Defining a Taxonomical Map for Craniosynostoses: An Integrated Nomenclature for Interdisciplinary Workflow and Problem Mapping in Craniosynostoses Management: A Feasibility Study.

Craniosynostoses is a complex clinical and management challenge. This is further complicated by varying phenotypes and specific challenges. Most complex craniosynostoses are approached and managed the same way despite the clinical variability. The goals for craniofacial surgery are common, but the nomenclature based on phenotype, genotype, sutural involvement, or syndromic nature does not shed light on the management workflow. The descriptive diagnosis and terminologies do not highlight the priorities and the operational issues, and most specialists dealing with these conditions remain in their field of tunnelled vision. In this article, the authors propose a concept of categorization based on initial patient presentation. In addition, the etiology and the treatment status, if available, are incorporated along with the presentation. The utility of our proposed categorization is to create a goal-based universal language among various specialists involved.

Trichosporon asahii infection presenting as chronic meningo-ventriculitis and intra ventricular fungal ball: a case report and literature review.

Central nervous system trichosporonosis is a rare clinical entity and so far only six cases including three each of brain abscess and meningitis has been on record. We report a rare case of chronic meningo-ventriculitis and intraventricular fungal ball due to Trichosporon asahii in an 18-year-old immunocompetent male from Burundi, east Africa. Neuroendoscopy showed multiple nodules and a fungal ball within the ventricle, which on culture grew T. asahii. He was initially empirically treated with liposomal amphotericin B. However, the antifungal susceptibility testing of T. asahii isolate revealed high minimum inhibitory concentration for amphotericin B (2 µg ml⁻¹), flucytosine (16 µg ml⁻¹) and caspofungin (2 µg ml⁻¹) but exhibited potent activity for voriconazole, posaconazole, itraconazole and fluconazole. The patient rapidly succumbed to cardiac arrest before antifungal therapy could be changed. Although disseminated trichosporonosis has been increasingly reported the diagnosis represents a challenge especially in rare clinical settings such as intraventricular fungal ball in the present case, which has not been described previously.

Extradural temporopolar approach for parahypothalamic hypothalamic hamartoma and use of posterior communicating artery as resection margin pointer.

BACKGROUND: Hypothalamic hamartomas (HH) are malformations originating from the hypothalamus and are associated with seizures, hormonal and behavioral abnormalities. METHOD: Most patients, especially those with a typical syndrome characterized by gelastic seizures, precocious puberty, cognitive decline, and behavior problems, are diagnosed in childhood. Pedunculated and parahypothalamic types of hamartomas are attached to the floor by a narrow or wide peduncle in the absence of distortion of the overlying hypothalamus. This location is most commonly associated with a clinical presentation of precocious puberty, and surgical removal has proved curative in small case series. Enthusiastic resection of hypothalamic lesions are known to produce severe hypothalamic disturbance while under resection might mean inadequate response to surgery. CONCLUSIONS: In this article, the authors describe the use of extradural temporopolar approach to hypothalamic hamartoma as an improvisation to improve access with reduced morbidity and describe a surgical nuance of using posterior communicating artery to determine a safe but maximal resection margin.

Role of endoscopy in recalcitrant intraventricular tuberculoma-innovative novel treatment adjunct.

INTRODUCTION: Intraventricular tuberculomas are rare entities. To the best of our knowledge, only 14 cases have been reported in English literature. Medical management of cerebral tuberculomas is well accepted. Intraventricular tuberculomas may be recalcitrant for unclear reasons. An effective management protocol for this entity is unclear. To the best of our knowledge, the definitive indication, timing, and possible role of surgery in these lesions have not been discussed in literature. MATERIALS AND METHODS: A 27-year-old nursing professional who was undergoing treatment for miliary tuberculosis at another center presented to us in 2008 with right hemiparesis, deteriorating vision, and progressive decline in consciousness. In addition to antituberculous therapy (ATT), she underwent multiple CSF diversion procedures for the obstructive hydrocephalus secondary to a recalcitrant third ventricular tuberculoma. Finally, she underwent endoscopic decompression of the lesion with a very good clinical response at 1-year follow-up. DISCUSSION: We discuss a patient with recalcitrant intraventricular tuberculoma managed using neuroendoscopy along with the standard antituberculous therapy. We also discuss in detail the technique we utilized for endoscopic management of this lesion. CONCLUSION: Being a rare entity, a consensus for management of these lesions is not possible, but we have demonstrated that neuroendoscopic management of these rare entities is an option giving the advantage of definitive diagnosis when required, hastening the resolution, and clearing the CSF pathway.

Dilemmas and challenges in the management of a neonate with Adams-Oliver syndrome with infected giant aplasia cutis lesion and exsanguination: a case-based update.

BACKGROUND: Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with a variable extent of defective formation of the scalp. Adams-Oliver syndrome is a condition mainly characterized by the congenital absence of skin, known as "aplasia cutis" which is usually limited to the vertex scalp and transverse limb defects. CASE REPORT: A 17-day-old term female neonate was referred to us with an infected scalp lesion of the vertex. The lesion which is about 10 × 9 cm had signs of infection with necrotic eschar. We started the neonate on systemic parenteral antibiotics with local dressings. On day 3 of conservative management, the neonate had exsanguination due to bleeding from the midline with severe hemodynamic compromise requiring cardiopulmonary resuscitation. After controlling the bleeding with local tamponade and resuscitating the child, she was taken for early surgery. Debridement and bipedicled rotation flap of the scalp to cover the raw area was performed. On day 18, the flap started showing signs of necrosis. The neonate was taken up for debridement, and subsequently, maternal allograft of split-thickness skin was placed as a temporary wound cover. Meanwhile, the wound showed progressive epithelialization. At 1 year, the patient continued to have a non-healing area, which was later successfully covered with a split-thickness skin graft. We plan to revaluate the need for cranioplasty at around 3-4 years of age. DISCUSSION: We discuss the dilemmas and challenges involved in the successful management of a neonate with Adams-Oliver syndrome with infected aplasia cutis and an episode of life-threatening exsanguination. CONCLUSION: Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with defective formation of the scalp to a varying extent and severity, requiring various timely strategies.

Modified Nasoseptal Flap Technique to Prevent the Recurrence of Rathke's Cleft Cyst.

Background: Rathke's cleft cysts (RCCs) are benign epithelial lesions arising from the Rathke's pouch remnants that fail to regress during embryogenesis. Some RCCs become symptomatic and require treatment. Cyst fenestration and drainage of its contents is the preferred procedure to treat symptomatic cases but carries a risk of recurrence. We propose the use of a novel modified nasoseptal flap technique to partially line the cyst wall to avoid recurrence. Methods: This was a prospective, observational study that included all RCC patients admitted to the Department of Neurosurgery, Aster Medcity, from April 2015 to May 2018. The modified nasoseptal flap technique was performed in all patients. They underwent preoperative and postoperative ophthalmological, endocrine, endoscopic, and MRI evaluations to look for recurrence. Results: Ten patients underwent the modified nasoseptal flap technique. The median follow-up was 36 months. Postoperatively, all patients were relieved from headaches. Moreover, their visual fields and pituitary functions normalized. None of the patients developed recurrence of RCC on follow-up brain MRI. On endoscopic examination, all patients had retained patency of the fenestra. The longest follow-up was 72 months. Conclusions: The modified nasoseptal flap technique maintains patency and avoids recurrence of RCCs on long-term follow-up.

Intracranial nanomedicine-gel with deep brain-penetration for glioblastoma therapy.

Glioblastoma Multiforme (GBM) is one of the challenging tumors to treat as it recurs, almost 100%, even after surgery, radiation, and chemotherapy. In many cases, recurrence happens within 2-3cm depth of the resected tumor margin, indicating the inefficacy of current anti-glioma drugs to penetrate deep into the brain tissue. Here, we report an injectable nanoparticle-gel system, capable of providing deep brain penetration of drug up to 4 cm, releasing in a sustained manner up to >15 days. The system consists of ∼222 nm sized PLGA nanoparticles (NP-222) loaded with an anti-glioma drug, Carmustine (BCNU), and coated with a thick layer of polyethylene glycol (PEG). Upon release of the drug from PLGA core, it will interact with the outer PEG-layer leading to the formation of PEG-BCNU nanocomplexes of size ∼33 nm (BCNU-NC-33), which could penetrate >4 cm deep into the brain tissue compared to the free drug (< 5 mm). In vitro drug release showed sustained release of drug for 15 days by BCNU-NP gel, and enhanced cytotoxicity by BCNU-NC-33 drug-nanocomplexes in glioma cell lines. Ex vivo goat-brain phantom studies showed drug diffusion up to 4 cm in tissue and in vivo brain-diffusion studies showed almost complete coverage within the rat brain (∼1.2 cm), with ∼55% drug retained in the tissue by day-15, compared to only ∼5% for free BCNU. Rat orthotopic glioma studies showed excellent anti-tumor efficacy by BCNU-NP gel compared to free drug, indicating the potential of the gel-system for anti-glioma therapy. In effect, we demonstrate a unique method of sustained release of drug in the brain using larger PLGA nanoparticles acting as a reservoir while deep-penetration of the released drug was achieved by in situ formation of drug-nanocomplexes of size <50 nm which is less than the native pore size of brain tissue (> 100 nm). This method will have a major impact on a challenging field of brain drug delivery.

Postulating the concept of compensated trapped fourth ventricle: a case-based demonstration with long-term clinicoradiological follow-up.

BACKGROUND: A trapped fourth ventricle (TFV) is diagnosed when the inlet and outlets of the fourth ventricle are obstructed with a resultant dilated fourth ventricle. CASE REPORT: A 3-month-old male child had a ventriculoperitoneal shunt for post-infectious hydrocephalus. At the age of 9 years, he became symptomatic with altered sensorium. He had a CT head which showed enlarged ventricles with a large fourth ventricle. A subsequent ventriculogram revealed dilated lateral ventricles with an aqueductal obstruction in association with an enlarged fourth ventricle. Although diagnosed as a TFV radiologically, his lateral ventricle shunt was revised with a diagnosis of shunt malfunction as there was a simultaneous dilatation of the lateral ventricle. He was never treated for the TFV. At his latest follow-up at 25 years of age, his MRI brain showed well-decompressed lateral ventricles with a large fourth ventricle. On clinical examination, he had truncal ataxia noticed only during turning which, according to the mother, "was always there". His MR flow study showed obstruction at the level of the aqueduct with some evidence of bidirectional flow at the foramen of Magendie. DISCUSSION: The concept of compensated hydrocephalus represents a stage where a previously active hydrocephalus attains a nonprogressive state clinically and radiologically. We postulate the concept of a "compensated" TFV with a long-term clinical and radiological follow-up. CONCLUSION: We demonstrate and describe a compensated TFV and reiterate that a TFV is a functional concept with imaging being at most only corroboratory.

Quality of life after resection of a meningioma-A cross-cultural comparison of Indian and Australian patients.

PURPOSE: To compare health-related quality of life (HRQoL) and symptom burden following meningioma resection in patients from two samples from Australia and India. This will add to the body of data on the longer-term consequences of living with a meningioma in two socio-economically and culturally different countries. METHODS: The European Organisation for Research and Treatment of Cancer (EORTC) Core Quality of Life Questionnaire (QLQ-C30), Brain Neoplasm Module (QLQ-BN20) and the Hospital Anxiety and Depression Scale (HADS) were administered to 159 Australian and 92 Indian meningioma patients over 24 months postoperative. A linear mixed model analysis identified differences between groups over time. RESULTS: Australian patients reported better physical functioning in the early months after surgery (T1: mean diff: 19.8, p<0.001; T2: mean diff: 12.5, p = 0.016) whereas Indian patients reported better global HRQoL (mean: -20.3, p<0.001) and emotional functioning (mean diff:-15.6, p = 0.020) at 12-24 months. In general, Australian patients reported more sleep and fatigue symptoms while Indian patients reported more gastro-intestinal symptoms over the 2-year follow-up. Future uncertainty and symptoms common for brain tumour patients were consistently more commonly reported by patients in Australia than in India. No differences for depression and anxiety were identified. CONCLUSION: This is the first cross cultural study to directly compare postoperative HRQoL in meningioma patients. Some differences in HRQoL domains and symptom burden may be explained by culturally intrinsic reporting of symptoms, as well as higher care support from family members in India. Although there were differences in some HRQoL domains, clinically meaningful differences between the two samples were less common than perhaps expected. This may be due to an Indian sample with high literacy and financial resources to afford surgery and follow up care.

Brevibacterium casei as a cause of brain abscess in an immunocompetent patient.

Coryneform bacteria belonging to the genus Brevibacterium have emerged as opportunistic pathogens. Of the nine known species of Brevibacterium isolated from human clinical samples, Brevibacterium casei is the most frequently reported species from clinical specimens. We report the first case of B. casei brain abscess in an immunocompetent patient successfully treated by surgery and antimicrobial therapy.

Isolated intracranial Rosai Dorfman disease.

Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder mainly affecting the lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. We report a case of isolated intracranial RDD occurring in a relatively elder patient, which was shown by histological examination to have a dura-based involvement.

Long-term stability of effects of subthalamic stimulation in Parkinson's disease: Indian Experience.

Reports of long-term effects of subthalamic (STN) stimulation for Parkinson's disease (PD) are few, mostly open-label evaluations and from Western centers. We used single-blind and open-label motor, cognitive and quality of life (QOL) evaluations to study the effects of bilateral STN stimulation in 45 patients over 5 years. Our patients showed a stable and substantial reduction in the cardinal signs of PD, motor fluctuations, and dyskinesias but less so for axial signs. The reduction in medications and the intensity of electrical stimulation needed also remained stable during follow up. Although the total QOL and its parkinsonism and social components showed sustained benefits till 5 years, the gains in emotional and systemic subsets were short lasting. Global scores for mood and cognition did not show significant worsening. Benefits of STN stimulation on the cardinal signs, motor complications, and QOL of advanced PD were substantial and sustained till 5 years. The initial benefits in axial motor signs and emotional and psychological aspects of QOL did not show similar stability. In general, the procedure had insignificant impact on cognition and mood. This is the first report of STN stimulation in Asian patients with PD.

Nocardia araoensis Causing Brain Abscess.

Nocardial brain abscess is a rare central nervous system infection with high morbidity and mortality. Most of the human infections, i.e., about 90%, are due to Nocardia asteroides group comprising N. asteroides complex, Nocardia farcinica, and Nocardia nova. Other species rarely cause human infections. Here, we report a case of left parieto-occipital abscess caused by a rare species, Nocardia araoensis, its diagnosis, treatment options, and review of literature. A 73-year-old male, known case of diabetes mellitus, on prolonged oral corticosteroid for autoimmune hemolytic anemia presented with a 1-month history of memory deficit and gait imbalance. On examination, he had a right inferior quadrantanopia and hemiparesis. Magnetic resonance imaging showed a multiloculated ring-enhancing lesion in the left parieto-occipital region. Navigation-assisted biopsy was done. The organism isolated was N. araoensis. He was treated successfully with prolonged course of antibiotics which resulted in complete clinical and radiological resolution. N. araoensis is a rare cause of brain abscess and needs to be suspected in immunocompromised individuals. Early diagnosis and prolonged treatment can result in complete clinical and radiological resolution.

Persisting Embryonal Infundibular Recess Masquerading as a Nasal Mass.

Persisting embryonal infundibular recess (PEIR) is a rare anomaly of the development of the posterior pituitary wherein there is a defect in the third ventricular floor. Earlier reports have found PEIR descending only up to the sella. However, this is the first case of PEIR presenting as a pulsatile nasal mass. A 35-year-old female presented to the otorhinolaryngologist with hyposmia. Diagnostic nasal endoscopy revealed a pulsatile nasal mass. Magnetic resonance imaging revealed a cystic lesion extending from the third ventricular floor to the nasal cavity. There was no associated hydrocephalus or empty sella. The case was successfully managed by the endoscopic endonasal transsphenoidal approach.

Decompressive hemicraniectomy in malignant middle cerebral artery infarction: an analysis of long-term outcome and factors in patient selection.

OBJECT: Middle cerebral artery infarction often occurs at a younger age than other strokes and is associated with significant rates of mortality and morbidity. After a period of pessimism regarding decompressive hemicraniectomy in the management of acute stroke, the method has reemerged in the past decade. The present study was undertaken to assess the immediate and long-term outcome of this intervention and to help better define the selection criteria for surgery. METHODS: The authors conducted a nonrandomized prospective study using decompressive hemicraniectomy with duraplasty in patients at various stages of clinical deterioration due to a space-occupying middle cerebral artery infarct. Patients were assessed at 6 and 12 months postinfarction by using functional scales. Subjective reconsideration was assessed using a questionnaire. Twenty-six patients were included in the study. The mean age was 48.4 +/- 11.2 years, and the mean preoperative Glasgow Coma Scale score was 9.9 +/- 3.2. The median time from ictus to surgery was 54 hours (range 13-288 hours). The rate of survival at 1 year postsurgery was 73%. Among survivors, 33.3% were independent (Barthel Index [BI] > 95) and 55.6% were partially dependent (BI 60-95) at 1 year postsurgery, with 72% attaining the ability to walk independently by 1 year postsurgery. No patient remained in a vegetative state. The 1-year BI score was inversely related to patient age (r = -0.47, p = 0.048). CONCLUSIONS: Survival after decompressive hemicraniectomy was better than previously reported using medical management alone. A vegetative state was avoided and functional independence was possible, especially in younger patients. Increasing age was a statistically significant predictor of disability and long-term functional dependence.