simple algorithm to treat scalp pediculosis is suggested.

Aneurysms unsuitable for endovascular intervention: surgical outcome and management challenges over a 5-year period following International Subarachnoid Haemorrhage Trial (ISAT).

OBJECTIVE: To analyse outcome of surgical management of aneurysms unsuitable for endovascular intervention in a tertiary referral neurosurgical unit over a 5-year post-ISAT period. To compare secondary parameters such as operating time, number of clips required, and training over last 5 years with similar number of patients in the pre-ISAT period. METHODS: Consecutive 54 patients harbouring 62 uncoilable aneurysms admitted from May 2002 to April 2007 in a tertiary neurosurgical unit. Outcome analysed at 3 months using Glasgow Outcome Score and Modified Rankin Scale based on standard questionnaires. Comparison with outcome of surgical arm of ISAT study performed. RESULTS: At 3 months 28 (90%) of Grades I-II and 9 (39%) of Grades III-V patients harbouring uncoilable aneurysms had good clinical outcome (MRS 0-2) comparing favourably with the surgical arm of the ISAT study at 2 months. (relative risk=0.28, 95% CI 0.08-0.45; P=0.01) Surgical time, number of clips required for aneurysmal obliteration had however increased significantly compared to pre-ISAT period. The training potential was severely restricted with uncoilable aneurysms. CONCLUSIONS: Ruptured cerebral aneurysms deemed unsuitable for endovascular intervention are also difficult cases to treat surgically. However, with neurovascular sub-specialisation, it is possible to achieve favourable surgical outcome in a higher percentage of cases than reported in the ISAT study and the National Study of Subarachnoid Haemorrhage. These cases, due to their technical complexities, unfortunately offer limited training potential for pre-certification Neurosurgical trainees.