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.

Role of prophylactic surgery in spinal tuberculosis: A retrospective study from Eastern India.

Background: Tuberculosis (TB) is broadly classified into pulmonary and extrapulmonary TB. Skeletal TB is considered to be a form of extrapulmonary TB whose incidence is around 1% of all forms of TB. The incidence of spinal TB is more than 50% of the entire skeletal TB. Methods: A total of 92 consecutive patients, treated over a period of 2 years (January 2021-January 2023), are retrospectively analyzed. Results: In our study, out of 92 patients, the pain had subsided in 90% of cases, and 12 patients showed improvement in motor power postoperatively. In our study, the patients have benefited due to the surgical intervention in decrease of pain and improvement in motor deficits in patients. The 29 patients who were operated on prophylaxis with pain as the only symptom benefitted from the resolution of the symptom of pain and did not develop any morbidity in their long-term follow-up. The sensory and bladder/bowel symptoms did not improve after surgery. Conclusions: In the author's view, prophylactic surgery for spinal TB is safe and effective with confirmation of the disease. However, a well-designed randomized controlled trial, to definitely and objectively prove the usefulness of prophylactic surgery, is needed.

Spinal fungal abscess mimicking as potts spine.

Spinal epidural abscess (SEA) is a rarest form of spinal infections and is characterized by the presence of pus in the space between the dura mater, and the osseoligamentous confines of the vertebral canal. This can cause spinal injury due to direct compression or local ischemia. The major etiology of SEA is bacterial and tuberculous in endemic regions. The incidence of fungal spinal abscess is relatively low and <5% of SEA is attributable to fungi. We, here, report a case of 77-year-old known patient of chronic renal disease on hemodialysis that presented with low-back pain for 15 days and was subsequently diagnosed with SEA by magnetic resonance imaging, and causative organism was identified as Candida by culture. The abscess was surgically drained after laminectomy. The patient improved with surgery and antifungal treatment.

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.

Pedunculated Chiari 3 malformation with proatlas defect.

Chiari III malformation is considered to be a rare congenital abnormality in human with very high mortality rates. Seventy percent of Chiari III is found to be associated with C1 arch defect as reported by Cakirer (Clin Imaging 27:1-4, 2003). The herniation of posterior fossa elements or dysplastic neural tissue is a must to stamp it as Chiari 3 malformation. The malformation is a result of the abnormal development of craniovertebral junction (CVJ). The CVJ developed from the occipital somites and the first spinal sclerotome. The major role in the development of the CVJ is played by the fourth occipital somite, which is otherwise known as "proatlas." The Chiari III anomalies are due to a result of proatlas defect, which results from failures of segmentation, failures of fusion of different components of each bone, or hypoplasia and ankylosis. We are presenting a case of a 1-year 4-month-old female child who presented with pedunculated swelling at the suboccipital region. The swelling was cystic and with pulsation. On evaluation, we found Chiari III anomaly with C1 posterior arch deficiency (proatlas defect). He was surgically managed. The outcome of the patient was good. Despite literature concluding Chiari 3 malformation with an unfavorable outcome, however, meticulous management and good pre- and postoperative care, physical therapy, and follow-up are necessary for good outcome.

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.

Complete intraventricular migration of ventriculo-peritoneal shunt: A rare case report.

INTRODUCTION AND IMPORTANCE: A ventriculoperitoneal (VP) shunt is a cerebral shunt used to treat hydrocephalus. This is used to remove the excessive accumulation of cerebrospinal fluid inside the ventricles. CASE PRESENTATION: We are reporting a rare case of complete intracranial migration of a ventriculoperitoneal shunt, a potentially fatal complication, presenting to us with hydrocephalous. The baby was taken up for surgery with endoscopic assisted shunt removal and shunt revision. CLINICAL DISCUSSION: Ventriculoperitoneal shunt is one of the common procedures used in infants and children, however, it is used in all age groups for hydrocephalous. The shunt is associated with multiple large number of complications like obstruction, infection, migration, and separation from the connected site. Etc. Shunt migration is a less common complication of which cranial migration is still less common. CONCLUSION: Optimum creation of the sub-galeal space for the shunt chamber, a smaller burr hole, a smaller dural opening, and proper anchorage of the chamber to the pericranium, are some of the measures that may be useful in obviating this complication.

A rare case of intramedullary tuberculosis with paraparesis.

Intramedullary tuberculoma (IMT) is considered to be a rare form of spinal tuberculosis (TB). Overall, TB of the central nervous system accounts for approximately 1% of all cases of TB and 50% of these involve the spine. The clinical presentation of spinal intramedullary TB is similar to an intramedullary spinal cord tumor mass. The factors attributable could be malnutrition, poor socioeconomic conditions, and immunodeficiency syndromes. As per the reports, the incidence of primary intramedullary TB is 2 in 100,000 cases among patients with TB. We describe one such patient who presented with progressive asymmetrical paraparesis due to histologically confirmed intraspinal tuberculoma. Paraparesis in spinal IMT is considered to be rare. Hereby, we present the case of a 29-year-old female who presented with asymmetric onset paraparesis of 6 months with associated numbness and tingling began in the left foot 3 months which was ascending in nature. There was no history of stiffness, involuntary movements, flexor spasms, thinning, or fasciculations of muscles. There was a loss of sensation pain, touch, and temperature below L3 with normal reflexes. Power in both the lower limbs was 1/5 as per Medical Research Council (MRC) grading. She underwent a contrast magnetic resonance imaging spine which was suggestive of an intramedullary SOL at D12 vertebral level. The patient underwent surgical intervention with resection of the SOL. Histopathology was confirmed to be an IMT. She was started on Category 1 (antitubercular drugs) and further investigated for primary source, which was found to be negative. We want to emphasize that TB can involve any part of the body. It should be kept as a differential diagnosis of any chronic inflammatory lesion involving the bony skeleton, especially in endemic countries where combined surgical and medical treatment is usually sufficient to provide a cure.

Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Clinicoradiological Poorly Recognized Entity- An Institutional Experience.

AIM: Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare nonmeningothelial mesenchymal tumors sharing fusion of NGF1-A binding protein 2 (NAB2) and signal transducers and activators of transcription (STAT6.).The WHO classification of central nervous system (CNS) tumors (2016) highlights that molecular confirmation of NAB2/STAT6 fusion or immunohistochemical nuclear expression of STAT6 is mandatory for the diagnosis of SFT/HPC.Herein, we present a series of four cases of SFT/HPC of the brain, which mimicked other CNS tumors both clinically and radiologically. MATERIAL AND METHODS: This is a retrospective study over a period of two and a half years. Out of the 156 operated cases of brain tumors, four patients (2.56%) were diagnosed with SFT/HPC. The clinicoradiological details with the surgical procedure were retrieved from the archived hospital records. RESULTS: All cases were males, of which three were in their 5th decade while one was a 14-month-old baby. Two cases were primary and the rest were recurrent.The location of tumors was extra-axial left cerebellotentorial,clivaldural-based,left cerebellar, and in the left frontoparietal region,respectively. The clinical impression was meningioma in three cases,while it was primitive neuroectodermal tumor (PNET) /atypical teratoid/rhabdoid tumor (ATRT) in one case.With the detailed histomorphology and immunohistochemistry, the final diagnosis was anaplastic hemangiopericytoma(WHO grade III) for all the cases. During our follow-up, one patient died with the disease, while the rest are doing well. CONCLUSION: SFT/HPC should be kept in the differential diagnosis of all dura-based hypervascular masses, especially in recurrent cases, due to its aggressiveness and high recurrence rate.

Primary cerebellopontine angle glioblastoma in an adult.

Supratentorial glioblastoma is the most frequent primary intracranial tumor in adults. Its localization in the cerebellum is extremely rare and such tumor presenting as cerebellopontine angle (CPA) mass in an adult is even rarer. We report the case of primary cerebellar glioblastoma in a 52-year-old woman who presented clinically and radiologically as CPA mass. Its clinical presentation, radiological findings, and treatment instituted are discussed in view of pertinent literature.

Recurrence of spinal schwannoma: Is it preventable?

Spinal schwannomas account for about 25% of primary intradural spinal cord tumors in adult. The prognosis for spinal schwannomas is excellent in most cases. Complete resection is curative. However following subtotal removal, recurrence develops after several years. We describe a case of recurrent spinal schwannoma who had been operated twice before for same disease. The possible cause of recurrence and difficulties in reoperation are discussed.

Development of contralateral extradural hematomas after evacuation of primary one.

The occurrence of bilateral extradural hematomas (EDH) is an uncommon consequence of closed head injuries. Incidence of bilateral EDH has been reported in various studies ranging from 2 to 25%. Bilateral EDH may develop simultaneously or second EDH develops few hours after first one. Development of second EDH after evacuation of primary one is rarely seen. We are reporting one such case. Awareness of this entity is required to detect such cases as timely intervention gives an excellent result like an acute EDH.

Transorbital penetrating brain injury by a wooden stick: A neuro-ophthalmologic emergency.

Accidental penetrating brain injury through transorbital route is an unusual occurrence in emergency practice of civilian cases. At time, it could be potentially life threatening. We report an interesting case of transorbital penetrating brain injury with a wooden stick in a 44-year-old male epileptic patient. The stick was removed successfully through transorbital exploration, without any neurovascular complications. The pertinent literature is reviewed and emergent management of such cases is discussed.

Malignant peripheral nerve sheath tumor presenting as orbito temporal lump: Case report and review of literature.

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. To our knowledge, few patients with a cranial or facial MPNST have been reported. We report such a lesion in a 35-year-old woman who presented with left sided rapidly progressive proptosis and visual loss due to an orbital lump extending up to the temporal lobe. Cranial imaging showed a huge mass invading the orbital wall and temporal bone. The presumptive diagnosis was a malignant orbital tumor. Preoperative fine needle aspiration cytology of the orbital mass came to be neurofibroma. Near total resection of the tumor was done. Histopathology revealed MPNST which was subsequently confirmed on the basis of immunopositivity for S-100. The patient recovered uneventfully and was discharged 8 days after surgery with an advice to attend cancer institute for possible radiotherapy.

Giant aneurysmal bone cyst of cervical spine: Surgical management and circumferential spinal fusion in a 13-year-old girl.

Aneurysmal bone cysts (ABCs) of the cervical spine are rare occurrences accounting for about 2% of all cases. Having a propensity to develop in the pediatric population, it generally involves the posterior elements with occasional extension to the facets and body of one vertebral level. Surgical treatment of high cervical ABC in pediatric patients is challenging. Where as complete tumor resection should be attempted for the best chance for cure, spinal fusion should be added to correct the defects created by resection to minimize the risk of postoperative instability. This paper presents a multi-level giant expansile ABC of the high cervical spine involving all three spinal columns in a 13-year-old girl and discuss the surgical management. Surgical treatment in this patient included complete tumor excision and circumferential fusion in two stages without any neurovascular injury. The safety and efficacy of this method, especially in the pediatric population is discussed in view of pertinent literature.