Esthetic subunit approach in massive facial plexiform neurofibroma: a case report. / Abordaje por subunidades estéticas en el neurofibroma plexiforme facial masivo: a propósito de un caso.

Plexiform neurofibroma is a presentation of neurofibromatosis type 1 (NF1) which can cause great facial deformities. Treatment rarely has a healing effect, so the surgical approach is aimed at improving esthetics and function. It requires a cross-disciplinary approach and typically needs multi-stage surgery. This is the case of a 16-year-old male patient with NF1 presenting with left periorbital and malar facial plexiform neurofibroma with slow-growth intraconal and extraconal invasion. He presented at the plastic surgery consultation for facial soft tissue deformity correction. Removal was performed using an esthetic subunit approach, with canthopexy and orbital cavity reconstruction, resulting in facial region symmetrization. This allowed for remarkable esthetic and functional improvement, facilitating ocular prosthesis adaptation. The subsequent use of selumetinib allowed the lesion to be stabilized.

Los neurofibromas plexiformes son una forma de presentación de la neurofibromatosis tipo 1 (NF1) que pueden originar grandes deformaciones faciales. El tratamiento de estas tumoraciones casi nunca es curativo, el abordaje quirúrgico tiene por objetivo mejorar la estética y la función. Requiere un abordaje multidisciplinar y suele necesitar cirugía por etapas. Se presenta el caso de un paciente varón con NF1 que presenta un neurofibroma plexiforme facial periorbitario y malar izquierdo con invasión intra y extraconal de crecimiento lento. Acude con 16 años a la consulta de cirugía plástica para corrección de las deformidades faciales de partes blandas. Se realiza exéresis mediante abordaje por subunidades estéticas, realizando cantopexia y reconstrucción de la cavidad orbitaria, resultando en una simetrización de la región facial. Con ello se obtiene una notable mejoría estética y funcional, facilitando la adaptación de la prótesis ocular. El uso posterior de selumetinib ha permitido estabilizar la lesión.

[Periorbital lymphatic malformations: Report of two cases]. / Malformaciones linfáticas periorbitarias: A propósito de 2 casos.

INTRODUCTION: Periorbital lymphatic malformations are a rare entity in which diagnosis and treatment could be a challenge for surgeons. Management may be conservative, or they can be treated by sclerotherapy, surgery or combination of both. CLINICAL OBSERVATION: We present two cases of periorbital lymphatic malformations treated by sclerotherapy (OK-432 or bleomycin) and surgery in which we achieve good results with minimum morbidity. COMMENTS: Surgery combined with sclerotherapy is a good option of treatment for periorbital lymphatic malformations because surgery can be more conservative and therefore, sequelae are lower.

INTRODUCCION: Las malformaciones linfáticas periorbitarias son una entidad poco frecuente cuyo diagnóstico y tratamiento puede suponer un reto para el cirujano. El manejo puede ser conservador, o pueden tratarse mediante escleroterapia, cirugía o combinación de ambas. IBSERVACION CLINICA: Presentamos dos casos de malformaciones linfáticas periorbitarias tratadas mediante escleroterapia (con OK-432 o bleomicina) y cirugía con buenos resultados y mínima morbilidad. COMENTARIOS: La combinación de cirugía y escleroterapia es una buena opción de tratamiento de las malformaciones linfáticas periorbitarias; ya que nos permite ser más conservadores en la exéresis de las mismas y, por ello, las secuelas son menores.

Colgajo FAMM para reconstrucción de fístulas de paladar en pacientes con fisura palatina congénita: experiencia y resultados / Experience and results of palatal fistula repair with FAMM flap in patients with congenital cleft palate

Las fístulas de paladar suponen una complicación frecuente en la cirugía de los pacientes fisurados. Las opciones de reconstrucción secundaria en fístulas de tamaño moderado-grande son limitadas por la escasez de tejido donante local y las cicatrices de la palatoplastia. Presentamos nuestra experiencia en el tratamiento de fístulas palatinas moderadas-grandes con colgajo músculomucoso basado en la arteria facial (colgajo FAMM: facial artery musculo-mucosal flap) y describimos algunos detalles técnicos que hemos incorporado para mejorar su seguridad.Revisamos 8 pacientes, 5 mujeres y 3 varones, con edades comprendidas entre los 5 y los 18 años, nacidos con fisura palatina que desarrollaron fístulas palatinas tras la palatoplastia primaria y que tras evaluación multidisciplinaria fueron sometidos a reconstrucción con colgajo FAMM. Los tamaños de las fístulas oscilaron entre los 0,5 y los 2,3 cm. de diámetro mayor. El seguimiento postoperatorio de los pacientes fue de 1 a 6 años. Obtuvimos cierre completo de las fístulas de paladar en el primer tiempo quirúrgico y sin complicaciones en 5 casos. Los 2 primeros sufrieron necrosis de los bordes distales del colgajo y precisaron revisión quirúrgica y una paciente sufrió necrosis total que requirió una nueva reconstrucción con colgajo FAMM contralateral. No hemos registrado complicaciones de la zona donante en ninguno de los casos. Consideramos que el colgajo FAMM es una excelente opción para la reparación de fístulas palatinas de tamaño moderadogrande secundarias a fisura palatina congénita, ya que permite aportar una gran cantidad de tejido vascularizado con un arco de rotación amplio, lo que posibilita obtener resultados de obturación satisfactorios. La tasa de complicaciones, tras una curva de aprendizaje relativamente breve, es baja (AU)

Palatal fistulas are a common complication of cleft surgery. Options for secondary reconstruction of moderate to large defects are limited due to the scarcity of local donor tissue as well as the scars from the primary cleft repair. We present our experience in the treatment of moderate to large palatal fistulas with facial artery musculo-mucosal flap (FAMM flap), and we describe some technical details that we have introduced to improve safety. We present 8 patients, 5 female and 3 male, aged 5 to 18 years, with congenital cleft palate who developed moderate to large fistulae that after multidisciplinary evaluation were reconstructed with FAMM flap. The sizes of the fistulae ranged from 0,5 to 2,3 cm. in largest diameter. The follow-up was 1-6 years. We obtained a complete correction of palatal fistula in 5 patients without complications. Marginal necrosis occurred in the first 2 cases and required revision. One patient suffered total flap necrosis and required reconstruction with a contralateral FAMM flap. We did not experience complications of the donor site. We consider this flap an excellent option in moderate to large palatal fistula repair because it allows providing a large amount of well vascularized tissue with a wide arc of rotation. After a relatively short learning curve, complications are rare (AU)

[Intraparotid first branchial arch cyst: complex diagnostic and therapeutic process]. / Quiste de primer arco branquial intraparotídeo: complejo proceso diagnóstico y terapéutico.

First branchial arch cysts are uncommon. Therefore, together with its variable clinical and age presentation they are often misdiagnosed at first. The treatment is surgical, requiring a correct procedure to avoid future recurrences. In this paper we describe a typical case of first branchial arch cyst in which as described in other reports, we first made several misdiagnoses and therefore an inadequate treatment and lastly, with the correct diagnosis, we performed a meticulous complete excision under facial nerve monitoring.

[Extruded cochlear implant magnet covered with a temporoparietal fascial flap. A case report]. / Rescate de implante coclear expuesto mediante cobertura con colgajo de fascia temporoparietal. A propósito de un caso.

Complications are infrequent after cochlear implant surgery but they might occur despite careful preoperative planning and meticulous surgical technique. Among the most commonly encountered problems are those associated with the postauricular flap. An exposed, and therefore contaminated, device requires immediate attention and intervention. Cochlear implantation revision surgery is justified by two main reasons, the high price of these devices and the difficulty of reimplantation, due to cochlear fibrosis and ossification after its removal. There are multiple options in cochlear implantation revision surgery with infected device. However, the temporoparietal fascia flap is highly vascularized and provides some advantages over other alternatives. We report a case of a 5 year old boy with bilateral sensor neural hearing loss, who suffered a device extrusion three years after its implantation.

[Rapidly involuting congenital hemangioma (RICH) that simulates a minimal cleft lip]. / Hemangioma congénito rápidamente involutivo (RICH) que simula una fisura labial mínima.

Hemangiomas are frequent tumors that manifest in variable forms. Rapidly Involuting Congenital Hemangiomas (RICH) are a rare subtype, only recently described, which, in contrast to the typical infantile hemangioma, undergo accelerated involution, sometimes within days. Their different initial appearance and posterior evolution may cause confusion with other tumors of infancy or even with congenital malformations. We present a case of RICH in the upper lip that after rapid involution produced a morphologic sequel that resembled a microform cleft lip. The authors review the specific characteristics of this rare tumor and describe the technical detail of surgical correction in this patient.

First Spanish Trauma Registry: analysis of 1500 cases.

OBJECTIVES: To develop the first Spanish Pediatric Trauma Registry to collect and evaluate infomation concerning aspects of injuries in our pediatric population. METHODS: From January 1995 to August 1998, 35,946 children younger than 16 years were treated in our hospital for acute injury: 1500 were admitted and included in our database. Our file registry consists of 108 data points including: patient identification, type, place and mechanism of injury, pre-hospital care, transport, assessment on admission, severity scores, diagnostic studies, injuries, treatment morbidity and mortality. RESULTS: Accidents were more frequent in males (68%) than in females. The predominant age group was 12-15 years of age (34%). Accidents were more frequent in the street (35.1%) than at home (18.9%) or school (13%). Falls and traffic-related accidents were the leading cause of injury (39% and 21.2%, respectively). Two hundred and thirty-five (15.7%) had a Pediatric Trauma Score < or = 8. Fifty of these sustained multiple trauma (33%) (Injury Severity Score > or = 15). Musculoskeletal and head trauma were the most frequent injuries (48.5% and 42.0%, respectively). Surgical or orthopedic procedures were performed in 906 patients (56.5%). The average length of stay was 4.5 days (range 1-93 days). Functional impairment in children older than 4 years of age was found in 413 children (33.3%). We encountered 7 deaths in the 1500 patients, or an overall mortality of 0.5%. These 7 deaths were only seen in the I.S.S. > or = 15 group (50 patients) with 14% mortality. CONCLUSIONS: The goals of this Registry are to establish the epidemiology of our injured pediatric population, to review patient care, to develop prevention programs and to compare results with other centers so that potential deficiencies can be corrected.

[Pediatric trauma registry: analysis of 1200 cases]. / Registro de trauma pediátrico: análisis de 1.200 casos.

OBJECTIVES: We developed the first Spanish Pediatric Trauma Registry in order to collect and evaluate information concerning aspects of injuries in our pediatric population. METHODS: From January'95 to September'97, 28,713 children younger than 16 years were treated in our Hospital for acute injury: 1,200 were admitted and included in our database. Our file-registry consists of 108 data points including: patient identification, type, place and mechanism of injury, pre-hospital care, transport, assessment on admission, severity scores, diagnostic studies, injuries, treatments and morbidity-mortality. RESULTS: Accidents were more frequent in males (69%) than in females. The age-group predominantly was 12-15 years old (34%). Accidents were more frequent in the street (35.3%) than at home (18.7%) or school (14%). Falls and traffic-related accidents were the leading cause of injury (38 and 21.1%, respectively). The 16.7% of cases had Pediatric Trauma Score < or = 8 (n = 201). The 3.6% of this sustained multiple trauma (43 cases with Injury Severity Score > or = 15). Musculoskeletal and head trauma was the most frequent (62 and 42.3%, respectively). Surgical or orthopedic procedures were performed in 678 patients (56.5%). Average length of stay were 4.8 days (range 1-93 days), and functional impairments at discharge were found in 33.9% of patients older than 3 years (n = 338). Mortality rate in our series was 0.5% (n = 6), and 13.9% in children with ISS > or = 15. CONCLUSIONS: The utility of this Registry is to know the epidemiology of our injured pediatric population, to review patient care, to develop prevention programs and to compare results with other centers so potential deficiencies can be identified and corrected.

[Epidemiologic study of injuries in childhood: the first pediatric trauma registry]. / Estudio epidemiológico de los accidentes en la infancia: Primer Registro de Trauma Pediátrico.

OBJECTIVE: In order to collect and evaluate information concerning aspects of injuries in our pediatric population, we developed the first Spanish Pediatric Trauma Registry. PATIENTS AND METHODS: During 1995, 11,307 patients were seen at our hospital for acute injuries: 521 were admitted to the hospital and are included in our database. Each patient's protocol consists of 103 data points including: patient identification, type, place and mechanism of injury, prehospital care, transport, assessment on admission, severity scores, diagnostic studies, injuries, treatment and morbidity-mortality. RESULTS: We found that accidents were more frequent in males (69) than in females. The predominant age group was 12-15 years (36%). Falls were the leading cause of injury (37%). A pediatric trauma score of 8 or less was seen in 13.4% of the cases. Twenty-nine patients were hospitalized in the Intensive Care Unit and 214 needed some surgical treatment. CONCLUSIONS: The utility of this registry is: 1) The knowledge of the epidemiology of our pediatric population. 2) To know and correct prehospital care deficiencies. 3) To improve hospital assistance and to recognize early prognosis factors.

[Femoral hernia in children]. / Hernia femoral en el niño.

Femoral hernia is rare in adults and even less common in children, to the point that expert surgeons have limited experience with it. Eleven patients with femoral hernia have been operated upon in our service during the past 15 years. Preoperative diagnosis was made in 6 cases (54.5%), and we underline that the ideal therapy began with this early diagnosis. We review the etiologies, clinical findings and treatment of femoral hernia in children.

[Urodynamic study of bladder dysfunction in recurrent vesico-ureteral reflux]. / Diagnóstico urodinámico de la disfunción vesical en el reflujo vesicoureteral recidivante.

In the last 5 years we have treated in our service 98 patients suffering vesicoureteral reflux (VUR) with 148 refluyents units (RU) in total. The VUR recurred in 9 ureters (6.08%) in children neurologically normal. A urodynamic study was made in them getting the following diagnosis, in 5 R. U. vesical dysfunction: 4 R. U. vesico-sphinteric dyssynergia and in 1 R. U. vesical hyperreflexia with uninhibited bladder contractions. In the remaining 4 R. U. the urodynamic study was normal.