Spina bifida transition care in India: strengths amidst challenges.

In India, adult neurosurgeons are required to care for children regularly because the concept of dedicated pediatric specialty care is not yet entirely established in the subcontinent. Likewise, pediatric neurosurgeons do not exclusively offer their services to the young, but they also provide care to adult patients with neurosurgical disorders. This creates a medical system where the transition between specialties is not often a formal and recognized aspect of neurosurgical care because most neurosurgeons provide care for patients of all ages. Additionally, there are very few teams geared toward caring for conditions in children that merit lifelong medical support, with spina bifida (SB) being one of them. Since there are no focused or structured pediatric programs on a large scale, developing a multidisciplinary clinic for adults becomes challenging. A pragmatic approach using technology-based education, supported by an organized system or a coordinator, may be a new strategy. A new system utilizing telemedicine and smartphones for established patients maybe an alternative option for SB children in India. During virtual video conferences, an established patient may benefit from multispecialty care and education toward a smooth transition that avoids significant issues with time, transportation, or financial constraints. Achieving a seamless transition among allied specialists from the pediatric to adult systems is a utopia. The current system in the subcontinent may be improved, with an opportunity to develop smooth transition care between coordinated specialists (who simultaneously treat children and adults). Learning from various global SB management styles, the Indian transition situation may offer another model in the near future.

Spinal Tuberculosis: An Exhaustive Diagnosis.

BACKGROUND: The most common organ affected due to tuberculosis (TB) is the lungs. Extrapulmonary TB is less common. Musculoskeletal organs are affected in around 8% of all tubercular patients, of which the spine is affected in almost half of the patients. The criteria for diagnosing spinal TB are quite difficult and we use an array of investigations for the same. METHODS: A retrospective study was carried out in the Neurosurgery and Microbiology Department at IMS and SUM Hospital between January 2021 and November 2023, and data were collected and tabulated in an Excel sheet. One hundred patients with spinal TB were evaluated, and their age, sex, samples sent, diagnostic investigation, duration of diagnosis from hospital admission, histopathology results, and surgical intervention (done or not) were recorded. RESULTS: The best investigation done to diagnose spinal TB was imaging and surgical/computed tomography (CT)-guided biopsy. The earliest result to diagnose spinal TB was histopathology. The yield of positivity in pus culture, smear microscopy, and true nucleic acid amplification test (NAAT) was found to be low even though sensitivity was on the higher side. CONCLUSION: Even though we have an array of investigations for diagnosing spinal TB, the best and the earliest diagnosing test was imaging plus CT-guided biopsy. The confirmation is made in the biopsy. Finding acid-fast bacteria (AFB) and NAAT tests are additional beneficial tests to supplement the diagnosis. Hence, we can conclude that sending for tests like AFB in pus, NAAT, and GeneXpert is a wastage of biological samples and delays in diagnosis.

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.

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.

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.

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.

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism.

Craniopagus conjoined twins are extremely rare, reported 1 in 2.5 million live births. To date, 62 separation attempts in 69 well-documented cases of craniopagus twins have been made. Of these, 34 were performed in a single-stage approach, and 28 were attempted in a multistage approach. One or both twins died of massive intraoperative blood loss and cardiac arrest in 14 cases. We report our surgical experience with conjoined craniopagus twins (JB) with type III total vertical joining and shared circumferential/circular sinus with left-sided dominance. A brief review of the literature is also provided. In our twins, the meticulous preoperative study and planning by the multidisciplinary team consisting of 125-member, first-staged surgical separation consisted of creation of venous conduit to bypass part of shared circumferential sinus and partial hemispheric disconnection. Six weeks later, twin J manifested acute cardiac overload because of one-way fistula development from blocked venous bypass graft necessitating emergency final separation surgery. Unique perioperative issues were abnormal anatomy, hemodynamic sequelae from one-way fistula development after venous bypass graft thrombosis, cardiac arrest after massive venous air embolism requiring prolonged cardiopulmonary resuscitation, and return of spontaneous circulation at 15 minutes immediately after separation. This is the first Indian craniopagus separation surgery in a complex total vertical craniopagus twin reported by a single-center multidisciplinary team. Both twins could be sent home, but one remained severely handicapped. Adequate perioperative planning and multidisciplinary team approach are vital in craniopagus twin separation surgeries.

Pre- and Postoperative Rehabilitation of Craniopagus Conjoined Twins: A Case Report.

AIMS: Describe the pre-operative and post-operative stages of physical therapy examination, rehabilitation interventions and outcomes in craniopagus conjoined twins (CCT). METHODS: 27-months old total vertical types III CCT were admitted for surgical treatment. A structured rehabilitation program (functional mobility, balance training, sensory stimulation, communication, orthotic management, and caregiver education) was delivered post-operatively at different stages of surgery. The total duration of post-separation rehabilitation was 15 months for Twin A and 23 months for Twin B. RESULTS: Pre-operative WeeFIM was 67/126 for both the twins. Post-separation, Twin A improved to a state of complete independence in walking, step climbing, and hand function with a discharge WeeFIM score of 84/126. However, Twin B was completely dependent in bed mobility and transfer activities during discharge (WeeFIM-18/126). Minimal change in functional status was noted in Twin B (discharge FSS score-19/30). Both the twins were transferred to a regional hospital for long-term care. At 67 months of age, Twin B lost his life due to chest infection. CONCLUSIONS: Vestibular based therapies, balance, and postural control may be prioritized in the rehabilitation process of CCT. Greater communication and coordination among physical, occupational therapists and other rehabilitation professionals are recommended for successful outcome in these unique cases.

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.

Cervical Split Cord Malformations: A Systematic Review.

Cervical split cord malformations are extremely rare with less than 75 cases reported in the literature worldwide. The widely different terminologies used to describe the same pathoanatomy make the documentation of all reported cases a difficult task. We conducted a systematic review of 71 cases documented over 38 reports from 1889 to 2016. The controversy regarding prophylactic surgery in asymptomatic individuals remains. We advocate prophylactic surgery in all asymptomatic individuals to preclude severe neurological deficit following trivial trauma in future. The results of surgery in asymptomatic individuals are excellent while those in symptomatic individuals are good as well.

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.

Drug Refractory Epilepsy - A Series of Lesions with Triple Pathology.

The associations between gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), and cortical dysplasias remain debatable. We report five cases of drug refractory epilepsy with temporal lobe lesions. On resection, histopathological examination showed distinctive areas of gangliogliomas and DNETs with cortical dysplasia. The coexistence of the above three lesions as distinct entities in a single lesion is virtually unknown. This points to the presence of a possible etiological relationship among them. Finally, we also delve into a plausible hypothesis for such a pathogenesis.

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.