Síndrome del microftalmos posterior-drusas papilares-retinosis pigmentaria asociado a puntos blancos. Caso clínico / Posterior microphthalmos, retinitis pigmentosa and optic disc drusen with white dots. A case report

Objetivo: Presentar el caso de un paciente con un síndrome del microftalmos posterior-drusas papilares-retinosis pigmentaria asociado por primera vez a puntos blancos en el polo posterior. Material: El síndrome del microftalmos posterior-drusas papilares-retinosis pigmentaria fue descrito por primera vez en la literatura en 1991. Posteriormente se asoció a un patrón de engrosamiento foveolar o foveosquisis. Distintas formas de mutaciones en los cromosomas 11 y 14 han sido identificadas como responsables de la aparición de este síndrome, sin que se haya podido esclarecer, hasta el momento, un patrón de herencia determinado. Discusión: Presentamos el caso de un varón de 37 años, sin antecedentes personales ni familiares de interés, con este síndrome asociado a puntos blancos en el polo posterior. Esta asociación morfológica nunca ha sido descrita en la literatura. Conclusión: El síndrome del microftalmos posterior-drusas papilares-retinosis pigmentaria es una entidad muy poco común y nunca ha sido descrita asociada a puntos blancos en el polo posterior. Es necesaria más casuística para establecer patrones claros tanto de la enfermedad como en su herencia (AU)

Objective: To present the case of a patient with a posterior microphthalmos-optic disc drusen-retinitis pigmentosa syndrome associated, for the first time, with white dots in the posterior pole. Methodology: The posterior microphthalmos, retinitis pigmentosa and optic disc drusen syndrome was described for the first time in literature in 1991. Later, it was associated with a pattern of foveal thickening and/or foveoschisis. Different forms of mutations on chromosomes 11 and 14 have been identified as being responsible for the appearance of this syndrome, but the inheritance pattern is unknown. Discussion: The case is reported of a 37 year-old man, with no personal or family history of interest, diagnosed with this syndrome in association with white dots in the posterior pole. Such a morphological association has never been published before in literature. Conclusion: The posterior microphthalmos, retinitis pigmentosa and optic disc drusen syndrome is a very rare entity, and has never been described associated with white dots in the posterior pole. More case reports are needed to establish clear patterns of both the disease and inheritance (AU)

Posterior microphthalmos, retinitis pigmentosa and optic disc drusen with white dots. A case report. / Síndrome del microftalmos posterior-drusas papilares-retinosis pigmentaria asociado a puntos blancos. Caso clínico.

OBJECTIVE: To present the case of a patient with a posterior microphthalmos-optic disc drusen-retinitis pigmentosa syndrome associated, for the first time, with white dots in the posterior pole. METHODOLOGY: The posterior microphthalmos, retinitis pigmentosa and optic disc drusen syndrome was described for the first time in literature in 1991. Later, it was associated with a pattern of foveal thickening and/or foveoschisis. Different forms of mutations on chromosomes 11 and 14 have been identified as being responsible for the appearance of this syndrome, but the inheritance pattern is unknown. DISCUSSION: The case is reported of a 37 year-old man, with no personal or family history of interest, diagnosed with this syndrome in association with white dots in the posterior pole. Such a morphological association has never been published before in literature. CONCLUSION: The posterior microphthalmos, retinitis pigmentosa and optic disc drusen syndrome is a very rare entity, and has never been described associated with white dots in the posterior pole. More case reports are needed to establish clear patterns of both the disease and inheritance.

[Transplant coordination and logistics of intra and extra-hospital cadaver donor tissue. "The Pamplona Model". Sequence of tasks performed from 1992-2006]. / Coordinación y logística del trasplante de tejidos de donantes de cadáver intra y extra-hospitalarios. "Modelo Pamplona". Cadena de actuación 1992-2006.

INTRODUCTION: Tissue and organ donations are the only option for many patients. Cerebral death (CD) facilitates this approach. However, hospitals that do not provide CD donors have to adapt in order to obtain donors, referred to as tissue donors (TD), who have died from cardiac arrest. OBJECTIVE: Is this paper it descripte the model for coordination and donation of intra and extra-hospital TD in the Autonomous Community of Navarra. It creats a program for detection, donation and extractions called the Pamplona Model, from 1992-2006. MATERIAL AND METHODS: In 1990, a transplant team was created by an Intensive Medicine Physician of HVC, INML and SOS-Navarra. In 1996, VCH Transplant Coordination is defined as a reference centre for the Tissue Transplant Programme in the Autonomous Community of Navarra. RESULTS: Consensus protocols for "intra and extra-hospital detection" of persons having died from cardiac arrest are developed: - Alerts from NHS-O hospitals, SOS-Navarra; judges and INML forensic pathologists. - Criteria for selection, search and contacts with relatives. - Alert serology, extraction and transport teams. - Logistics and distribution of tissue. - Agreed incentives: Economic, administrative and relevant regulations. CONCLUSIONS: The Pamplona Model, with the Virgen Del Camino hospital has made important contributions and is unique in the world. Intra and extra-hospital coordination of cadaver donor from a referred hospital, it is a scientific and organizational advance to have in it counts for the creation of extraction and transplant tissues teams.

[Cornea transplant]. / Trasplante de córnea.

The keratoplasty, or cornea transplant, is one of the oldest surgical techniques in opthalmology, whose indication are: 1) tectonic, in order to preserve corneal anatomy and integrity; 2) clinical, in order to eliminate the inflamed corneal tissue in cases refractory to medical treatment; 3) optical, in order to improve visual acuity; and 4) cosmetic, in order to improve the appearance of the eye. Improvements in technique and instruments, as well as in post-operative treatment and the means of preserving donated tissue, have improved survival of the grafts. The Pamplona Model of transplant coordination of the Virgen del Camino Hospital is considered to be original and unique in Spain. The logistics of this program include the protocol for detection and extraction of corneas as well as for keratoplasties.

Coordinación y logística del trasplante de tejidos de donantes de cadáver intra y extra-hospitalarios. “Modelo Pamplona”. Cadena de actuación 1992-2006 / Transplant coordination and logistics of intra and extra-hospital cadaver donor tissue. “The Pamplona Model”. Sequence of tasks performed from 1992-2006

Fundamento. La donación de tejidos y órganos constituye la única alternativa para muchos pacientes. La muerte cerebral (MC) facilita esta actitud. Aquellos hospitales no-generadores de donantes con MC, tienen que generar donantes a partir de fallecidos por parada cardiorrespiratoria, llamados donantes de tejidos (DT). En este trabajo se expone el modelo de coordinación y donación de tejidos de DT intra y extra-hospitalarios en la Comunidad Foral de Navarra. Se crea un Programa de detección, donación, extracción, denominado Modelo Pamplona, desde 1992-2006. Material y métodos. A partir de 1990 se organiza el Equipo de Trasplantes formado por un médico de la Unidad de Cuidados Intensivos del hospital Virgen del Camino, Instituto Navarro de Medicina Legal (INML) y SOS-Navarra. En 1996 la Coordinación de Trasplantes del HVC es definido como centro de referencia para el Programa de Trasplantes de Tejidos de la Comunidad de Navarra. Resultados. Se desarrollan protocolos consensuados para la detección intra y extrahospitalaria de los fallecidos en parada cardiorrespiratoria: – Alarma desde hospitales del SNS-O, SOS-Navarra, jueces y forenses del INML. – Criterios de selección, búsqueda y contacto con familiares. – Aviso a equipos de serología, extracción y transporte. – Logística y reparto de los tejidos. – Incentivos pactados: económicos, administrativos y normativa al respecto. Conclusiones. El modelo Pamplona, desde el hospital Virgen del Camino resulta positivo y es único en el mundo. La coordinación intra y extrahospitalaria de trasplantes de donantes cadáver desde un hospital de referencia, es un avance científico y organizativo a tener en cuenta para la creación de equipos de extracción y trasplante de tejidos

Introduction. Tissue and organ donations are the only option for many patients. Cerebral death (CD) facilitates this approach. However, hospitals that do not provide CD donors have to adapt in order to obtain donors, referred to as tissue donors (TD), who have died from cardiac arrest. Objective. Is this paper it descripte the model for coordination and donation of intra and extra-hospital TD in the Autonomous Community of Navarra. It creats a program for detection, donation and extractions called the Pamplona Model, from 1992-2006. Material and methods. In 1990, a transplant team was created by an Intensive Medicine Physician of HVC, INML and SOS-Navarra. In 1996, VCH Transplant Coordination is defined as a reference centre for the Tissue Transplant Programme in the Autonomous Community of Navarra. Results. Consensus protocols for “intra and extra-hospital detection” of persons having died from cardiac arrest are developed: – Alerts from NHS-O hospitals, SOS-Navarra; judges and INML forensic pathologists. – Criteria for selection, search and contacts with relatives. – Alert serology, extraction and transport teams. – Logistics and distribution of tissue. – Agreed incentives: Economic, administrative and relevant regulations. Conclusions. The Pamplona Model, with the Virgen Del Camino hospital has made important contributions and is unique in the world. Intra and extra-hospital coordination of cadaver donor from a referred hospital, it is a scientific and organizational advance to have in it counts for the creation of extraction and transplant tissues teams

Trasplante de córnea / Cornea transplant

La queratoplastia o trasplante de córnea es una de las técnicas quirúrgicas más antiguas de la oftalmología cuyas indicaciones son: 1) tectónicas, para preservar la anatomía e integridad corneal, 2) clínicas, para eliminar el tejido corneal inflamado en casos refractarios al tratamiento médico, 3) ópticas, para mejorar la agudeza visual y 4) cosméticas para mejorar el aspecto del ojo. El perfeccionamiento en la técnica y en el instrumental, así como en el tratamiento postoperatorio y en los medios de conservación del tejido donante han mejorado la supervivencia de los injertos realizados. El Modelo Pamplona de coordinación de trasplantes del Hospital Virgen del Camino (HVC) está considerado como original y único en España, y en la logística de este programa se incluye el protocolo de detección y extracción de córneas así como el de queratoplastias

The keratoplasty, or corena transplant, is one of the oldest surgical techniques in opthalmology, whose indication are: 1) tectonic, in order to preserve corneal anatomy and integrity; 2) clinical, in order to eliminate the inflamed corneal tissue in cases refractory to medical treatment; 3) optical, in order to improve visual acuity; and 4) cosmetic, in order to improve the appearance of the eye. Improvements in technique and instruments, as well as in post-operative treatment and the means of preserving donated tissue, have improved survival of the grafts. The Pamplona Model of transplant coordination of the Virgen del Camino Hospital is considered to be original and unique in Spain. The logistics of this program include the protocol for detection and extraction of corneas as well as for keratoplasties