Pakistan Journal Of Neurological Surgery
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    Von Hippel – Lindau Disease, Involvement of Multiple Members of the Same Families

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    Von Hippel – Lindau disease is a heritable multisystem cancer syndrome that is associated with a germline mutation of the VHL tumour suppressor gene on the short arm of chromosome 3. This disorder is not rare (about one in 36 000 live births) and is inherited as a highly penetrant autosomal dominant trait (i.e. with a high individual risk of disease). Affected individuals are at risk of developing various benign and malignant tumours of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive adnexal organs. Because of the complexities associated with management of the various types of tumours in this disease, treatment is multi-disciplinary.Objectives: We present an overview of the clinical aspects, management, and treatment options for von Hippel-Lindau disease in three families, a total number of 7 patients.Study Design: It was a descriptive observational study.Material and Methods: In this study we included 7 patients belonging to 3 families. In the first family there was a father and a son, both of them had Von Hippel-Lindau disease. In the second family two sisters and one brother was involved, while in the third family one brother and one sister had this disease. This study was conducted in the Department of Neurosurgery, Lahore General Hospital, Lahore.Results: In this study we observed that 1 out of 7 patients died, 1 is bed ridden with paraparesis. The rest 5 patients are passing their normal routine life. We observed that in all of our patients (100%) there was CNS involvement, the recurrence in our series is quite high. (70%), 3 of our patients required permanent V.P. Shunt while 1 patient required per operative EVD. In 2 of our patients eye surgery was required. 1 of our patient underwent the Gama Knife (Radio surgery)

    Early Ultrasonic Detection of Neural Tube Defects

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    Objective: Evaluation of early neural tube defects by ultrasound examination. Study Design: Observational study. Material and Methods: Public and private hospitals of Lahore, from October 2011 to January 2014. (Amna Inayat Medical College, Avicenna Medical College, Shaikh Zayed Hospital, Fatima Memorial Hospital, National Hospital, Defence Medical Group). Ultrasound done in public and private centers of Lahore, Pakistan. Routine anomaly scan (18 – 23 weeks gestation) done for 2034 pregnant women to assess the effectiveness of diagnostic ultrasound in the diagnosis of neural tube defects. During the anomaly scan the fetus with neurological defects was recorded separately and followed up. Results: Out of the 2034 pregnant women screened, 27 fetus (.01%) were diagnosed with neural tube defects. Out of these, 4 fetus had anencephaly. Two of the women opted to terminate the pregnancy immediately and the other two mothers opted to continue to term and they delivered stillborns. There were 3 women whose fetus were diagnosed with acrania. Encephalocele was seen in 5 patients. Three had frontal encephaloceles and 2 had posterior encephaloceles. Fourteen cases of spina bifida with associated congenital anomalies were detected. The most common associated anomaly was hydrocephalus (seen in 9 of the fetus), lumbar meningocele (seen in 8 of fetus). Scoliosis (seen in 6 fetus). One patient was diagnosed with hydrocephalus and after birth it was deter-mined to be agenesis of corpus callosum. Conclusion: Ultrasound is a beneficial investigation to make early diagnosis in neural tube defects. Anomaly scan is a routine investigation assisting the neurosurgeon in treatment and prognosis for the fetus. Such cases with neural tube defects should be assessed early and delivered in tertiary care hospitals so that early treatment may be initiated for better prognosis

    Frequency of Gross Total Resection in Intra-axial Brain Tumors with Help of Neuronavigation

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    Introduction: Neuronavigation has become a ubiquitous tool in the surgical management of brain tumors. Neuronavigation is most useful as an adjunct to other brain-mapping techniques such as awake mapping and electrocorticography in the resection of lesions within eloquent motor and language areas. Neuronavigation is also commonly used in skull base tumors, especially for planning an operative trajectory in regions containing vital neurovascular structures and may be used for cerebrovascular surgery. The current study was planned to determine the frequency of gross total resection in intra-axial brain tumors with the help of neuronavigation.Material and Methods: This cross sectional study was carried out in the Department of Neurosurgery, Nishtar Medical College & Hospital, Multan, from September 2014 to March 2015. After approval from institutional ethical committee, seventy seven patients fulfilling the inclusion criteria were selected from the patient admitted in the Neurosurgical Department through the Out Patient Department and patients referred from other departments. After thorough counseling with the patient and his/her relatives, informed consent for procedure was taken.Results: Total 78 patients were included in the study. Out of these 78 (100%), 41 (52.6%) were male and 37 (47.4%) were female. As concern to the outcome variable (gross total resection), out of 78 (100%), in 61 (78.2%) patients gross total resection was present. On cross tabulation it was further clarified that in male patients’ gross total resection present in 32 patients and absent in 9 patients. Similarly in female patients gross total resection present in 29 patients and absent in 8 patients. P value was 0.747. Conclusion: Conclusion of our study is that neuronevigation is a usefull technique in for better gross total resection of intra axial brain tumor

    Traumatic Epidural Hematoms in Posterior Cranial Fossa: a Clinical Study in Tertiary Care Hospital

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    Objective: To determine clinical presentation and outcome of traumatic epidural hematoma in posterior cranial fossa. Material and Methods: This descriptive case series was conducted in department of Neurosurgery Mardan Medical Complex Mardan from May 2016 to April 2017. All patients presenting with extradural hematoma in posterior fossa of either gender and age were included in the study while patients with recurrent posterior fossa extradural hematoma and hematoma at other side of the brain were excluded. CT scan Brain with bone window was done in all patients. Patients were followed for one month. Outcome was measured on the basis of GCS and GOS. Results: Total 23 patients were included in the study having age range from 8 to 58 years with mean age was34 ± 5.23 years. 16 (69.56%) were males and 7 (30.4%) were female. Road traffic accident was the most common cause of head injury in 11 (47.8%) cases. Headache and vomiting was seen in 16 (69.5%) cases, drowsiness in 8 (33%), occipital swelling in 4 (19%), otorrhea in 3 (13%) and coma in 1 (4%) case. 11 (47.8%) were managed conservatively while 12 (52.1%) were manage by surgery. Outcome measured by GOS, 15 (65.2%) fell into GOS 5, 3 (13.04%) fell into GOS 4, 7 (30.4%) into GOS 3, 6(26%) into GOS 2 and 6 (26%) into GOS 1. Conclusion: PFEDH is a traumatic substrate with poor clinical picture. Failure to diagnose the condition, level of consciousness at the time of intervention, clinical course and associated cranial injuries are the significant factors influencing morbidity and mortality from this condition. One should maintain high index of suspicion for the possibility of PFEDH in any patient with head injury

    Early Diagnosis and Intervention in Epidural Hematoma of the Posterior Cranial Fossa

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    Objective is to emphasize the importance of early diagnosis based on head CT scan and aggressive surgical treatment. The authors present their experience in the management of patients with posterior cranial fossa epidural hematoma (PFEDH) which involved an aggressive diagnostic approach based on symptoms and signs and early use of head computerized tomography (CT) scanning.Material and Methods: The authors treated 25 patients in DHQ Teaching Hospital/Sahiwal Medical College, Sahiwal from January 2007 to June 2014. Diagnosis was established in all the patients with the aid of CT Scan-ning because the clinical manifestations were frequently non-specific. Cases were stratified by clinical course, Glasgow coma score (GCS) and their radiological status. Based on clinical and radiological parameters, patients underwent surgical or conservative management.Results: Male to female ratio was 1.6:1. Patients ranged in age from 5 – 45 years. All hematomas were of traumatic origin. The most frequent cause was motor vehicle accidents (17 cases) and fall from height (8 cases). Out of cases of PFEH, male to female ratio was 1.6:1. The most important findings were occipital, sub-occipital or retro-mastoid swelling (18 patients – 72%) headache (16 patients-64%) vomiting (16 patients – 64%). Clinical signs and symptoms are summarized in the table. Ten out of 23 patients (40%) had disturbed conscious level, three patients had ear bleed and then developed CSF leakage and two patients were asymptomatic. On admission a GCS score of 5 – 6 was noted in two patients, a score of 7 – 8 was seen in three cases, a score of 9 – 12 in five and score of 13 – 15 in fifteen cases. In two patients in whom there were small hematomas not exerting mass effect as well as absence of other associated traumatic lesions, conservative therapy was elected. Admission score was 15 which remained unchanged and patients were free of deficits after hospitalization for one week or until the clinical and radiological deterioration was excluded. Twenty three patients in whom the hematoma caused mass effect, even if the lesion was small and associated with either traumatic lesions, were treated surgically. The mortality rate in this group was 4% as one patient died despite prompt evacuation of the clot and supportive intensive care treatment. Nine patients in which GCS score was less than 13 on admission improved after surgery, making an excellent recovery without neurological deficit except one patient who remained disabled after surgery. Thirteen patients in whom the GCS score was 13-15 on admission and in whom large hematomas exerted mass effect underwent surgery and all improved of consciousness remained unchanged and discharged without neurological deficit.Conclusion: Compared with outcomes reported in the available literature, good outcome was found in our series. This is primarily due to the early use of CT scanning for diagnostic and observational purposes, which in the authors’ opinion, led to early diagnosis, prompt treatment and ultimately good outcome

    Surgical Management and Outcome of Depressed Skull Fracture

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    Objective: To know the surgical management and outcome of depressed skull fracture.Materials and Methods: This descriptive study was conducted in Head injury unit Hayatabad Medical Complex, Peshawar from October 2006 to October 2009. Detail history regarding the cause of depressed skull fracture, duration since injury, clinical condition, and CT Scan picture were documented in a Performa. Depressed fracture of more than 5 mm, cosmetically disfiguring fractures and fracture over the sinuses were operated. All the patients were given prophylactic antibiotics and anticonvulsants. These patients were followed up for six months. History of fits, clinical examination of the wound, CSF leak and neurological assessment of patients were done on every visit. The data was analyzed in SPSS 16.Results: This study includes 48 consecutive, surgically treated, depressed skull fractures patients. Male-to-female ratio was 2.2 : 1. The age range of the patients was 1-63 years with a mean age of 14.1 years. The pediatric population constituted the largest group 26 (54.1%) with the 16-30 year-old age group the next largest 12 (25.0%). 10 (20.8%) patients were older than 30 years. Etiological factors include fall from height in 26 (54.16%) cases, road traffic accident in 15 (31.25%), recreational activities injury in 1 (2.08%), physical violence in 3 (6.25%) and miscellaneous in 3 (6.25%) cases. 35 (72.91%) patients presented with mild head injury, 7 (14.58%) had moderate head injury and 6 (12.5%) with severe head injury. Closed depressed skull fracture was noted in 11 (22.91%) cases, while compound depressed skull fracture was in 37 (71.09%) cases. The incidence of depressed skull fracture was 8 (16.67%) in frontal bone, 8 (16.67%) in fronto-parietal, 10 (20.8%) in temporal, 13 (27.08%) in parietal, 5 (10.41%) in occipital, 2 (4.17%) on superior saggital sinus, 1 (2.08%) on confluence of sinuses and 1 (2.08%) on transverse sinus. Associated intracranial lesions were Extradural hematoma 16 (33.3%) Contusions 15 (31.25%), Dural tear 23 (47.92%) and in driven bone fragment 7 (14.58%). Postoperatively, 35 (72.9%) patients showed excellent recovery, 6 (12.5%) were hamiparetic, 4 (8.33%) were having seizures and 2 (4.17%) remained in vegetative state, and 3 (6.25%) developed meningitis. 2 (4.17%) patients had surgical site wound infections. 1 patient developed pseudomeningocele. One patient had CSF leak.Conclusion: Depressed skull fractures are common in children. Anticonvulsants and antibiotics are effective in prevention of epilepsy and infection during perioperative period. Early surgical treatment is extremely desirable when fracture is more than 5 mm depressed and open depressed skull fractures. The outcome of depressed skull fracture depends on severity of injury and presence of other associated intracranial lesion

    Craniovertebral Junctional Injuries and Management

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    Objective: Craniocervical junction injuries are less common. They are unique in their presentation and need specialized management. The objective was to determine diagnosis initial management and ultimate surgical procedures performed and efficacy of these procedures.Materials and Methods: A five year study from April 2003 to Oct. 2008 was conducted at department of neurosurgery unit II Lahore general hospital Lahore. A total of fifteen patients were included. All patients with upper cervical trauma with all modes of injuries were included irrespective of their age and sex. All patients were evaluated with routine X-rays cervical spine, anterioposterior, lateral and open mouth views. While dynamic views were advised only in those having osodontoideum. C.T with saggital reconstruction and MRI were performed in all patients to further augment and detect bony and soft tissue details. In all modes of injuries we maintain their airway breathing and circulation.Clinical Presentation: Out of total fifteen patients mostly were young in their twenties and thirtees, only two patients (13.33%) were below twenty and one patient (6.66%) was above fourty years. The main culprit was road traffic accident in most of patients (thirteen patients 80%) followed by fall in two patients (13.33%) and assault in one patient (6.66%). The odontoid fracture with reductable atlantoaxial instability was appeared to the most common problem in five patients (33.33%). In two patients (13.33%) transverse ligament found to be intact. In two other cases (13.33%) atlas fracture was simultaneously found. Osodontoideum detected in two patients (13.33%) while basilar invagination seen in one patient (6.66%). Irreducable atlantoaxial instability was seen in three patients (20%). Out of fifteen patients, three patients (20%) were neurologically intact, while one patient (6.66%) had complete injury. Eleven patients (73%) had partial injury.Surgical Procedures: In order to achieve stability, we performed posterior instrumentation and bony fusion in all nine reducible injury patients (60%). Atlanto axial fusion performed in seven patients (46.66%), while in two patients (13.33%) having concomitant C1 injury occipitocervical fusion was done. Initial transoral decompres-sion, prior to posterior fusion was done in all four (26.66%) non reducible injury patients. Transodontoid screw fixation was done in two patients (13.33%) having intact transverse ligament.Outcome: Overall 07 (46.66%) cases revealed excellent results all recovered without any complication. Four (26.66%) cases had some complication but recovered within 02 weeks and result was labeled as good. Two cases who had neurological deterioration, recovered slowly within 03 months. Recovery was labeled as fair. One patient who suffered neurological deterioration did not recovered and result was labeled as poor.Complications: One patient (6.66%) died after severe chest infection, although severe chest infection observed in three patients (20%). Mild wound infection and wound dehiscence seen in one patient (6.66%) each. These patients managed conservatively successfully. Neurological deterioration observed in three patients (20%), out of them two patients (13.66%) improved with 3 months

    Lumbar Disc Herniation, Clinical Analysis Muzaffarabad, AK

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    Objective: Analysis of different variables in patients operated for lumbar disc prolapsed.Materials and Method:Study design: Observational study.Sample size: Twelve hundred forty eight consecutive patients operated for lumbar disc herniation.Settings: Abbas Institute of Medical and Health Sciences.Duration: The study duration was four years from January 2005 to December 2009.Inclusion criteria: Patients irrespective of age and sex with lumbar disc herniation having. Failed conservative treatment for 3 weeks. Intractable pain or impending neurological deficit.Exclusion criteria: Spinal stenosis. Spinal tumors. Spinal tuberculosis. Spondylolysthesis. Psychological problems.Results: Out of 1248 patients 812 were male and 416 were female with male to female ratio of 2:1. The age ranged from 20 to 60. Majority 672 (56%) of patients were from Muzzafrabad and its suburbs. Straight leg raising test was impaired in1180 cases. The ratio between contained and ruptured disc was 3:1. About 44.9% of patients (560) had left lateral disc prolapse, while right sided in 29.5% cases and bilateral in 25.6% cases. The common level affected was L4-L5 in 900 (72.1%) patient. Fenestration and discectomy was suitable in 900 patients, 232 patients had Hemilaminectomy and in 116 laminectomy was carried out. The removed disc tissue was sent for histopathology in all cases. Out of which three were reported as tuberculosis, two metastasis and two plamacytoma. Forty patients developed discitis postoperatively.Conclusion: From this study we conclude that lumber disc herniation is common in male gender in 4th decade. Left lateral disc at L4-L5 level is involved in the majority of patients. Sciatica with backache and impaired straight leg raising test are important clinical features. Contained disc is more common than ruptured discs. Minimal invasive procedures like fenestration and disectomy gives good results. It is also concluded that all disc material should be sent for histopathology examination to over look unusual pathologie

    Clinical Risk Factors Affecting Survival of Primary Cerebral Malignant Astrocytoma at a University Hospital in Western Saudi Arabia

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    Objective: Malignant astrocytomas are the most aggressive tumors affecting the brain. The natural history of survival of malignant astrocytomas differs significantly between anaplastic astrocytoma and glioblastoma multiforme (1). The clinical risk factors affecting the survival of malignant astrocytomas are scarcely studied (2). The aim of this study is to describe the clinical risk factors affecting the survival of malignant astrocytomas at king abdulaziz university hospital in Jeddah.Materials and Method: From January 2004 to December 2008, medical files of patients with a diagnosis of anaplastic astrocytoma and glioblastoma multiforme were retrospectively reviewed. Only those with characteristic pathologic findings suggestive of malignant astrocytomas were enrolled. Demographic data and clinical manifestations with outcome were analyzed. All data were processed using SPSS 16 software (SPSS Inc., Chicago Illinois).The data were analysed for age, symptoms, signs and clinical risk factors influencing the survival of both tumours. Differences with p < 0.05 were considered significant.Results: Forty eight cases were evaluated. The number of anaplastic astrocytoma was 15 (31.25%) and the number of GBM was 33 (68.75%). The mean age for glioblastoma multiforme was 55.3 ± 27.5 years. The mean age of anaplastic astrocytoma was 27.4 ± 21.3 years. Risk factors that worsen the prognosis were: A- old age p = .00002, B- Vomiting p < .02, C- Numbness p < .0001, D- Cranial nerves abnormalities p < .0004 and E- Abnor-mal mental status p < .003.Conclusion: Clinical risk factors affecting the survival negatively of patients with malignant astrocytomas are age, presentation with vomiting, numbness, abnormal mental status and abnormal cranial nerves examination

    Burr Hole Evacuation of Extradural Hematoma in Mass Trauma, a Life Saving and Time Saving Procedure: Our Experience in Earth Quake of Oct 8th, 2005

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    Background: Traumatic brain injury represents a significant cause of mortality and permanent disability in the adult population. Extradural hematoma is one of the conditions most strongly associated with severe brain injury. Knowledge on the natural history of the illness and the outcomes of patients surgically managed may help the neurosurgeon in the decision-making process.Objective: To observe the outcome of burr hole evacuation of extradural hematoma in mass head injury.Methods: This study included patients of any age who sustained head injury in the earth quake of October 8th, 2005. Who has been diagnosed as a case of extradural hematoma on CT-Scan and has been admitted in the neurosurgery ward presenting over a period of 3 days (8th October 2005 to12th October 2005). Patients with any other associated intracranial abnormalities, such as cerebral contusions, as shown on CT, were excluded from this study. All patients were followed by serial CT scanning, and neurological assessments.Results: A total of 36 patients were included in this study. There were 25 male and 11 female patients with age ranges from 5 years to 50 years. All cases were the victim of earth quake. All patients underwent surgery for evacuation of extradural hematoma through a single burr hole. 1 patient required craniotomy for extradural hematoma due to neurological deterioration on the second postoperative day, and 1 patient died.Conclusion: From this study we concluded that as extradural hematoma is potentially fatal lesion, so in mass head injured patients like in earth quakes, evacuation of extradural hematoma through a single burr hole has good outcome with less chances of recurrence and complications

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    Pakistan Journal Of Neurological Surgery
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