Pulsatile giant sacrococcygeal teratoma appearing like 2nd head.

Sacrococcygeal teratoma is a congenital tumor of early infancy. Eighty percent are present in the first month of life. It is considered to be an extragonadal germ cell tumor that can be diagnosed in utero. It is considered to be a rare tumor in infancy and childhood. Incidences in girls are more common as compared to boys. Association with other congenital abnormalities of the gastrointestinal system, cardiovascular system, and urological manifestations may be present. Association with spinal dysraphism may be present. A complete systemic evaluation needs to be done before any surgical intervention. We hereby present you a case of a 5-day-old male child, born from a non-consanguineous marriage, who presented to us with a large swelling at the coccygeal region. The mass had a variegated consistency with an ear-like appendage over it with associated hair, bones, and necrotic tissue in it. The patient was evaluated with imaging (MRI) which revealed a heterogeneously enhancing mass at the sacrococcygeal region with mild extension into the pre-sacral space suggesting sacrococcygeal teratoma. After a complete evaluation for congenital abnormal abnormalities of other sites, gross total excision of the mass was performed. The post-operative condition of the baby was uneventful. A biopsy of the mass revealed a mature cystic teratoma. The baby is under follow-up at present.

Unusual spinal injury in a child caused by a wooden foreign body.

Traumatic penetrating spinal injuries with wooden objects are rare. A 6-year-old boy presented with history of fall from tree 1 month back. It resulted in a penetrating injury at the lumbosacral area, followed by a discharging sinus without any neurological deficit. Radiological imaging showed a foreign body at the spinous process level traversing L4-5 and is lodged in L3-4 intervertebral body causing listhesis of L3 over L4. The wooden stick was retrieved with a meticulous surgical procedure. Proper imaging and early surgery are necessary to prevent any complications and early recovery.

Thoracic bony human tail in a newborn baby: a rare case and review of literature.

BACKGROUND: The dorsal cutaneous appendage or so-called human tail is considered to be a cutaneous marker of underlying occult dysraphism. CASE PRESENTATION: We are presenting an unusual case of spinal dysraphism with a bony human tail at midthoracic region occurring in a newborn baby with tethered cord (conus at L4). Physical examination was unremarkable except for a thoracic appendage and a dermal sinus over coccyx region. Magnetic resonance imaging (MRI) scan of spine revealed bony outgrowth arising from D7 posterior element with multiple butterfly-shaped vertebrae found at D2 D4 D8 D9 D10 with low-lying conus at L4-L5 level. Surgical excision of the tail and untethering of the spinal cord along with excision of dermal sinus were performed. The infant had an uneventful postoperative period and was unchanged neurologically. CONCLUSION: To our knowledge, no similar case has been reported in English literature till date. DISCUSSION: The specific features of this rare case of human tail treated surgically are discussed in light of the available literature.

First Successful Separation of Craniopagus Twins in India-Plastic Surgical Considerations.

Separation of total vertical craniopagus with shared venous sinuses poses multiple challenges. Provision of soft-tissue cover to the exposed brains at the time of total separation is one of them, due to the large size of the defect and paucity of local tissues. Staged separation of twins is advised with partial venous and parenchymal disconnection in the first stage and total separation in the second stage. Tissue expanders are inserted in the first stage, and second stage planned to coincide with the period of adequate expansion. In the child being reported, emergency second stage was done due to the deteriorating general condition of the children. Left with inadequate expanded skin, the critical defect in a twin was managed with bilateral trapezius myocutaneous flaps. High ionotropic support of the postoperative period resulted in superficial necrosis of the flap, which was managed by debridement, allograft application and autograft later. Both twins had well-healed wounds by 3 months.

Detection of metabolic pattern following decompressive craniectomy in severe traumatic brain injury: A microdialysis study.

OBJECTIVE: The aim of the study was to detect mitochondrial dysfunction and ischaemia in severe traumatic brain injury and their relationship with outcome. METHODS: Forty-one patients with severe traumatic brain injury (TBI) who underwent decompressive craniectomy were prospectively monitored with intracerebral microdialysis catheters (MD). Variables related to energy metabolism were studied using microdialysis. RESULTS: Twentysix patients (63.4%) had a good outcome in terms of Glasgow outcome score (GOS) at 6 months while the rest (15 patients) had poor GOS at 6 months. Mitochondrial dysfunction was defined as Lactate Pyruvate ratio (LP ratio) > 25 and pyruvate <70 while ischaemia was defined as LP ratio > 25 and pyruvate >70. The poor outcome group showed significantly higher proportion of mitochondrial dysfunction 65.9% vs. 55.9% (p<0.001) and ischemia 13.9% vs. 7.2% (p<0.001) Conclusions: After decompressive craniectomy in severe TBI, patients with higher incidence of mitochondrial dysfunction and ischaemia were more likely to have poorer outcome with ischaemia having a more profound effect. ABBREVIATIONS: Traumatic brain injury (TBI), microdialysis (MD), lactate pyruvate ratio (LP ratio), Glasgow coma scale (GCS), Glasgow outcome scale (GOS), cerebral perfusion pressure (CPP), intracranial pressure (ICP), mitochondrial transition pore (MTP), non-contrast computed tomography (NCCT), traumatic axonal injury (TAI).

Pediatric giant pituitary adenomas: are they different from adults? A clinical analysis of a series of 12 patients.

OBJECTIVES: To evaluate clinical presentation and microsurgical outcome of giant pituitary adenomas (GPAs) in pediatric age. METHODS: All patients <18 years, who were operated on at our center for GPA (tumor >40 mm in maximum diameter) were included in study. Clinical features, hormonal profile, radiology, surgical approach, results and complications were analysed. RESULTS: A total of 12 children with GPA were managed microsurgically. Visual deterioration (73 %) was most common presentation. Functioning adenomas were found in 83 % patients, with prolactinomas being most common. Twelve patients underwent a total of 16 microsurgical procedures, with a single surgery done in eight (75 %) patients. Out of the 12 primary surgeries, eight (67 %) were performed trans-sphenoidally. A near-total excision (>90 % tumor removal) could be achieved in six (50 %) patients. Visual improvement was observed in 44 % patients. However, there was no improvement in those where the eye was negative to perception of light prior to surgery. At the last follow-up, all the patients with functioning adenomas were in hormonal remission, and there was no residual/recurrent tumor in patients with non-functional adenomas. 25 % experienced single or multiple perioperative or postoperative complications. There was one perioperative death (8 %). CONCLUSIONS: GPAs are very rare in the pediatric population, with majority being functional and more aggressive in nature as compared to in adults. However, most of them can be approached trans-sphenoidally. The combination of surgery and radiotherapy, as well as medical therapy with bromocriptine, achieves good tumor control, despite a high rate of residual tumor and tumor recurrence.

Role of simvastatin in prevention of vasospasm and improving functional outcome after aneurysmal sub-arachnoid hemorrhage: a prospective, randomized, double-blind, placebo-controlled pilot trial.

BACKGROUND: Vasospasm plays a major role in the morbidity and mortality after aneurysmal subarachnoid hemorrhage (aSAH). The preliminary studies suggest that statins protect against cerebral vasospasm. OBJECTIVE: The aim of the study was to determine the role of simvastatin in preventing clinical vasospasm and improving functional outcome in patients with aSAH. METHODS: All patients with aSAH admitted within 96 h of ictus were randomized to receive either Simvastatin or placebo - 80 mg/day for 14 days. Thirty eight patients were recruited in the study- 19 received Simvastatin and 19 placebo. All the patients underwent surgical clipping of the aneurysm. The primary outcome of the study was the development of clinical cerebral vasospasm. The secondary outcomes included Glasgow Outcome Score (GOS), Modified Rankin Scale (MRS) and Barthel Index Score (MBI) at follow-up at 1, 3 and 6 months. RESULTS: 16% of the patients in the simvastatin group had high Middle Cerebral Artery velocities (> 160 cm/sec) on transcranial Doppler on one or more than one day during the study duration as compared to 26% of the patients in the placebo group (p = 0.70). Neurological deterioration occurred in 26% and 42% of the patients in simvastatin group versus placebo group, respectively (p = 0.31). There was an improvement in the functional outcome in the simvastatin group at 1, 3 or 6 months in the follow-up; however, this difference was not statistically significant. CONCLUSIONS: There was benefit of simvastatin in terms of reduction in clinical vasospasm, mortality or improved functional outcome, however, this was not statistically significant.

Spinal dural arterio-venous fistula: clinico-radiological profile and outcome following surgical occlusion in an Indian neurosurgical center.

BACKGROUND: Spinal dural arteriovenous fistula (SDAVF) is a common type of spinal vascular lesion. However, there has not been any published study on its clinico-radiological characteristics or surgical outcome from India. AIM: The aim of this study was to determine the clinico-radiological features of patients with SDAVF, outcomes following surgical ligation of the fistula and the various factors involved. MATERIALS AND METHODS: Patients who were operated for SDAVF were studied for demographic details, symptoms, clinical severity, radiological features and neurological outcome in the form of improvement in gait disability grades. Appropriate statistical tests were performed. RESULTS: There were 22 (19 males, 3 females) patients of SDAVF who underwent surgical ligation with a mean age of 55 years. The mean duration of symptoms at presentation was 15 months. Three patients had acute onset while the rest had insidious onset of symptoms. Out of the 22 patients, 11 (50%) had motor weakness as the first symptom, 13 (59%) were bedridden and 19 (86.4%) had bladder involvement at presentation. Thirteen patients had fistulae in thoracic spine, whereas eight had fistulae in the lumbar spine. All had a favorable outcome in the form of at least non-progression of gait disability (14 had improvement while 8 had stabilized). The improvement was non-significantly associated with younger age, acute onset, ambulant status and fistula below T9. It was inversely associated with pain as the first symptom and fluctuant clinical course. CONCLUSION: Surgical occlusion of SDAVF is usually associated with either improvement or stabilization of motor weakness.

Von-Hippel Lindau (VHL) syndrome with bilateral cerebellar hemangioblastomas and retinal angiomas: A rare presentation.

INTRODUCTION AND IMPORTANCE: Hemangioblastomas are benign vascular neoplasms which originate almost exclusively from central nervous system (CNS). They account for 2 % of all intracranial neoplasms, 7 %-12 % of posterior fossa tumours. 60-75 % cases of hemangioblastomas occur sporadically and rest 25 % to 40 % occur in genetically inherited in the autosomal dominant neoplasia syndrome known as Von Hippel-Lindau (VHL) disease with mutation occurring on chromosome 3p. CASE PRESENTATION: We, hereby, report a case of one such rare case of a 30-year old male who presented to us with the complaint of holocranial headache for past 2 months associated with vomiting along with bilateral cerebellar signs and symptoms. MRI Brain revealed multiple cysts with enhancing mural nodules present on both cerebellar hemispheres. Due to recurrence and multi-focality of lesions, VHL syndrome was suspected. CLINICAL DISCUSSION: VHL is a rare autosomal dominant disease. MRI is the gold standard test. It demonstrates specific appearances depending on the tumour anatomy. Hemangioblastoma of the CNS in VHL usually develop from childhood at an age of <10 years or early teen until the age of 30 years. The most common locations for hemangioblastomas in VHL are cerebellum and spinal cord as seen in our patient. CONCLUSION: VHL being a lifelong disease with no cure till date, the patients should be frequently screened for lesions at various potential sites in his/her lifetime so as to provide required intervention at the earliest. Hence, a genetic screening for VHL (VHL tumour suppressor gene), should be performed in cases of hemangioblastoma.

Cerebral Myiasis Secondary to Burr Hole Evacuation: A Rare Illustrative Case Report.

Myiasis (maggot infestation) is a condition in which fly maggots feed off and develop in the tissues of living organisms. Most common in tropical and subtropical regions, human myiasis, is prevalent among individuals in close association of domestic animals and those inhabiting the unhygienic conditions. We, hereby, describe a rare case of cerebral myiasis (17th in the world, 3rd in India) that presented to our institution in Eastern India secondary in the operated site of craniotomy and burr hole few years back. Cerebral myiases are exceedingly rare conditions, especially in high-income countries with only 17 previously published cases with the reported mortality as high as 6 out of 7 cases dying of the disease. We additionally also present a compiled review of previous case literatures to highlight the comparative clinical, epidemiological features and outcome of such cases. Although rare, brain myiasis should be a differential diagnosis of surgical wound dehiscence in developing countries where conditions do exist in this country that permit myiasis. This differential diagnosis should be remembered, particularly when the classic signs of inflammation are not present.

Vertebral choristoma in lipomyelomeningocele: a case report.

Lipomeningocele is a type of occult spinal dysraphism characterized by a subcutaneous lipomatous mass that protrudes through a midline bony defect. We report a rare presentation of this condition--a vertebral choristoma wherein the lipomatous mass displaced the normally formed posterior elements--lamina and spinous process of L4 vertebra dorsally to an abnormal location in the absence of a bony defect.

Primary tethered cord syndrome--clinical and urological manifestations, diagnosis and management: a prospective study.

Primary tethered cord syndrome refers to a group of neural tube defects that are not externally obvious, and, if detected at an early age, surgical intervention may prevent the significant irreversible neurological deficits. This study was performed to evaluate the presenting clinical features of patients with primary tethered cord syndrome and the indications of surgery in such patients as well as the clinical and urological outcome. In all cases, the indication for surgery was the presence of a tethered cord on magnetic resonance imaging, the criteria for tethering being a low-lying conus (below L1-L2) and a thickened filum (>2 mm). Urodynamic studies were performed before detethering. Microsurgical detethering of low-lying cord was then performed, and the patients were then followed clinically and urologically for 6 months. Pain responded the most to detethering while limb weakness and urological symptoms responded the least. Clinical improvement in urological symptoms correlated with improvement in urodynamic parameters. A urodynamic study identified improvement in a larger number of patients and also deterioration in a few patients which was not visible clinically; this may point to its high sensitivity and usefulness in preceding clinical manifestations in a future follow-up.

MAGAT gel and EBT2 film-based dosimetry for evaluating source plugging-based treatment plan in Gamma Knife stereotactic radiosurgery.

This work illustrates a procedure to assess the overall accuracy associated with Gamma Knife treatment planning using plugging. The main role of source plugging or blocking is to create dose falloff in the junction between a target and a critical structure. We report the use of MAGAT gel dosimeter for verification of an experimental treatment plan based on plugging. The polymer gel contained in a head-sized glass container simulated all major aspects of the treatment process of Gamma Knife radiosurgery. The 3D dose distribution recorded in the gel dosimeter was read using a 1.5T MRI scanner. Scanning protocol was: CPMG pulse sequence with 8 equidistant echoes, TR = 7 s, echo step = 14 ms, pixel size = 0.5mm × 0.5mm, and slice thickness of 2 mm. Using a calibration relationship between absorbed dose and spin-spin relaxation rate (R2), we converted R2 images to dose images. Volumetric dose comparison between treatment planning system (TPS) and gel measurement was accomplished using an in-house MATLAB-based program. The isodose overlay of the measured and computed dose distribution on axial planes was in close agreement. Gamma index analysis of 3D data showed more than 94% voxel pass rate for different tolerance criteria of 3%/2 mm, 3%/1 mm and 2%/2 mm. Film dosimetry with GAFCHROMIC EBT 2 film was also performed to compare the results with the calculated TPS dose. Gamma index analysis of film measurement for the same tolerance criteria used for gel measurement evaluation showed more than 95% voxel pass rate. Verification of gamma plan calculated dose on account of shield is not part of acceptance testing of Leksell Gamma Knife (LGK). Through this study we accomplished a volumetric comparison of dose distributions measured with a polymer gel dosimeter and Leksell GammaPlan (LGP) calculations for plans using plugging. We propose gel dosimeter as a quality assurance (QA) tool for verification of plug-based planning.

Monitored gradual occlusion of the internal carotid artery followed by ligation for giant internal carotid artery aneurysms.

OBJECTIVE: To demonstrate a technique of gradual monitored occlusion of the internal carotid artery (ICA) followed by ligation for giant aneurysms as an option for balloon test occlusion followed by permanent ligation of ICA. MATERIALS AND METHODS: Authors retrospectively analyzed 27 patients with giant and complex ICA aneurysms who underwent carotid artery ligation between January 2001 and December 2010. Clinical presentation included headache, vision loss and diplopia. There were 19 patients with cavernous aneurysm, 5 supraclinoid, 1 ophthalmic, 1 petrous segment and 1 cervical segment aneurysm located extracranially. All demonstrated good cross-circulation. Selverstone clamp was used for gradual occlusion of the ICA over 72 h under closed observation in the intensive care unit. RESULTS: Six patients developed hemiparesis in the postoperative period. Improvement occurred in one patient over two to three weeks while the remaining five patients had residual hemiparesis. One patient developed malignant MCA infarct for which decompressive craniectomy had to be done. There was no mortality in the present series. CONCLUSIONS: Gradual monitored occlusion and ICA ligation may be a simple, safe alternative procedure to clipping in surgically inaccessible and complex aneurysms, especially for surgeons with limited experience. Cross circulation study is an absolute requisite for carotid ligation.

Giant encephalocele: a study of 14 patients.

BACKGROUND: Giant encephalocele is a rare condition and few published reports are available in the English literature. It is a challenge to neurosurgeons, even today. This series consists of 14 patients with giant encephaloceles treated at our institute. MATERIAL AND OBSERVATION: Over a period of 8 years, from 2002 to 2009, 110 patients with encephaloceles were managed at our institute. Amongst them, 14 were children with giant encephaloceles. All patients had CT/MRI or both prior to surgery, and all were operated upon. Four patients were neonates, under 1 month of age, and 9/14 patients (64%) were under 3 months. The youngest child was a newborn baby aged 2 days. Except for 1 with an anterior encephalocele, the rest were patients with occipital encephaloceles. A CT scan was performed on 5 and an MRI on 1 patient. Both CT and MRI scans were performed on the other 8 patients. MRI/CT showed hydrocephalus in 10/14 patients. Of these, 7 required ventriculoperitoneal (VP) shunt, and the remaining 3 with mild to moderate hydrocephalus did not. Of the 7 patients who underwent VP shunt, 5 had a shunt during the encephalocele repair and 2 had a postoperative shunt for increasing hydrocephalus. RESULTS: Other associated anomalies recorded were acquired Chiari malformation in 3 patients, secondary craniostenosis with microcephaly in 5, and syringomyelia in 1 patient. All the patients underwent repair of encephalocele and 4 had suturectomy of coronal suture for the secondary craniostenosis. There were 2 postoperative deaths due to hypothermia. Among the 12 surviving patients, 9 had a good outcome and 3 had poor mental development. The present study shows overall good outcomes in 9/14 (66%) patients.

An unusual case of spinal dysraphism with four splits including three posterior spurs.

BACKGROUND: Composite-type split spinal cord malformation (SCM) is very rare and results from 2 separate foci of ectoendodermal adhesions and endomesenchymal tracts leading to the development of SCM with an intervening normal cord in the same patient. Posterior spurs are even rarer. We report a unique case of composite SCM, where the patient had 3 posterior bony spurs and 1 fibrous spur at different levels. To our knowledge, this is the first reported case of a patient having composite-type SCM with posterior spurs at 3 different levels. CLINICAL PRESENTATION: A 3-year-old girl presented to us with progressively worsening scoliosis. Neurological examination was unremarkable except for mild weakness in the left lower limb. Three-dimensional CT imaging demonstrated posterior bony spurs located at T(6), T(12) and L(3) levels. The patient underwent T(11)-L(5) laminoplasty and T(5)/T(6) laminectomy and excision of SCM type 1 with division of the thickened filum terminale. All the bony spurs arose from the inner surface of the lamina, where the base of the spur was located. CONCLUSIONS: The unique point of this case report is the rarity of such a presentation. Careful planning and knowledge of the occurrence of such conditions can make surgery in these children safe, and all the levels can be treated in a single setting.

Long-segment type 1 split cord malformation with two-level split cord malformation and a single dural sac at the lower split.

OBJECTIVE AND IMPORTANCE: Two-level bony spurs are rare and also a very long segment of bony spur is very rare. Bony spur with a single dural covering is not reported in the literature. Here, we report a case of prophylactic surgery performed on a patient with a bony spur with a single dural covering who showed a good postoperative outcome. This case highlights the importance of prophylactic surgery in such patients to avoid cord injuries. CLINICAL PRESENTATION: Since birth a 1-year-old child had had a tuft of hair at the dorsal (D6 and D7) region. The patient was examined and found to have scoliosis with convexity towards the right. There was no neurological deficit. MRI and CT spine scans showed 2-level type 1 split cord malformation (SCM) and there was a low-lying conus at the L(3-4) level. INTERVENTION: The patient was operated on at the age of 1 year and 2 separate bony septae were observed. The upper one extended from D5 to D9 and the lower one was at the D11 level. The bony spurs were excised. The filum was sectioned at the L(4-5) level by a separate incision. The patient had a good postoperative recovery. CONCLUSION: With meticulous planning and careful surgery, prophylactic surgery can have a very good outcome in long-segment and two-level type 1 SCM.

Transsellar transsphenoidal encephalocele: a series of four cases.

Transsellar transsphenoidal encephalocele is the least common type of basal encephalocele. We present a series of four cases of transsellar transsphenoidal encephalocele. Clinical findings, imaging reviews, surgical repair techniques and postoperative morbidity are discussed with the relevant literature. Non contrast CT scan head with 3D reconstruction and magnetic resonance imaging should be done in all patients of transsphenoidal encephalocele. Endocrine assessment is also essential. Repair of a transsphenoidal encephalocele should be coordinated between a team of neurosurgeons and ENT surgeon. Our surgical outcome supports the transpalatal/ transnasal approach over the transcranial approach.

Microsurgical management of prolactinomas - clinical and hormonal outcome in a series of 172 cases.

AIMS AND OBJECTIVES: To report hormonal outcome after surgery in a special subgroup of prolactinomas in whom medical therapy is not effective or not indicated. PATIENTS AND METHODS: All patients who underwent surgery for prolactinomas, between December 2002 and December 2009, were analyzed retrospectively. The study group consisted of patients who were either intolerant or noncompliant to dopamine agonist (DA) therapy or those in whom medical treatment could not be given due to various reasons. The surgical results were assessed according to whether patients had microadenoma (<1 cm diameter), macroadenoma (>1 cm diameter), or giant prolactinoma (>4 cm diameter). Initial and final hormonal remission was defined as normalization of serum prolactin levels (<25 ng/ml) at 7 days after surgery and at last follow up, respectively. RESULTS: Of the 172 patients, 133 (77%) were operated by primary transsphenoidal approach and 25 (14.5%) were operated transcranially and 14 patients required reoperation, either transsphenoidally (6 patients) or transcranially (8 patients). Overall, at last follow-up, hormonal remission could be achieved in 44% patients (83% microadenomas, 48% macroadenomas, and 16% of giant adenomas). There were three deaths (1.7%), 12 patients (7%) had single/multiple postoperative treatable complications. 59% of those patients, who did not achieve remission after surgery, finally attained normoprolactinemia with either DA therapy or radiotherapy, at last follow-up. CONCLUSIONS: Medical treatment of prolactinomas with DA should be considered as treatment of choice for these tumors. However, there is a major subgroup of patients who respond better to surgery rather than protracted medical therapy.

Use of O-arm for spinal surgery in academic institution in India: experience from JPN apex trauma centre.

There is a relatively high incidence of screw misplacement during spinal instrumentation due to distortion of normal anatomy following spinal trauma. The O-arm® is the next-generation spinal navigation tool that provides intraoperative 3-D imaging for complex spine surgeries. In this prospective study over 1-month period, 25 patients (mean age 29.16 years (range 7-58 years), 22 (88%) males) with spinal injury who underwent spinal instrumentation under O-arm® guidance were included. Fall from height (64%) was the most common etiology seen in 16 patients. The majority (68%) had dorsolumbar fractures. Spinal canal compromise was seen in 21 patients (84%). Ten patients (40%) had American Spinal Injury Association (ASIA) grade A injuries, two patients (8%) had grade B, five patients (20%) had grade C, four patients (16%) each had grade D, and grade E injuries. A total of 140 screws were inserted under O-arm guidance. Of these, 113 (81%) were dorsolumbar pedicle screws, 2 were odontoid screws, 12 were anterior cervical screws, and 12 screws (48%) were lateral mass screws. Mean duration of surgery was 4.5 h with a mean blood loss of 674 mL. The mean postoperative stay was 6.3 days. None of the patients had screw malplacement ort canal breach. No patient deteriorated in ASIA grade postoperatively. The system was rated as excellent for ease of use by all faculty using the system. Accurate screw placement provides better patient safety and reduces the in hospital stay thereby leading early patient mobilization and may reduce the cost incurred in patient management.