A Photo Score for Aesthetic Outcome in Sagittal Synostosis:An ERN CRANIO Collaboration

European Reference Network (ERN) CRANIO is focused on optimizing care for patients with rare or complex craniofacial anomalies, including craniosynostosis and/or rare ear, nose, and throat disorders. The main goal of ERN CRANIO is to collect uniform data on treatment outcomes for multicenter comparison. We aimed to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of sagittal synostosis and aesthetic outcome of treatment. We conducted a retrospective study among nonsyndromic sagittal synostosis patients aged &lt;19 years. We included preoperative and postoperative photo sets from 6 ERN CRANIO centers. Photo sets included bird's eye, lateral, and anterior-posterior views. The sagittal synostosis photo score was discussed in the working group, and consensus was obtained on its contents. Interrater agreement was assessed with weighted Fleiss' Kappa and intraclass correlation coefficients.The photo score consisted of frontal bossing, elongated skull, biparietal narrowness, temporal hollowing, vertex line depression, occipital bullet, and overall phenotype. Each item was scored as normal, mild, moderate, or severe. Results from 36 scaphocephaly patients scored by 20 raters showed kappa values ranging from 0.38 [95% bootstrap CI: 0.31, 0.45] for biparietal narrowness to 0.56 [95% bootstrap CI: 0.47, 0.64] for frontal bossing. Agreement was highest for the sum score of individual items [intraclass correlation coefficients agreement 0.69 [95% CI: 0.57, 0.82]. This is the first large-scale multicenter study in which experts investigated a photo score to assess the severity of sagittal synostosis phenotypical characteristics. Agreement on phenotypical characteristics was suboptimal (fair-moderate agreement) and highest for the summed score of individual photo score items (substantial agreement), indicating that although experts interpret phenotypical characteristics differently, there is consensus on overall phenotypical severity.</p

Pediatric colloid cysts: a multinational, multicenter study. An IFNE-ISPN-ESPN collaboration

OBJECTIVE Colloid cysts (CCs) are rare at all ages, and particularly among children. The current literature on pediatric CC is limited, and often included in mixed adult/pediatric series. The goal of this multinational, multicenter study was to combine forces among centers and investigate the clinical course of pediatric CCs. METHODS A multinational, multicenter retrospective study was performed to attain a large sample size, focusing on CC diagnosis in patients younger than 18 years of age. Collected data included clinical presentation, radiological characteristics, treatment, and outcome. RESULTS One hundred thirty-four children with CCs were included. Patient age at diagnosis ranged from 2.4 to 18 years (mean 12.8 ± 3.4 years, median 13.2 years, interquartile range 10.3–15.4 years; 22% were \u3c 10 years of age). Twenty-two cases (16%) were diagnosed incidentally, including 48% of those younger than 10 years of age. Most of the other patients had symptoms related to increased intracranial pressure and hydrocephalus. The average follow-up duration for the entire group was 49.5 ± 45.8 months. Fifty-nine patients were initially followed, of whom 28 were eventually operated on at a mean of 19 ± 32 months later due to cyst growth, increasing hydrocephalus, and/or new symptoms. There was a clear correlation between larger cysts and symptomatology, acuteness of symptoms, hydrocephalus, and need for surgery. Older age was also associated with the need for surgery. One hundred three children (77%) underwent cyst resection, 60% using a purely endoscopic approach. There was 1 death related to acute hydrocephalus at presentation. Ten percent of operated patients had some form of complication, and 7.7% of operated cases required a shunt at some point during follow-up. Functional outcome was good; however, the need for immediate surgery was associated with educational limitations. Twenty operated cases (20%) experienced a recurrence of their CC at a mean of 38 ± 46 months after the primary surgery. The CC recurrence rate was 24% following endoscopic resection and 15% following open resections (p = 0.28). CONCLUSIONS CCs may present in all pediatric age groups, although most that are symptomatic present after the age of 10 years. Incidentally discovered cysts should be closely followed, as many may grow, leading to hydrocephalus and other new symptoms. Presentation of CC may be acute and may cause life-threatening conditions related to hydrocephalus, necessitating urgent treatment. The outcome of treated children with CCs is favorable

Disección aguda de aorta

La disección de aorta es un emergencia vital asociada a una tasa elevada de morbilidad y mortalidad. El diagnóstico de la disección de aorta es difícil y frecuentemente confundido. Objetivos: Evaluar la presentación clínica, manejo y resultados de la disección de aorta en el servicio de urgencias de un hospital de tercer nivel. Métodos: Se realizó un estudio descriptivo observacional y retrospectivo, analizando 46 pacientes que ingresaron en el servicio de urgencias del Hospital Clínico San Carlos, con el diagnóstico primario de disección de aorta, durante el periodo de enero de 2000 a enero de 2003. Participantes: Un total de 46 pacientes (64 años de media, 76% varones), 63% de los cuales presentaron disección tipo A. Datos analizados: Síntomas de presentación, signos, pruebas realizadas, tratamiento y mortalidad. Resultados: La presentación clínica fue diversa, pero la mayoría de los pacientes con disección de aorta presentaron dolor punzante de comienzo brusco. Los hallazgos físicos clásicos, como el déficit de pulso, sólo se registró en un 19,5%. En la exploración, el 24% de los pacientes tenía tensión arterial (TA) elevada, el 26% soplo diastólico y solamente el 2% presentaba déficit neurológico focal. Los resultados de la radiografía de tórax fueron frecuentemente anormales. En el electrocardiograma no se encontraron alteraciones en el 60% de los pacientes. La Tomografía Computarizada fue la prueba de imagen inicial en el 56,5%. La mortalidad intrahospitalaria supuso un 28,2%. La mortalidad de los pacientes con disección tipo A tratados quirúrgicamente fue un 38,4%; entre los que no recibieron tratamiento quirúrgico (generalmente por edad avanzada y comorbilidad) la mortalidad fue del 61,5%. Conclusiones: La disección de aorta es poco frecuente, pero las complicaciones se desarrollan rápidamente y el desenlace es con frecuencia fatal. Se presenta con un amplio rango de manifestaciones y los hallazgos clásicos muchas veces no se encuentran