Linfedema genital crónico gigante. Contribución de 2 casos, abordaje quirúrgico y revisión de la literatura / Chronic giant genital lymphedema: contribution of 2 cases, description of surgical approach and literature review

Objetivo. Exponer una descripción detallada de la técnica quirúrgica para el linfedema genital crónico gigante. Comentar otras opciones quirúrgicas de extirpación y reconstrucción. Método. Se presentan los casos de 2 varones con linfedema genital crónico gigante de más de 2 años de evolución, con gran afectación funcional y un estado de ánimo y autoestima bajos. Se realiza una descripción por pasos de la técnica quirúrgica y los cuidados posteriores. Resultados. Se realizó la extirpación completa del linfedema genital crónico gigante hasta tejido sano, la disminución de la oquedad condicionada por la extirpación, con flaps de piel y tejido celular subcutáneo, y la reparación del defecto de la piel con injertos cutáneos libres de espesor parcial obtenidos del abdomen. El resultado estético fue muy bueno y se obtuvo un magnífico resultado psicológico, con mejoría de la autoestima y el estado de ánimo. Conclusiones. El tratamiento quirúrgico en casos avanzados es la única opción posible. Con la técnica descrita se obtienen buenos resultados (estéticos, funcionales y psicológicos) (AU)

Aim. To provide a detailed description of the surgical technique for the chronic giant genital lymphedema. To explain other surgical methods of removal and reconstruction. Method. Based in 2 males with a chronic giant genital lymphedema with more than 2 years of evolution, with high functional impairment, low mood and low self-esteem. Description, step by step, of the surgical procedures and postoperative care. Results. The removal of the chronic giant genital lymphedema up to healthy tissue was performed. Reduction of the cavity conditioned by the removal, with flaps of skin and subcutaneous tissue. Repair of skin defect with partial thickness skin grafts obtained from the abdomen. Very good aesthetic result and splendid psychological outcome with improved self-esteem and mood. Conclusions. Surgical treatment in advanced cases is the only option. With the described technique good results (aesthetic, functional and psychological) are obtained (AU)

Retroperitoneal fibrosis: single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature.

OBJECTIVE: The aims of this study were to investigate retroperitoneal fibrosis in a Spanish hospital and present a review of the international literature to attempt to elucidate a diagnostic and therapeutic approach to this unusual pathology. MATERIAL AND METHODS: A database search was performed in the pathology department and in the documentation service using the key words "retroperitoneal fibrosis" and "Ormond's disease", limiting the search to the years 1990-2010. Cases in which secondary retroperitoneal fibrosis was considered were excluded. In addition, a PubMed literature search was performed using the terms "retroperitoneal fibrosis" and "Ormond's", limiting the search to 1985-2011. RESULTS: Twenty-two patients were diagnosed with idiopathic retroperitoneal fibrosis (IRF) or Ormond's disease. The most common symptom at the time of diagnosis was flank pain. With regard to laboratory findings, five patients (22.7%) had anaemia and eight (36.3%) had renal failure. Computed tomography (CT) was performed in 20 patients (90.9%) and the most common finding observed was retroperitoneal mass. Eighteen patients were started on corticosteroids, in six cases in association with azathioprine. Three patients had recurrence at 12, 24 and 72 months, respectively, and 15 patients required emergency surgery. Nine open surgical procedures were performed. CONCLUSIONS: At present, IRF is considered an autoimmune disease that presents with local and systemic signs and symptoms. CT and magnetic resonance imaging are the two tests of choice in IRF diagnosis and follow-up. [(18)F]Fluorodeoxyglucose positron emission tomography is starting to be used for assessment and treatment response. A combination of medical and surgical treatment is usually applied. It is essential to administer corticosteroids alone or in association with other immunosuppressive drugs such as azathioprine. Laparoscopic ureterolysis, or robotic ureterolysis, if available, is the technique of choice.