Fatal postoperative tension pneumocephalus after acute subdural hematoma evacuation: a case report.

PURPOSE: Tension pneumocephalus (TP) represents a rare pathology characterized by constant accumulation of air in the intracranial space, being associated with increased risk of herniation, neurologic deterioration and death. Regarding neurosurgical trauma cases, TP is majorly encountered after chronic subdural hematoma evacuation. In this case report, we present a rare case of fatal postoperative TP encountered after craniotomy for evacuation of acute subdural hematoma (aSDH). CASE PRESENTATION: An 83-year old gentleman was presented to the emergency department of our hospital with impaired level of consciousness. Initial examination revealed Glascow Coma Scale (GCS) 3/15, with pupils of 3 mm bilaterally and impaired pupillary light reflex. CT scan demonstrated a large left aSDH, with significant pressure phenomena and midline shift. Patient was subjected to an uneventful evacuation of hematoma via craniotomy and a closed subgaleal drain to gravity was placed. The following day and immediately after his transfer to the CT scanner, he presented with rapid neurologic deterioration with acute onset anisocoria and finally mydriasis with fixed and dilated pupils. Postoperative CT scan showed massive TP, and the patient was transferred to the operating room for urgent left decompressive craniectomy, with no intraoperative signs of entrapped air intracranially. Finally, he remained in severe clinical status, passing away on the eighth postoperative day. CONCLUSION: TP represents a rare but severe neurosurgical emergency that may be also encountered after craniotomy in the acute trauma setting. Involved practitioners should be aware of this potentially fatal complication, so that early detection and proper management are conducted.

X-linked hypophosphatemia due to a de novo novel splice-site variant in a 7-year-old girl with scaphocephaly, Chiari syndrome type I and syringomyelia.

X-linked hypophosphatemia (XLH) is a rare X-linked dominant inherited disorder caused by loss-of-function variants in the PHEX gene and characterized by renal phosphate wasting, hypophosphatemia, abnormal vitamin D metabolism, growth retardation and lower limb deformities. We describe a case of XLH-rickets in a 7-year-old girl with scaphocephaly, Chiari syndrome type I and syringomyelia, with a de novo non-canonical splice variant (c.1080-3C > G) in intron 9 of the PHEX gene, that has not been previously described.

Cerebrospinal Fluid Homeostasis and Hydrodynamics: A Review of Facts and Theories.

BACKGROUND AND PURPOSE: According to the classical hypothesis, the cerebrospinal fluid (CSF) is actively secreted inside the brain's ventricular system, predominantly by the choroid plexuses, before flowing unidirectionally in a cranio-caudal orientation toward the arachnoid granulations (AGs), where it is reabsorbed into the dural venous sinuses. This concept has been accepted as a doctrine for more than 100 years and was subjected only to minor modifications. Its inability to provide an adequate explanation to questions arising from the everyday clinical practice, in addition to the ever growing pool of experimental data contradicting it, has led to the identification of its limitations. Literature includes an increasing number of studies suggesting a more complex mechanism than that previously described. This review article summarizes the proposed mechanisms of CSF regulation, referring to the key clinical and experimental developments supporting or defying them. METHODS: A non-systematical literature search of the major databases was performed for studies on the mechanisms of CSF homeostasis. Gray literature was additionally assessed employing a hand-search technique. No restrictions were imposed regarding the time, language, or type of publication. CONCLUSION: CSF secretion and absorption are expected to take place throughout the entire brain's capillaries network under the regulation of hydrostatic and osmotic gradients. The unidirectional flow is defied, highlighting the possibility of its complete absence. The importance of AGs is brought into question, potentiating the significance of the lymphatic system as the primary site of reabsorption. However, the definition of hydrocephalus and its treatment strategies remain strongly associated with the classical hypothesis.

Paediatric spinal trauma presenting to a UK major trauma centre.

PURPOSE: Paediatric spine trauma is uncommon and is managed differently from adults due to the anatomical differences of the paediatric spine. The paediatric spine is less ossified, with lax ligaments and a higher fulcrum in the c-spine which results in a different pattern of injuries. The aim of this study is to provide a contemporary audit of paediatric spinal trauma. METHODS: A retrospective review was conducted using the Trauma and Audit Research Network database at a major trauma centre (2011-2018). All patients < 18 years old with a spine injury underwent case note and radiology review. RESULTS: A total of 72 patients (37, 51.4% male with an average age of 13.3 (± 5.9) years old) were identified. The most common mechanism of injury was road traffic collisions (n = 39, 54.2%). The most common sporting cause was motocross accidents (n = 6, 8.3%), and a further 6 (8.3%) patients had a suspected inflicted injury. Eight patients (11.1%) sustained a spinal cord injury. Twenty-seven (37.5%) patients underwent surgical intervention to treat their spinal injury. CONCLUSION: This series demonstrates the profile of injury mechanisms causing paediatric spinal injuries. Paediatric spine injuries continue to have the potential for lifelong disability and require careful, specialist management. This series also highlights certain causes such as motocross accidents and suspected inflicted injury which are more frequent than expected and raise potential public health concerns.

Ruptured intra-cranial arachnoid cysts: a case series from a single UK institution.

INTRODUCTION: Intracranial arachnoid cysts are a common incidental finding; however presentation with cyst rupture is a recognised complication. Patients are advised to avoid contact sports to reduce the risk of cyst rupture but the risk of rupture is not currently known. The aim of this study is to describe a single institution's experience of managing ruptured intra-cranial arachnoid cysts. METHOD: A retrospective case note review of all patients admitted to a single institution with a ruptured intra-cranial arachnoid cyst between 2005 and 2016 (inclusive). Medical records were reviewed for demographics, history of trauma, surgical treatment and radiological evidence of cyst rupture. RESULTS: Fourteen patients were identified for inclusion with an average age of 23.4 years (range 7-57) and 10 (71%) were male. Nine patients (64%) had a documented history of head trauma. Eleven patients (78.6%) required neurosurgical intervention which included hygroma/haematoma evacuation only (n = 5), haematoma evacuation with cyst fenestration (n = 4) and cyst fenestration/shunting only (n = 2). Twelve patients (85%) experienced full recovery and the remaining two had persisting headaches or neurological symptoms. DISCUSSION: Cyst rupture remains an uncommon cause for presentation with arachnoid cysts. However the majority are associated with head trauma and thus current advice to avoid contact sports seems justified.

Prenatal diagnosis of arachnoid cysts: a case series and systematic review.

INTRODUCTION: Primary cysts are believed to arise from the splitting of the arachnoid membrane during prenatal development and can be diagnosed in utero. Prenatal diagnosis is uncommon; therefore, the evidence base for counselling expectant mothers is limited. The purpose of this article is to present a case series and review the current literature on prenatally diagnosed arachnoid cysts. METHOD: A keyword search of hospital electronic records was performed for all patients with a prenatally diagnosed arachnoid cyst at a tertiary neurosurgical centre. Case notes were reviewed for all patients diagnosed between 2005 and 2017. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to structure a systematic review of all English language articles published up to May 2018. RESULTS: A total of eight eligible patients were identified from our own records and 123 from the literature. Sixty-eight per cent of patients had a normal outcome. Sixty-three per cent of patients underwent surgical intervention which was not associated with abnormal outcome. The diagnosis of syndromic/genetic diagnosis (p < 0.001) and the presence of other intra-cranial anatomical abnormalities (p = 0.05) were significant predictors of abnormal outcome. CONCLUSION: The pathogenesis and prognosis of a prenatal arachnoid cyst diagnosis remain unclear. These results suggest favourable outcomes from simple cysts without associated abnormalities and expectant mothers should be counselled accordingly. A wider prospective review is required to better established evidence-based practice.

Paediatric brain abscesses: a single centre experience.

Introduction: Brain parenchymal abscesses are relatively infrequent but potentially serious infections in the paediatric population. Surgical intervention in addition to a prolonged administration of antibiotics is generally appropriate management. This study presents our centre's experience of managing such patients in the context of relevant literature. Method: A single-centre retrospective case note review was conducted over a 15 year period (2003-2017). Patients were selected from electronic hospital records using ICD10 code G06.0. Patients < 18 years of age with a confirmed intra-parenchymal abscess were included. Patient records were reviewed for abscess location, microbiology results, surgical intervention, and outcome using the Glasgow Outcome Score at 3 months. Results: Twenty-four patients were identified (mean age: 7.4 ± 5.3 years, male n = 11). Twelve (50.0%) patients had an abscess in the frontal lobe and Streptococcus was the most common causative microorganism (n = 15). Nineteen patients (79.2%) had an identifiable source which included: ENT infections, congenital cardiac malformations, recent dental surgery and meningitis. All 24 patients underwent surgery with 20 patients having a total of 32 aspirations between them and the other 4 having craniotomy and excision. Twenty patients had 3 month follow-up data of which 18 patients scored GOS: 5, one was GOS: 4 and one was GOS: 3. Conclusions: Brain parenchymal abscess remains an uncommon pathology in the paediatric population. The majority of patients have a preceding infection with Streptococcus as the most common causative organism. Antimicrobial therapy should be selected accordingly. All of our patients underwent surgical intervention and received intravenous antibiotics with favourable outcome and no mortality.

Natural History of Intracranial Arachnoid Cysts.

BACKGROUND: Intracranial arachnoid cysts are a common incidental finding. Most of these remain asymptomatic with surgical intervention being reserved for those cysts causing symptoms from mass effect or hydrocephalus. It is not properly understood why cysts enlarge nor is it possible to predict in which cysts this will occur. The aim of this study is to review the natural history of incidental arachnoid cysts. METHODS: A retrospective case review was conducted of all patients diagnosed with an arachnoid cyst between 2007 and 2016. Case notes and radiology reports were reviewed for demographics, symptoms, and changes in cyst sizes. RESULTS: Four hundred and eighty-five patients were diagnosed with an arachnoid cyst during this time period; of these, 425 (87.6%) were asymptomatic. Patients with symptomatic cysts were significantly younger and more likely to have associated hydrocephalus. The most common indications for imaging which resulted in the diagnosis of the cyst were headaches (n = 106), stroke (n = 51), and seizures (n = 41). The cyst size remained stable or reduced in 147 patients (99.3%) with follow-up imaging (n = 148), and the enlarging cyst remained asymptomatic. CONCLUSIONS: Asymptomatic arachnoid cysts have a low rate of cyst enlargement on follow-up imaging, which supports not routinely imaging these patients. However, serial imaging and surgery may still be indicated in asymptomatic patients at risk of obstructive hydrocephalus which, in this series, was only seen in the pediatric population. A better understanding of the natural history of intracranial arachnoid cysts allows for more efficient follow-up planning.

Clinical and radiological outcomes following surgical treatment for intra-cranial arachnoid cysts.

OBJECTIVES: Intra-cranial arachnoid cysts are benign lesions which are usually incidental, however can produce neurological symptoms due to mass effect as they enlarge. Controversy still exists regarding the optimal option for the surgical management of these cysts. These options are neuroendoscopic fenestrations, microsurgical fenestrations +/- marsupialisation and insertion of a cysto-peritoneal shunt. PATIENTS AND METHODS: A retrospective case note review of all patients with intra-cranial arachnoid cysts treated surgically at a single UK neurosurgical centre over a 15 year period. Data on clinical presentations and outcomes was collected from the patient notes and the pre- and post-operative cyst volumes were calculated by creating 3-dimensional volumetric models. RESULTS: Eighty-two patients were identified of which 45 were treated endoscopically, 34 microscopically and 3 underwent cysto-peritoneal shunting. The most common cyst location was the middle fossa (n = 25). Amongst the symptomatic patients, improvement or resolution of symptoms was seen in 35 out of 40 cysts treated endoscopically (88%), 28 out of 32 treated microsurgically (88%) and 3 out of 3 treated by shunting (100%, p = 0.79). The reoperation rate was not significantly different between the endoscopic and microsurgical groups (24.4% vs 14.7%, p = 0.49). The endoscopic and shunted groups had a shorter length of stay than the microsurgical group (3.0 vs 3.0 vs 4.5 days, p = 0.04). All three treatment modalities had a similar percentage reduction in cyst volume after surgery (30.0 vs 41.7 vs 30.9%, p = 0.98). CONCLUSIONS: This cohort series shows that endoscopic and microsurgical approaches to treat intracranial arachnoid cysts produce comparable clinical and radiological outcomes. Endoscopic fenestration is associated with a shorter length of stay as would be expected from a minimally invasive procedure.

Clinical Significance of Venous Anomalies in Syndromic Craniosynostosis.

BACKGROUND: The pattern of cranial venous drainage in syndromic craniosynostosis is unpredictable and not adequately understood. Collateral channels substitute for stenotic venous sinuses and pose potential risk for surgical intervention. The purpose of this study was to analyze the patterns of venous drainage in patients with syndromic craniosynostosis and their influence on operative planning and morbidity. METHODS: A retrospective study of patients with syndromic craniosynostosis from 2000 to 2013 was performed. Demographic data were collected including phenotype and associated pathologies. Pre- and/or postoperative venous imaging was reviewed for venous sinus stenosis, collateral emissaries, and persistent fetal sinuses. Categorization of anomalous venous drainage was performed, and the relationship with surgical morbidity was assessed. RESULTS: Forty-one patients were identified. Anomalies were present in 31 patients (76%) consisting of dural sinus stenosis in 28 (68%), dilated emissaries in 26 (63%), and fetal sinuses in 7 (17%). Pfeiffer syndrome was most commonly associated with anomalous drainage (100%). Venous anomalies were associated with elevated intracranial pressure (ICP), shunted hydrocephalus, Chiari malformations, and sleep apnea. In 5 cases, the surgical plan was adjusted based on anomalous anatomy. No mortalities occurred. Intraoperative complication rate was 7.3%, all with anomalous drainage. Median estimated blood loss was 1,100 cc for patients with anomalies versus 400 cc without anomalies (P = 0.181). CONCLUSION: Cranial venous anomalies are commonly detected in patients with syndromic craniosynostosis and may affect surgical morbidity and outcome with a higher estimated blood loss, alteration of procedure, and postoperative morbidity. Detailed preoperative imaging of the venous drainage is therefore recommended in cases of syndromic synostosis.

Congenital Glioblastoma Multiforme: An Unusual and Challenging Tumor.

Congenital glioblastoma multiforme is a rare tumor of the central nervous system with unique features. The existing evidence on its pathogenesis, genetic and molecular profile, special characteristics, treatment, and prognosis is reviewed. An increased number of antenatal diagnoses and prolonged survival for those individuals who can tolerate combined surgical resection and chemotherapy has been noted. The overall prognosis, however, remains poor. A better understanding of this unusual entity is important. Further research is needed to discern tumor's pathogenesis and natural history. This will likely lead to the development and implementation of treatment strategies that may decrease mortality and morbidity in these patients.

Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature.

Chordomas are tumors arising from remnants of the embryological notochord, most commonly found in the spheno-occipital, spinal, or sacro-coccygeal areas. They are rare tumors in the pediatric population and are challenging to manage due to their difficult accessibility, proximity to important anatomy and extension into adjacent structures. We report a series of 10 children treated for chordoma at The Hospital for Sick Children focusing on their surgery, adjuvant therapy and long-term outcomes. A retrospective review involving patient charts, radiographic imaging, and pathology slides was performed for 10 chordoma patients during the period from 1987-2015. Important variables, including patient demographics, chordoma location, presentation, imaging characteristics, pathology subtype, treatment options, and long-term outcome were analysed. The series consists of seven girls and three boys with cranial or upper cervical spine chordomas. One patient presented with an extradural left cerebellopontine angle chordoma demonstrating aggressive and dedifferentiated features, which, to our knowledge, has not been previously described in the literature. All patients received surgical resection followed by photon or proton radiotherapy. Four patients with chondroid or atypical pathology also received chemotherapeutic adjuvants. All patients with classical pathology achieved favourable outcome, while the four patients with atypical pathology progressed quickly despite aggressive therapy, suggesting that pathology subtype is a crucial prognostic factor. This study summarizes 30years of surgical and adjuvant therapy experience in a large academic center for pediatric chordoma patients. Patient outcomes were dependent on pathology subtype, and a multidisciplinary approach involving surgery, radiotherapy, and chemotherapy can be considered on an individual basis.

Cerebellar mutism.

Mutism of cerebellar origin is a well-described clinical entity that complicates operations for posterior fossa tumors, especially in children. This review focuses on the current understanding of principal pathophysiological aspects and risk factors, epidemiology, clinical characteristics, treatment strategies, and outcome considerations. The PubMed database was searched using the term cerebellar mutism and relevant definitions to identify publications in the English-language literature. Pertinent publications were selected from the reference lists of the previously identified articles. Over the last few years an increasing number of prospective studies and reviews have provided valuable information regarding the cerebellar mutism syndrome. Importantly, the clarification of principal terminology that surrounds the wide clinical spectrum of the syndrome results in more focused research and more effective identification of this entity. In children who undergo surgery for medulloblastoma the incidence of cerebellar mutism syndrome was reported to be 24%, and significant risk factors so far are brainstem involvement and midline location of the tumor. The dentate-thalamo-cortical tracts and lesions that affect their integrity are considered significant pathophysiological issues, especially the tract that originates in the right cerebellar hemisphere. Moderate and severe forms of the cerebellar mutism syndrome are the most frequent types during the initial presentation, and the overall neurocognitive outcome is not as favorable as thought in the earlier publications. Advanced neuroimaging techniques could contribute to identification of high-risk patients preoperatively and allow for more effective surgical planning that should focus on maximal tumor resection with minimal risk to important neural structures. Properly designed multicenter trials are needed to provide stronger evidence regarding effective prevention of cerebellar mutism and the best therapeutic approaches for such patients with a combination of pharmacological agents and multidisciplinary speech and behavior augmentation.