Surgical treatment of pediatric low-grade glioma in developing countries.

Pediatric low-grade gliomas constitute the most common brain tumors worldwide, though with some peculiarities in the presentation and surgical care in different parts of the world. The symptomatology in developing countries is likely to be over longer periods with a tendency to delayed diagnosis due to cultural, religious beliefs, manpower, and infrastructural deficits. Thus, the children present with large tumors with attendant morbidities and an increased risk of mortalities from surgery. Surgery is mainly by "general" neurosurgeons due to the paucity of trained pediatric neurosurgeons. The pre-operative imaging may be limited to anatomic MR imaging, and in some cases, CT scans, without expansive neuropsychological evaluation. The armamentarium available to the neurosurgeon may warrant large openings to access the tumor, and there may be limited possibility for intra-operative mapping of "eloquent" brain functions when this is deemed necessary. Complicating pre-operative acute hydrocephalus can result in two operations that further worsen the catastrophic spending associated with brain tumor surgeries in these climes. While these challenges appear daunting but certainly have not been enough to deter the "can do" spirit of neurosurgeons in developing countries, it is essential to strengthen the training of pediatric neurosurgeons in LMICs and provide a platform for the advocacy of better infrastructure for the surgical management of these tumors.

Split cord malformation associated with congenital dermoid cyst and myeloschisis - case-based literature review on possible embryonic derivation and implications.

Split cord malformation co-existing with either congenital dermoid or myeloschisis has been previously reported. Theories exist which explain the underlying embryopathy behind the occurrence of each of these anomalies in conjunction with split cord malformation. However, the occurrence of all three anomalies in the same patient, to the best of our knowledge, has not yet been reported in the literature. We report two cases on the co-existence of congenital dermoid cyst with both myeloschisis and split cord malformation and review the literature on previous postulations of mechanisms that could possibly explain the simultaneous occurrence of all three distinct anomalies. They involve all three primary germ layers and therefore share a common embryonic origin from the epiblast. We try to explain their co-existence based on the theory of notochordal splitting and endomesechymal tract formation consequent on endodermal-ectodermal adhesion as postulated by Beardmore and Wigglesworth and discuss on implications in terms of treatment paradigms. The unusual combination of all three anomalies in both patients probably highlights a need for further research on their pathogenesis despite these previous theories. These cases demonstrate the fact that currently accepted embryological explanations of most pathologies especially outliers still fall short with much remaining to be understood.

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.

Avoiding the antenatal counselling faux pas: bridging the gap between prenatal prognostication and postnatal outcome of closed spina bifida.

PURPOSE: Closed spina bifida (CSB) is rare in prenatal literature, and various lesions are grouped under this broad nosological entity CSB, leading to confusing and misleading prognostic conclusions. METHODS: This is a retrospective observational cohort study of prenatally detected CSB cases using two-dimensional ultrasound, complemented by three-dimensional ultrasonography and foetal MRI in indicated cases, from October 2014 to October 2021 in a tertiary-level single centre. RESULTS: The most common upper vertebral level of CSB was lumbar in 66.6% (10/15). The sub-classification of lesions based on prenatal ultrasound showed an agreement in 53% of the cases. Sixty percent had associated abnormalities identified postnatally, the most common being anorectal malformation seen in 33.3%. On postnatal follow-up, 46.6% had bowel incontinence and bladder dysfunction, and 33.3% developed lower limb deformities. CONCLUSIONS: All CSBs do not have a uniformly favourable prognosis. The prognosis of CSB depends on the pathological type, the presence of associated abnormalities and the management.

Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome.

OBJECTIVE: In this study, the authors aimed to 1) retrospectively analyze the early functional outcomes in a cohort of very young children with craniofacial dysostoses who underwent robot-assisted frontofacial advancement (RAFFA) or robot-assisted midface distraction (RAMD), and 2) analyze the utility of robotic assistance in improving the accuracy and safety of performing transfacial pin insertion for RAFFA or RAMD. METHODS: A retrospective analysis of a cohort of 18 children (age range 1-42 months at presentation), who underwent RAFFA or RAMD from February 2015 to February 2021 in the craniofacial unit at Amrita Institute of Medical Sciences and Research Centre in Kochi, India, was performed. Inclusion criteria were patients who had undergone RAFFA in a single stage or RAMD where the cranial vault had been addressed earlier, had been addressed on follow-up, or had not been addressed and had follow-up of at least 6 months. RESULTS: Overall, 18 children with syndromic craniosynostosis underwent LeFort level III midface distraction, with or without RAFFA, from February 2015 to February 2021 at a single center in India. The patients' ages ranged from 6 to 47 months at the time of the procedure. All patients had significant obstructive sleep apnea (OSA), significant ocular issues, and disturbed sleep as determined by the authors' preoperative protocol. Clinically significant intracranial pressure issues were present in 17 patients. None of the patients had injury due to the transfacial pin trajectory such as globe injury, damage to the tooth buds, or the loss of purchase during the active distraction phase. The mean distraction achieved was 23 mm (range 18-30 mm) (n = 16/18). Of the 18 patients, 10 (56%) had an excellent outcome and 6 (33%) had a satisfactory outcome. In all cases, the degree of OSA had significantly reduced after surgery. Eye closure improved in all patients, and complete closure was seen in 11 patients. On follow-up, the functional gain remained in 14 of 16 patients at the final follow-up visit. The distraction results were stable during the follow-up period (mean 36 months [range 6-72 months]). CONCLUSIONS: The early RAFFA and RAMD protocols investigated in this study gave a significant functional advantage in very young patients with craniofacial dysostoses. The results have demonstrated the accuracy and safety of robotic assistance in performing transfacial pin insertion for RAFFA or RAMD.

Should not we be using aspirin in patients with a ventriculoatrial shunt? Borrowing a leaf from other specialities: a case for surrogate evidence.

INTRODUCTION: Ventriculoatrial (VA) shunts are life-saving in circumstances where ventriculoperitoneal shunts (VP) have failed. They are at risk for different complications, and more specific of them are cardiopulmonary complications. Currently, there are no standard recommendations concerning screening for risk factors, prophylaxis, or anticoagulation treatment in patients after VA shunt placement. Our study aims to prospectively study the possible role and efficacy of the use of aspirin to increase the survival of shunts in children with VA shunt and avoid secondary morbidity. In this article, the authors describe the interim results of an ongoing prospective study which supports the use of aspirin for VA shunt. MATERIALS AND METHODS: The study design is prospective. The duration of the study is 2011 onwards and is ongoing. Hospital ethics board clearance and consent from the family were taken before inclusion in the study. All patients who had VA shunt were given a once-a-day low-antiplatelet dose of aspirin 5 mg/kg, from the first postoperative day onwards. Primary endpoints of the study are as follows: (1) major distal end malfunction documented on echocardiography or (2) any cardiac complications associated with the VA shunt catheter. RESULTS: We have 6 patient since march 2011, who are being followed up. None of the shunts had malfunctioned until the reporting. None of the patients had any cardiac issues reported. The patients are to be followed continually. The present follow-up ranges from 2.5 to 10 years. The patient follow-up is being continued. CONCLUSIONS: Aspirin is a drug with well-accepted safety profile, and its use and our preliminary observation and outcome of the use of aspirin in VA shunt are promising.

Intraoperative Neuromonitoring for Tethered Cord Surgery in Infants: Challenges and Outcome.

OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of intraoperative neuromonitoring (IONM) in surgery for tethered cord in infants. MATERIALS AND METHODS: The study included 87 infants who underwent surgery for closed spinal dysraphism under IONM. Their preoperative neurological and urological statuses were compared with postoperative status clinically. The study design was prospective, and the study's duration was from January 2011 to February 2020. IONM was performed (TcMEP and direct mapping) with an Xltek Protektor 32 IOM system, Natus Neurology/medical Inc., Middleton, USA. Statistical analysis in the form of χ2 is conducted using SPSS. RESULTS: Overall, among 87 patients, clinical improvement was seen in 28 (28/29) patients with motor deficits, 17 (17/24) with bladder deficits, and 18 (18/24) with bowel deficits. The monitorability for motor and sphincter was 97.3% and 90.7%, respectively. The sensitivity of IONM in predicting new motor deficit was 100%, whereas the specificity was 100%. The negative predictive value of predicting motor deficit was 100%, with a diagnostic accuracy of 100%. There were no complications in this cohort related to the IONM. CONCLUSIONS: The study has highlighted that the use of IONM is sensitive in identifying motor injury in infants with reliable outcome correlation. Assessment, monitoring, and outcome correlation of bladder and sphincteric functions are a challenge in this cohort.

Prognostication of the neurological outcome of tethered cord based on intraoperative neuromonitoring findings: how close can we get?

PURPOSE: To evaluate the correlation of intraoperative neuromonitoring (IONM) data in surgery for tethered cord syndrome (TCS) in children to the neurological outcome at 1-year follow-up. METHODS: 208 consecutive patients operated on for TCS, between January 2011 to February 2020, under electrophysiological monitoring in the Division of Paediatric Neurosurgery, AIMS, Kochi, India, were included. Their preoperative neurological, urological and orthopaedic status were compared with the postoperative status at 1 year follow-up. RESULTS: Our study prospectively collected the IONM data and retrospectively correlated it to the children's neurological outcome on follow-up. Out of 208 children, 28% (n = 59/208) had motor, 35% (n = 73/208) had bladder and 26% (n = 54/208) had bowel disturbances. Postsurgery, at one-year follow-up, 91% (n = 52/57) of the patients who had motor deficits had improvement, 82.3% (n = 51/62) of patients who had bladder deficits showed an improvement, and 88.8% (n = 48/54) with bowel deficits showed improvement. The monitorability for motor and sphincter potentials were 99.4% and 89.3%, respectively. Except for four patients (3 with bladder and 1 with motor worsening), all the patients who were monitorable with no deficits remained intact except for four patients, all the patients who had deficits and were monitorable improved after detethering (at 1 year). Clinical worsening corresponded to those who had a drop in amplitude of baseline TcMEP (n = 4). 18 events showed an increase in amplitude compared to the baseline TcMEP. These patients improved clinically on follow-up (at 1 year). CONCLUSIONS: IONM complements the preoperative clinical details in predicting immediate and long-term outcomes.

Four novel mutations in EFNB1 in Indian patients with craniofrontonasal syndrome.

Craniofrontonasal syndrome (CFNS) (OMIM #304110) is a very rare, X-linked developmental disorder characterized by facial stigmata, including hypertelorism, frontonasal dysplasia, craniosynostosis, bifid nasal tip, and digital abnormalities. CFNS is caused by mutations in the Ephrin 1 gene (EFNB1) located at Xq13.1, which encodes the transmembrane protein Ephrin B1. Interestingly, heterozygous females are more severely affected than hemizygous males. We report on four individuals from four unrelated Indian families with mild-to-severe CFNS. All patients had variable degrees of hypertelorism and nasal bridge depression, which did not correlate with changes in other tissues. Although patients 3 and 4 showed the most severe facial dysmorphism and syndactyly, there were no structural CNS changes or developmental delay. In contrast, patient 1 displayed agenesis of corpus callosum and developmental delay, although facial and finger abnormalities were milder. Patients 1, 2, and 4 showed different degrees of clefting. DNA sequencing revealed four previously undescribed heterozygous mutations in exons 1 and 2 of EFNB1. Patient 1 carried the second single amino acid deletion reported up to date. The other three affected individuals harbored frameshift mutations, leading to premature termination codons. Our findings broaden the spectrum of EFNB1 mutations and illustrate the absence of an obvious correlation between mutation type, severity, and expression of symptoms.

Why do metopic sutural synostoses angulate? The concept of nasion sutural complex and its implication on the management of hypotelorism-early results and proof of concept.

OBJECT: Angulation at the suture is a hallmark of metopic synostoses amongst all craniosynostoses. No other sutural synostoses demonstrate angulation at synostoses consistently. We look into the possible aetiology and the implication of the understanding in the treatment goals of trigonocephaly. We hypothesise that the nasal bone and nasofrontal suture viz. "nasion sutural complex" are involved in trigonocephaly along with the well-accepted role of metopic suture. We propose that it is the angulation at this junction which leads to trigonocephaly and its secondary features. MATERIALS AND METHODS: The study included seven infants, who underwent correction for trigonocephaly at our paediatric craniofacial division at Amrita Institute of Medical Sciences and Research Centre, Kochi, India, between the period July 2015 to March 2018. The cohort included were infants with trigonocephaly who had CT head for diagnosis. We analysed the multidimensional CT (MDCT) of these infants and compared to an equal number of age-matched controls. The controls were infants with other forms of sutural synostosis with metopic uninvolved and normal infants where MDCT was done for other reasons. Sutural characteristic at the nasion and metopic suture recorded in comparison with an equal number of age-matched controls. We performed spring cranioplasty for three infants after metopic suturectomy, extending the release beyond the nasion sutural complex, placing springs to distract the suture. The infants who underwent spring cranioplasty were followed up for the aesthetic outcome. Remaining infants of the study underwent standard frontorbital correction for metopic craniosynostoses. RESULTS: We could demonstrate a fusion of nasofrontal and nasal suture in all cases (n = 7) of trigonocephaly included in the study on MDCT and intraoperatively. We performed spring cranioplasty for three infants (n = 3/7), where we released the internasal suture. At 3 months follow-up, along with correction of the angulation, the hypotelorism improved significantly. Other infants in the study (4/7) underwent classical frontorbital advancement. CONCLUSIONS: Fusion of nasion sutural complex along with metopic sutures may explain the angulation in trigonocephaly. We propose that all minimally invasive techniques for correction of trigonocephaly and associated hypotelorism should consider this fact for an improved outcome.

A proposal for a tailored protocol for focal suppurative infection of the central nervous system: analysis of an institutional experience in pediatric patients.

OBJECTIVE: The incidence of focal suppurative infections (FSIs) of the brain has significantly decreased owing to the better health and fundamental conditions of the population on the one hand and earlier detection and the availability of more potent antibiotics on the other. Interestingly, the antibiotic protocols have not been well defined in terms of duration despite a prompter diagnosis, definitive management of the etiology, and the advent of various higher-generation antibiotics. In this study, the authors evaluated the current treatment protocol. Their aim was to optimize management protocols for FSIs of the central nervous system based on clinical parameters. METHODS: The study was a retrospective analysis of all children who had undergone surgical management for an FSI at the Division of Paediatric Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, in the period from January 2001 to February 2018. In addition to demographic characteristics, the details of culture reports and antibiotic regimens were collected. The response to treatment was compared to changes in the infective profile (C-reactive protein) and imaging. Instances of reaspiration were compared with clinical signs, imaging findings, and infective profiles. Treatment response was separated into two groups: responders within or at 2 weeks and responders beyond 2 weeks. The clinical characteristics of these two groups were compared. RESULTS: Forty-eight children were treated in the study period. Nineteen patients benefited from the 2-week (short-term) protocol of intravenous antibiotics. Twenty-nine patients required more than 2 weeks (approximately 4 weeks; long-term protocol) for resolution. Of those requiring more than 2 weeks, 69% had cardiogenic etiology. All patients were followed up with a minimum of 3 weeks of oral antibiotics. In a comparative analysis between short-term and long-term responders, only etiology was significantly different. None of the patients who had the short-term protocol had a recurrence. CONCLUSIONS: A shorter antibiotic protocol can be used successfully in carefully selected patients who are surgically treated and followed up. It is clear that the 2-week intravenous antibiotic protocol is more suitable for immunocompetent patients who have a noncardiogenic etiology.

Single-stage deformity correction in children with neurogenic kyphoscoliosis: nuances and a proposal for risk stratification.

OBJECTIVE Neurogenic kyphoscoliosis in pediatric patients has varied causes and diverse management options. The most common management strategy is to stage the orthopedic and neurosurgical aspects of the correction at an interval of 3-6 months from each other. The aim of this study was to report the authors' experience with correction of deformity in children with associated neurological abnormalities requiring intervention as a single-stage surgical treatment. METHODS Of 591 pediatric patients with scoliosis treated at the Amrita Institute of Medical Sciences and Research Centre between January 2001 and January 2011, the authors identified all patients with neurogenic kyphoscoliosis who underwent deformity correction and a neurosurgical procedure in the same surgical session when they were younger than 15 years. Data regarding the demographic details, preoperative Cobb angle/kyphotic angle, surgical details, postoperative complications, cost of the procedure, and long-term neurological outcome were collected for each case and analyzed. Based on the results of this analysis, the authors propose a 2-category risk stratification system for the timing of deformity correction depending on the primary neurogenic etiology. RESULTS Ten cases involving patients with neurogenic kyphoscoliosis requiring both deformity correction and neurosurgical procedure met the criteria and were included in the analysis. All 10 patients were younger than 15 years; their mean age was 10.8 years. The average Cobb angle was 59.5°. Five patients underwent foramen magnum decompression, 5 patients underwent spinal cord detethering, and 1 patient had intraspinal intradural tumor excision. The mean estimated blood loss was 1177 ml (range 700-1550 ml), and the mean operative time was 4.4 hours (range 3-5.5 hours). There were no intraoperative complications. None of the patients had any new postoperative neurological deficits. The mean postoperative Cobb angle of the main thoracic curve was 12.6°. CONCLUSIONS Simultaneous deformity correction along with a definitive neurosurgical procedure in patients with neurogenic kyphoscoliosis can be performed without added morbidity and avoids both the added costs and the risk of complications associated with a second surgery. The risk stratification system proposed in this paper may serve as a guideline for case selection and help to optimize outcome.

An Analysis of Outcome of Endoscopic Fenestration of Cavum Septum Pellucidum Cyst - More Grey than Black and White?

BACKGROUND: Cavum septum pellucidum (CSP) and cavum vergae are actually fluid-filled, generally communicating midline cavities located between the third ventricle and corpus callosum. There have been various reports of their association with many behavioral and psychiatric disorders. Infrequently, they have been associated with an obstructive hydrocephalus-like picture. Although the structure and management of CSP has long been known, it has been an enigma as far as functional significance and management indications are concerned. The authors of this article try to analyze the significance of a persistent cavum and involvement of the same entity in varied presentations ranging from an incidental imaging finding to acute hydrocephalus, and propose a possible implication on the present surgical intervention paradigm. PURPOSE: To assess the surgical outcome of fenestration of a CSP cyst. METHODS: Retrospective analysis of 3 patients who underwent endoscopic fenestration for CSP with obstructive hydrocephalus between 2012 and 2014 was done, and data were analyzed for symptomatic clinical improvement in particular behavior. RESULTS: Pre- and postoperative brain MRI showed a significant decrease in the size of the cyst as well as the ventricles. There were no recurrences during follow-up. All of the patients improved. CONCLUSIONS: (1) Endoscopic fenestration of symptomatic CSP cysts is a safe treatment option. (2) Neurocognitive assessment is essential in the evaluation and outcome assessment of CSP.

Customizable rigid head fixation for infants: technical note.

INTRODUCTION: The need and advantages of rigid fixation of the head in cranial surgeries are well documented (Berryhill et al., Otolaryngol Head Neck Surg 121:269-273, 1999). Head fixation for neurosurgical procedures in infants and in early years has been a challenge and is fraught with risk. Despite the fact that pediatric pins are designed, rigid head fixation involving direct application of pins to the head of infants and slightly older children is still generally not safe (Agrawal and Steinbok, Childs Nerv Syst 22:1473-1474, 2006). Yet, there are some surgeries in which some form of rigid fixation is required (Agrawal and Steinbok, Childs Nerv Syst 22:1473-1474, 2006). We describe a simple technique to achieve rigid fixation of the head in infants for neurosurgical procedures. TECHNICAL NOTE: This involves applying a head band made of Plaster of Paris (POP) around the head and then applying the fixation pins of the fixation frame directly on to the POP. CONCLUSION: We have used this technique of head fixation successfully for infants with no complications.

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.

Beckwith-Wiedemann syndrome and Chiari I malformation--a case-based review of central nervous system involvement in hemihypertrophy syndromes.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is an unusual complex of abnormalities that includes mainly omphalocele, macroglossia, gigantism, visceromegaly, and neonatal hypoglycemia. Type I Chiari malformation, on the other hand, is defined as ectopia of the cerebellar tonsils below the plane of the foramen magnum. Only one case of association of BWS with Chiari I malformation has been previously reported in the literature. DISCUSSION: Several conditions involving congenital hemihypertrophy have been previously reported in association with Type I Chiari malformation. The pathophysiological mechanism for most of these associations is thought to be quite complex and still remains unclear. However, the presence of tonsillar herniation in BWS has been explained by Tubbs and Oakes in the only one existing case report of BWS with Type I Chiari malformation in the literature, to be due to associated hemihypertrophy of the skull base. We additionally suggest that cerebellar hypertrophy may also contribute to the tonsillar herniation and fourth ventricular outlet obstruction. ILLUSTRATIVE CASE: We now report our recent experience on this association following a review of the literature on association of other hemihypertrophy syndromes with the central nervous system anomalies. CONCLUSION: We believe that a common pathogenesis of Type I Chiari malformation occurs in conditions of hemihypertrophy including BWS, probably secondary to dysmorphology involving the posterior cranial fossa, and is not just an associated finding.

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.