The MBS is a useful adjunct in spinal surgery with particular value in revision cases where scar tissue distorts the normal anatomy. There was a low complication rate with a trend to reduced blood loss. This was most apparent to the senior authors during cervical and thoracic corpectomies.

Cerebral aspergillosis, is an infrequent, opportunistic infection of the central nervous system that accounts for 5-10% of all intracranial fungal pathology. It is uncommon in immunocompetent patients and has a significant disease burden, with high morbidity and mortality, even with appropriate treatment. Basic principles of abscess management should be employed, including aspiration and targeted anti-fungal therapy for 12-18 months. However, reported outcomes with a purely minimally invasive approach are poor and there should be a low threshold for surgical excision, especially in resource poor settings and in patients with deteriorating neurology harbouring sizeable masses. Evidence favouring gross total excision over subtotal resection is lacking, however. It is notable that these recommendations are largely based on retrospective case series and isolated case reports. There is a need therefore for international collaboration to evaluate management strategies for immunocompetent patients with cerebral aspergillosis.

No abstract

Heterozygous activating variants in platelet‐derived growth factor, beta (PDGFRB) are associated with phenotypes including Kosaki overgrowth syndrome (KOGS), Penttinen syndrome and infantile myofibromatosis (IM). Here, we present three new cases of KOGS, including a patient with a novel de novo variant c.1477A > T p.(Ser493Cys), and the oldest known individual age 53 years. The KOGS phenotype includes characteristic facial features, tall stature, scoliosis, hyperelastic thin skin, lipodystrophy, variable intellectual and neurological deterioration, and abnormalities on brain imaging. Long‐term outcome is unknown. Our cases confirm the phenotypic spectrum includes progressive flexion contractures, camptodactyly, widely spaced teeth, and constriction rings. We also propose novel occasional features including craniosynostosis, ocular pterygia, anterior chamber cleavage syndrome, early osteoporosis, increased pigmentation, recurrent haematomas, predisposition to cellulitis, nail dystrophy, carpal tunnel syndrome, recurrent hypoglycaemia in infancy, joint dislocation, and splenomegaly. Importantly, we report fusiform aneurysm of the basilar artery in two patients. Complications include thrombosis and stroke in the oldest reported patient and fatal rupture at the age of 21 in the patient with the novel variant. We conclude that cerebrovascular complications are part of the phenotypic spectrum of KOGS and KOGS‐like disorders and suggest vascular imaging is indicated in these patients.

No abstract

Introduction: Deep brain stimulation (DBS) markedly improves motor function in advanced Parkinson’s disease (PD), but its effect on sleep is less clear. Patients and methods: Forty PD patients who had subthalamic DBS (STN-DBS) were identified from an on-going non-motor naturalistic longitudinal study (NILS). All patients were followed up for at least 6 months, 26 patients had a 1 year follow-up. A total PDSS score of 100 or less, a score in any PDSS-item of 6 or less, and a Epworth score of 10 or more were classified as being significant. Results: Forty-five percent of patients reported significant improvement in the total PDSS score at 6 months, and 35% at 12 months. In terms of magnitude, the total PDSS score at 6 months was significantly improved from baseline while the improvement at 12 months was not statistically significant. The most frequently reported improvements were overall sleep quality and maintenance of sleep. Some patients reported worsening of the total PDSS score. More than half of the patients reporting daytime sleepiness at baseline had persistent sleepiness at 6 and 12 months. The mean Epworth Score did not improve because a significant number of patients without sleepiness at baseline reported new-onset sleepiness at 6 and 12 months. Neither medication changes nor motor improvement were consistently related to sleep changes after DBS. Conclusion: Subthalamic DBS is associated with a statistically and clinically significant, but variable, improvement in sleep as measured by the PDSS. The most frequent improvements were better overall sleep quality and better sleep maintenance.

Background: Isolated sagittal synostosis is the most common form of nonsyndromic craniosynostosis. The surgical management of this condition ranges from strip craniectomy to total cranial vault remodeling. In our unit, passive correction of sagittal synostosis utilizing open extended midline strip craniectomy with bilateral micro-barrel staving is performed before 22 weeks corrected age. The aim of this prospective study is to identify the effectiveness and morbidity associated with this technique. Patients and Methods: Nonsyndromic patients who underwent OMEC at a tertiaryreferral-center (Alder Hey Children's Hospital, Liverpool, UK) over a 3-year period (2014–2016) were included. In particular, we looked at pre- and postoperative cranial index (CI), as well as other secondary outcomes such as rates of transfusion and reoperation. Results: A total of 44 patients (12 female, 32 male) met the inclusion criteria. Median age at operation was 132 (range 99–171) days. Median operative time was 117 (range 89–171) minutes. Twenty-four patients underwent a blood transfusion. Median pre-op CI was 66 (range 61–74.7)%. The first post-op CI, taken a median of 56 (range 12–107) days postoperatively, was 78 (range 73–87)%. No patients required reoperation. There were no perioperative deaths. Conclusion: Early open extended midline strip craniectomy with bilateral micro-barrel staving for correction of isolated nonsyndromic sagittal synostosis is a safe and effective technique, associated with minimal morbidity, producing sustained satisfactory head shape morphology on short to medium-term follow-up.

Unilateral synostotic frontal plagiocephaly is most commonly due to a premature fusion of the frontoparietal suture. However, the coronal ring comprises of major and minor sutures and these sutures in isolation or in combination can result in similar clinical presentations which can make diagnosis challenging and result in a delay in referral to a craniofacial surgeon for timely management. Isolated frontosphenoidal craniosynostosis is a rare clinical entity with only 49 cases reported in the English literature to date. The authors present our series of 4 patients to add to this cohort of patients and describe key characteristics to distinguish frontoparietal from isolated frontosphenoidal synostosis and introduce a means of differentiating these 2 diagnoses from posterior deformational plagiocephaly and unilateral lambdoid synostosis. All previous case reports have been diagnosed after radiological imaging but the authors have devised a novel algorithm to aid the clinician in diagnosis of craniosynostosis before any radiological imaging.