Spontaneous Vertebral Artery Arteriovenous Fistula with Neurofibromatosis Type I and Its Management with Covered Stent.

Vertebro-vertebral fistulas (VVFs) are uncommon vascular pathology. It can be either primary (spontaneous) or secondary (iatrogenic or mechanical trauma). Spontaneous vertebral arteriovenous malformation is often associated with connective tissue disorders. Cases associated with neurofibromatosis type I (NF I) are even rarer. Management of VVF with covered stent is an emerging option for construction of vertebral artery. It not only preserves the flow of the parent artery but also has immediate exclusion of the fistula from the parent artery. A 30-year-old pregnant female patient presented with cervical bruit and left upper limb radiculopathy. She was a known case of NF I. Magnetic resonance imaging cervical spine revealed multiple flow voids compressing the cervical spinal cord and nerve roots. Digital subtraction angiography revealed a vertebral artery arteriovenous fistula. She underwent endovascular treatment in the form of a covered stent. Her clinical symptoms immediately improved. She was asymptomatic at the 1-year follow-up.

Life-Threatening Intracerebral Hemorrhage in Adult with ITP: Challenging Entity.

Intracerebral hemorrhage (ICH) is a rare and fatal complication of immune thrombocytopenia. ICH is more common in children than in the adult population. A 30-year-old male patient, a known case of immune thrombocytopenia, presented with sudden onset severe headache and vomiting. Computed tomography scan showed a large right frontal intracerebral hematoma. His platelet counts were low, and he received multiple transfusions. Though he was initially conscious, his neurological condition progressively deteriorated, so the decision was taken for an emergency craniotomy. Despite multiple transfusions, his platelet counts were 10,000/µL, so craniotomy was very risky. He underwent an emergency splenectomy and received one unit of single donor platelets. Subsequently, his platelets count increased a few hours after, and he underwent successful evacuation of intracerebral hematoma. He eventually had an excellent neurological outcome. Though intracranial hemorrhage carries significant morbidity and mortality, a timely decision of emergency splenectomy followed by craniotomy can result in an excellent clinical outcome.

Mature Teratoma with Somatic-Type Malignancy: An Entity of Unacquaintance-A Case Report.

Primary intracranial teratomas are nongerminomatous germ cell tumors. They are infrequent lesions along the craniospinal axis, with their malignant transformation extremely uncommon. A 50-year-old-male patient presented with one episode of generalized tonic-clonic seizure (GTCS), without any neurological deficit. Radiological imaging revealed a large lesion in the pineal region. He underwent gross total excision of the lesion. Histopathological examination was representative of teratoma with adenocarcinomatous malignant transformation. He underwent adjuvant radiation therapy and had an excellent clinical outcome. The present case highlights the rarity of malignant transformation of the primary intracranial mature teratoma.

Atlantoaxial Instability with Persistent Second Intersegmental Artery.

Understanding the anatomy of the vertebral artery is essential while manipulating the craniovertebral joint during surgery. Its anomalous course in congenital atlantoaxial dislocation makes it more vulnerable to injury. Preoperative dedicated computed tomography (CT) angiography helps identify the artery's position and plan for surgical procedure. A 13-year-boy presented with neck pain and spastic quadriparesis for 1 year. Radiological imaging of the craniovertebral junction revealed atlantoaxial instability with basilar invagination. His CT angiography of neck and brain vessels revealed an anomalous course of the vertebral artery due to a persistent second intersegment artery. He underwent posterior atlantoaxial fixation after mobilization of the vertebral artery. His clinical condition significantly improved after surgery. We report a case of management of an atlanto axial dislocation with persistent second intersegment artery and describe the role of vertebral artery mobilization during surgery.

Endovascular Management of Falcine Dural Arteriovenous Fistula-A Case Report and Review of Literature.

Cranial dural arteriovenous (AV) fistulas are abnormal connections between the branches of dural arteries to dural veins or venous sinuses. They are most frequently located at the transverse sinus and cavernous sinus. They can occur at every cranial dural sinus. Dural AV fistula of falx cerebri is rare. A 62-year-old female presented with signs and symptoms of raised intracranial pressure. Radiological imaging revealed a dural AV fistula at the posterior one-third falx cerebri. She underwent transarterial embolization, and complete obliteration of the fistula was achieved. A detailed digital subtraction angiography study is warranted in patients with seemingly benign complaints like recurrent headaches, and falcine dural AV fistula should be identified and treated in the nick of time. We describe a very rare falcine dural AV fistula case and its management.

Cranial fibrous dysplasia: An institutional experience and review of the literature.

BACKGROUND: Cranial or craniofacial fibrous dysplasia (CFD) is a rare entity which most often presents with either incidental finding or with pain/cosmetic disfigurement or visual/hearing problems. Multidisciplinary treatment with close follow-up or medical management/surgery is options. Management of these lesions can often give satisfying results. There is a dearth of neurosurgical literature on this subject matter. Our objective was to review the clinical symptomatology and outcome of CFD patients managed in our institution. METHODS: This is a retrospective observational study of CFD patients managed in our institution over a period of 5 years. Clinical and radiological data were collected from departmental database. Outcomes were evaluated immediately and on 1-4 years follow-up. RESULTS: A total of 21 patients were managed over a period of 5 years with age ranging from 12 to 55 years and symptoms of cosmetic issues or visual disturbance. Preoperative computed tomography scan with 3D reconstruction with bone window was done in all patients. In most of the patients (16/20), immediate reconstruction was done following excision. Five patients were managed conservatively. Follow-up was obtained over a period ranging from 1 to 4 years and all (except one) patients are doing well. Only one patient had permanent visual impairment in spite of early intervention. CONCLUSION: Craniofacial dysplasia has various modalities of management. Careful selection of patients for surgical or conservative management is feasible with good results both in short and long term.

Carotid Endarterectomy and Carotid Artery Stenting for Symptomatic Carotid Stenosis: An Experience of a Hybrid Neurosurgeon in a Developing Nation.

Background: Stenosis of the ICA is an important cause of ischemic stroke and associated morbidity and mortality. Carotid artery stenting (CAS) and carotid endarterectomy (CEA) help to prevent impending or subsequent ischemic stroke in such patients. Aim and Objective: To study the outcome and adverse events associated with CEA and CAS. To determine the generalization of results obtained with multicentric trials such as CREST, etc., by comparing the results obtained by a single neurosurgeon in a community setting. Material and Methods: From Jan 2014-Dec 2017, 80 patients presented with symptomatic carotid stenosis. Out of these 80 patients, 65 underwent intervention; 34 patients underwent CEA and 31 patients underwent CAS. Pre-defined variables like age, sex, and degree of stenosis were assessed as potential risk factors, and the patients' clinical features, radiological imaging, and procedural complications were documented. Results: The primary outcome of procedure-related stroke, major adverse events (MAEs), and death at 30 days follow-up and long-term outcomes of restenosis at 1 year were analyzed. Peri-procedural stroke occurred in 2 cases (6.4%) of CAS; one suffered an ischemic stroke and other suffered a hemorrhagic stroke. Three cases of CEA suffered procedure-related events; one (2.9%) suffered TIA while the other two developed postoperative local hematoma without neurological deficit; one was treated conservatively while the other required re-exploration due to pressure symptoms. Restenosis occurred in one case that underwent CAS. Conclusion: CAS and CEA are complementary approaches in treating symptomatic carotid stenosis even when performed by a single hybrid neurosurgeon as results obtained are commensurable to major studies like CREST.

Extra-Axial Cystic Meningioma without Dural Attachment in an Adult: Case Report and Review of Literature.

Intraparenchymal meningiomas, meningiomas without dural attachment, and cystic meningioma are atypical and extremely rare, especially in adults. Only four cases of intraparenchymal cystic meningioma without dural attachment have been reported. A 47-year-old female presented with an altered sensorium. She had a progressive bifrontal headache for 2 months. Computed tomography scan of the brain showed an 8 cm × 6 cm cystic lesion with a solid component in the left frontoparietal region with a midline shift. The solid part of the lesion was enhancing on contrast but the cyst rim was not. Intraoperatively, the cyst was filled with amber-colored fluid, which was drained, and the solid component was completely excised. Histopathological examination of the solid tumor component confirmed cystic meningioma. At 2 years of follow-up, she has no evidence of recurrence. We report the fifth case of this very rare entity and review the literature.

Life-threatening perforating brain injury by a rusty iron rod - A case report.

Background: When an object traverses through the cranium leaving behind both an entry and exit wound, it is called perforating brain injury. Perforating open brain injury is rare. A paucity of published literature on such cases and a lack of a standard management protocol pose significant challenges in managing such cases. Case Description: We present a case of a 24-year-old man who worked as a carpenter at the construction site. He slipped while working and fell from a height of 13 feet onto a rusty, vertically placed 3 feet iron rod located on the ground. Iron rod entered his body from the right upper chest, came out from the neck, and again re-entered through the right upper neck medial to the angle of the mandible and finally came out from the posterosuperior surface of the right side of the head. He presented to the emergency department in a conscious state, but his voice was heavy and slow-paced, and he showed signs of lower cranial nerve palsy on the right side. He underwent numerous radiological investigations. The iron rod was removed in the operation theater under strict aseptic precautions. On day 7 after surgery, he developed right lobar pneumonia, and on day 21, he developed an altered sensorium, followed by a loss of consciousness. He did not regain consciousness and, unfortunately, succumbed after 30 days of sustaining the injuries. Conclusion: Perforating open brain injuries are rare, especially in civilian society, and are usually associated with significant morbidity and mortality. Due to a lack of standard guidelines for managing such severe injuries and limited knowledge, many patients with these injuries do not survive. Although each case presents differently, certain management principles must be followed.

Surgical treatment and results in growing skull fracture.

Growing skull fracture is a rare complication of skull fracture and remains almost undetected in the first few years of life. Here, we report a series of 11 patients with growing skull fracture treated at our institute over a period of five years and discuss their clinical features, radiological findings, and principles of management. Of the 11 patients, six were females and five males, with the age ranging between 9 months and 12 years (mean, 3 years). Progressive scalp swelling was the most common presenting feature. Other clinical features included generalised tonic clonic seizures, eyelid swelling, and proptosis. Computed tomography scan of the head defined the growing skull fracture in all 11 patients and detected the underlying parenchymal injury. Postoperatively, all patients had a complete resolution of the scalp swelling. Two patients had postoperative seizures and one had cerebrospinal fluid leak. Early recognition and surgical repair is essential to prevent the development of neurological complications and cranial asymmetry.

Concomitant occurrence of subfrontal extradural hematoma and orbital subperiosteal hematoma: a rare entity.

Subfrontal extradural hematomas are uncommon, similar are orbital subperiosteal hematomas. Co-occurrence of both following head trauma is very rare. We describe co-occurrence of sub frontal extradural and orbital subperiosteal hematomas in four patients. The presenting symptoms were proptosis and visual complaints. Diagnosis was confirmed on computed tomography in three patients and magnetic resonance imaging in one patient. Frontal craniotomy and superior orbitotomy with evacuation of hematoma resulted in complete resolution of proptosis and visual symptoms. We emphasize on the early diagnosis of this rare condition and also emergency treatment to prevent permanent visual loss.

Intriguing case of giant intra-abdominal pseudocyst: Diagnostic dilemma.

We present a case of a giant intra-abdominal pseudocyst in a 24-year-old male as a complication of ventriculoperitoneal (VP) shunt. Ultrasonography and computed tomography abdomen detected a 20 × cm 14.5× cm 9 cm thin-walled cystic lesion with few septae occupying a large space in the left side of the abdomen with a VP shunt tip within it. Histopathological findings suggested a pseudocyst. However, multiple epithelioid cell granulomas on cyst wall resulted in a diagffignostic dilemma.

Calvarial tuberculosis: A report of eleven patients.

BACKGROUND: Tuberculosis is endemic in developing countries. However, calvarial tuberculosis is rare, even in areas where tuberculosis is endemic. In the literature, only few case series of calvarial tuberculosis have been reported. AIM: To report a case series of 11 patients with calvarial tuberculosis, and discuss their presentation and management. MATERIALS AND METHODS: This study is a retrospective analysis of case records of 11 patients with calvarial tuberculosis treated between 2001 and 2005 in a tertiary hospital. Clinical features, radiological findings, surgical and medical management, and outcomes were reviewed. RESULTS: Of the 11 patients, seven were male and the age ranged between 1 and 25 years (mean 15.09 years). The mean duration of symptoms was 2.9 months (range 1-5 months). The most common presenting features were scalp swelling, discharging sinus, and pain. Computed tomography (CT) scan of brain showed punched out bony defect, with a peripherally enhancing extradural collection in most of the cases. Ten patients underwent surgical excision of necrotic bone and granulation tissue with primary closure of the scalp flap and antituberculous therapy. One patient is being managed with antituberculous therapy only. Of the 10 patients treated surgically and with antituberculous therapy, nine recovered well and one died of tuberculous meningitis and hydrocephalus. The patient being treated with antituberculous therapy is under follow-up. CONCLUSION: A high index of suspicion and knowledge is required for early diagnosis of calvarial tuberculosis. Surgery and antituberculous therapy remains the mainstay of treatment.

Ventral Craniovertebral Junction Arachnoid Cyst in an Elderly Patient-A Case Report of a Unique Occurrence at Extremes of Age.

BACKGROUND: Craniovertebral junction arachnoid cysts are uncommon. Among those reported, ventrally located arachnoid cysts at the extremes of age have been even rarer. We report a successfully managed case of a ventrally placed arachnoid cyst in an 88-year-old man using an unconventional surgical approach. CASE DESCRIPTION: An 88-year-old man presented to us with complaints of tingling and numbness in both upper and lower limbs. He had a weak handgrip on both sides. Spinal magnetic resonance imaging (MRI) showed a non-contrast-enhancing cystic lesion over the anterior lip of the foreman magnum that had displaced the cervicomedullary junction posteriorly. The lesion was homogenously hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. The lesion was excised using a posterior approach. The cyst contents were drained, followed by complete wall excision. Complete improvement in clinical symptoms and an absence of neurological deficits were noted in the patient. CONCLUSION: Arachnoid cysts are congenital lesions and can present at the extremes of age. Ventrally placed small symptomatic arachnoid cysts can be managed successfully through the posterior approach, especially in the elderly population.

A Unique Pediatric Case of Intratumoral Hemorrhage in a Sporadic Vestibular Schwannoma.

Vestibular schwannomas (VSs) are tumors that commonly occur in the eighth cranial nerve. They are usually associated with type 2 neurofibromatosis. They are uncommon in children, and sporadic cases of pediatric VS are even rarer. In general, VSs are benign lesions with less than 1% chance of intratumoral hemorrhage. Adult cases of hemorrhage in VS are well documented. We present the first pediatric case of intratumoral hemorrhage in VS in the absence of any features of NF in an 11-year-old child who complained of holocranial headache and sensorineural hearing loss in the left ear. We further discuss the pathogenesis and clinical features, and review the literature of intratumoral hemorrhage in patients of VS.

Concurrent Spinal Epidural Tubercular and Pyogenic Abscess of Cervical Spine without Bony Involvement.

Spinal epidural abscess (SEA) presents with vertebral body involvement. SEA is mostly pyogenic in developed countries, but in developing countries, tuberculosis is more common cause. Young female presented with fever, neck, and right upper limb pain for 1 month followed by acute onset weakness in the right upper limb. Magnetic resonance imaging study of cervical spine showed contrast-enhancing lesion in C2-C3 epidural region. Surgery-aided by initiation of antibacterial and antitubercular treatment based on culture and histopathological study of pus helped us to get good clinical outcome. One should always keep in mind possibility of tubercular abscess while treating cases of spinal epidural lesions, though it is rare in the absence of osseous involvement. Pyogenic and tubercular abscess can present concurrently and sending pus for culture and sensitivity is must as it plays important role in identifying dual organisms.

Giant Craniospinal Intramedullary Neurenteric Cyst in Infant-Case Report and Review of Literature.

BACKGROUND: Neurenteric cysts are rare congenital lesions derived from an anomalous connection between the primitive ectoderm and endoderm. CASE DESCRIPTION: A 2-month-old infant presented with an insidious onset of upper limb weakness. Magnetic resonance imaging of the spine revealed a contrast-enhancing solid mass located anterior to the spinal cord at the level of the cervicothoracic junction, which was isointense on T1-weighted images and hyperintense on T2-weighted images. The lesion was associated with marked spinal cord compression and also with a giant intramedullary cyst that extended proximally into the cranium. Spinal dysraphism was noted. The patient underwent cervicothoracic laminotomy, drainage of the cyst contents, and a complete excision of the solid component. Neurologic function recovery was observed postoperatively. CONCLUSIONS: We report and discuss the clinical presentation, pathogenesis, and neuroradiologic findings in an infantile case of a giant craniospinal intramedullary neurenteric cyst. Early recognition is beneficial because of its benign nature and good clinical outcome following total surgical resection.

Cerebral Arteriovenous Malformation Associated with a Cyst.

An 11-year-old child presented with chronic occipital headache and vomiting. Computed tomography (CT) scan revealed a cystic mass lesion in the right occipital region. Magnetic resonance imaging brain revealed a cystic mass lesion which was hypointense on T1-weighted images and hyperintense on T2-weighted images. A mural nodule was noted in the inferolateral wall of the lesion. CT angiography revealed small arteriovenous malformations (AVM) related to the lesion. The AVM associated with the cyst was excised. The patient recovered well. Cystic AVM is a rare vascular lesion, and only five such cases have been reported in the past. AVMs should be considered in the differential diagnosis of cystic lesions of the brain, especially when associated with a mural nodule.

Orbital Tuberculosis with Intracranial Extension.

We report a case of orbital tuberculosis (OTB) with intracranial extension without active tuberculosis in the rest of the body organs or a history of tuberculosis infection. A 29-year-male patient presented with left-sided painful periorbital swelling with pus discharging sinus and visual impairment. Orbital computed tomography revealed contrast enhancing cystic mass lesion in the left orbit with erosion of the lateral and superior orbital wall with intracranial extension. After the failure of 3 weeks' course of oral antibiotics, the patient underwent left lateral orbitotomy, pus was drained out, and granulation tissues were excised. Histopathological examination confirmed OTB. The patient had received antituberculous treatment. Periorbital swelling completely disappeared and vision improved over a period of 2 weeks. OTB should be considered in differential diagnosis of periorbital swelling especially when it does not respond to oral antibiotics.