Enfermedad de Castleman: dos variantes, dos pacientes / Castleman's disease: two variants, two patients

Introducción: La enfermedad de Castleman (EC) es un trastorno linfoproliferativo poco frecuente, localizada más frecuentemente en mediastino y abdomen. Se describen dos casos de EC de localización mediastínica presentados en nuestro servicio. El primer caso una mujer de 33 años con EC variante plasmocelular asociada a enfermedad de Hodgkin, y el segundo caso, una mujer de 32 años con EC tipo hialinovascular. Discusión: De etiología desconocida, clínicamente se distinguen dos formas de EC: a) la multicéntrica, que afecta a más de un órgano, cursa con síntomas generales y puede estar acompañada de otras afecciones como linfoma, y b) la localizada, más frecuente, que cursa de forma asintomática o con síntomas compresivos por efecto de masa. En estos casos presentados, se puede observar la diferencia en la presentación clínica de esta enfermedad en sus dos variedades. Conclusiones: La EC es una rara enfermedad linfoproliferativa cuyo tratamiento es la resección tumoral y su pronóstico es bueno (AU)

Introduction: Castleman's disease (CD) is an uncommon lymphoproliferative disorder most frequently localized in the mediastinum and abdomen. Two cases of CD with mediastinal localization in our service are described. The first case was found in a 33-year old woman with the plasma cell variant of CD associated to Hodgkin's disease and the second case was found in a 32 year old woman with hyalinevascular type CD. Discussion: CD, of unknown etiology, is clinically distinguished with two forms, multicentric that affects more than one organ and occurs with general symptoms and can be accompanied by other involvements such as lymphoma. The second one is the localized one, which is more frequent and evolves asymptomatically or with compressive symptoms due to mass effect. In these cases presented, the difference in the clinical presentation of this condition in its two variants can be seen. Conclusions: CD is an uncommon lymphoproliferative disease whose treatment is tumor resection and whose prognosis is good (AU)

[Traumatic rupture of the diaphragm]. / Rotura diafragmática traumática.

OBJECTIVE: Traumatic rupture of the diaphragm (TRD) is a rare occurrence, with variable morbidity and mortality. The aim of this study was to analyze cases of TRD in a tertiary hospital and assess prognostic factors associated with mortality. PATIENTS AND METHODS: A retrospective study was performed of patients diagnosed with TRD in Hospital Universitario La Fe, Valencia, Spain, between 1969 and 2006. The following variables were analyzed: sex, age, cause, diagnosis, associated lesions, surgical procedure, side and size of the lesion, visceral herniation, and postoperative morbidity and mortality. RESULTS: The study group comprised 132 patients (105 men, 79.5%) with a mean (SD) age of 39.64 (17.04) years. Traffic accidents were the most common cause of TRD. Rupture involved the left hemidiaphragm in 96 cases (72.7%), and 113 patients (85.6%) had associated lesions, most often affecting the abdomen. Thoracotomy was performed in 83 cases (62.9%) and laparotomy in 41 (31.1%). Visceral herniation was reported in 90 patients (68.3%), most often involving the stomach. The rates of perioperative morbidity and mortality were 62.8% and 20.5%, respectively. Diagnostic delay and the presence of morbidity and serious associated lesions all had a statistically significant impact on mortality (P< .05). In the case of serious associated lesions, the odds ratio was 2.898 (95% confidence interval, 1.018-8.250) and for perioperative morbidity it was 1.488 (95% confidence interval, 1.231-1.798). CONCLUSIONS: TRD is an infrequent occurrence in young men, is generally caused by traffic accidents, and is more common on the left side. Associated lesions are present in most cases and represent the main prognostic factor affecting morbidity and mortality. TRD can be considered a relative surgical emergency when not accompanied by other lesions that in themselves constitute surgical emergencies.

Rotura diafragmática traumática / Traumatic rupture of the diaphragm

Objetivo: La rotura diafragmática traumática (RDT) es una lesión infrecuente, con tasas variables de morbimortalidad. El objetivo del estudio ha sido analizar la experiencia en RDT de un hospital terciario y los factores pronósticos de mortalidad. Pacientes y métodos: Se ha realizado un estudio analítico y retrospectivo de los pacientes diagnosticados de RDT entre 1969 y 2006 en el Hospital La Fe. Se analizaron: sexo, edad, causa, diagnóstico, lesiones asociadas, procedimiento quirúrgico, lado y tamaño, herniación visceral y morbimortalidad postoperatoria. Resultados: Se incluyó en el estudio a 132 pacientes (105 varones; 79,5%) con una edad media ± desviación estándar de 39,64 ± 17,04 años. Los accidentes de tráfico fueron la causa más frecuente de RDT. En 96 casos (72,7%) se afectó el hemidiafragma izquierdo y 113 pacientes (85,6%) asociaron lesiones, de las cuales las abdominales fueron las más frecuentes. Se abordaron por toracotomía 83 casos (62,9%) y por laparotomía 41 (31,1%). En 90 pacientes (68,3%) se evidenció herniación visceral, siendo el estómago la más frecuente. Las tasas de morbilidad y mortalidad perioperatorias fueron del 62,8 y el 20,5%, respectivamente. La presencia de morbilidad y de lesiones asociadas graves, y el retraso diagnóstico tuvieron un impacto significativo en la mortalidad (p < 0,05. Lesiones graves: odds ratio = 2,898; intervalo de confianza del 95%, 1,018-8,250. Morbilidad perioperatoria: odds ratio = 1,488; intervalo de confianza del 95%, 1,231-1,798). Conclusiones: La RDT es una entidad infrecuente que se da en varones jóvenes, generalmente por accidentes de tráfico, y es más frecuente en el lado izquierdo. Las lesiones asociadas están presentes en la mayoría de los casos y son el principal factor pronóstico que condiciona la morbimortalidad. La RDT puede considerarse una urgencia quirúrgica diferida, en ausencia de otras lesiones que constituyan una urgencia quirúrgica en sí mismas

Objective: Traumatic rupture of the diaphragm (TRD) is a rare occurrence, with variable morbidity and mortality. The aim of this study was to analyze cases of TRD in a tertiary hospital and assess prognostic factors associated with mortality. Patients and methods: A retrospective study was performed of patients diagnosed with TRD in Hospital Universitario La Fe, Valencia, Spain, between 1969 and 2006. The following variables were analyzed: sex, age, cause, diagnosis, associated lesions, surgical procedure, side and size of the lesion, visceral herniation, and postoperative morbidity and mortality. Results: The study group comprised 132 patients (105 men, 79.5%) with a mean (SD) age of 39.64 (17.04) years. Traffic accidents were the most common cause of TRD. Rupture involved the left hemidiaphragm in 96 cases (72.7%), and 113 patients (85.6%) had associated lesions, most often affecting the abdomen. Thoracotomy was performed in 83 cases (62.9%) and laparotomy in 41 (31.1%). Visceral herniation was reported in 90 patients (68.3%), most often involving the stomach. The rates of perioperative morbidity and mortality were 62.8% and 20.5%, respectively. Diagnostic delay and the presence of morbidity and serious associated lesions all had a statistically significant impact on mortality (P<.05). In the case of serious associated lesions, the odds ratio was 2.898 (95% confidence interval, 1.018-8.250) and for perioperative morbidity it was 1.488 (95% confidence interval, 1.231-1.798). Conclusions: TRD is an infrequent occurrence in young men, is generally caused by traffic accidents, and is more common on the left side. Associated lesions are present in most cases and represent the main prognostic factor affecting morbidity and mortality. TRD can be considered a relative surgical emergency when not accompanied by other lesions that in themselves constitute surgical emergencies

Carcinoma broncogénico e infección por virus de la inmunodeficiencia humana / Bronchogenic carcinoma and virus infection in human immunodeficiency

Propósito: Mostrar nuestra experiencia en pacientes con carcinoma broncogénico (CB) e infección por el virus de la inmunodeficiencia humana (HIV).- Pacientes y método: Se presentan cuatro pacientes con CB y VIH tratados mediante cirugía. - Resultados: La edad media fue de 43 años y tres pacientes tenían antecedentes tuberculosos. La tasa media de CD4 fue de 211/mm3. Se realizaron tres lobectomías y una neumonectomía. El diagnóstico histológico más frecuente fue el de carcinoma epidermoide. Sólo un enfermo vive en la actualidad tras cinco años de la intervención. - Conclusión: La aparición de un CB en pacientes HIV, población con una alta incidencia de patología pulmonar por infecciones oportunistas, es cada día más frecuente por lo que hay que tener en cuenta la posible asociación de ambos procesos (AU)

[Postoperative survival in stage II non-small-cell bronchogenic carcinoma]. / Supervivencia posquirúrgica en el estadio II del carcinoma broncogénico no anaplásico de células pequeñas.

OBJECTIVE: This study aimed to validate in our population changes in the stage II criteria for non-small cell bronchogenic carcinoma. PATIENTS AND METHODS: We retrospectively reviewed and followed the course of disease in 336 patients who underwent complete resection in our hospital between January 1969 and December 1995 with stage II disease, classified as T1N1M0 (41), T2N1M0 (144) and T3N0M0 (151). RESULTS: The expected five-year survival in our population was 43.19 +/- 2.90%. Estimated mean survival was 3 +/- 0.71 years (95% confidence interval: 1.60-4.40). Mean survival was 8.82 +/- 0.67 years (95% confidence interval 7.51-10.13). Five-year survival was 53.32 +/- 8.55% for tumors classified as T1N1M0, 38.57 +/- 4.40% for T2N1M0, and 44.46 +/- 4.30% for T3N0M0. We observed significant differences in survival depending on histological type, tumor size, and IIA or IIB staging, degree of tumor invasion (T), number of nodes involved (N1) and location. T3N0M0 tumors displayed great variation in expected survival rates in relation to structures involved (27.53% to 59.98%). Multivariate analysis confirmed degree of tumor invasion, size and histological type to be the main prognostic factors. CONCLUSIONS: We conclude that the new staging system gives a more realistic prognosis for patients in our practice. The stage IIA and IIB division is appropriate and gives significantly different prognoses. However, the T3N0M0 category is heterogeneous and is not significantly different from T1-2N1M0, such that stage II overall continues to be an indivisible, homogeneous group of patients. Other prognostic variables, such as histological type, affect survival in our patients.

Supervivencia posquirúrgica en el estadio II del carcinoma broncogénico no anaplásico de células pequeñas / Postoperative survival in stage II non-small cell bronchogenic carcinoma

Objetivo: El presente trabajo trata de validar en nuestra población las modificaciones del estadio II del nuevo sistema de estadificación del carcinoma broncogénico no anaplásico de células pequeñas. Pacientes y métodos: Revisamos retrospectivamente y seguimos en su evolución a 336 pacientes operados con resección completa en nuestro hospital desde enero de 1969 a diciembre de 1995, con estadio II patológico y distribuidos como T1N1M0 (41), T2N1M0 (144) y T3N0M0 (151). Resultados: La supervivencia esperada en nuestra población fue de 43,19 ñ 2,90 por ciento a los 5 años. La estimación de la mediana fue de 3 ñ 0,71 años (intervalo de confianza [IC] del 95 por ciento, 1,60-4,40). El tiempo medio de supervivencia fue de 8,82 ñ 0,67 años (IC del 95 por ciento, 7,51-10,13). Los tumores clasificados como T1N1M0 presentaron una supervivencia del 53,32 ñ 8,55 por ciento a los 5 años; en los T2N1M0 el porcentaje fue del 38,57 ñ 4,40 por ciento, y en los T3N0M0 fue del 44,46 ñ 4,30 por ciento. Encontramos diferencias significativas entre supervivencias en función del tipo histológico, tamaño tumoral, estadio II A o II B, grado de invasión tumoral (T), número de ganglios afectados (N1) y localización de los mismos. Los tumores clasificados como T3N0M0 presentaron una amplia variabilidad en los porcentajes esperados de supervivencia a los 5 años en función de las estructuras afectadas (27,53 a 59,98 por ciento). Un análisis multivariante confirmó como principales factores pronósticos el grado de invasión tumoral, el tamaño tumoral y el tipo histológico. Conclusiones: El nuevo sistema de estadificación está más cerca de la realidad pronóstica de los pacientes en nuestra población. La división en estadio II A y II B es adecuada y presenta diferencias pronósticas significativas. Sin embargo, el apartado T3N0M0 es heterogéneo y no presenta diferencias significativas respecto al T1-2N1M0, por lo que el estadio II, en conjunto, continúa sin estar configurado por un grupo homogéneo de pacientes. Otras variables pronósticas, como el tipo histológico, condicionaron la supervivencia en nuestra población. (AU)

[Prognosis of T3N0M0 small-cell non-anaplastic bronchogenic carcinoma]. / Pronóstico del carcinoma broncogénico no anaplásico de células pequeñas T3N0M0.

We analyzed the survival after surgery for non-small cell lung cancer (NSCLC) classified as T3N0. Between January 1969 and 1995, 151 patients underwent surgery for NSCLC in our hospital. Survival analysis was performed using the Kaplan-Meier statistical method and the curves were compared using Mantel-Cox, Breslow and Tarone-Ware tests. The estimated five-year survival in the studied population was 44.46 +/- 4.30%. Four groups were defined based on degree of tumoral invasion of mediastinal structures, parietal pleura, chest wall or superior sulcus. Significant differences in five-year survival were observed between groups. Patients in the mediastinal group (59.98 +/- 8.71%) had the best prognosis, followed by patients with parietal pleura involvement (52.79 +/- 6.69%). Survival in the chest wall group was 27.53 +/- 7.22%. No patients with superior sulcus tumors survived over five years (median survival 1.50 +/- 1.16 years; 95% confidence interval 0.00 to 3.77 years). Prognosis is clearly determined by degree of tumoral invasion in T3N0 patients. In spite of the evident conceptual improvements achieved with the revised International Staging System, the system still fails to fully define prognosis in such cases.

Pronóstico del carcinoma broncogénico no anaplásico de células pequeñas T3N0M0 / Prognosis of non-small cell lung cancer classified as T3N0M0

Estudiamos la supervivencia posquirúrgica del carcinoma broncogénico no anaplásico de células pequeñas (CB-NACP) clasificado como T3N0. Para ello seguimos prospectivamente a 151 pacientes intervenidos por este motivo en nuestro hospital desde enero de 1969 a diciembre de 1995. El análisis de la supervivencia se realizó por el método estadístico de Kaplan-Meier, y las curvas fueron comparadas empleando los tests de Mantel-Cox, Breslow y Tarone-Ware. El porcentaje de supervivencia esperado en nuestra población fue del 44,46 ñ 4,30 por ciento a los 5 años. En función del grado de invasión tumoral definimos cuatro grupos de pacientes según el tumor afectase a estructuras mediastínicas, pleura parietal, pared costal o sulcus superior. Los porcentajes de supervivencia a 5 años pusieron de manifiesto diferencias significativas entre grupos con un mejor pronóstico para los enfermos del grupo mediastínico (59,98 ñ 8,71 por ciento), seguido de la afectación de pleura parietal (52,79 ñ 6,69). Entre los casos del grupo de pared, la supervivencia fue del 27,53 ñ 7,22 por ciento, mientras que entre los pacientes con tumor de sulcus superior ninguno sobrevivió por encima de los 5 años (mediana de supervivencia de 1,50 ñ 1,16 años; límites del intervalo de confianza del 95 por ciento 0,00-3,77 años). En conclusión, en los T3N0 el mal pronóstico está determinado por el grado de invasión tumoral y, a pesar de las evidentes mejoras conceptuales conseguidas con la nueva revisión del International Staging System (ISS), éste continúa sin definir completamente el pronóstico de la afectación T3N0. (AU)

[The indications for and results of video thoracoscopic surgery. Reflections on 152 procedures]. / Indicaciones y resultados de la cirugía videotoracoscópica. Consideraciones sobre 152 procedimientos.

Since thoracoscopy was first described in 1910, its application has been confined mainly to diagnosis and symptomatic treatment of pleural diseases. Recent technological advances in endoscopy and the refinement of surgical technique have brought wider applications, giving rise to video-assisted thoracoscopy (VAT). VAT surgery allows us to view, access and act upon internal thoracic organs without recourse to thoracotomy, thus circumventing inherent risk. We have reviewed our experience from April 1994 through November 1998 in 152 procedures with 141 consecutive patients. Diagnoses were pneumothorax in 94 cases, sympathetic nervous system alteration in 10, diffuse lung disease in 10, lung tumors in 9, pulmonary metastasis in 4, pleural tumors in 5, mediastinal tumors in 2, pericardial effusion in 2, spinal disease in 2 and chronic pancreatitis in 1. No deaths associated with the procedure occurred. The incidence of non-fatal postoperative complication was 11%. The most common complications were prolonged air leak (5%) and bloody pleural effusion (3.5%). The mean length of postoperative hospital care was 3.8 days (range 1 to 18 days). Our experience indicates that VAT is increasingly used to diagnose and treat a variety of chest lesions. Complications are fewer than in procedures in which thoracotomy is needed. Prolonged air leakage does not occur significantly more often with VAT than with thoracotomy. VAT is apparently safe and is particularly useful in some situations, as postoperative morbidity is low and clinical tolerance good.

[1990-1996: the experience of the La Fe Lung Transplant Group (Valencia)]. / 1990-1996: experiencia del Grupo de Trasplante Pulmonar La Fe (Valencia).

Objective to review the experience of the lung transplantation unit at Hospital La Fe (Valencia). Between February 1990 and March 1996 we performed 40 lung transplants. The following causes were most common: cystic fibrosis (9 cases), emphysema (8), pulmonary fibrosis (8) and bronchiectasis (7). Types of intervention were 27 double lung transplants (25 sequential and 9 blocked), 9 single lung transplants, and 4 heart-lung transplants. We then reviewed the 36 single and double lung transplants. The main exclusion criteria were age over 65 years, malignant disease, kidney or liver disease, severe or non reversible central nervous system disease, and drug addiction. Prior surgery, mechanical ventilation and the presence of Aspergillus were considered lower-order contraindications. Mean patient age was 37.7 years (14-59). Six patients were colonized by Aspergillus before transplantation. Five had undergone earlier surgery and two were mechanically ventilated before the transplant. The most common complication was respiratory infection, which was present in 6 of the 7 patients who died. Other complications in order of frequency were dehiscence and/or bronchial stenosis, corticoid myopathy and postoperative bleeding. The actuarial survival rate of single and double lung transplants was 67.85 after 3 years, and 87.5% in patients with cystic fibrosis. Lung transplantation is a well-established procedure that is gradually being extended to treat more conditions. The main obstacle is the scarcity of donors. The main challenge at present is bronchiolitis obliterans.

[Benign acquired tracheoesophageal fistulas]. / Fístulas traqueoesofágicas benignas adquiridas.

Benign acquired tracheoesophageal fistula (TEF) is a rare though serious occurrence associated with a high degree of morbidity and mortality. We review 18 cases of TEF treated over 16 years in our hospital. The variables analyzed descriptively were demographic (age, sex and others), clinical (etiology, time of intubation, time and symptoms leading to clinical suspicion, diagnostic techniques, and others), and therapeutic (dependence on mechanical ventilation, location of tissues, tracheal resection, tissue interposition, postoperative course of disease, and others). Fifteen of the 18 patients required surgery. The rate of success (80%) was high in terms of respiration, swallowing and phonation. Surgery involved simple closure of the TEF, with half the patients requiring a second operation to correct tracheal stenosis. Resection and anastomosis were performed in the remaining 11 cases, with only one requiring a second operation for recurrence of stenosis. Two cases of perioperative mortality and one TEF recurrence related to assisted ventilation were recorded. Tracheoscopy was the most effective diagnostic technique, complemented by computerized tomography of the trachea. Presurgical evaluation of tracheal stenosis, the absence of mechanical ventilation, preoperative preparation and postoperative care are the factors that determine success in this type of surgery. When tracheal stenosis is associated with TEF, resection and anastomosis should be performed in the affected zone.

[Lung transplantation in cystic fibrosis]. / El trasplante pulmonar en la fibrosis quística.

OBJECTIVE: Since 1990 we have performed 40 lung transplants in the Hospital "La Fe" in Valencia. Nine of them have been performed in cystic fibrosis patients, which is the subject of this paper. PATIENTS AND METHODS: The mean age of the patients was 19.8 years, with the youngest patient being 14 years of age. In regards to patient selection, it is important to mention that one had a previous lobectomy, another one a thoracic deformity due to long term atelectasis and one needed intubation for hemoptysis within the 7 days before the lung transplant. Prophylaxis with imipenem and cyprofloxicin, aerosolized colistin and amphotericin B, prompt weaning and intensive respiratory physiotherapy were important for controlling postoperative infection. RESULTS: With 15.3 months as the mean follow-up (range 36-3), 3 year survival was 87.5%. Pulmonary infection, which was the most frequent complication, had a good response to adequate antibiotic treatment. The main postoperative problem pertained to the bronchial suture with 2 partial dehiscences, 2 stenoses and one bronchopleural fistula by Aspergillus, all of which were resolved with conservative procedures without surgery. CONCLUSIONS: Middle and long term evolution in these patients shows an excellent quality of life with spirometric and ergometric tests within the normal range.