Papilloedema: a highly specific predictor of raised intracranial pressure in a complex neurosurgical paediatric cohort.

PURPOSE: Papilloedema is recognised as an indicator of raised intracranial pressure, although there is a paucity of literature describing the utility of fundoscopy in screening for raised ICP in children with craniofacial synostosis, particularly young children. We sought to investigate the association of optic disc morphology with ICP in children, and to define the sensitivity and specificity of papilloedema as a clinical indicator of raised ICP and determine if age, or underlying conditions impact the findings. METHOD: Retrospective analysis of all patients undergoing ICP monitoring at a designated paediatric neurosurgical and craniofacial unit in the United Kingdom between October 2009 and October 2018. The fundoscopy findings and ICP monitoring data were analysed for 31 children with craniosynostosis and 29 children without craniosynostosis. RESULTS: All children who had papilloedema had raised ICP confirmed with monitoring. Across the 60-patient cohort, confirmed papilloedema on fundoscopy had Positive Predictive Value (PPV) of 1.00, Negative Predictive Value (NPV) of 0.64 with sensitivity 48% and specificity 100% for the presence of raised ICP (p = < 0.0001). In the craniosynostosis group, PPV was 1.00, NPV was 0.39, sensitivity 48% and specificity 100% (p = < 0.03). There is no correlation between severity of optic disc swelling using Frisen grading and elevation of ICP. Age did not affect the presence of papilloedema in those with raised ICP. CONCLUSION: The presence of papilloedema is a strong indicator of raised ICP in a child, regardless of underlying aetiology. Detailed fundoscopy can prevent the need for further investigations including imaging-related radiation and invasive CSF pressure monitoring.

Trampoline-Associated Cranial and Spinal Injuries: A 10-Year Study in a Pediatric Neurosurgery Center.

OBJECTIVE:  There has been an increasing use of trampolines for recreation by children in recent years. Many studies have explored the different types of injuries sustained due to falls from trampolines, but so far none have focused specifically on cranial and spinal injuries. In this study, we describe the pattern of cranial and spinal injuries sustained by pediatric patients that were associated with the use of trampolines and their management in a tertiary pediatric neurosurgery unit over a period of 10 years. METHODS:  This is a retrospective study of all children less than 16 years of age with suspected or confirmed trampoline-associated cranial or spinal injuries, managed by a tertiary pediatric neurosurgery unit from 2010 to 2020. Data collected included the patient's age at the time of injury, gender, neurological deficits, radiological findings, management, and clinical outcome. The data were analyzed to highlight any trends in the pattern of injuries. RESULTS:  A total of 44 patients with a mean age of 8 years (ranging from one year and five months to 15 years and five months) were identified. 52% patients were male. 10 patients (23%) had a reduced Glasgow Coma Scale (GCS) score. In terms of imaging findings, 19 patients (43%) had a radiologically positive head injury, nine (20%) had a craniovertebral junction (CVJ) injury, including the first (C1) and second (C2) cervical vertebrae, and six (14%) had an injury involving other parts of the spine. No patient sustained concurrent head and spinal injuries. Eight (18%) patients had normal radiological findings. Two (5%) had incidental findings on radiology that required subsequent surgery. A total of 31 patients (70%) were managed conservatively. 11 patients (25%) underwent surgery for their trauma, of which seven were cranial. Two further patients underwent surgery for their incidental intracranial diagnoses. One child died from an acute subdural hemorrhage. CONCLUSIONS:  This study is the first to focus on trampoline-associated neurosurgical trauma and report the pattern and severity of cranial and spinal injuries. Younger children (less than five years of age) are more likely to develop a head injury, whereas older children (more than 11 years of age) are more likely to develop a spinal injury following the use of a trampoline. Although uncommon, some injuries are severe and require surgical intervention. Therefore, trampolines should be used prudently with the appropriate safety precautions and measures.

Infantile Macrocephaly: Complicated Familial Benign Enlargement of Subarachnoid Space in Twins of Suspected Nonaccidental Injury.

A 3-month-old first of dizygotic male twins, born at 34 + 5 weeks gestational age, presented with a 4-week history of increasing head circumference and vomiting. He had a tense anterior fontanelle and a head circumference above the 97th percentile. Magnetic resonance imaging showed large bilateral subdural collection with hemosiderin deposits suggestive of hemorrhage. Nine days of bilateral subdural drainage reduced the collection size and blood load. On postoperative day 16, magnetic resonance imaging confirmed persistent but smaller subdural collections, unmasking the underlying subarachnoid space enlargement. On day 18, a right subdural-peritoneal valveless shunt was inserted as definitive treatment. As part of a nonaccidental injury investigation, Twin 2 was also found to have macrocephaly secondary to benign enlargement of subarachnoid space, which was managed conservatively. Benign enlargement of subarachnoid space has an assumed autosomal/multifactorial inheritance and predisposes to subdural hemorrhage. Ultimately, no safeguarding issues were raised. Both twins continued to be neurologically stable at 2-year follow-up with head circumferences between the 98th and 99th percentiles.

Atlantoaxial limited dorsal myeloschisis: A report of two cases and review of literature.

Introduction: Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism that is characterised by a distinctive fibroneural stalk connecting the spinal cord to the overlying skin lesion. The skin lesions associated with LDM can appear benign clinically and careful evaluation with an MRI scan is essential for diagnosing LDM and to differentiate this entity from other forms of spinal dysraphism and benign causes of skin lesions. Research question: There is a lack of reported atlantoaxial LDM in the literature. We sought to report the clinical presentation, radiological features and surgical management of the first two reported atlantoaxial LDM. Material and methods: Clinical findings and radiological images of the two cases of atlantoaxial LDM that underwent surgical intervention at our institution were retrieved from the medical notes, operative records and imaging system. Results: Both cases of atlantoaxial LDM (C0-1 and C1-2 respectively) underwent successful resection of the overlying cutaneous lesions and stalks to release the tethered spinal cords. Discussion: The surgical management of LDM have been associated with good outcomes and consists of resecting the fibroneural stalk close to the underlying cord, releasing the tethered spinal cord and removing the overlying cutaneous lesion. Conclusion: These are the first two reported cases of atlantoaxial LDM in the literature. We aim to raise awareness of this pathological entity and highlight the importance of establishing the correct diagnosis to guide definitive management, and report the favourable neurological outcome in these cases despite the rostral location.