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Background: Headaches are the most common complaints among pediatric populations. Determining the cause and appropriate treatment for headaches may be challenging and costly, and the impact of headaches on the lives of patients and their families is not well understood., Objective: A systematic literature review was conducted to examine what PROMs are currently used, and to identify quality of life (QoL) concepts important to children suffering from headaches and any known determinants of QoL., Methods: Embase, Medline, Web of Science, CINAHL, EBSCOhost, PsychINFO, Cochrane CENTRAL and Google Scholar were searched from their inception through to June 2021. Studies investigating QoL, using a validated outcome measure in pediatric patients with headaches, were included. Relevant studies were identified through title and abstract screening and full text review by two independent reviewers. A citation review of included studies was performed. QoL concepts were extracted from the outcome measures that were used in each study to develop a preliminary conceptual model of QoL in children suffering from headaches. Determinants of QoL were also identified and categorized., Results: A total of 5421 studies were identified in the search. Title and abstract screening resulted in the exclusion of 5006 studies. Among the 415 studies included for full text review, 56 were eligible for final analysis. A citation review resulted in the addition of five studies. Most studies were conducted in high-income countries and included a patient-sample accordingly (n = 45 studies). Sixteen different PROMs were identified in the included studies, of which the PedsQL was used the most often (n = 38 studies). The most common health concepts reported were physical functioning (n = 113 items), social and psychological wellbeing (N = 117, n = 91 resp.). Twenty-five unique determinants of QoL were extracted from the included studies., Conclusion: There is a need for a condition-specific PROM to facilitate the measurement of QoL outcomes in the pediatric headache population. A conceptual model was developed based on the findings from the health concepts. Findings from this review could be used for future qualitative interviews with pediatric patients with headaches to elicit and refine important QoL concepts., (© 2023. Springer-Verlag Italia S.r.l., part of Springer Nature.)

Introduction: Neurofibromatosis Type 1 (NF-1) is a genetic disorder that occurs in 1:2500-3000 live births and may involve multiple organs. An uncommon but well-known phenomena is sphenoid dysplasia, which appears in 3-11% of the patients, and may result in significant lowering of the orbit. Understanding the cause and development of this process might predict its course and its treatment., Methods: The records of 29 patients with PNF in the head and neck region were searched for location of the PNF and type of surgery. Photographs were studied for facial disturbances, including vertical dystopia, hypertelorism, ptosis, exophthalmos and enophthalmos. MR and CT scans were studied for the presence of PNF, aspect of the sphenoid bone, alteration in the CSF circulation, and other tumour involvement., Results: Fourteen of 29 patients with PNF in the head and neck presented with sphenoid dysplasia. All 14 patients had their PNF located in the periorbital region. Increased locoregional CSF collections were seen in almost all of these patients. Besides glioma of the optical nerve or hamartomas in the cerebrum and cerebellum, there were no other tumours found. Surgical treatment varies from simple excision to excessive debulking of the PNF, reconstruction of the orbit and even enucleation., Conclusion: Although sphenoid dysplasia is uncommon in NF-1 patients, early recognition is important to prevent facial disfigurement and vision loss. Clinicians should be aware that abnormalities in the periorbital region and especially vertical dystopia might be the first sign of sphenoid dysplasia. Performing a CT or black-bone MR scan would be the next step to confirm or exclude sphenoid dysplasia and abnormal CSF dynamics., Competing Interests: Declaration of competing interest None., (© 2022 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.)

Introduction: Apert syndrome is a rare syndrome characterized by a consistent phenotype including bilateral coronal suture synostosis with an enlarged anterior fontanel, midface hypoplasia, and complex symmetric syndactyly of hands and feet., Case Report: We present a boy with Apert syndrome caused by the pathogenic c.755C > G p.Ser252Trp mutation in the FGFR2 gene with atypical characteristics, including premature fusion of the metopic suture with a small anterior fontanel, hypotelorism, and a massive posterior fontanel. Directly after birth, he showed papilledema, epilepsy, and central apneas., Conclusion: We present a newborn with Apert syndrome with atypical craniofacial presentation.

Introduction: Although meningiomas are frequently diagnosed in adults, it is a rare (intracranial) tumor in the pediatric population, with an incidence of 0.06/100,000. The pathology and treatment of meningiomas in adulthood has been a topic of increasing investigation. So far, the treatment of pediatric meningiomas has been extrapolated from these results. The question remains, however, whether translation of adult meningioma data into the childhood population is legitimate., Methods: We present the case of a 3-year-old girl diagnosed with an intraventricular malignant meningioma and type 2 neurofibromatosis. She was operated on multiple times to achieve complete resection and received adjuvant chemotherapy. Since, she has been stable with no neurological sequelae and/or recurrence of the meningioma., Conclusion: Pediatric meningiomas are rare tumors and differ from their adult counterparts in various aspects. We believe that gross total resection of meningioma in the pediatric population, when possible, is the treatment of choice. In the event of a subtotal resection, repeat resection is recommended. Any adjuvant treatment with chemotherapy or radiation therapy should be carefully considered during multidisciplinary meetings.

Object: Following shunt placement for treatment of normal-pressure hydrocephalus (NPH), several patients suffered hearing loss. The authors undertook a study to analyze this outcome., Methods: Sixteen patients in whom NPH was diagnosed were treated by placement of a ventriculoperitoneal shunt. Their hearing was assessed pre- and postoperatively by using pure tone audiometry. Two thirds of the ears tested showed a postoperative hearing loss of more than 10 dB. Recovery of the hearing loss occurred 6 to 12 weeks after shunt placement in 75% of the ears examined., Conclusions: Although shunt insertion for treatment of NPH results in a decrease in hearing, most of the loss can be recovered.