Dorsal bony spur in pediatric split cord malformations: eight-year experience from a tertiary care hospital.

INTRODUCTION: Dorsal spurs in Type I split cord malformations (SCM-I) are infrequent findings. The pathogenesis of the same is debatable. The objective of this study is to analyze our experience with SCM-I patients having dorsal bony spurs. METHODOLOGY: Retrospective analysis of SCM patients operated from 2010 to 2017 was performed. Their demographic profile, clinic-radiological features, operative findings, and outcome following surgery were recorded. RESULTS: Twenty-four cases of Type I SCM harboring dorsal bony spurs were identified with mean age of 4.96 years. The commonest split site was lumbar, documented in 62.5%. Scoliosis was observed in 58.3%. Pre-operative neurological deficits were seen in 66.6% cases with asymmetric weakness of limbs seen in 16.6%. There was no new neurological deficit observed post-operatively. CONCLUSIONS: This is the largest series of dorsal spurs occurring in SCM, reported in literature so far. Meticulous pre-operative evaluation and imaging are important to identify dorsal spurs for appropriate management and good clinical outcome. Differentiating dorsal spur from ventral spur is important as it has a bearing on surgical approach.

Craniovertebral Junction Anomalies: An Overlooked Cause of 'Posterior Circulation Stroke'.

Background: Vertebral artery dissection (VAD) is a treatable cause of vertebrobasilar ischemic stroke and can be spontaneous or more commonly traumatic. Craniovertebral junction (CVJ) anomalies are a rare and often overlooked cause of VAD. Objective: The objective of this study was to study cases where CVJ anomaly presented as posterior circulation infarct and to conduct a relevant literature review. Materials and Methods: The medical records of seven patients who were managed for posterior circulation infarct associated with CVJ anomaly at our center from January 2009 through August 2013 were reviewed. PubMed and MEDLINE databases were also searched for similar cases, and the published case reports/series were reviewed. Results: Seven patients met our inclusion criteria and were included in the study. The mean age was 17.4 years (range: 10-35 years). All the patients were males. The most common symptoms were headache, vomiting, and gait ataxia. Slurring of speech was seen in one patient. One patient had repeated episodes of gait ataxia with left-sided weakness with complete recovery in between the episodes. One patient presented in unconscious state. Four patients complained of vertigo. The median duration of symptoms was 7 days (range: 3 days-12 months). Conclusions: CVJ anomalies can present as posterior circulation infarct. One must evaluate all patients with posterior circulation stroke, especially young patients, for possible CVJ anomalies. Dynamic lateral cervical spine X-ray is an important tool to diagnose AAD. CVJ anomalies represent a treatable cause of VAD.

Hydrocephalus Research.

OBJECTIVE: This paper highlights the hydrocephalus research efforts undertaken at AIIMS, New Delhi, supporting progress in the field. MATERIAL: Over a period of three decades, basic research, clinical investigations, and multicentric studies were undertaken. This report will review the work mainly to emphasize the need for future generations to pursue further research. Studies that impacted hydrocephalus care (mainly in India) are described, and some of these findings may be useful in other resource-challenged situations. RESULTS: Investigative studies on the effect of shunting on brainstem auditory evoked responses (BAER), transcranial Doppler (TCD), and CT-SPECT were published offering management options for patients. Participation in the International Infant Hydrocephalus Study (IIHS) study offered opportunities to compare our approaches and develop modifications in patient care. This effort proved shunting was equal or better for young children with congenital aqueductal stenosis. Shunt infection protocols and changes made in a systematic manner helped develop local protocols to reduce postoperative shunt infections. CONCLUSIONS: Hydrocephalus research over three decades at AIIMS, New Delhi was productive and educational, confirming that locally performed investigative work can help in decision making. Further studies and active participation in international efforts are necessary to advance the field.

Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS).

PURPOSE: The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. METHODS: We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC z-score, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. RESULTS: Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up. CONCLUSION: ETV and shunting led to improvements in HC centile, z-score, and FOR measurements during long-term follow-up of infants with hydrocephalus secondary to aqueductal stenosis. Head size did not significantly differ between the treatment groups during follow-up, however ventricle size was greater in those undergoing ETV when measured at 1 and 3 years following treatment.

Role of Decompressive Craniectomy in Traumatic Brain Injury - A Meta-analysis of Randomized Controlled Trials.

OBJECTIVE: Several studies have indicated that decompressive craniectomy (DC) for traumatic brain injury (TBI) is lifesaving. However, there is lack of level 1 evidence to define the role of DC in TBI. We performed a meta-analysis of all the randomized controlled trials (RCTs) published so far on the role of DC in adult patients with TBI. MATERIALS AND METHODS: A systematic literature search was performed for articles published until September of 2016 for RCTs of DC in adult patients with TBI. The primary end-point was mortality at six-months. We also evaluated the overall adverse outcomes at six months. Assessment of risk of bias of the RCTs was also performed. RESULTS: Three trials evaluating adult population satisfied the eligibility criteria. Pooled analysis involved 285 and 288 patients in DC group and control groups respectively. Patients undergoing DC for TBI had a lower mortality association of nearly 50 percent. However, patients surviving DC were more likely to have a poor neurological outcome compared to patients undergoing medical management. CONCLUSION: Based on the available RCTs on DC in TBI, the results of our meta-analysis show that there is a mortality benefit of performing a DC over the best medical management in adult patients. Furthermore, surviving following DC, a greater incidence of a poor neurological outcome is noted. In the event of small number of high-quality RCTs, our results must be interpreted with caution.

Neurogenic Fever in Severe Traumatic Brain Injury Treated with Propranolol: A Case Report.

The causes of intractable fever in severe traumatic brain injury (TBI) patients can be diverse. Neurogenic fever (NF) which is a rare entity can develop due to autonomic dysregulation in the absence of infection or any other cause of fever. It manifests as fever, tachycardia, paroxysmal hypertension, dilated pupils, tachypnea, and extensor posturing in cases of severe TBI, brain neoplasms or brain haemorrhage. We found propranolol to be effective in controlling many of the manifestations of neurogenic fever in our patients with severe TBI. Fever in severe TBI patients is not an uncommon phenomenon, but when intractable with negative fever workup, a central cause should be considered. Propranolol is deemed as one of the most efficacious drugs for managing NF due to dysautonomia. We want to apprise the readers about this entity and its treatment with beta-blockers.

The role of mifepristone in the management of meningiomas: A systematic review of literature.

BACKGROUND: Surgery is challenging in patients with multiple or recurrent meningiomas. With the discovery of progesterone receptors (PR) on meningioma cells, there is an increased interest in the hormonal treatment using mifepristone, a PR blocker. MATERIALS AND METHODS: A systematic review of clinical studies evaluating the efficacy and side effects of mifepristone in recurrent, unresectable, or multiple meningiomas was done. The primary outcome of this review was to study the efficacy in terms of tumor regression and clinical symptoms. Secondarily, we also reviewed the frequency and severity of different side effects reported by various studies. RESULTS: A total of 7 studies, including one Phase III randomized controlled trial, were found relevant to the topic. Though a few studies showed some response in terms of clinical improvement and tumor size reduction, the response was either minimal or temporary. The only subset showing a good response was the "diffuse meningiomatosis" group. None of the studies evaluated the relation of the PR isoform with mifepristone responsiveness. However, long-term mifepristone administration was well tolerated in most of the patients. CONCLUSIONS: Use of mifepristone as a hormonal agent for meningiomas has produced mixed results. We propose that the possible mechanisms of action of mifepristone on meningioma cells must be studied in further detail by in-vitro studies. This may help in the identification of a mifepristone responsive subset of meningioma. This must be followed up with appropriately designed clinical studies with detailed baseline evaluation and standardized clinical and radiological follow-up.

An Unusual Case of Split Cord Malformation with Simultaneous Ventral and Dorsal Bony Spur at a Single Site: A Technical Challenge.

Split cord malformation (SCM) with simultaneous ventral and dorsal bony spur at a single site is an extremely rare entity with only one other case reported so far. We present a second such case in a 13-month-old female child, who presented with a skin dimple over the lumbar region with overlying hairy patch without any associated lower limb weakness or urinary complaints. Imaging demonstrated a complex Type I SCM with simultaneous ventral and dorsal bony spur at L4-L5 level. Intraoperatively, the patient was confirmed to have a Type Ic SCM with a ventrally based bony spur going dorsally to attach on the hypertrophied posterior arch of L4 and a dorsal spur going ventrally to attach on the L4 vertebral body. After meticulous microsurgical excision of the spur, the child was stable and developed no fresh neurological deficits. We discuss the relevant surgical implications and possible embryological mechanisms involved in such a complex SCM.

Microvascular decompression versus stereotactic radiosurgery as primary treatment modality for trigeminal neuralgia: A systematic review and meta-analysis of prospective comparative trials.

OBJECTIVE: The current opinion among neurosurgeons regarding the selection between microvascular decompression (MVD) and gamma knife radiosurgery for trigeminal neuralgia is not based on clear evidence. In this meta-analysis, we have attempted to synthesize the findings of the prospective trials comparing the efficacy and complications of the two procedures as primary treatment modality for medically refractory trigeminal neuralgia. MATERIALS AND METHODS: The authors performed a systematic review of PubMed for manuscripts comparing the efficacy or complications of MVD and stereotactic radiosurgery for medically refractory trigeminal neuralgia. The data of the identified studies was pooled and a meta-analysis was done. RESULTS: Five prospective studies fulfilling the eligibility criteria were identified. The mean age of the patients subjected to gamma knife therapy (GKT) was more than those who underwent MVD. The initial success rate in the pooled data with MVD was 96% (95% confidence interval [C.I.] 93.3%-98.6%) as compared to GKT which was 71.8% (95% C.I. 64.9%-78.7%) with the ratio of 1.309 (95% C.I. 1.217-1.409; P= <0.001). This superiority was sustained till the last follow up available in all the studies. Out of the complications common to both procedures, MVD had a lower rate of facial numbness, with a risk ratio of 0.481 (95% C.I. 0.297-0.778); and dysesthetic pain, with a risk ratio of 0.470 (95% C.I. 0.172-1.286). CONCLUSIONS: MVD seems to be more efficacious than GKT as a first line treatment for trigeminal neuralgia immediately as well as on a long term basis. However, the dilemma regarding the choice of treatment to be adopted still remains for special subgroups of patients, like the elderly patients and those in whom no vascular compression has been found during surgery. Further studies are needed for elucidating the unequivocal treatment plan under these circumstances.

Standardization of the technique of silicon injection of human cadaveric heads for opacification of cerebral vasculature in Indian conditions.

A surgeon's understanding of the surgical anatomy can be greatly enhanced by the dissection of preserved cadaveric specimens. A reliable and inexpensive biological model for testing and standardization of dye injection concentrations is proposed utilizing the goat's head as a biological model. The first phase was concerned with standardization of the dye by titrating its concentration and injecting various amounts into cerebral vessels of a goat's head until an optimal concentration had been ascertained. In the second phase, this optimum concentration of the dye was injected into four human cadaveric heads following the same technique standardized using the goat's head. Upon dissecting the four cadaveric human heads which were injected with silicon dyes and preserved in 10% formalin, the vessels were all well-opacified and the brain was of near normal consistency and good for dissection, without showing any features of putrefaction. The goat model, having similar color, texture, and the handling as the cadaveric head, offers an opportunity to test indigenously manufactured polymerizing dyes in the future. This biological model, therefore, has the potential to considerably reduce the cost of cadaver preparation.

Traumatic Atlantoaxial Spondyloptosis Associated with Displaced Odontoid Fracture: Complete Reduction via Posterior Approach Using "Joint Remodeling" Technique.

BACKGROUND: Atlantoaxial spondyloptosis (AAS), which is defined as complete displacement of facets of atlas anterior to the facets of axis such that there is no contact between the 2 articulating surfaces, is an extremely rare manifestation of atlantoaxial instability. The reason for an extreme rarity of traumatic AAS is probably the severity of injury in traumatic AAS that is usually incompatible to life. It represents the most severe form of atlantoaxial dislocation, and complete reduction in such a case presents a real technical challenge because of the interlocking of C1-C2 facets. Cranial traction fails to achieve reduction in such cases. CASE DESCRIPTION: In this report, we describe a case of traumatic odontoid fracture associated with AAS and discuss our technique of complete reduction of deformity via posterior approach. An 11-year-old child presented to us 4 months after a road traffic accident with progressive spastic quadriparesis. On evaluation, displaced type II odontoid fracture with AAS was detected. The patient underwent surgery via posterior approach. The techniques of "joint manipulation" and "joint remodeling" were used to achieve complete reduction of spondyloptosis. Postoperative imaging showed complete reduction of deformity. The patient also improved neurologically after surgery. CONCLUSION: This case report aims to present the ability of "joint manipulation" and "joint remodeling" techniques in achieving excellent reduction in even one of the most difficult post-traumatic deformities that affect the craniovertebral junction.

Traumatic Lateral Spondyloptosis: Case Series.

OBJECTIVE: To apprise readers about this rare but severest form of traumatic spine injury and its surgical management. BACKGROUND: Complete fracture dislocation and subluxation (>100%) of 1 vertebral body in the coronal or sagittal plane with respect to the adjacent vertebra is defined as spondyloptosis. In coronal spondyloptosis the subluxated vertebral bodies lie beside each other, and the condition is lateraloptosis. Patients with lateraloptosis present unique surgical challenges because reduction and achieving realignment of spinal column require meticulous planning and execution. METHODS: A retrospective analysis of patients admitted with lateraloptosis over a 4-year period (2013-2016) was done. Lateraloptosis was defined on computed tomography as complete subluxation of the spinal column with more than 50% of adjacent vertebral bodies lying directly lateral to each other. RESULTS: Five men, ranging from 18 to 50 years (mean, 35.2 years) old were included in the study. Three patients had thoracic spine lateraloptosis, and in 2 the injury was at the thoracolumbar junction. All patients underwent single-stage posterior surgical reduction and fixation. Intraoperatively, cord transection was seen in 3 patients, and dural tear with cerebrospinal fluid leak was seen in 1 patient. The mean follow-up period was 14 months (range, 1-36 months), during which 1 patient died of complications arising from bedsores. All patients remained at American Spinal Injury Association grade A neurologically. CONCLUSION: Lateraloptosis is difficult to treat, and the aim of surgery is to stabilize the spine. Rehabilitation remains the most crucial factor, but the scarcity of proper rehabilitation centers results in high mortality and morbidity.