RNA exosome mutations in pontocerebellar hypoplasia alter ribosome biogenesis and p53 levels.

The RNA exosome is a ubiquitously expressed complex of nine core proteins (EXOSC1-9) and associated nucleases responsible for RNA processing and degradation. Mutations in EXOSC3, EXOSC8, EXOSC9, and the exosome cofactor RBM7 cause pontocerebellar hypoplasia and motor neuronopathy. We investigated the consequences of exosome mutations on RNA metabolism and cellular survival in zebrafish and human cell models. We observed that levels of mRNAs encoding p53 and ribosome biogenesis factors are increased in zebrafish lines with homozygous mutations of exosc8 or exosc9, respectively. Consistent with higher p53 levels, mutant zebrafish have a reduced head size, smaller brain, and cerebellum caused by an increased number of apoptotic cells during development. Down-regulation of EXOSC8 and EXOSC9 in human cells leads to p53 protein stabilisation and G2/M cell cycle arrest. Increased p53 transcript levels were also observed in muscle samples from patients with EXOSC9 mutations. Our work provides explanation for the pathogenesis of exosome-related disorders and highlights the link between exosome function, ribosome biogenesis, and p53-dependent signalling. We suggest that exosome-related disorders could be classified as ribosomopathies.

Differences in nonlinear heart dynamics during rest and exercise and for different training.

OBJECTIVE: In this work we want to analyze differences in nonlinear properties between rest and exercise and also to study the permanent effects of physical exercise on heart rate dynamics. APPROACH: It has been shown that physical exercise alters heart dynamics by increasing heart rate and decreasing variability, modifying spectral power and linear correlations, etc. We hypothesize that physical exercise should also reduce nonlinearity in the heartbeat time series. To quantify nonlinearity in the heartbeat time series, we use an index of nonlinearity recently proposed by Bernaola et al based on correlations of the magnitude time series. MAIN RESULTS: Our results confirm our initial hypothesis of loss of nonlinearity during physical exercise. Moreover, regarding the permanent effects of physical exercise on heart rate dynamics, we also obtain that aerobic physical training tends to increase nonlinearity in heart dynamics during rest. SIGNIFICANCE: It is well-known that heart dynamics are controlled by complex interactions between the sympathetic and parasympathetic branches of the autonomic nervous system. Moreover, these two branches act in a competing way, resulting in a clear parasympathetic withdrawal and sympathetic activation during physical exercise. We associate these interactions during physical exercise with a drastic loss of nonlinear properties in the heartbeat time series, revealing the importance of nonlinearity measures in the study of complex systems.

Correlations in magnitude series to assess nonlinearities: Application to multifractal models and heartbeat fluctuations.

The correlation properties of the magnitude of a time series are associated with nonlinear and multifractal properties and have been applied in a great variety of fields. Here we have obtained the analytical expression of the autocorrelation of the magnitude series (C_{|x|}) of a linear Gaussian noise as a function of its autocorrelation (C_{x}). For both, models and natural signals, the deviation of C_{|x|} from its expectation in linear Gaussian noises can be used as an index of nonlinearity that can be applied to relatively short records and does not require the presence of scaling in the time series under study. In a model of artificial Gaussian multifractal signal we use this approach to analyze the relation between nonlinearity and multifractallity and show that the former implies the latter but the reverse is not true. We also apply this approach to analyze experimental data: heart-beat records during rest and moderate exercise. For each individual subject, we observe higher nonlinearities during rest. This behavior is also achieved on average for the analyzed set of 10 semiprofessional soccer players. This result agrees with the fact that other measures of complexity are dramatically reduced during exercise and can shed light on its relationship with the withdrawal of parasympathetic tone and/or the activation of sympathetic activity during physical activity.

Endovascular treatment of intracranial aneurysms using the Pipeline Flex embolization device: a case series of 30 consecutive patients.

BACKGROUND: The Pipeline Flex embolization device has some peculiarities in comparison with the previous generation device. Despite recent reports of the modified delivery system, its safety is still unknown. OBJECTIVE: To illustrate the intraprocedural and periprocedural complication rate with this new device in 30 consecutive patients. MATERIAL AND METHODS: Clinical, procedural, and angiographic data, including aneurysm size and location, device or devices used, angiographic and clinical data were analyzed. RESULTS: 30 patients harboring 30 aneurysms were analyzed. 39 devices were placed properly. Multiple Pipeline embolization devices (PEDs) were used in 7 cases. In 28 devices the distal end opened fully from the beginning with a complete wall apposition. In the remaining 11 devices, distal-end opening of the devices was instant but partial, but fully opened easily after recapture. Among the 30 procedures, recapture and reposition of the Pipeline Flex was performed four times owing to proximal migration/malposition of the device during delivery. Four intraprocedural/periprocedural complications occurred, of which 2 resulted in major complications, with neurologic deficits persisting for longer than 7 days. The 30-day morbidity rate was 6.6%, with no deaths. No aneurysm rupture or parenchymal hemorrhage was seen. CONCLUSIONS: The Pipeline Flex embolization device allows more precise and controlled deployment than the first-generation device. The number of devices and the complication rate during the learning curve are lower than reported with the first-generation PED. The new delivery system and the resheathing maneuvers do not seem to increase the intraprocedural complication rate in comparison with the first-generation PED.

Pipeline endovascular device for the treatment of intracranial aneurysms at the level of the circle of Willis and beyond: multicenter experience.

BACKGROUND AND PURPOSE: The aim of our study was to evaluate the safety and efficacy of the pipeline endovascular device for the treatment of anterior circulation aneurysms at the level of the circle of Willis and beyond. METHODS: A consecutive series of 25 patients (24 unruptured and one ruptured) with anterior circulation aneurysms treated with a pipeline endovascular device were included in the analysis. RESULTS: We found two minor clinical events (resolved within 7 days of the procedure), one major event (symptoms present after 7 days), and no mortality. There were no aneurysm ruptures or parenchymal hemorrhages during follow-up. The modified Rankin Scale (mRS) scores at 3 and 6 months did not change from the prior mRS score for all cases except 1. There was one asymptomatic periprocedural event. There were three intraprocedural complications which resolved without clinical consequences. Six month follow-up angiograms were obtained for 22 aneurysms, showing complete occlusion in 14 (64%) and significantly decreased residual filling in 8 (36%). The status of branches originating from the aneurysm sacs was evaluated in 14 angiograms: 11 were patent (79%), 2 had moderate reduction (14%) and 1 (7%) was occluded. We found six cases of in-stent stenosis (27%) on 6 month DSA, with only one symptomatic case. CONCLUSIONS: The pipeline embolization device provides a feasible and technically safe solution for aneurysms at and beyond the circle of Willis. Preliminary results are promising but larger series with longer term follow-up examinations are required to show the long term safety and durability of this treatment alternative.

Management of oral lesions in Lesch-Nyham syndrome.

Lesh-Nyhan Syndrome is a hereditary disorder that affects the way in which the body handles the production and breakdown of purines. One of its main characteristic is self-mutilation. We present a new appliance which allows healing to occur.

Bioabsorbable osteofixation devices in craniosynostosis. Clinical experience in 216 cases.

INTRODUCTION: The pursuit of bone fixation systems capable of affording appropriate stability for osteosynthesis has gone through several stages from the use of metal wires, plates, and screws to the current stage of bioabsorbable systems. In our Pediatric Neurosurgery Service and Craniofacial Surgery Unit we began employing these systems in June 1997. The object of this paper is to present a review of the bioabsorbable materials most commonly used in pediatric age, and more specifically in treating craniosynostosis, to describe the characteristics of each one of them and our experience. PATIENTS AND METHODS: From June 1997 to May 2006 we implanted bioabsorbable fixation systems in 216 patients diagnosed with craniosynostosis. Age at treatment ranged between 4 and 24 months (mean age 6.38 months). Brain computed tomography (CT) scans, and three-dimensional (3-D) reconstruction of CT scans were performed before and after treatment. An 82: 18 L-lactic acid: glycolic acid copolymer was used in 92.2% of these cases, and a 70:30 L-lactic acid: D-lactic acid copolymer was used in the remaining 7.8% of cases. The follow-up of the patients ranged between six months and five years. RESULTS: There was no displacement of bioabsorbable plates or screws in any case. In one case (0.46%), radiological imaging revealed osteolysis underneath the implant eight months after the surgical procedure. Prominences caused by the plates and screws employed were visible in two cases (0.93%). We found fractures in the osteosynthesis mesh in two patients (0.93%). Four patients (1.85%) presented local inflammation. No alterations of cranial morphology secondary to inadequate stability were observed. CONCLUSIONS: 1) Bioabsorbable fixation systems provide excellent stability during the bone "healing" period, without a higher complication rate than with other systems. 2) They help the bone grafts keep their remodeled shape. 3) They promote reossification by preventing the bone grafts from moving after osteosynthesis. 4) No interference with normal growth of the cranial vault has been observed.

Bioabsorbable osteofixation devices in craniosynostosis. Clinical experience in216 cases

Introduction: The pursuit of bone fixation systems capable of affording appropriate stability for osteosynthesis has gone through several stages from the use of metal wires, plates, and screws to the current stage of bioabsorbable systems. In our Pediatric Neurosurgery Service and Craniofacial Surgery Unit we began employing these systems in June 1997. The object of this paper is to present a review of the bioabsorbable materials most commonly used in pediatric age, and more specifically in treating craniosynostosis, to describe the characteristics of each one of them and our experience. Patients and methods: From June 1997 to May 2006 we implanted bioabsorbable fixation systems in 216 patients diagnosed with craniosynostosis. Age at treatment ranged between 4 and 24 months (mean age 6, 38 months). Brain computed tomography (CT) scans, and three-dimensional (3-D) reconstruction of CT scans were performed before and after treatment. An 82: 18 L-lactic acid: glycolic acid copolymer was used in 92.2% of these cases, and a 70:30 L-lactic acid: D-lactic acid copolymer was used in the remaining 7.8% of cases. The follow-up of the patients ranged between six months and five years. Results: There was no displacement of bioabsorbable plates or screws in any case. In one case (0.46%), radiological imaging revealed osteolysis underneath the implant eight months after the surgical procedure. Prominences caused by the plates and screws employed were visible in two cases (0.93%). We found fractures in the osteosynthesis mesh in two patients (0.93%). Four patients (1.85%) presented local inflammation. No alterations of cranial morphology secondary to inadequate stability were observed. Conclusions: 1) Bioabsorbable fixation systems provide excellent stability during the bone "healing" period, without a higher complication rate than with other systems. 2) They help the bone grafts keep their remodeled shape. 3) They promote reossification by preventing the bone grafts from moving after osteosynthesis. 4) No interference with normal growth of the cranial vault has been observed (AU)

Introducción: La búsqueda de sistemas de fijación ósea que proporcionen la estabilidad adecuada para favorecer una osteosíntesis, ha dado lugar a diferentes etapas. Desde la utilización de hilos de alambre, placas y tornillos metálicos hasta la etapa actual en la que se utilizan sistemas bioabsorbibles. En nuestro Servicio de Neurocirugía Pediátrica y Unidad de Cirugía Craneofacial comenzamos a utilizar estos sistemas en Junio de 1997. El objetivo de este trabajo es realizar una revisión de los materiales bioabsorbibles utilizados en la edad pediátrica, concretamente en craneosinostosis, aportando información sobre las características de cada uno de ellos y nuestra experiencia. Pacientes y métodos: Desde Junio de 1997 a Mayo de 2006, hemos utilizado sistemas de fijación bioabsobible en 216 pacientes diagnosticados de craneosinostosis. Las edades oscilaron entre 4 y 24 meses cuando se realizó el tratamiento (la edad media fue 6,38 meses). Todos los casos fueron estudiados con escáner cerebral y reconstrucción tridimensional antes y después del tratamiento. En el 92,2% de los casos el copolímero utilizado fue el formado por los ácidos L-Láctico y Glicolico en las proporciones de 82/18 y en el 7,8% restante el sistema formado por el copolímero de los ácidos L-Láctico y D-Láctico en la proporción de 70/30. El período de seguimiento ha oscilado entre 6 meses y 5 años. Resultados: No hubo desplazamientos de placas o tornillos bioabsorbibles en ningún caso. En un caso (0.46%), 8 meses después de la intervención se diagnosticó radiológicamente una imagen de osteolísis subyacente al implante. En dos pacientes (0.93%) se apreciaron relieves cutáneos debidos a las placas y tornillos utilizados. En dos casos (0.93%) fueron visibles a nivel frontal, prominencias por fracturas de las mallas de osteosíntesis. En cuatro ocasiones (1.85%) se apreciaron zonas de inflamación cutánea local. En ningún caso hemos observado alteraciones de la morfología craneal secundarias a una insuficiente estabilidad. Conclusiones: 1) Los sistemas de fijación bioabsorbibles proporcionan una estabilidad ideal durante la fase de “curación” ósea sin aumentar el porcentaje de complicaciones con respecto a los sistemas que previesen nuestra experiencia. 2) Favorecen el mantenimiento de la forma dada a los fragmentos óseos en la remodelación. 3) Facilitan la reosificación al impedir los movimientos de los fragmentos óseos una vez realizada la osteosíntesis. 4) No se han detectado interferencias con el crecimiento normal de la bóveda craneal (AU)

[Endovascular treatment of cerebral vasospasm due to aneurysmal subarachnoid hemorrhage]. / Tratamiento endovascular del vasospasmo cerebral inducido por hemorragia subaracnoidea aneurismática.

Cerebral vasospasm remains a leading cause of death and disability in patients with aneurysmal subarachnoid hemorrhage. When vasospasm becomes refractory to maximal medical treatment, endovascular therapies may be considered as an option to increase cerebral blood flow to prevent cerebral infarction. Endovascular techniques include transluminal balloon angioplasty and intra-arterial infusion of vasorelaxants. This article reviews the various endovascular techniques for the treatment of cerebral vasospasm and discusses the mechanisms of action, techniques of administration, clinical results, and limitations of these treatment strategies.

Surgical treatment of isolated and syndromic craniosynostosis. Results and complications in 283 consecutive cases.

OBJECTIVE: To review the results and complications of the surgical treatment of craniosynostosis in 283 consecutive patients treated between 1999 and 2007. PATIENTS AND METHODS: Our series consisted of 330 procedures performed in 283 patients diagnosed with scaphocephaly (n=155), trigonocephaly (n=50), anterior plagiocephaly (n=28), occipital plagiocephaly (n=1), non-syndromic multi-suture synostosis (n=20), and with diverse craniofacial syndromes (n=32; 11 Crouzon, 11 Apert, 7 Pfeiffer, 2 Saethre-Chotzen, and 2 clover-leaf skull). We used the classification of Whitaker et al. to evaluate the surgical results. Complications of each technique and time of patients' hospitalization were also recorded. The surgeries were classified in 12 different types according to the techniques used. Type I comprised endoscopic assisted osteotomies for sagittal synostosis (42 cases). Type II included sagittal suturectomy and expanding osteotomies (46 cases). Type III encompassed procedures similar to type II but that included frontal dismantling or frontal osteotomies in scaphocephaly (59 cases). Type IV referred to complete cranial vault remodelling (holocranial dismantling) in scaphocephaly (13 cases). Type V belonged to fronto-orbital remodelling without fronto-orbital bandeau in trigonocephaly (50 cases). Type VI included fronto-orbital remodelling without fronto-orbital bandeau in plagiocephaly (14 cases). In Type VII cases of plagiocephaly with frontoorbital remodelling and fronto-orbital bandeau were comprised (14 cases). Type VIII consisted of occipital advancement in posterior plagiocephaly (1 case). Type IX included standard bilateral fronto-orbital advancement with expanding osteotomies (30 cases). Type X was used in multi-suture craniosynostosis (15 cases) and consisted of holocranial dismantling (complete cranial vault remodelling). Type XI included occipital and suboccipital craniectomies in multiple suture craniosynostosis (10 cases) and Type XII instances of fronto-orbital distraction (26 cases). RESULTS: The mortality rate of the series was 2 out of 283 cases (0.7%). These 2 patients died one year after surgery. All complications were resolved without permanent deficit. Mean age at surgery was 6.75 months. According to Whitaker et al's classification, 191 patients were classified into Category I (67.49%), 51 into Category II (18.02%), 30 into Category III (10.6%) and 14 into Category IV (4.90%). Regarding to craniofacial conformation, 85.5 % of patients were considered as a good result and 15.5% of patients as a poor result. Of the patients with poor results, 6.36% were craniofacial syndromes, 2.12% had anterior plagiocephaly and 1.76% belonged to non-syndromic craniosynostosis. The most frequent complication was postoperative hyperthermia of undetermined origin (13.43% of the cases), followed by infection (7.5%), subcutaneous haematoma (5.3%), dural tears (5%), and CSF leakage (2.5%). The number of complications was higher in the group of re-operated patients (12.8% of all). In this subset of reoperations, infection accounted for 62.5%, dural tears for 93% and CSF leaks for 75% of the total. In regard to the surgical procedures, endoscopic assisted osteotomies presented the lowest rate of complications, followed by standard fronto-orbital advancement in multiple synostosis, trigonocephaly and plagiocephaly. The highest number of complications occurred in complete cranial vault remodelling (holocranial dismantling) in scaphocephaly and multiple synostoses and after the use of internal osteogenic distractors. Of note, are two cases of iatrogenic basal encephalocele that occurred after combined fronto-facial distraction. CONCLUSIONS: The best results were obtained in patients with isolated craniosynostosis and the worst in cases with syndromic and multi-suture craniosynostosis. The rate and severity of complications were related to the type of surgical procedure and was higher among patients undergoing re-operations. The mean time of hospitalization was also modified by these factors. Finally, we report our considerations for the management of craniosynostosis taking into account each specific technique and the age at surgery, complication rates and the results of the whole series.

Surgical treatment of isolated and syndromic craniosynostosis. Results andcomplications in 283 consecutive cases

Objective. To review the results and complications ofthe surgical treatment of craniosynostosis in 283 consecutivepatients treated between 1999 and 2007.Patients and methods. Our series consisted of 330procedures performed in 283 patients diagnosed withscaphocephaly (n=155), trigonocephaly (n=50), anteriorplagiocephaly (n=28), occipital plagiocephaly (n=1),non-syndromic multi-suture synostosis (n=20), and withdiverse craniofacial syndromes (n=32; 11 Crouzon, 11Apert, 7 Pfeiffer, 2 Saethre-Chotzen, and 2 clover-leafskull). We used the classification of Whitaker et al. toevaluate the surgical results. Complications of eachtechnique and time of patients’ hospitalization were alsorecorded. The surgeries were classified in 12 differenttypes according to the techniques used. Type I comprisedendoscopic assisted osteotomies for sagittal synostosis(42 cases). Type II included sagittal suturectomy andexpanding osteotomies (46 cases). Type III encompassedprocedures similar to type II but that included frontaldismantling or frontal osteotomies in scaphocephaly (59cases). Type IV referred to complete cranial vault remodelling(holocranial dismantling) in scaphocephaly (13cases). Type V belonged to fronto-orbital remodellingwithout fronto-orbital bandeau in trigonocephaly (50cases). Type VI included fronto-orbital remodellingwithout fronto-orbital bandeau in plagiocephaly (14cases). In Type VII cases of plagiocephaly with frontoorbitalremodelling and fronto-orbital bandeau werecomprised (14 cases). Type VIII consisted of occipitaladvancement in posterior plagiocephaly (1 case). TypeIX included standard bilateral fronto-orbital advancementwith expanding osteotomies (30 cases). (..) (AU)

bjetivos. Revisar y analizar los resultados y las complicacionesdel tratamiento en nuestra serie de 283 casosconsecutivos de craneosinostosis, tratados por medio de330 procedimientos quirúrgicos transcraneales entrelos años 1999 hasta el 2007.Pacientes y métodos. La serie consta de 155escafocefalias, 50 trigonocefalias, 28 plagiocefaliasanteriores, 1 plagiocefalia occipital, 20 craneosinostosismúltiples no sindrómicas y 32 síndromes craneofaciales(11 Crouzon, 11 Apert, 7 Pfeiffer, 2 Saethre-Chotzen y2 cráneos en hoja de trébol). Para la evaluación de losresultados quirúrgicos hemos empleado la conocida clasificaciónde Whitaker. Por otra parte, para el estudio delas complicaciones, los procedimientos quirúrgicos utilizadosse han clasificado en 12 tipos: Tipo I: osteotomíasasistidas por endoscopia en las escafocefalias (42 casos).Tipo II: suturectomía sagital y osteotomías expansivas(46 casos). Tipo III: la misma técnica que en el grupoanterior, pero incluyendo el desmontaje frontal o bienosteotomías de la región frontal en escafocefalias (59casos). Tipo IV: remodelación completa de la bóvedacraneal (desmontaje holocraneal) en escafocefalias (13casos). Tipo V: remodelación fronto-orbitaria sin barrafronto-orbitaria en trigonocefalias (50 casos). TipoVI: remodelación fronto-orbitaria sin barra frontoorbitariaen plagiocefalias anteriores (14 casos). TipoVII: remodelación fronto-orbitaria con barra frontoorbitariaen plagiocefalias anteriores (14 casos). TipoVIII: avance occipital en plagiocefalias posteriores (1caso). Tipo IX: avance fronto-orbitario standard conosteotomías expansoras (30 casos) (..) (AU)

Tratamiento endovascular del vasospasmo cerebral inducido por hemorragia subaracnoidea aneurismática / Endovascular treatment of cerebral vasospasm due to aneurysmal subarachnoid hemorrhage

El vasospasmo cerebral es una de las principales causas de morbimortalidad en los pacientes con hemorragia subaracnoidea por rotura de un aneurisma cerebral. Cuando el vasospasmo se hace resistente al tratamiento médico máximo, el tratamiento endovascular es una opción terapéutica para incrementar el flujo sanguíneo cerebral y evitar lesiones isquémicas cerebrales. Los rápidos avances en técnicas endovasculares permiten utilizar la angioplastia transluminal percutánea y la infusión intraarterial de diversos fármacos vasodilatadores para revertir el vasospasmo. En este artículo se revisan las diferentes técnicas endovasculares disponibles y se describen sus mecanismos de acción, técnicas de administración, resultados clínicos y complicaciones

Cerebral vasospasm remains a leading causeof death and disability in patients with aneurysmalsubarachnoid hemorrhage. When vasospasm becomesrefractory to maximal medical treatment,endovascular therapies may be considered as anoption to increase cerebral blood flow to preventcerebral infarction. Endovascular techniques includetransluminal balloon angioplasty and intraarterialinfusion of vasorelaxants.This article reviews the various endovasculartechniques for the treatment of cerebral vasospasmand discusses the mechanisms of action,techniques of administration, clinical results,and limitations of these treatment strategies

Spanish Registry for Embolization of Small Intracranial Aneurysms with Cerecyte Coils (SPAREC) Study. Early Experience and Mid-Term Follow-up Results.

SUMMARY: This study aimed to report the results obtained in treating small ruptured and unruptured intracranial aneurysms using Cerecyte coils. A prospective, non-randomized multicenter registry operating in Spain with a reporting period between May 2005 and September 2007.We present clinical and angiographic results for 48 small aneurysms (26 ruptured, five with III cranial nerve paresis, and 17 incidental) that had undergone six months' follow-up. The volumetric percentage occlusion (VPO) achieved and percentage bioactive coils (PBC) used were assessed. No episodes of bleeding occurred during the follow-up period. The technical complication rate was 10.4% (five cases): four thromboembolic complications resolved with medication (8.3%) and one asymptomatic protrusion of a coil into the parent vessel. The clinical complication rate for the procedure was 2.1% (occlusion of the anterior choroidal artery in a ruptured anterior choroidal anaeurysm). Mean VPO was 25.2%. Balloon-assisted technique (BAT) was used in 60.4% of cases. The VPO was higher in the BAT-treated cases (P < 0.05). The overall six-month recanalization rate was 16.7% (12.5% minor and 4.2% major recanalizations). Neck size and VPO were unrelated to the recanalization rate. The PBC was higher in cases with progressive Deployment of the device is safe from the standpoint of periprocedural technical and clinical complications. No episodes of hemorrhage were recorded during follow-up. The sixmonth recanalization and retreatment rates compared favorably with most endovascular platinum and bioactive coil series.

[Positional plagiocephaly. Diagnosis and treatment]. / Diagnóstico y tratamiento de la plagiocefalia posicional. Protocolo para un Sistema Público de Salud.

INTRODUCTION: Positional plagiocephaly is nowadays the most frequent consultation at pediatric neurosurgical departments in Spain and western countries. Another important issue is the confusion existing in literature regarding terminology, physiopathology, differential diagnosis with true synostosis and, of course, proper recommendations for treatment. OBJECTIVES: To clarify all these concepts and present a protocol that was recently asked by the Health Administration of the Community of Madrid. PROTOCOL: Pretends to achieve coordination among paediatricians and neurosurgeons, as much as to offer precise information about data concerning this entity for parents, paediatricians and neurosurgeons. MATERIAL AND METHODS: Previous consensus is reached about some data. Children are classified in three categories of deformation (mild; moderate; severe), according to measurements on digital photography. Diagnosis and treatment follows two phases: Paediatric phase (up to 5 months of age) and Neurosurgical phase (from 5 months on). Children would be referred to neurosurgical consultation only after being treated with postural changes and physiotherapy by the age of 5 months. Reasons are explained in the protocol that defines also functions and responsibilities for each speciality. CONCLUSION: Treatment proposed here is staged, starting with postural changes and physiotherapy, followed by orthotic cranial devices and finally surgical treatment.

Diagnóstico y tratamiento de la plagiocefalia posicional. Protocolo para un Sistema Público de Salud / Positional plagiocephaly. Diagnosis and treatment

Introducción. La plagiocefalia posicional es actualmente la causa más frecuente de asistencia en una consulta de neurocirugía pediátrica, tanto en España como en todos los países occidentales. A ello se suma la considerable confusión existente en la literatura en relación a aspectos como la terminología, conceptos fisiopatológicos, diagnóstico diferencial con la craneosinostosis y por supuesto en el tratamiento más adecuado a seguir. Objetivos. Intentar clarificar estos conceptos y además presentar un protocolo de asistencia que nos ha solicitado recientemente la Administración Sanitaria dela Comunidad de Madrid. Protocolo. Pretende lograr la coordinación entre pediatras y neurocirujanos, así como conseguir una información precisa de los principales datos de esta patología para los familiares, pediatras y neurocirujanos. Material y métodos. Se establecen una serie de datos de consenso. Los niños son clasificados en tres grados de deformación (leve, moderada y grave) según los índices medidos en fotografías digitales. Además el proceso del diagnóstico y tratamiento tiene dos fases: fase pediátrica (hasta los 5 meses de edad) y fase neuroquirúrgica (desde los 5 meses de edad). Los niños serán enviados a Neurocirugía después de haber sido tratados con tratamiento posicional y rehabilitación y solamente a partir de los 5 meses de edad. Las razones de todo ello son explicadas en el protocolo que define también las funciones y responsabilidades de cada especialista. El tratamiento que se propone es escalonado, comenzando por las medidas posicionales y de rehabilitación, seguidas de ortesis craneal y en último lugar del tratamiento quirúrgico

Introduction. Positional plagiocephaly is nowadays the most frequent consultation at pediatric neurosurgical departments in Spain and western countries. Another important issue is the confusion existing in literature regarding terminology, physiopathology, differential diagnosis with true synostosis and, of course, proper recommendations for treatment. Objectives. To clarify all these concepts and presenta protocol that was recently asked by the Health Administration of the Community of Madrid. Protocol. Pretends to achieve coordination among paediatricians and neurosurgeons, as much as to offer precise information about data concerning this entity for parents, paediatricians and neurosurgeons. Material and methods. Previous consensus is reached about some data. Children are classified in three categories of deformation (mild; moderate; severe), according to measurements on digital photography. Diagnosis and treatment follows two phases: Paediatric phase (up to 5 months of age) and Neurosurgical phase (from 5 months on). Children would be referred to neurosurgical consultation only after being treated with postural changes and physiotherapy by the age of5 months. Reasons are explained in the protocol that defines also functions and responsibilities for each speciality. Conclusion. Treatment proposed here is staged, starting with postural changes and physiotherapy, followed by orthotic cranial devices and finally surgical treatment

[Endoscopically assisted fronto-orbitary correction in trigonocephaly]. / Remodelación frontoorbitaria endoscópicamente asistida en la trigonocefalia.

The development of multidisciplinar Units for Craneofacial Surgery has led to a considerable decrease in morbidity even in the cases of more complex craniofacial syndromes. The use of minimally invasive techniques for the correction of some of these malformations allows the surgeon to minimize the incidence of complications by means of a decrease in the surgical time, blood salvage and shortening of postoperative hospitalization in comparison to conventional craniofacial techniques. Simple and milder craniosynostosis are best approached by these techniques and render the best results. Different osteotomies resembling standard fronto-orbital remodelling besides simple suturectomies and the use of postoperative cranial orthesis may improve the final aesthetic appearence. In endoscopic treatment of trigonocephaly the use of preauricular incisions achieves complete pterional resection, lower lateral orbital osteotomies and successful precoronal frontal osteotomies to obtain long lasting and satisfactory outcomes.

Remodelación frontoorbitaria endoscópicamente asistida en la trigonocefalia / Endoscopically assisted fronto-orbitary correction in trigonocephaly

La aparición de equipos multidisciplinares en las Unidades de Cirugía Craneofacial ha permitido disminuir considerablemente la incidencia de morbimortalidad incluso en el caso de los síndromes craneofaciales más complejos. La posibilidad de ofertar técnicas mínimamente invasivas mediante osteotomías endoscópicamente asistidas para algunas de estas malformaciones permite disminuir el número de complicaciones, así como acortar el tiempo quirúrgico, las pérdidas hemáticas y la estancia hospitalaria postoperatoria respecto a las técnicas convencionales de cirugía craneofacial. Los resultados más satisfactorios se consiguen en las craneosinostosis simples o más leves. La realización de diversas osteotomías junto a la suturectomía simple imitando las técnicas de remodelación frontoorbitaria clásica y la aplicación postoperatoria de cascos de ortesis permiten mejorar la apariencia final de la deformidad. En el tratamiento endoscópico de la trigonocefalia la utilización de dos incisiones preauriculares permite una osteotomía orbitaria lateral más baja, la resección pterional completa y la realización de las osteotomías frontales en posición retrocoronal, con lo que es posible obtener un resultado más satisfactorio que tras la resección simple de la sutura metópica (AU)

The development of multidisciplinar Units for Craneofacial Surgery has led to a considerable decrease in morbidity even in the cases of more complex craniofacial syndromes. The use of minimally invasive techniques for the correction of some of these malformations allows the surgeon to minimize the incidence of complications by means of a decrease in the surgical time, blood salvage and shortening of postoperative hospitalization in comparison to conventional craniofacial techniques. Simple and milder craniosynostosis are best approached by these techniques and render the best results. Different osteotomies resembling standard fronto-orbital remodelling besides simple suturectomies and the use of postoperative cranial orthesis may improve the final aesthetic appearence. In endoscopic treatment of trigonocephaly the use of preauricular incisions achieves complete pterional resection, lower lateral orbital osteotomies and successful precoronal frontal osteotomies to obtain long lasting and satisfactory outcomes (AU)

[Presurgical orthopedic treatment for cleft lip and palate]. / Uso de la ortopedia prequirúrgica en pacientes con fisura palatina: nuestra experiencia.

INTRODUCTION: A multidisciplinary approach with several specialits allows a complete treatment for Cleft Lip and Palate. We show our experience in presurgical orthopedic treatment in these patients, their advantages, their problems and the results. MATERIAL AND METHODS: Since 1999 presurgical orthopedy has been applied to 12 patients (3 bilateral cleft lip and palate and 9 unilateral cleft lip and palate). This approach was applied when there was a long distance between the alveolar segments. A palate mould and the location of Latham's appliance have been made in the operating room under general anesthesia. The patients were controlled by the orthodoncist and Latham's appliance was removed when cleft lip was closed. RESULTS: Latham's appliance was kept for 4-7 weeks with once a week controls until the distance between the maxillary segments was less than 1 mm; in bilateral cases of cleft lip and palate the premaxilla was moved between lateral segments. Then, lip closure and nasoplasty was made and, sometimes, an obturador was placed. CONCLUSIONS: Latham's appliance permit to achieve a perfect alignment of alveolar segments decreasing the soft tissues tension and facilitating the lip surgery, thus, a better aesthetic and functional results can be achieved. A more anatomic position of palate can be made and easier future orthopedic treatments are possible.

Fronto-orbital remodeling without orbito-naso-frontal bandeau.

INTRODUCTION: Fronto-orbital bilateral advance is the procedure of choice for the treatment of craniosynostosis affecting most of the anterior area of the skull and orbitomalar regions. The aim of the technique is to achieve a supra-orbital bilateral bar and a frontal bone. We have introduced a modification in order to simplify the technique. PATIENTS AND METHODS: From November 1998 to January 2002, 18 patients with craniosynostosis have been surgically treated using our technique. The mean age when the treatment was performed was 6.93 months (range 3 to 22 months). Brain computed tomography (CT) scans and three-dimensional (3-D) reconstruction of CT scans were performed before and after treatment. SURGICAL TECHNIQUE: A bifrontal craniotomy was performed taking the osteotomy up to the supraorbital rim. A new frontal bone was obtained from another region of the cranium creating new orbital edges. The osteosynthesis was conducted using absorbable materials. RESULTS: The follow-up of the patients ranged from 3 months to 3 years. All patients were studied using CT scans and 3-D reconstruction of CT after treatment, which demonstrated the persistence of the fronto-orbital advance. No secondary complications related to the new technique were found in any of the patients. CONCLUSIONS: The frontal-orbital advance obtained was stable. The technique was simplified by not creating a supraorbital bar and by reducing the bone fixation points. The manipulation of both frontal lobes and orbital globes was negligible. The aesthetic results were excellent.

Surgical treatment of trigonocephalies and associated hypoteleorbitism.

Premature closure of metopic suture is a relatively uncommon form of craniosyostosis with an estimated incidence of 0,3 per 1000 live births, comprising about 7% of surgical craniosynostosis referred to craniofacial centers. A broad phenotypical spectrum spreads from minor metopic ridges to severe trigonocephaly with pterional indentation supraorbital bar retrusion, temporal and parietal compensating bossings and hypotelorism. Most of the cases arise spontaneously although autosomal dominant inheritance has been described and association with cromosomal abnormalities and different syndromes has been widely reported. Surgical correction has been attempted with good cosmetic results using several variations of the standard frontoorbitary advance. However there is still a number of questions to be solved in relation to this entity, mainly on its pathogenesis, but also on its development, natural history and treatment. Direct surgical approach to associated hypotelorism is a matter of argument when considering the reestablishment of normal interorbitary distances. We have conducted a retrospective analysis of our serie consisting of twenty-eight cases of trigonocephalies. Surgical correction of hypotelorism was attempted in eleven cases while the resting seven children remained "not treated". The objective was to review the functional outcome and cosmetic results comparing the different techniques applied to the frontal bone and to observe evolution of the hypoteleorbitism after the treatment with or without osteotomies and grafting of the nasoethmoidal area.