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.

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.

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.

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.

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.

Molecular Genetics involved in Neural Tube Defects: Recent Advances and Future Prospective for Molecular Medicine.

BACKGROUND: Folic acid and multivitamin supplements ((FAMVS) and genetics involvement is one of the major roles in the development of neural tube defects (NTDs). OBJECTIVE: Our prior aim and objective is to establish an unique guideline and helps the policy decision making for our country India and the World. MATERIALS AND METHODS: We have collected the data through the literature from the World for their necessary action, rehabilitation part all objectively in PubMed/Medline, Scopous, Embase, Cochrane Review, Hinari, and Google scholar. STATISTICAL ANALYSIS: Statistical analysis was performed with very simple and logistic statistics, percentage, mean, total as collection through the available software SPSS with new version 17.0. RESULTS: The overall (70-95%) we find out those infants with neural tube defects (NTDs) associated with genes involvement and maternal vitamin intake (MVI). Before pregnancy relative risk (PRR) prior to non intake noted as 90% significantly reduced their risk of the NTDs. Now (40-60%) of the women of child-bearing age (CBA) don't use the folic acid intake and supplements (FAISs) in proper way in villages, urban, industrial and sewage areas. We find out that the genetic variants of the fourteen special reported genes, had the major risk factor (MRF) for the (NTDs) and associated abnormalities rate (AAR) within the developmental process in the human brain. CONCLUSIONS: The (45-55%) people still having at ignorant zone, due to lack of education, genetic counseling, and awareness till date.

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

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).

Anal extrusion of migrated ventriculo-peritoneal shunt catheter: An unusual complication and review of literature.

Authors present an unusual case of anal extrusion of peritoneal end of ventriculo-peritoneal shunt in a 2-year-old male child. Pertinent literature is reviewed regarding this rare complication of a very commonly performed neurosurgical procedure.

Paired discharging sinuses at medial canthus of left eye and dorsum of nose in a 2-year toddler since birth associated with interfalcial dermoid.

Twin nasal dermal sinus with associated intracranial dermoid located in interfalcial region is a very rare occurrence and is reported only in the occipital and temporal regions. However, multiple sinuses located in the canthus and dorsum of nose are not reported till date. Authors report an interesting first case of interfalcial dermoid cyst associated with twin discharging dermal sinuses, who underwent successful surgical repair in the world literature. The authors report the management of an unusual case and the review has been discussed briefly.

Pain perception following different neurosurgical procedures: a quantitative prospective study.

BACKGROUND: Pain following neurosurgery has never been given due attention. OBJECTIVE: This was a prospective study to assess pain following various neurosurgical procedures. METHODS: Patients underwent pain assessment on 11-point scale(0-10) for 24 hours following neurosurgery, and analyzed in relation to various factors. RESULTS: Among total 159 patients, 88(55%), 58(37%) and 13(8%) had undergone cranial, spinal and peripheral nerve procedures respectively. The mean pain score within 12 hours was 3.51(SD ± 2.53), which increased significantly during 13-24 hours to 5.06(SD ± 2.6)(P<0.001). During 13-24 hours, the pain score among those who underwent infratentorial procedures (8.02 ± 2.77) was significantly higher than among those who underwent supratentorial procedures (3.48 ± 1.99)(P<0.001). The pain score of patients who underwent lumbar surgery (6.5 ± 1.93) was significantly higher than of those who underwent cervical surgery (4.04 ± 2.43)(P<0.001). Age and gender did not show any significant influence on pain. CONCLUSION: Pain is significantly greater during 13-24 hours after neurosurgery, especially after infratentorial and lumbar surgical procedures, compared to others.

Giant pediatric glioblastoma multiforme causing primary calvarial erosion and sutural diastasis presenting with enlarged head.

Authors report a rare case of supratentorial glioblastoma multiforme in a 13-year-old boy, who had headache, vomiting and left sided hemiparesis for last 6 months. On evaluation by primary physician he was labeled as hydrocephalus in view of enlarged head with papilledema on fundoscopic evaluation and no imaging was carried out. On current admission, magnetic resonance imaging brain revealed a large heterogeneous mass lesion involving right frontoparietal region associated with massive perilesional edema causing significant mass effect. He underwent right fronto-temporal craniotomy and intraoperatively erosion of parietal bone was observed, unassociated with any extradural deposit of tumor. After surgery, he noticed improvement in headache along with hemiparesis. Primary calvarial erosion in glioblastoma is extremely rare, and there is paucity of literature as evident from the few case reports reported previously and all occurred in elderly, so current case is the first pediatric case having primary calvarial erosion. Management of such case and pertinent literature is briefly discussed.

Pediatric posterior cerebral artery stroke as a presentation of atlantoaxial dislocation.

We report an uncommon case of posterior circulation stroke in a young patient due to occlusion of posterior cerebral artery with reducible atlantoaxial dislocation (AAD). Plain dynamic radiography showed reducible AAD and intra-arterial digital subtraction angiography demonstrated occlusion of left posterior cerebral artery. Patient underwent stabilization of craniovertebral junction by occipito cervical fixation using occipit-C2/C3 lateral mass screws and rod fixation and has since experienced no recurrent symptoms. Vertebrobasilar insufficiency is a known entity in a patient with reducible AAD; however, isolated involvement of posterior cerebral artery is very rare. So this condition should be kept in mind, and necessary interventions must be undertaken at the earliest to avoid further irreversible brain damage.

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.

A giant occipital encephalocele with spontaneous hemorrhage into the sac: A rare case report.

In giant encephalocele, head size is smaller than the encelphalocele. Occipital encephalocele is the commonest of all encephalocele. In our case, there was rare association with giant encephalocele with old hemorrhage in the sac. This was a unique presentation. In world literature, there was rare association with giant encephalocele with hemorrhage.

Compound elevated skull fracture: a clinical series of three patients with a review of the literature.

AIM: Compound elevated fracture of the skull is an unusual variety of fracture of the cranial vault that has been rarely described in the currently available literature. The authors describe three such patients with post-traumatic compound elevated skull fracture. The pertinent literature is reviewed, the injury mechanism is highlighted and the management issues are discussed in detail. CASE DESCRIPTION: All three patients in this series presented with elevation of free skull fracture fragments above the surface. The mode of injury was rail accident in two patients and injury by a rapidly moving crane in one patient. Two patients had dural laceration with extrusion of brain matter through the dural defect. The remaining patient had an elevated fracture fragment simulating a formal craniotomy with an intact dura. The patient who suffered crane injury was brought dead. Surgery was performed in remaining two patients. Wound debridement and duraplasty was performed. Both patients received broad-spectrum antibiotics in anti-meningitic doses. Both of these patients did well after surgery. CONCLUSION: Elevated skull fractures are usually compound injuries. High velocity tangential impact to the skull vault is the most likely causative mechanism in such type of injury. Delay in surgery could lead to intracranial sepsis including meningitis and brain abscess. Thus, these fractures should be aggressively treated.