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.

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)

[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

[Doubts and confusions in the diagnosis and treatment of positional plagiocephaly. Management protocol]. / Enigmas y confusiones en el diagnóstico y tratamiento de la plagiocefalia posicional. Protocolo asistencial.

INTRODUCTION: Positional plagiocephaly is currently the most frequent cause of consultation at pediatric neurosurgical departments in Spain and other western countries. There is considerable confusion in the literature on the terminology and physiopathology of this deformity, as well as its differential diagnosis with true synostosis and treatment recommendations. OBJECTIVES: To clarify these concepts and present a protocol for the management of positional plagiocephaly, which was recently requested by the Health Administration of the Community of Madrid. PROTOCOL: The protocol aims to achieve coordination among pediatricians and neurosurgeons, as well as to provide precise information on this deformity for parents, pediatricians and neurosurgeons. MATERIAL AND METHODS: Previous consensus was reached on a series of data. Infants were classified into three categories of deformity (mild, moderate, severe) according to measurements on digital photographs. Diagnosis and treatment follows two phases: a pediatric phase (up to 5 months of age) and a neurosurgical phase (from 5 months onwards). Infants are referred to neurosurgical consultation only after being treated with postural changes and physiotherapy and only after reaching the age of 5 months. The reasons for this approach are explained in the protocol, which also defines the functions and responsibilities of each specialty. CONCLUSION: The treatment proposed in the protocol is staged, starting with postural changes and physiotherapy, followed by orthotic cranial devices and finally surgical treatment.

Enigmas y confusiones en el diagnóstico y tratamiento de la plagiocefalia posicional. Protocolo asistencial / Doubts and confusions in the diagnosis and treatment of positional plagiocephaly. Management protocol

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 especializada en relación a aspectos como la terminología, conceptos fisiopatológicos, diagnóstico diferencial con la craneosinostosis y por supuesto en el tratamiento más adecuado que hay que seguir. Objetivos Intentar clarificar estos conceptos y además presentar un protocolo de asistencia que nos ha solicitado recientemente la Administración Sanitaria de la 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 3 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. Conclusiones 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 currently the most frequent cause of consultation at pediatric neurosurgical departments in Spain and other western countries. There is considerable confusion in the literature on the terminology and physiopathology of this deformity, as well as its differential diagnosis with true synostosis and treatment recommendations. Objectives To clarify these concepts and present a protocol for the management of positional plagiocephaly, which was recently requested by the Health Administration of the Community of Madrid. Protocol The protocol aims to achieve coordination among pediatricians and neurosurgeons, as well as to provide precise information on this deformity for parents, pediatricians and neurosurgeons. Material and methods Previous consensus was reached on a series of data. Infants were classified into three categories of deformity (mild, moderate, severe) according to measurements on digital photographs. Diagnosis and treatment follows two phases: a pediatric phase (up to 5 months of age) and a neurosurgical phase (from 5 months onwards). Infants are referred to neurosurgical consultation only after being treated with postural changes and physiotherapy and only after reaching the age of 5 months. The reasons for this approach are explained in the protocol, which also defines the functions and responsibilities of each specialty. Conclusion The treatment proposed in the protocol 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)

Tiroides lingual: un nuevo abordaje quirúrgico / Lingual thyroid: a new surgical approach

El tiroides lingual es una rara anomalía congénita del desarrollo tiroideo resultante de la ausencia de descenso del mismo desde el foramen caecum hasta la localización prelaríngea habitual. Presentamos el caso de una paciente en edad postmenopáusica con diagnóstico anatomopatológico de tiroides lingual de gran tamaño y localización profunda en la base de la lengua que producía disfagia y dificultad respiratoria crecientes. Asimismo, planteamos un nuevo abordaje quirúrgico para la resección combinando cervicotomía media, pulí-through lingual y glosotomía media. Se discuten las distintas pruebas complementarias para llegar a su diagnóstico y se revisan las diferentes técnicas quirúrgicas habitualmente empleadas en su tratamiento concluyendo con las ventajas del abordaje empleado en este caso (AU)

Utilización del colgajo de músculo temporal en cirugía reconstructiva maxilofacial. Revisión de 104 casos / Use of the temporalis muscle flap in maxillofacial reconstruction surgery. A review of 104 cases

Introducción: el colgajo de músculo temporal ha sido empleado en reconstrucción craneofacial desde hace más de 100 años. El primer caso descrito en la literatura fue publicado por Lentz en 1895. Hoy en día el uso de colgajos locales con músculo temporal parece ser desplazado por el uso de colgajos libres microvascularizados en la reconstrucción craneofacial. En nuestra experiencia dichos colgajos locales constituyen una opción segura en muchos de nuestros pacientes. Objetivos: mostrar nuestras indicaciones y resultados en reconstrucción craneofacial con el empleo del colgajo de músculo temporal. Material y método: análisis retrospectivo de nuestra experiencia con el colgajo de músculo temporal en la última década. Resultados: hemos empleado un total de 108 colgajos miofasciales temporales en pacientes adultos con las siguientes indicaciones: reconstrucción de defectos postmaxilectomía (44); cirugía de base de cráneo (25) incluyendo fosa craneal anterior, media y posterior; cavidad oral y orofaringe (23); tras exenteración orbitaria en pacientes oncológicos (6); anquilosis de ATM (6); secuelas faciales postraumáticas (2); reanimación facial (2).Conclusiones: el colgajo de músculo temporal es una de las primeras opciones en cirugía reconstructiva oncológica craneofacial, de la ATM y base de cráneo. La disección traumática del colgajo y la sutura a tensión predisponen la aparición de complicaciones como necrosis o dehiscencia de la sutura (AU)

Adenoma pleomorfo recurrente del paladar. Reconstrucción con colgajo bilateral de bola adiposa bucal de Bichat

No disponible

Reconstrucción de grandes defectos de cabeza y cuello con el colgajo libre de rectoabdominal / Reconstruction of huge head and neck defects with the rectus abdominis free flap

Introducción. La utilidad del colgajo libre de recto abdominal en la reconstrucción de grandes complejos defectos tridimensionales de cabeza y cuello, oncológicos o de otra etiología, permite ofrecer una alternativa terapéutica en aquellos pacientes que, debido a la extensión de su enfermedad o al haber agotado previamente otras modalidades de tratamiento, serían desahuciados.Material y método. Se presenta un estudio retrospectivo sobre 16 colgajos realizados en el período comprendido entre 1-1-1996 y el 1-7-1999, en el Servicio de Cirugía Oral y Maxilofacial del Hospital Universitario 12 de Octubre de Madrid. Resultados. La tasa global de éxito en la transferencia tisular microvascularizada fue superior al 93 por ciento, registrándose una muy baja incidencia de complicaciones en la zona donante y en la receptora. Cerca de un 50 por ciento de los pacientes con un estadio IV en la evolución de su enfermedad siguen vivos y con un control efectivo locorregional a los 2 años de su seguimiento postoperatorio. Conclusión. La reconstrucción de grandes defectos en cabeza y cuello mediante el colgajo libre de recto abdominal es un método seguro y fiable, útil en aquellos pacientes con avanzados estadios de evolución en su enfermedad (AU)