Study of Neurodevelopmental Outcome in Patients with Non-tumoral Hydrocephalus with Shunt Surgery Done in Infancy.

BACKGROUND: Hydrocephalus (HC) is a common neurological disorder presenting in infancy, with a myriad of etiologies requiring early neurosurgical intervention. OBJECTIVE: To study neurodevelopmental outcome in patients with HC with shunt surgery done in infancy. MATERIALS AND METHODS: This was an observational retrospective cohort study of 50 pediatric patients (2 years to 16 years of age). These patients were diagnosed with HC and were operated on with ventriculo-peritoneal shunt (VP shunt) insertion in infancy (did not include patients with brain tumors) and then later following in the neurology outpatient department (OPD). Clinical records and neurodevelopmental assessment (intelligence quotient [IQ]/development quotient [DQ] and vision and hearing assessment) were reviewed. RESULTS: Only 50% of the patients with congenital HC were diagnosed at birth, which included patients who had been diagnosed antenatally and they had lesser complications and better intellectual outcome (P = 0.12), compared with those who presented later with HC. Patient-related factors such as etiology of HC, antenatal diagnosis, and requirement of shunt revisions had poor correlation with neurodevelopmental outcome. Patients with late postoperative complications had significantly poor neurodevelopmental outcome (P ≤ 0.001). Patients with post-meningitis HC required a significantly higher number of shunt revisions than patients with other causes (P = 0.04). CONCLUSION: Better neurodevelopmental outcome depends on early diagnosis and early referral for the management than the cause of HC. Regular head circumference monitoring is the most feasible and sensitive screening tool for early pickup. Larger studies are needed for accurate prognostication.

Experience with Management of Intracranial Arachnoid Cysts.

OBJECTIVES: The aim of this study was to analyze the demographics, clinical presentation, and surgical outcome of intracranial arachnoid cysts and to review the surgical options. METHODS: This is a retrospective observational study of 56 cases of symptomatic arachnoid cysts among all age groups treated between 2004 and 2020 at the Bombay Hospital, Mumbai. Endoscopic fenestration, microsurgical cyst excision, and shunt insertion were the interventions performed. Clinical presentation, cyst reduction post-intervention, and complications were studied. The follow-up period varied from 1 month to 16 years. Statistical analysis was done for 43 patients with a minimum of 3 years' follow-up. RESULTS: In all, 75% of patients were <18 years of age. Of these, the majority were between the age of 1 and 10 years. There were 14 cases of temporal, 13 cases of retrocerebellar, 10 cases of quadrigeminal cistern, and 7 cases each of interhemispheric and suprasellar arachnoid cysts. The most common clinical presentation was headache and vomiting. Concomitant hydrocephalus was seen on imaging in 24 cases. Endoscopic fenestration of cyst was the most routinely performed procedure (35 cases). Four patients of endoscopic fenestration underwent a redo endoscopic procedure on follow-up. Post-operative reduction in cyst size was found to be significantly better after endoscopic fenestration (p < 0.05). CONCLUSION: Though the best available treatment for arachnoid cysts remains controversial, surgery has been found to be beneficial in symptomatic cases. Endoscopic fenestration is considered the first-line surgical option and it may be followed by shunt, if necessary. Shunts may be preferred in very young children where there is associated hydrocephalus/macrocephaly.

Subdural empyema in children.

BACKGROUND: Subdural empyema denotes the collection of purulent material in the subdural spaceand is commonly seen in infants and older children. In infants, the most common cause is bacterialmeningitis. In older children, sinusitis and otitis media are usually the source for subdural empyema. Theclinical symptomatology is varied and has a wide range including prolonged or recurrent fever, seizures,meningeal irritation, and raised intracranial pressure. It can mimic as well as complicate meningitis and aheightened clinical awareness is therefore paramount. AIMS AND OBJECTIVES: The clinical profile, etiopathogenesis, imaging features and management of subdural empyema in children is discussed and the relevant literature is reviewed. CONCLUSION: Subdural empyema is a neurosurgical emergency and rapid recognition and treatment canavoid life-threatening complications. In most cases, surgical decompression through burr hole or craniotomyis warranted. Near complete evacuation of the purulent material and appropriate long-term intravenous antibiotics are necessary for a gratifying outcome.

Moyamoya Vasculopathy in Indian Children: Our Experience.

BACKGROUND: Moyamoya vasculopathy is a chronic progressive vaso-occlusive disease affecting the distal intracranial carotid arteries and their proximal branches. It is an important cause of recurrent strokes in children. Surgical revascularization procedures are now considered as the treatment option for moyamoya vasculopathy. The data from Indian children with moyamoya vasculopathy are limited to a very few studies. STUDY DESIGN: We analyzed the records of children with moyamoya vasculopathy treated at our tertiary care center from 2000 to 2014. Our study population included all patients (aged 0-18 years) with moyamoya disease/syndrome (MMD/MMS). The demographic data, clinical characteristics, imaging, treatment details, and surgical procedures performed were reviewed. RESULTS: A total of 41 patients (females-19, males-22) were identified. Thirty-three (80.48%) had MMD and eight (19.5%) had MMS. The mean age (±standard deviation) at presentation was 6.26 ± 3.79 years (range: 6 months-14 years). Majority had ischemic events at onset; none had hemorrhagic manifestations. Twenty-eight (68.29%) patients underwent surgery (a total of 33 surgical procedures, bilateral in five and unilateral in 23) and 13 (31.7%) were managed conservatively. The median duration of follow-up was 2.2 ± 1.85 years (range: 4 months-7 years). Two/thirteen patients (15%), who were managed conservatively, had recurrent strokes as against none (0/28) in the operated patients. No mortality was observed in our cohort. CONCLUSION: We agree with previous studies that Indian patients with moyamoya vasculopathy differ from their Asian and European counterparts. The availability of expertise in revascularization surgeries in various centers should prompt surgery as an efficient and safe treatment option.

Frontal lobe tuberculoma.

Tuberculomas are usually infratentorial in children, and supratentorial lesions predominate in adults. We present a 4-year-old girl with multiple seizures, papilloedema and brisk reflexes. On investigation, she was found to have a large left parafalcine tuberculoma. She was treated with antitubercular treatment (ATT) and steroids. The child improved, seizures stopped and the papilloedema gradually disappeared. Follow-up magnetic resonance imaging brain after 8 months showed a mild reduction in the size of the lesion. Child is on regular follow-up.

Real-time PCR assay based on the differential expression of microRNAs and protein-coding genes for molecular classification of formalin-fixed paraffin embedded medulloblastomas.

BACKGROUND: Medulloblastoma has recently been found to consist of 4 molecularly and clinically distinct subgroups: WNT, Sonce hedgehog (SHH), Group 3, and Group 4. Deregulated microRNA expression is known to contribute to pathogenesis and has been shown to have diagnostic and prognostic potential in the classification of various cancers. METHODS: Molecular subgrouping and microRNA expression analysis of 44 frozen and 59 formalin-fixed paraffin embedded medulloblastomas from an Indian cohort were carried out by real-time RT-PCR assay. RESULTS: The differential expression of 9 microRNAs in the 4 molecular subgroups was validated in a set of 101 medulloblastomas. The tumors in the WNT subgroup showed significant (P < .0001) overexpression of miR-193a-3p, miR-224, miR-148a, miR-23b, and miR-365. Reliable classification of medulloblastomas into the 4 molecular subgroups was obtained using a set of 12 protein-coding genes and 9 microRNAs as markers in a real-time RT-PCR assay with an accuracy of 97% as judged by the Prediction Analysis of Microarrays. Age at diagnosis, histology, gender-related incidence, and the relative survival rates of the 4 molecular subgroups in the present Indian cohort were found to be similar to those reported for medulloblastomas from the American and European subcontinent. Non-WNT, non-SHH medulloblastomas underexpressing miR-592 or overexpressing miR-182 were found to have significantly inferior survival rates, indicating utility of these miRNAs as markers for risk stratification. CONCLUSIONS: The microRNA based real-time PCR assay is rapid, simple, inexpensive, and useful for molecular classification and risk stratification of medulloblastomas, in particular formalin-fixed paraffin embedded tissues, wherein the expression profile of protein-coding genes is often less reliable due to RNA fragmentation.

Combined intraoperative magnetic resonance imaging and navigated neuroendoscopy in children with multicompartmental hydrocephalus and complex cysts: a feasibility study.

OBJECT: The rationale for using endoscopy to treat complex cysts and multiloculated hydrocephalus is to combine several CSF compartments into a minimum number, establish a connection to functioning CSF compartments (that is, ventricles), and decrease shunt dependency. The aim is to decrease the number of proximal shunt catheters, the number of shunt revisions, and in selected cases even to avoid a shunt. In cases of distorted anatomy and multiloculated cysts, endoscopy may be problematic because of orientation issues. Standard navigation becomes useless soon after CSF loss due to brain shift. Therefore, the concept of "real-time" navigation and intraoperative imaging in combination with endoscopic surgery has been previously suggested. The goal of the present study was to assess the feasibility and efficacy of combining intraoperative MR (iMR) imaging and navigated neuroendoscopy in infants. METHODS: The authors report their experience in treating 5 infants (aged 6-14 months), who underwent surgery for multicystic hydrocephalus presenting with shunt malfunction (4 patients) and a quadrigeminal fetal arachnoid cyst (1 patient). In all infants, a low-field portable iMR imaging system (0.12-T PoleStar N-10/0.15-Tesla PoleStar N-20) was used in conjunction with navigated endoscopy. The authors used e-steady, T1-weighted, and T2-weighted sequences (acquisition time 24 seconds to 3.5 minutes). RESULTS: The iMR imaging system provided clear images that correlated with the endoscopic appearance of the cystic membranes in all patients, and the images were helpful in determining trajectories and redefining targets. The iMR images documented brain shift and changes in CSF spaces during surgery. There were no intraoperative complications or technical difficulties of visualization. No infection or any other immediate postoperative complication occurred. Patients were followed up for 9 months to 7 years. The infant presenting with the quadrigeminal cyst remains shunt free since surgery, and the patients with multicystic hydrocephalus have 1-2 shunts each. Following endoscopic, iMR imaging-guided surgery, shunt catheter positioning was found to be optimal and as planned according to the postoperative imaging. CONCLUSIONS: Navigated neuroendoscopy and iMR imaging may complement each other, offering an advantage over other modalities in complicated cases of hydrocephalus. Whenever targets and trajectories need to be redefined, the iMR images provided an updated navigation data set, allowing accurate navigation of the endoscope and minimizing the number of CSF compartments. Direct vision through the endoscope provides microanatomical details for the optimization of fenestration and catheter positioning. The combined usage of the two modalities may transform a conventional procedure into a visually controlled real-time navigated process.

Posterior fossa craniotomy for trapped fourth ventricle in shunt-treated hydrocephalic children: long-term outcome.

OBJECT: Trapped fourth ventricle (TFV) is a rare late complication of postinfectious or posthemorrhagic hydrocephalus. This entity is distinct from a large fourth ventricle because TFV entails pressure in the fourth ventricle and posterior fossa due to abnormal inflow and outflow of CSF, causing significant symptoms and signs. As TFV is mostly found in children who were born prematurely and have cerebral palsy, diagnosis and treatment options are a true challenge. METHODS: Between February 1998 and February 2007, 12 children were treated for TFV in Dana Children's Hospital by posterior fossa craniotomy/craniectomy and opening of the TFV into the spinal subarachnoid space. The authors performed a retrospective analysis of relevant data, including pre- and postoperative clinical characteristics, surgical management, and outcome. RESULTS: Thirteen fenestrations of trapped fourth ventricles (FTFVs) were performed in 12 patients. In 6 patients with prominent arachnoid thickening, a stent was left from the opened fourth ventricle into the spinal subarachnoid space. One patient underwent a second FTFV 21 months after the initial procedure. No perioperative complications were encountered. All 12 patients (100%) showed clinical improvement after FTFV. Radiological improvement was seen in only 9 (75%) of the 12 cases. The follow-up period ranged from 2 to 9.5 years (mean 6.11 ± 2.3 years) after FTFV. CONCLUSIONS: Fenestration of a TFV via craniotomy is a safe and effective option with a very good long-term outcome and low rate of morbidity.

Modified bilateral subtemporal decompression for resistant slit ventricle syndrome.

PURPOSE: Slit ventricle syndrome (SVS) remains a major problem for early shunted children. Several conservative and surgical treatment paradigms have been suggested; however, there is no consensus on the optimal surgical treatment. We present our experience using bilateral subtemporal decompressions with dura and arachnoid opening for the treatment of a subgroup of children with severe and resistant SVS. METHODS: Fifteen children with severe and resistant SVS underwent a modified bilateral subtemporal craniectomy, with dura and arachnoid opening. Their clinical and radiological data were retrospectively reviewed. RESULTS: Seven (46.6%) patients had a complete recovery from their symptoms with a follow-up of 5.9 ± 2.6 years.The remaining eight (53.3%), underwent additional surgeries. Four (26.6%), had a single proximal shunt revision after dilatation of their ventricles. Following these procedures these four children are well and stable with a follow-up of 1.8 ± 2 years. The other four had further cranial vault expansion, one of which was followed by a proximal shunt revision. Thus, 11 of these 15 patients (73.4%) had a very good outcome, attributable to this technique, with a mean follow-up of 4.5 ± 3 years. CONCLUSIONS: Modified bilateral subtemporal decompression with dura and arachnoid opening yields a high cure rate for severe and resistant slit ventricle syndrome. Proximal shunt revision may be safely performed in a subset of patients that dilate their ventricles following the procedure. Further cranial expansion may be reserved for children with recurrent SVS symptoms who do not respond to STD and remain with very small ventricles.

Real-time neuronavigation with high-quality 3D ultrasound SonoWand in pediatric neurosurgery.

Intraoperative ultrasound (IOUS) serves as a basic imaging tool in neurosurgery. However, its low quality and difficulty in interpreting the images make its use as a resection control tool and navigation system cumbersome. We present our experience using a high-resolution 3D IOUS system combined with a navigation system in pediatric cranial surgery. We retrospectively reviewed 16 pediatric neurosurgical procedures in which a high-resolution 3D IOUS combined with a navigation system was used. The system enables basic navigation based on preoperative computed tomography or magnetic resonance imaging scans. In addition, IOUS images serve as a data set for updated intraoperative navigation. The indications for IOUS were preoperative navigation to define the skin incision and exact craniotomy site, and for real-time neuronavigation and resection control during tumor removal. The added time per case was short and no technical difficulties were encountered. High-resolution 3D IOUS combined with navigation systems has advantages for the pediatric neurosurgical population, including both real-time basic navigation and improved resection control.

Classical and real-time neuronavigation in pediatric neurosurgery.

INTRODUCTION: Neuronavigation has become a cornerstone of neurosurgery. Navigation systems are categorized into two main groups: those based on preoperative imaging and those based on real-time intraoperative acquired images. OBJECTIVES: The preoperative imaging systems, either computed tomography (CT)- or magnetic resonance imaging (MRI)-based, are straight-forward systems that are routinely used in most institutions. Image accuracy, however, decreases secondary to brain and lesion shifts that occur during surgery. Intraoperative, real-time navigation systems overcome anatomical shifts by updating the image base of the navigation during surgery, thus, maintaining precise navigation capabilities throughout the surgical procedure. CONCLUSIONS: In this article, we review the main neuronavigation systems and their applications, emphasizing their unique advantages and usage within the pediatric population.

Intraoperative portable 0.12-tesla MRI in pediatric neurosurgery.

OBJECTIVES: Intraoperative MRI (iMRI) is used mainly in the adult neurosurgical population. The main indications for iMRI usage are resection control and updated intraoperative navigation capabilities. In this paper we present our experience using this technique in children. Specific advantages of iMRI for this age group are discussed. METHODS AND RESULTS: We retrospectively reviewed 31 pediatric neurosurgical procedures in which a portable iMRI system was used. The indications for iMRI usage were preoperative navigation, resection control during tumor removal, shunt placements, and needle biopsy. In 7 children the use of the iMRI changed the course of the surgical procedure. Operative morbidity and mortality were not increased with use of the iMRI. CONCLUSIONS: iMRI systems have advantages for the pediatric neurosurgical population, including both real-time basic navigation and improved resection control.