Primary intracranial myxoma - Report of a rare case and review of literature

Myxomas are benign primary tumors of the heart of mesenchymal origin. Neurological complications attributed to atrial myxoma occurs in 10% to 12% of  patients, with ischemic presentation due to cerebral infarct in 83%-89% of cases. Few case reports are available of multiple metastatic myxomas from primary inthe heart, despite its slow growing and innocuous histological appearance. Primary intracranial myxomas are extremely rare and only six cases have been reported in literature till date, out of which four were supratentorial in location. As on account of its benign nature, complete surgical resection of the tumor is the recommended treatment

Acromesomelic dysplasia (Marotaeux type) associated with craniovertebral junction anomaly: A report of a rare case and review of literature

Acromesomelic dysplasia Maroteaux type (AMDM) is a rare autosomal recessive osteochondrodysplasia. The responsible gene, AMDM gene, in human beings, has been mapped on 9p13-q12 chromosome by homozygous mapping and pathogenic mutation was later identified in natriuretic receptor B (NPR-B) which has been implicated in the regulation of skeletal growth. Till now, around 40 to 50 cases of AMDM have been described in the world literature. Association of the congenital craniovertebral (CV) junction anomaly has not been reported. Here we are presenting a case of AMDM, with CV junction anomaly. A 10-year boy presented with short stature (122 cm) with short distal limbs, symptomatic for thoracic kyphoscoliosis with back pain. On examination there were no neurological deficits. On radiological investigation, he was found to have short and broad phalanges and toes, thoracic kyphoscoliosis, abnormal pelvic ring, mild ventriculomegaly, cervical syringomyelia and tonsillar descent below foramen magnum, hydrocephalus, os odontoideum with Klippel-Feil anomaly. This was diagnosed as AMDM with congenital os odontoideum, Klippel-Feil anomaly with Arnold-Chiari malformation (ACM) type-1. The patient underwent posterior fossa decompression by removal of foramen magnum ring along with C1 arch for ACM type-1. Kyphosis was left for conservative treatment till further observation and required orthopedic correction in his further age. To the best of our knowledge this is a very rare entity of AMDM with congenital CV junction anomaly

Spinal Dermoid and Epidermoid Cyst: An Institutional Experience and Clinical Insight into the Neural Tube Closure Models

Objectives The spinal dermoid and epidermoid cysts (SDECs) are rare entities comprising less than 1% of pediatric intraspinal tumors. The present study aims to extrapolate the clinicoradiological data, in order to identify the most plausible neural tube closure model in human and provide a retrospective representation from our clinical experience. Materials and Methods We collected the details of all histologically proven, newly diagnosed primary SDECs who underwent excision over the past 20 years. Secondary or recurrent lesions and other spinal cord tumors were excluded. Surgical and follow-up details of these patients as well as those with associated spinal dysraphism were reviewed. Clinical and radiological follow-up revealed the recurrence in these inborn spinal cord disorders. Results A total of 73 patients were included retrospectively, having a mean age of 22.4 ± 13.3 years, and 41 (56.2%) cases fell in the first two decades of life. Twenty-four (32.9%) dermoid and 49 (67.1%) epidermoid cysts comprised the study population and 20 of them had associated spinal dysraphism. The distribution of SDECs was the most common in lumbosacral region (n = 30) which was 10 times more common than in the sacral region (n = 3). Bladder dysfunction 50 (68.5%) and pain 48 (65.7%) were the most common presenting complaints. During follow-up visits, 40/48 (83.3%) cases showed sensory improvement while 11/16 (68.7%) regained normal bowel function. There was no surgical mortality with recurrence seen in eight till the last follow-up. Conclusions The protracted clinical course of the spinal inclusion cysts mandates a long-term follow-up. The results of our study support the multisite closure model and attempt to provide a retrospective reflection of neural tube closure model in humans by using SDECs as the surrogate marker of neural tube closure defect

Knock and Drill Technique: A Simple Tips for the Instrumentation in Complex Craniovertebral Junction Anomalies without using Fluoroscopy

Context: Existence of complex variable bony and vertebral artery (VA) anomalies at craniovertebral junction (CVJ) in subset of complex CVJ anomalies demands individualized instrumentation policy and placing screws in each bone requires strategic preoperative planning and intraoperative skills. Aim: To evaluate the clinical accuracy of knock and drill (K and D) technique for the screw placement in complex CVJ anomalies. Settings and Design: Prospective study and operative technical note. Materials and Methods: Totally 36 consecutive patients (16 - pediatrics, 20 - adult patients) of complex CVJ: Complete/partial occipitalized C1 vertebra; at least one hypoplastic (C1/C2) articular mass, rotational component, and variations in the third part of VA were included in this study. Preoperative detail computed tomography (CT) CT CVJ with three-dimensional reconstruction was done for the assessment of CVJ anatomy and facet joint orientation. The accuracy of novel technique was assessed with postoperative CT to evaluate cortical breach in between 5th and 7th postoperative day in all the patients. All patients were underwent clinico-radiological evaluation at 6-month follow-up. Results: Totally 144 screws were placed using K and D technique (pediatric group - 64 screws, adult patients - 80 screws). Total of 12 screws were placed in C1 lateral mass in both age group without any bony cortical breach and complication. Sixteen C2 pedicle screws and 12 C2 pars screw in pediatrics and 18 C2 pedicle screws in adult patients were placed without any bony breach or VA injury. Out of thirty subaxial lateral mass screws in pediatric group, the bony breach was encountered with one screw (3.3%). Total of 38 C2 pars screws was placed in adult group in which bony breach along with VA injury was encounter with 1screw (2.6%). Conclusion: A simple technique of K and D for placing a screw increases the accuracy and spectrum of bony purchase and has the potential to reduce the complication in patients with complex CVJ anomalies

Symptomatic extradural spinal arachnoid cyst: More than a simple herniated sac

Introduction and Study Design: Spinal arachnoid cyst is an uncommon entity, presenting with radiculopathy or paraparesis. These cysts are usually found in intradural extramedullary region; and the extradural region is a rare location. The exact pathogenesis in the existence of these cysts in the extradural region is still debated. In this article, we have retrospectively analyzed the clinical profile of the rare extradural arachnoid cyst (EDAC). Methodology: In this study, 19 patients of EDAC operated at our institute between January 2006 to June 2016 are analyzed. All patients with the Oswestry disability index score of >20 were managed surgically (open laminectomy and cyst excision). The clinical outcome was assessed at using 5-point satisfaction scale and McCormick grading. Results: All 13 operated patients had EDACs with communication with the intradural compartment. In 11 (84.6%) patients, cyst wall was excised completely, in 2 (15.3%) patients underwent partial excision of cyst wall; dural communication was closed in all patients (n = 13). None of the patients had clinical deterioration or radiological recurrence till the last follow-up. Mean follow-up was 52.2 months (range 1–160); all patients were satisfied after surgery (median score was 3). Discussion: Symptomatic EDACs account <2% of all spinal tumors. The EDACs have communication with the intradural compartment. In our article, we have discussed the approach and management of EDAC, including minimally invasive percutaneous procedures. Conclusion: One should aim for preoperative or intraoperative localization of dural communication and try to disconnect the extradural cyst from the intradural connection to prevent recurrence. Surgical treatment is complete excision of the cyst

Cervicomedullary intramedullary peripheral primitive neuroectodermal tumor with intratumoral bleed: Report of one case and review of literature

Primitive neuroectodermal tumors (PNET) are highly malignant, yet relatively uncommon neoplasms of the central nervous system. Although a host of different parts of the nervous system can be affected, intramedullary location of PNET is extremely rare. Most reports on intramedullary PNET have reported central PNET (cPNET); peripheral PNET (pPNET) affecting intramedullary spinal location is extremely rare. Till now, seven such cases of intramedullary pPNET have been described in medical literature in English. Here, we report an 11-year-old boy with cervicomedullary junction intramedullary pPNET who presented with intratumoral bleed, wherein the clinical presentation and radiological features gave us no clue preoperatively about the underlying diagnosis. In this report, we additionally review certain salient aspects of this dreaded disease in light of the existing evidence

Clinicoradiological Features and Treatment Outcome of Supratentorial Intraparenchymal Epidermoid Cyst: A Report of Five Cases and Literature Review

Objectives Intraparenchymal epidermoid cysts (IECs) are rare lesions. They represent less than 1% of the intracranial epidermoid cysts. The supratentorial IEC is a clinically and prognostically distinct subset. Given the rarity, most of the articles are case reports. We present a series of five cases of supratentorial IEC to characterize their clinical presentation and outcome, with emphasis on the surgical features. Materials and Methods We searched our database for all cases of intracranial epidermoid cysts operated between January 2005 and January 2020. Five patients were identified having IEC from the hospital information system and the neurosurgical operation record book. Standard craniotomy and decompression of the lesion were performed in all these patients. Standard postoperative care includes computed tomography scan of head on the day of surgery and magnetic resonance imaging of brain after 6 weeks to look for the residual lesion, if any. Subsequent follow-up visits in outpatient department to look for resolution of the presurgical symptoms. Results The mean age of the patients in our series was 28.8 years (range: 28–40 years.). All the five patients were male. Four patients had IEC involving frontal lobe and one in parietal lobe with a small occipital lobe extension. Seizure was the most common presenting complaint followed by headache. Complete excision was achieved in all the cases. All the three patients with seizure attained seizure freedom postlesionectomy. Focal neurological deficits resolved gradually in postoperative period. There was no recurrence of lesion during follow-up. Conclusion Supratentorial IEC most commonly affects young males, involve frontal lobe and present clinically with seizure. Complete surgical excision offers best outcome in the form of remission of seizure disorder