Torcular dural sinus malformation

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.

The cost of serial cerebrospinal fluid aspirations between ventricular access device (VAD) and ventriculosubgaleal shunt (VSGS) for treatment of post-hemorrhagic ventricular dilatation (PHVD) in premature infants

Introduction: Ventriculosubgaleal shunts (VSGS) require fewer cerebrospinal (CSF) aspirations than ventricular access devices (VAD) for temporization of post-hemorrhagic ventricular dilatation (PHVD) in preterm infants. Cost of postoperative CSF aspiration has not been quantified. Methods: We reviewed CSF aspiration and laboratory studies obtained in preterm infants with PHVD and VAD at our institution between 2009-2020. Cost per aspiration was calculated for materials, labs, and Medicare fee schedule for ventricular puncture through implanted reservoir. We searched PubMed, Cochrane Library, Embase, CINAHL, and Web of Science for meta-analysis of pooled mean number of CSF aspirations and proportion of patients requiring aspiration. Results: Thirty-five preterm infants with PHVD had VAD placed with 22.2±18.4 aspirations per patient. Labs were obtained after every aspiration per local protocol. Cost per aspiration at our institution was $935.51. Of 269 published studies, 77 reported on VAD, 29 VSGS, and 13 both. Five studies on VAD (including the current study) had a pooled mean of 25.8 aspirations per patient (95%CI:16.7-34.8). One study on VSGS reported a mean of 1.6±1.7 aspirations. 3 studies on VAD (including the current study) had a pooled proportion of 97.4% of patients requiring aspirations (95%CI: 87.9-99.5). Four studies on VSGS had a pooled proportion of 36.5% requiring aspirations (95%CI:26.9-47.2). Frequency of lab draws ranged from weekly to daily. Based on costs at our institution, mean number of aspirations, and proportion of patients requiring aspirations, cost difference ranged between $4,243 to $23,235 per patient and $500,903 to 2.36 million per 100 patients depending on frequency of taps and Medicare locality. Discussion/Conclusion: Lower number of CSF aspirations using VSGS can be associated with considerably lower cost compared to VAD.

Gamma Knife Radiosurgery in the Management of Hypothalamic Glioma: A Case Report with Long-term Follow-up

Background Optic pathway/hypothalamic gliomas are rare pediatric brain tumors. The management paradigm for these challenging tumors includes chemotherapy, radiotherapy, or surgical resection, but the optimal management strategy remains elusive. Gamma Knife radiosurgery (GKRS) has emerged as a promising treatment for such lesions as documented by a small number of cases in the literature. Case Presentation Herein, we present a rare case of hypothalamic glioma in a 13-year-old girl who was referred to our service due to growth of a an incidentally diagnosed hypothalamic lesion following head injury at the age of 8 years. The lesion demonstrated hypointensity on T1 and hyperintensity on T2 without enhancement. Given the growth of the lesion on serial imaging, a stereotactic biopsy was performed demonstrating low-grade glioma. The patient underwent GKRS treatment with a marginal dose of 15 Gy at 50% isodose line for a tumor volume of 2.2 ml. Annual radiological surveillance over the next 17 years demonstrated a gradual shrinkage of the lesion until it completely disappeared. The patient is currently a healthy 31-year-old female without any visual, endocrine, or neurocognitive complaints. Conclusion The outcome obtained after extended follow-up in our patient highlights the safety and efficacy of GKRS in the management of hypothalamic gliomas in pediatrics, which in turn can avoid potentially serious complications of surgery in this vulnerable patient population in this sensitive location.

Orbitofrontal Extradural Hematoma: Unusual Cause of Proptosis

Traumatic orbital subperiosteal hematoma are a distinct entity and should not be confused with orbital extradural hematoma. Most of the orbital subperiosteal hematoma are small in volume and resolve spontaneously. Management should be based on the clinico-radiological condition. A delayed proptosis may be simply due to orbital venous congestion due to a substantial hematoma or due to a carotid-cavernous fistula. Differentiation between the two is paramount to decide management strategy.

The Case for Early Antibiotic Commencement and Source Control in Paediatric Subdural Empyema: A Single-Centre Retrospective Case Series

<b><i>Background:</i></b> Subdural empyema is a neurosurgical emergency requiring prompt diagnosis and treatment. There is a debate between the benefits and risks of starting early antibiotics prior to surgical drainage as this is purported to reduce the rate of microbiological diagnosis. Here, we describe our experience of treating this potentially life-threatening condition, advocating for the early commencement of antibiotics and importance of source control in its treatment. <b><i>Methods:</i></b> Retrospective review of a prospectively collected electronic departmental database included all patients who were admitted to our unit with a diagnosis of subdural empyema over an 11-year period (2008–2018). Basic demographic data were collected. Further data pertaining to mode of presentation, surgical approach, causative organism, post-operative antibiotic regime, anti-seizure medications, length of hospital stay, further surgery, and neurological outcomes were extracted. <b><i>Results:</i></b> Thirty-six children underwent 44 operations for subdural empyema at our institution during the study period. Median age was 11.0 (range 0.2–15.8); 47.2% (17/36) were female. Over time, there was decreasing use of burr holes and increasing use of craniectomy as the index surgery. Using a combination of extended culture and polymerase chain reaction, a microbiological diagnosis was achieved in all 36 cases; the commonest causative microorganism was of the <i>Streptococcus anginosus</i> group of bacteria. Seven patients underwent repeat surgery, and 4 patients underwent a concurrent ENT procedure. No risk factors were significant in predicting the likelihood of re-operation (location of subdural empyema, age, index surgery type, inflammatory markers, concurrent ENT procedure, and microorganism) although it was notable that none of the patients undergoing a concurrent ENT procedure underwent repeat surgery (<i>p</i> = 0.29). Median length of stay was 12 days (range 3–74), and there were no inpatient or procedure-related mortalities. Clinical outcomes were good with 94.4% (34/36) categorized as modified Rankin Scale 0–3 at discharge and there were 2 cranioplasty-related complications. <b><i>Conclusions:</i></b> We observed an evolution of practice from limited surgical approaches towards more extensive index surgery over the study period. Given that a microorganism was isolated in all cases using a comprehensive approach, initiation of antibiotic therapy should not be delayed on presentation. Concurrent ENT surgery may be an important factor in providing aggressive source control thereby reducing the need for repeat surgery.

Validation of Sonographic Fronto-Occipital Ratio Based on Anatomical Landmarks compared to MR/CT-Derived Indexes in Children with Chiari II and Ventriculomegaly

Introduction: Ultrasound (US) based indexes such as fronto-occipital ratio (FOR) can be used to obtain an acceptable estimation of ventricular volume. Patients with colpocephaly present a unique challenge due to the shape of their ventricles. In the present study, we aim to evaluate the validity and reproducibility of modified US-FOR index in children with Chiari II-related ventriculomegaly. Methods: In this retrospective study, we evaluated Chiari II patients younger than one year who underwent head US and MR or CT scans for ventriculomegaly evaluation. MR/CT based FOR was measured in the axial plane by identifying the widest diameter of frontal horns, occipital horns, and the interparietal diameter (IPD). US based FOR (US-FOR) was measured using the largest diameter based on the following landmarks: frontal horn and IPD in the coronal plane at the level of the foramen of Monro, IPD just superior to the Sylvian fissures, and occipital horn posterior to the thalami and inferior to the superior margins of the thalami. Interclass correlation coefficients (ICC) were used to evaluate inter-rater reliability and Pearson correlation coefficients and Bland-Altman plots were applied to assess agreement between US and other two modalities. Results: Ninety paired US and MR/CT exams were assessed for agreement between US-FOR and MR/CT-FOR measurements. ICC showed an excellent inter-rater reliability for US-FOR (ICC=0.99, p<0.001) and MR/CT-FOR measurements (ICC=0.99, p<0.001). The mean (range) values based on US-FOR showed a slight overestimation in comparison to MR/CT-FOR [0.51 (0.36-0.68) vs 0.46 (0.34-0.64)]. Pearson correlation coefficient showed high cross-modality agreement for the FOR index (r=0.83, p<0.001). Bland-Altman plot showed excellent concordance between US-FOR and MR/CT-FOR with a bias of 0.05 (95% CI, -0.03-0.13) Conclusion: US-FOR in the coronal plane is a comparable tool for evaluating ventriculomegaly in Chiari II patients when compared with MR/CT-FOR, even in the context of colpocephaly.

The burden of pediatric subarachnoid hemorrhage healthcare disparities in Senegal: A retrospective cohort study

Introduction Pediatric aneurysms are uncommon but potentially deadly clinical conditions with varied etiology and outcomes. In low-resource countries, numerous barriers prevent the timely diagnosis and management of pediatric aneurysmal subarachnoid hemorrhage (aSAH). Thus, this study aimed to assess the mortality of pediatric aSAH stemming from limited access to pediatric neurological surgery care in Senegal. Methods Pediatric aSAH patients admitted at the authors’ institution from 2012 and 2020 were recruited. Spearman Rho’s correlation, McNemar’s test, and Wilcoxon signed-rank test were used. Odds ratios and their 95% confidence intervals were calculated, and the population attributable fraction (PAF) was used to quantify aSAH mortality attributable to lack of surgical care. Results Twenty-four pediatric patients (12 females and 12 males) aged 12.2 (95% CI=10.0-14.3) years presented with aSAH. They had 1 median aneurysm (range [1, 2]) measuring 12.6 (6.1-19.0) mm. The median WFNS grade was 3 (range [1, 4]), and the mean Fisher grade was 4 (range [1, 4]). Fifteen patients (62.5%) had surgical treatment on day 15.0 (IQR=23.0) of hospitalization. The overall mortality rate was 20.8%, and the PAF of mortality for lack of surgical treatment during hospitalization was 0.08. Conclusion Eight percent of deaths among pediatric aSAH patients who do not receive surgical treatment are attributable to lack of access to surgical treatment. Health systems strengthening policies should be implemented to address this health inequity.

Paediatric Spinal Tumours: Profile and Treatment Outcome in A Nigerian Tertiary Institution

Introduction Paediatric spinal tumours are rare, accounting for 1-10% of all childhood central nervous system tumours. There is a paucity of information on spinal tumours in Sub-Saharan Africans. This is particularly so in the Nigerian paediatric population where neuro-oncologic data is limited. Indeed, there is no previously published work on the profile of paediatric spinal tumours in the Nigerian neurosurgical literature. The authors aim to document the profile of paediatric spinal tumours in a Nigerian tertiary institution and to contribute to the available data on paediatric central nervous system tumours in West Africa. Methods We retrospectively evaluated data on paediatric patients who underwent surgery for spine tumours over a 20-year-period at our institution. Results A total of 12 patients were managed for paediatric spine tumours during the study period. These had a male: female ratio of 1:1.4 and their ages ranged between 3 and 18 years (mean: 12.83 ± 4.75 years). The highest incidence (6/12) was seen in the 15-18 years age group. The mean duration of symptoms was 10.2 months. More than half (7/12) of the patients presented with symptoms with duration of at least 6 months. Motor deficit was present in all patients at presentation. Two-thirds of the tumours were in the thoracic region, 1 was located in the cervical region, while the remaining 3 tumours were cervicothoracic. The tumour was extradural in location in 8 of our patients and intramedullary in the remaining 4. Astrocytoma (intramedullary in all cases) was the most predominant histological tumour type in (3/12) in our series. Postoperative neurological improvement occurred in 7 of the patients while 5 remained the same. There was no permanent postoperative neurological deterioration or perioperative mortality. Conclusion Paediatric spinal tumours mostly affected older children in our study group and were predominantly astrocytic in nature. The most common tumour location was extradural, involving mostly the thoracic spinal level. The preoperative neurological status correlates with the postoperative functional outcome. Therefore, the need for early diagnosis and treatment of these tumours cannot overemphasized.

Factors that Influence Intraoperative Decision-Making Among Pediatric Neurosurgeons: A Grounded Theory Study

Introduction: Pediatric neurosurgery is a sub-specialty of medicine that is responsible for diagnosing, managing, and treating neurological disease in children with the use of surgery. Good intraoperative decisions making is critical to ensuring patient safety, yet almost nothing is known about what factors play a role in intraoperative decisions. As such, the purpose of this paper was to explore the factors that influence intraoperative decisions when pediatric neurosurgeons encounter something unexpected or uncertain during surgery. Methods: The study utilized the Grounded Theory method of data collection and analysis. Twenty-six pediatric neurosurgeons from 12 countries around the world were interviewed between June-October 2020 about the factors that go into making intraoperative decisions. Data was analyzed line by line and constant comparison was used to examine relationships within, and across codes and categories. Results: Pediatric neurosurgeons reflected on six factors while operating in order to come to a decision about how to proceed when they encountered an uncertainty or complication. The study findings resulted in a conceptual model that describes how concrete data including biological and technological factors and contextual data including emotional/relational factors, surgeon factors, and cultural factors influence risk assessment when making an intraoperative decision during surgery. Conclusions: The findings from this research can be used for training and educating surgeons about intraoperative decision-making processes. Pedagogical modules can be developed that includes training sessions on factors that may implicitly and explicitly influence thinking processes during an operation. Surgeons may also benefit from having open discussions with surgical colleagues about the rich, emotional, intellectual scope of the work that they do with all the challenges that these relationships can bring into decision-making in the operating room.

Management of Lambdoid Craniosynostosis: A Comprehensive and Systematic Review

<b><i>Background:</i></b> Craniosynostosis is a condition characterized by the premature fusion of 2 or more skull bones. Craniosynostosis of the lambdoid suture is one of the rarest forms, accounting for 1–4% of all craniosynostoses. Documented cases are separated into simple (single suture), complex (bilateral), and associated with adjacent synostoses (“Mercedes Benz” Pattern) or syndromes (i.e., Crouzon, Sathre-Chotzen, Antley-Bixler). This condition can manifest phenotypic deformities and neurological sequelae that can lead to impaired cognitive function if improperly treated or left undiagnosed. Preferred surgical techniques have varied over time but all maintain the common goals of establishing proper head shape and preventing of complications that could contribute to aforementioned sequelae. <b><i>Summary:</i></b> This comprehensive review highlights demographic distributions, embryological development, pathogenesis, clinical presentation, neurological sequelae, radiologic findings, surgical techniques, surgical outcomes, and postoperative considerations of patients with lambdoid craniosynostosis presentation. In addition, a systematic review was conducted to explore the operative management of lambdoid craniosynostosis using PubMed, Embase, and Scopus databases, with 38 articles included after screening. <b><i>Key Messages:</i></b> Due to a low volume of published cases, diagnosis and treatment can vary. Large overlap in presentation can occur in patients that display lambdoid craniosynostosis and posterior plagiocephaly, furthering the need for comprehensive analysis. Possessing the knowledge and tools to properly assess patients with lambdoid craniosynostosis will allow for more precise care and improved outcomes.