Chaotic lipoma with proximal syrinx -a (not so) rare variant - review of the literature, possible embryology and management.

Chaotic lipomas are an extremely rare variant of spinal lipomas. This entity was first defined in 2009 by Pang and colleagues. Not much has been written about this variant. Its characteristic is the haphazard distribution of DREZ (Dorsal root entry zone), nerve roots and placode-lipoma interface. Thus complete/near-total excision of this lesion is quite difficult. We describe a case of chaotic spinal lipoma and elucidate the challenges faced in the management of this entity and review the literature. We performed a thorough systematic review with the keyword 'chaotic', 'Lipomyelomeningocele', 'Complex Lipomyelomeningocele', 'LMMC', 'Lumbar lipoma', 'spinal lipoma' in the google scholar and PUBMED data system for indexed literature on the above topic with no particular time frame. The studies quoted range earliest from 1970 till currently. Additional potential relevant articles were further retrieved through a manual search of references from original reports. Out of 42 studies, a total of 21 publications were selected which could have encountered a chaotic variant, but due to the term introduced only recently in 2009, may have been described differently. Studies encompassing true lipomeningomyelocele were excluded from our review. What we found out? Chaotic lipoma may not be a new entity. The scarce description in literature may be in part due to non-introduction and unclear description of this term earlier. The management of this variant is particularly challenging with basic principles remaining the same. Meticulous near-total excision and placode-lipoma construct are the major obstacles.

Intracranial high-grade glioma with malignant progression of spinal intramedullary metastasis: an atypical presentation with review of literature.

PURPOSE: High-grade gliomas (grade 3 and grade 4) are known to be highly locally invasive and distant metastasis though known, rarely manifest clinically due to poor survival. Recently, due to increasing survival in view of early diagnosis at relatively young age, more cases of extra neural symptomatic metastasis are being diagnosed and treated. Among these, symptomatic spinal metastasis is even more rare. Dissemination of GBM to the spinal compartment, albeit uncommon, has an even poorer outcome, with most patients dying within 2-3 months after diagnosis of metastatic disease. MATERIALS AND METHODS: Here, we present a rare case report of a 25-year-old female with transformed/histologically progressed tumour in cranial region along with ultra-rapid progression of the spinal disease following metastasis. Proper consent of the patient relatives was taken prior to production of material as patient had expired by the time of writing of this paper. We also present an extensive review of spinal metastasis secondary to intracranial high-grade gliomas starting from 1950. We conducted a thorough and exhaustive systematic search and review of the indexed databases available in PUBMED, COCHRANE and GOOGLE SCHOLAR with key words 'spinal metastasis of glioma', 'spinal metastasis', 'glioma progression', 'secondary glioma', multicentric glioma', 'secondary spinal metastasis' and formulated a comprehensive table of the studies that met the set standards. The studies that included (a) Number of cases, (b) Age and sex of patients, (c) operated primary or non-operated primary with spinal metastasis, (d) time period from the index cranial surgery, (e) outcome after diagnosis of spine metastasis and (f) histopathology of both cranial and spinal tumour either following surgery or autopsy have been elucidated herewith. We searched the databases with no particular time period. Out of 42 case reports and series, 28 studies were selected for our publication as they met the standards set, starting from 1950 to 2020. RESULTS: In this case, the primary histopathological diagnosis post cranial tumour removal was Grade-3 anaplastic astrocytoma, whereas Spinal autopsy report done 16 months after the primary diagnosis showed Grade-4 GBM suggestive of secondary transformation (Secondary GBM), it showed same genome of IDH mutation and ATRX loss, neoplastic fibrillary and gemistocytic astrocytes with de-differentiation, foamy histiocytes as seen in primary lesion suggestive of progression and metachronicity rather than multicentricity or synchronicity. What is more peculiar and rare in our case is that the spinal disease was very malignant and it progressed in course of just two days to involve the whole spine.

Malignant ultra-rapid progression of spinal metastasis.Thorough review of literatureMetachronicity of spinal metastasis Importance of the studyThis study presents a very atypical case of malignant progression of spinal metastasis documented with successive MRI radiology scans in a span of mere two days.It is different from other studies in the sense such malignant progression in a span of few days has never been documented with radiographs.This manuscript also provides an exhaustive review of literature and draws comparisons among the same.This study compares→ Time period to diagnosis of spinal metastasis following primary diagnosis, Outcome from diagnosis of spine metastasis, age along with other variables like histopathology of spinal metastasis if available, Treatment underwent, site of metastasis among different studies.

Patients with Invasive Tumors and eNOS Gene Polymorphisms with Subarachnoid Hemorrhage Tend to Have Poorer Prognosis.

Context Endothelial nitric oxide synthase ( eNOS) gene polymorphisms are found to predict predisposition to aneurysmal rupture and development of vasospasm in a patient of subarachnoid hemorrhage (SAH). eNOS gene polymorphisms are also found to predict invasiveness of malignant cells. Studies are not available in literature to describe the effect of eNOS gene polymorphisms and correlation between aneurysm and carcinoma. This study aims to investigate whether positive cancer history influences clinical outcome following SAH and eNOS gene polymorphisms. Materials and Methods The eNOS gene polymorphisms were analyzed in seven consecutive patients (mean age, 52.28 ± 20 years) with a diagnosis of invasive systemic tumors from 2011 to 2017. The eNOS 4a/4b eNOS -786T> eNOS 894G > T polymorphisms of the eNOS gene were determined by polymerase chain reaction and restriction fragment length polymorphism. Results Seven patients of aneurysmal SAH in association with malignancies were studied for eNOS polymorphisms expression and outcome. Three patients had carcinoma cervix: one patient of carcinoma breast and one each of transitional cell carcinoma of urinary bladder, spindle cell carcinoma of left kidney, and untreated patient of atypical pituitary (adenoma). A genotype study of eNOS gene polymorphisms in these patients shows common polymorphisms are involved in the determination of disease progression in malignancies and aneurysmal SAH. Conclusion Patients who expressed 4ab, eNOS -786T > TT/CC/TC, eNOS 894G > T GG/GT polymorphisms did better than patients who expressed only 4bb, though both were associated with poor prognosis.

Heartbroken Child: A Rare Case Report of Neurogenic Pulmonary Edema and Takotsubo Cardiomyopathy following Recurrent Medulloblastoma Excision with Possible Aetio-Patho-Bio-Physiological Mechanisms.

INTRODUCTION: Takotsubo (Japanese fishing pot for trapping octopus) cardiomyopathy is a rare phenomenon of acute coronary syndrome presenting usually with the presence of transient apical ballooning of the left ventricle in the absence of obstructive coronary artery disease. It is mainly seen in women of older age secondary to emotional, physical, or psychological stress. In age less than 18 years, it is mainly seen in adolescents suffering from psychiatric disorders and substance abuse. In children, it is rarely described. CASE PRESENTATION: We present here a peculiar case of a 12-year-old child with neurogenic pulmonary oedema and takotsubo cardiomyopathy syndrome after surgery for recurrent medulloblastoma. Also, management challenges are discussed. DISCUSSION/CONCLUSION: Takotsubo cardiomyopathy is not just a classical or inverted type but indeed a spectrum. It can be seen in any case, be it a child or adult secondary to handling or injury to the ponto-medullary junction, rostral pons, or dorsolateral medulla. The density and distribution of beta-adrenergic receptors may be different in children and adults which needs further research. Prognosis is usually excellent across all ages.

Vertebral artery dissection in acute cervical spine trauma.

Objective: The aim of this study was to study mechanism, risk factors, and prognosis of patients with vertebral artery dissection (VAD) from acute cervical spine trauma (CST). Methods: A total of 149 consecutive patients were chosen from 2014 to 2019 from our institute data base, and their records were retrospectively studied. Morphology of fracture and subluxation were studied in detail with respect to the presence or absence of VAD. Results: Patients were divided in subsets of axial spine injury and subaxial spine injury. Subgroup and group analysis was performed and computerized tomography angiogram, MR angiogram and T1/T2 axial scans were studied to identify VAD, an incidence of 14.1% was found. Patients having infarcts in posterior circulation were also identified. Conclusion: There is a significant contribution of biomechanics of CST and evolution of VAD. This is an important consideration to prevent significant morbidity and mortality. Hence, a diagnostic algorithm which can be applied in any hospital setup is the need of the hour.

Six-year longitudinal prospective comparative study between preoperative and postoperative heart rate variability indices in congenital craniovertebral junction anomalies.

Background: Craniovertebral junction (CVJ) anomalies involve mosaic interaction of multifaceted neurovascular and bony elements. Most of them present late in the course of illness usually as acute presentations following trivial trauma. Knowing subclinical autonomic dysfunction in such anomalies when managed medically can not only indicate progression but also provide en route to early intervention for better outcomes, especially in relatively asymptomatic patients. Materials and Methods: We conducted a 6-year longitudinal prospective study including 40 consecutive patients of CVJ anomalies with clinical, radiological, and heart rate variability (HRV) parameters and found their correlation in preoperative and follow-up period. Results: Twenty-eight patients were male and the rest were female. The mean age was 32 years with the least age being 8 years and maximum age being 75 years old. Mean Nurick's grade and Barthel's index were 1.8 and 83.75, respectively. 38% had severe-to-moderate compression. The mean follow-up was 17.4 months. Both sympathetic and parasympathetic oscillator HRV indices were significantly affected in the preoperative period (P ≤ 0.001) with no association with Nurick's grade or degree of compression although there was association with grade of Barthel's index. Poincare plots showed "fan," "complex," or "torpedo" patterns in 36 patients. Forty patients had both preoperative and follow-up clinical grade whereas 22 patients HRV tests in the above periods. None of the HRV indices showed significant improvement at follow-up. Nonetheless both sympathetic and parasympathetic did improve at follow-up with sympathetic tone registering better scores. Poincare plots showed improvement toward "comet" patterns in all patients. Conclusion: HRV indices not only help in prognosticating but may also help in predicting outcomes.