Presentation and outcomes of paediatric craniopharyngioma in the west of Scotland: a 25 year experience.

PURPOSE: Craniopharyngiomas can be aggressive leading to significant complications and morbidity. It is not clear whether there are any predictive factors for incidence or outcomes. Our aim was therefore to record the incidence, presentation, characteristics and progression of paediatric craniopharyngiomas in the West of Scotland. METHOD: Retrospective case note review for children diagnosed with paediatric craniopharyngiomas at the Royal Hospital for Children Glasgow, from 1995 to 2021 was conducted. All analyses were conducted using GraphPad Prism 9.4.0. RESULTS: Of 21 patients diagnosed with craniopharyngiomas, the most common presenting symptoms were headaches (17/21, 81%); visual impairment (13/21, 62%); vomiting (9/21, 43%) and growth failure (7/21, 33%). Seventeen (81%) patients underwent hydrocephalus and/or resection surgery within 3 months of diagnosis, usually within the first 2 weeks (13/21, 62%). Subtotal resection surgeries were performed in 71% of patients, and median time between subsequent resection surgeries for tumour recurrence was 4 years (0,11). BMI SDS increased at 5 year follow-up (p = 0.021) with 43% being obese (BMI > + 2SD). More patients acquired hypopituitarism post-operatively (14/16, 88%) compared to pre-operatively (4/15, 27%). A greater incidence of craniopharyngiomas were reported in more affluent areas (10/21, 48%) (SIMD score 8-10) compared to more deprived areas (6/10, 29%) (SIMD score 1-3). Five patients (24%) died with a median time between diagnosis and death of 9 years (6,13). CONCLUSION: Over 25 years the management of craniopharyngioma has changed substantially. Co-morbidities such as obesity are difficult to manage post-operatively and mortality risk can be up to 25% according to our cohort.

Use of Direct Intracranial Pressure and Brain Tissue Oxygen Monitoring in Perioperative Management of Patients with Moyamoya Disease.

Intracranial pressure monitoring and brain tissue oxygen monitoring are commonly used in head injury for goal-directed therapies, but there may be more indications for its use. Moyamoya disease involves progressive stenosis of the arterial circulation and formation of collateral vessels that are at risk of hemorrhage. The risk of ischemic events during revascularization surgery and postoperatively is high. Impaired cerebral autoregulation may be one of the factors that are implicated. We present our experience with monitoring of cerebral oxygenation and autoregulation in the pathological hemisphere during the perioperative period in four patients with moyamoya disease.

Telemetric Intracranial Pressure: A Snapshot Does not Give the Full Story.

Telemetric intracranial pressure (ICP) monitors are useful tools in the management of complex hydrocephalus and idiopathic intracranial hypertension (IIH). Clinicians may use them as a "snapshot" screening tool to assess shunt function or ICP. We compared "snapshot" telemetric ICP recordings with extended, in-patient periods of monitoring to determine whether this practice is safe and useful for clinical decision making.

Morphometric Analysis of the Posterior Cranial Fossa in Syndromic and Nonsyndromic Craniosynostosis.

Posterior cranial fossa (PCF) anatomy can be abnormal in craniosynostosis, and hindbrain herniation may occur. This study analyzed PCF anatomy in single suture and complex craniosynostosis.Children with craniosynostosis and age-matched controls were identified. Cephalic index (CI) for cranial vault and PCF as well as tentorial (TA) and occipital angles (OA) were measured on preoperative imaging.Children with syndromic (N = 6), bicoronal (N = 4), sagittal (N = 12), and metopic synostosis (N = 4) as well as controls (N = 10) were enrolled. Mean CI for cranial vault was 0.89, 0.93, 0.65, 0.74, and 0.78, respectively. Corresponding CI for PCF was 0.81, 0.93, 0.62, 0.74, and 0.78. Mean TA and OA were 45.4° and 96.6° in syndromic, 39.7° and 87.0° in bicoronal, 34.0 and 75.0° in sagittal, 39.7° and 87.0° in metopic synostosis, and 42.9° and 88.3° in controls.While CI, TA, and OA in metopic synostosis were similar to controls, abnormalities were found in syndromic, bicoronal, and sagittal synostosis. Syndromic and bicoronal craniosynostosis patients had a higher CI for both cranial vault and PFC as well as larger TA and OA, indicating a brachycephalic skull with steep tentorium and narrow PCF. In sagittal synostosis, CI for cranial vault and PCF were lower and TA and OA smaller, reflecting scaphocephalic deformity also at PCF, with a flat tentorium. This study provides basic PCF morphometry in craniofacial conditions.

Decompressive craniectomy for malignant middle cerebral artery infarction in a 16-year old boy: a case report.

BACKGROUND: Cryptogenic stroke frequently occurs in younger patients and has a high risk of recurrence. Consequently, secondary prevention is often suboptimal as there is no known risk factor to target. This case demonstrates an unexpected finding of middle cerebral artery infarction and extensive malignant transformation in a 16-year-old boy more than a day post-admission. The lack of a proven culprit lesion makes this case even more intriguing and subsequently raises questions of cryptogenic mechanisms in the context of unrelated trauma. CASE PRESENTATION: A 16-year-old white boy had been stabbed in his chest but had a Glasgow Coma Scale score of 15. Over a day later he developed sudden signs and symptoms consistent with a neurological event of unknown etiology. Computed tomography demonstrated significant cerebral edema but was equivocal in its list of differentials. A computed tomography scan of his chest demonstrated no cardiac wall or vascular injury and he was transferred to our neurosurgical unit for intracranial pressure monitoring. A computed tomography angiogram revealed an unexpected finding of malignant middle cerebral artery infarction. Failure to medically manage his intracranial pressure resulted in a decompressive craniectomy less than 12-hours postictus. Despite extensive diagnostic investigations no culprit lesion was identified and no patent foramen ovale found. Since discharge he has returned to full functional status. He was the youngest patient (mean age of 43 years) out of a 10-year institutional retrospective on decompressive craniectomies for malignant middle cerebral artery infarction (n = 40) and had the singularly best Glasgow Outcome Scale score of 5. CONCLUSIONS: This case highlights the preponderance of cryptogenic stroke in younger patients and its etiological elusiveness. It further demonstrates that age is predictive in terms of survival and functional outcome in the context of malignant middle cerebral artery infarction.

Telemetric Intracranial Pressure Monitoring in Syndromic Craniosynostosis.

Syndromic and nonsyndromic craniosynostosis can be associated with raised intracranial pressure (ICP). After corrective surgery, raised ICP persists or reoccurs in a subgroup of patients. The standard procedure for monitoring ICP is insertion of a percutaneous intraparenchymal probe for a limited time, usually 24 to 48 hours. However, in selected patients repeated ICP measurements might be useful in the clinical setting, and a noninvasive method for achieving this would be desirable. The authors present the use of a telemetric ICP monitoring system implanted during cranial vault expansion in a child with syndromic craniosynostosis. This system, once implanted, allows for noninvasive transdermal ICP readings and might represent a promising alternative to conventional ICP monitoring devices in selected patients with craniofacial conditions.

Melorheostosis causing lumbar radiculopathy: a case report and a review of the literature.

BACKGROUND CONTEXT: Melorheostosis is a rare sclerosing bone disorder with a predilection for the appendicular skeleton. Involvement of the spine is infrequent and largely asymptomatic. Surgical treatment for spinal involvement is therefore uncommon with only one reported case of lumbar fusion for painful lumbosacral melorheostosis. PURPOSE: We report a case of lumbar melorheostosis causing disabling radiculopathy treated with nerve root decompression. CONCLUSIONS: Melorheostosis of the lumbar spine causing radicular symptoms has not been reported before. Our message from the management of this particular patient is to consider surgical option in symptomatic individuals.

Electromagnetic-guided neuroendoscopy in the pediatric population.

OBJECT: Image-guided neuroendoscopy is being increasingly used in an attempt to reduce the morbidity associated with surgery and to make navigation easier. It has a particularly useful application in the pediatric population for the treatment of conditions such as complex hydrocephalus and arachnoid cysts. However, its use has been limited by the requirement for rigid head fixation, which may be difficult in infants because of the immaturity of the skull. In addition there can be line-of-sight issues, which can be a problem with optical-based systems. Electromagnetic navigation has eliminated the requirement for head immobilization, and its successful use in the infant population has been reported. The authors present their series to date, define its role, and discuss its advantages over other forms of image-guided navigation. METHODS: The authors used the electromagnetic StealthStation and software (Medtronic) for neuronavigation. A dynamic reference frame was attached to the head using an adhesive dressing. The patient was positioned without rigid fixation and was registered using a specially designed stylet. Navigation was through a stylet, which could be placed within the endoscope. Direct advantages were no rigid head fixation, the ability to maneuver the endoscope without the requirement for a bulky optical attachment, and no loss of navigation caused by user obstruction of reflective fiducial markers. The authors performed a total of 28 procedures in 23 patients. There were 9 arachnoid cyst marsupializations, 4 multiple fenestrations for multiloculated hydrocephalus, 4 aqueductal stenting procedures for encysted fourth ventricles, 5 endoscopic third ventriculostomies, 3 septum pellucidotomies, 2 tumor biopsies, and 1 tumor cyst decompression. RESULTS: Electromagnetic navigation was successful in all cases. Two complications were reported: a subdural collection, requiring bur hole drainage after a successful fenestration of the arachnoid cyst and failed treatment of complex hydrocephalus requiring subsequent placement of a ventriculoperitoneal shunt. CONCLUSIONS: The electromagnetic technology provides reliable image-guided endoscopy. It has several advantages over alternative forms of stereotaxy, and the ability to use it without the need for rigid head fixation makes it eminently suitable for the pediatric population. Its use and application in the treatment of a variety of different conditions has been demonstrated successfully.

Perimesencephalic nonaneurysmal subarachnoid hemorrhage caused by jugular venous occlusion: case report.

OBJECTIVE: The cause of perimesencephalic nonaneurysmal subarachnoid hemorrhage remains unknown. We describe a patient in whom jugular venous occlusion preceded the occurrence of perimesencephalic nonaneurysmal subarachnoid hemorrhage. This finding supports the theory that the source of the hemorrhage is venous in origin. CLINICAL PRESENTATION: A 25-year-old man presented with sudden onset of headache after his head was held in a headlock during a playful fight 48 hours before the ictus. His computed tomographic (CT) scan on admission demonstrated a perimesencephalic pattern of subarachnoid hemorrhage. CT angiography excluded the presence of an underlying aneurysm or vascular malformation but showed bilateral jugular venous obstruction with hematoma surrounding the right internal jugular vein. Magnetic resonance imaging and a 4-vessel cerebral angiogram confirmed the CT angiographic findings. INTERVENTION: The patient was observed as an inpatient and had no complication of his hemorrhage. Follow-up at 5 months with CT angiography showed resolution of his neck hematoma and reopening of his internal jugular veins. CONCLUSION: The presence of acute jugular venous occlusion as a cause of perimesencephalic nonaneurysmal subarachnoid hemorrhage supports a venous origin of hemorrhage.

The use of noninvasive electromagnetic neuronavigation for slit ventricle syndrome and complex hydrocephalus in a pediatric population.

OBJECT: The aim of this study was to prospectively evaluate the use of noninvasive electromagnetic neuronavigation in children, in particular its use in complex hydrocephalus and slit ventricle syndrome. METHODS: Prospective data was collected from all pediatric patients undergoing insertion of ventriculoperitoneal shunts using electromagnetic frameless neuronavigation from January 2006 to November 2007. RESULTS: Twenty-three patients fulfilled the study criteria. All ventricles were cannulated on the first pass. There were no immediate or early postprocedural complications. All but 1 patient had resolution of symptoms (mainly chronic headache) on follow-up (median 7 months, range 1-17 months). The proximal revision rate was 9% (2 of 23 patients). One patient required distal catheter revision. Infection occurred in 1 patient. CONCLUSIONS: Electromagnetic neuronavigation using a frameless and pinless system is especially suited for pediatric patients. The authors hypothesize that successful placement of ventricular catheters will reduce morbidity and improve shunt longevity.

Jugular foramen schwannoma in a child treated with complete surgical excision.

INTRODUCTION: A rare case of a large jugular foramen tumour presenting as a posterior fossa mass is described. MATERIALS AND METHODS: The patient, a 13-year-old boy presented with symptoms of raised intracranial pressure and on MRI imaging demonstrated the tumour origin within the jugular foramen, with appearances suggestive of schwannoma. He underwent a complete excision as a single-stage procedure thorough a lateral suboccipital craniotomy with minimal morbidity. Histology confirmed the diagnosis of schwannoma (WHO I). Jugular foramen schwannoma is virtually unheard of in the paediatric population. CONCLUSION: The differential diagnosis of a jugular foramen tumour includes tumours of the glomus jugulare, meningiomas, schwannomas, myxomas and chondrosarcomas. The imaging characteristics are important when considering this differential diagnosis.