Gastric pneumatosis, an uncommon complication of a lung transplant, prognosis, diagnostic and treatment management.

We present a case of a 40-year-old woman with double-lung transplant with extracorporeal circulation (EC) due to pulmonary hypertension (pulmonary veno-occlusive disease form (PVOD)) secondary to mixed connective tissue disease (MCTD). On day 6 postoperatively, abdominal pain and distension was noticed, since abdominal CT scan was performed, showing emphysematous gastritis with gastric wall ischemia and peritonitis. Therefore, emergent surgery was proposed. By open surgery approach, a total gastrectomy with Roux-en-Y and esophagojejunal anastomosis was performed. On day 6 after gastrectomy, intraluminal bleeding of the esophagojejunal anastomosis was detected in control CT, which was not need any aggressive treatment. Postoperative evolution was favourable, being discharged from the intensive care unit on the day 34 and from the hospital two months later.

Doble vena cava inferior con doble vena renal izquierda / Double inferior vena cava with double left renal vein

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Trasplante hepatorrenal simultáneo en pacientes adultos con hiperoxaluria primaria. Experiencia del Hospital Universitario 12 de Octubre / Liver-kidney simultaneous transplantation in adult patients with primary hyperoxaluria. Experience at Hospital Universitario 12 de Octubre

La hiperoxaluria primaria es un trastorno del metabolismo autosómico recesivo que origina una hiperproducción hepática de oxalato, que no puede ser metabolizado por el hígado y se elimina por vía renal formando litiasis, nefrocalcinosis y ocasionando un deterioro progresivo y precoz de la función renal que, generalmente, a pesar del tratamiento médico precisará de una terapia renal sustitutiva. La hiperoxaluria primaria (HOP) tipo 1 es el trastorno más frecuente, se debe a un déficit de la enzima alanina-glicolato aminotransferasa que se encuentra en los peroxisomas hepáticos. Por tanto, el trasplante hepatorrenal simultáneo (THRS) es el tratamiento definitivo para los pacientes con enfermedad renal crónica terminal. Sin embargo, algunos resultados sugieren que la morbimortalidad es mayor al realizar este procedimiento frente al trasplante renal aislado. Se presentan cinco pacientes adultos con hiperoxaluria primaria y un filtrado glomerular medio de 20,2 ± 1,3 ml/min/1,73 m2 a los que se les realizó un THRS entre 1999 y 2015 en el Hospital Universitario 12 de Octubre. No se observó recurrencia de la enfermedad ni pérdida del injerto hepático o renal durante el postoperatorio y únicamente un episodio de rechazo agudo tardío sin pérdida del injerto renal. La supervivencia de los receptores fue del 100% con una mediana de seguimiento de 84 meses. Debido a que el THRS permite la curación de la enfermedad y constituye una técnica segura, con una baja morbimortalidad y elevada supervivencia, debe considerarse como el tratamiento de elección en la hiperoxaluria primaria con enfermedad renal terminal (AU)

Primary hyperoxaluria (PH) is a metabolic liver disease with an autosomal recessive inheritance that results in oxalate overproduction that cannot be metabolized by the liver. Urinary excretion of oxalate results in lithiasis and nephrocalcinosis leading to a progressive loss of renal function that often requires renal replacement therapy despite medical treatment. Type 1 PH is the most common form and is due to a deficiency in the alanine-glycolate aminotransferase enzyme found in hepatic peroxisomes. Therefore, a liver-kidney simultaneous transplant (LKST) is the definitive treatment for end-stage renal disease (ESRD) patients. However, some studies suggest that the morbidity and mortality rates are greater when this procedure is performed instead of only a kidney transplant (IKT). Herein, we report five patients with PH and a mean glomerular filtration rate of 20.2 ± 1.3 ml/min/1.73 m2 who received a LKST between 1999 and 2015 at the Hospital Universitario 12 de Octubre. Recurrence and liver or kidney graft loss was not observed during the postoperative period and only one case of late acute rejection without graft loss was diagnosed. The recipient survival rate was 100% with a median follow up of 84 months. As LKST is a curative and safe procedure with a low mortality and high survival rate, it must be considered as the treatment of choice in adults with HP and ESRD (AU)

Trasplante de páncreas-riñón simultáneo. Experiencia del Hospital Doce de Octubre / Simultaneous pancreas-kidney transplantation. Experience of the Doce de Octubre Hospital

Introducción: El trasplante de páncreas-riñón simultáneo constituye el tratamiento de elección en la diabetes tipo 1 o tipo 2 con fallo renal terminal o preterminal (diálisis o prediálisis), por ser la única terapia que consigue el estado euglucémico (insulino-independiente) en el paciente diabético. Métodos: Estudio retrospectivo y descriptivo de una serie de 175 pacientes trasplantados de páncreas-riñón simultáneo entre marzo de 1995 y abril de 2016. Se analizan las características de los donantes y receptores, variables perioperatorias e inmunosupresión, morbimortalidad postrasplante, supervivencia del paciente e injerto y factores de riesgo de supervivencia del paciente e injerto. Resultados: La mediana de edad de los donantes fue de 28 años y la media de los receptores, de 38,8 ± 7,3años, siendo 103 hombres y 72 mujeres. La derivación duodeno-entérica se realizó en 113 casos y la duodeno-vesical, en 62. Las tasas de complicaciones postrasplante fueron las siguientes: infección global (70,3%), pancreatitis del injerto (26,3%), hemorragia intraabdominal (17,7%), trombosis del injerto (12,6%) y rechazo pancreático global (10,9%). Las causas de mortalidad fueron fundamentalmente cardiovasculares e infecciosas. La supervivencia del paciente a 1, 3 y 5 años fue del 95,4, del 93 y del 92,4%, respectivamente, mientras que la del injerto correspondió al 81,6, al 77,9 y al 72,3%, respectivamente, durante el mismo periodo. Conclusiones: En nuestra experiencia de 20 años de trasplante pancreático-renal simultáneo las tasas de morbilidad y supervivencia del paciente y del injerto a 5 años son similares a las referidas en los registros internacionales de trasplante pancreático (AU)

Introduction: Simultaneous pancreas-kidney transplantation (SPKT) constitutes the therapy of choice for diabetes type 1 or type 2 associated with end-stage renal disease, because is the only proven method to restore normo-glicemic control in the diabetic patient. Methods: Retrospective and descriptive study of a series of 175 patients who underwent SPKT from March 1995 to April 2016. We analyze donor and recipient characteristics, perioperative variables and immunosuppression, post-transplant morbi-mortality, patient and graft survival, and risk factors related with patient and graft survival. Results: Median age of the donors was 28 years and mean age of recipients was 38.8 ± 7.3 years, being 103 males and 72 females. Enteric drainage of the exocrine pancreas was performed in 113 patients and bladder drainage in 62. Regarding post-transplant complications, the overall rate of infections was 70.3%; graft pancreatitis 26.3%; intraabdominal bleeding 17.7%; graft thrombosis 12.6%; and overall pancreas graft rejection 10.9%. The causes of mortality were mainly cardiovascular and infectious complications. Patient survival at 1, 3 and 5-year were 95.4%, 93% and 92.4%, respectively, and pancreas graft survival at 1, 3 and 5-year were 81.6%, 77.9% y 72.3%, respectively. Conclusions: In our 20-year experience of simultaneous pancreas-kidney transplantation, the morbidity rate, and 5-year patient and pancreas graft survivals were similar to those previously reported from the international pancreas transplant registries (AU)

Analyzing predictors of graft survival in patients undergoing liver transplantation with donors aged 70 years and over.

AIM: To increase the number of available grafts. METHODS: This is a single-center comparative analysis performed between April 1986 and May 2016. Two hundred and twelve liver transplantation (LT) were performed with donors ≥ 70 years old (study group). Then, we selected the first cases that were performed with donors < 70 years old immediately after the ones that were performed with donors ≥ 70 years old (control group). RESULTS: Graft and patient survivals were similar between both groups without increasing the risk of complications, especially primary non-function, vascular complications and biliary complications. We identified 5 risk factors as independent predictors of graft survival: recipient hepatitis C virus (HCV)-positivity [hazard ratio (HR) = 2.35; 95% confidence interval (CI): 1.55-3.56; P = 0.00]; recipient age (HR = 1.04; 95%CI: 1.02-1.06; P = 0.00); donor age X model for end-stage liver disease (D-MELD) (HR = 1.00; 95%CI: 1.00-1.00; P = 0.00); donor value of serum glutamic-pyruvic transaminase (HR = 1.00; 95%CI: 1.00-1.00; P = 0.00); and donor value of serum sodium (HR = 0.96; 95%CI: 0.94-0.99; P = 0.00). After combining D-MELD and recipient age we obtained a new scoring system that we called DR-MELD (donor age X recipient age X MELD). Graft survival significantly decreased in patients with a DR-MELD score ≥ 75000, especially in HCV patients (77% vs 63% at 5 years in HCV-negative patients, P = 0.00; and 61% vs 25% at 5 years in HCV-positive patients; P = 0.00). CONCLUSION: A DR-MELD ≥ 75000 must be avoided in order to obtain the best results in LT with donors ≥ 70 years old.

Liver-kidney simultaneous transplantation in adult patients with primary hyperoxaluria. Experience at Hospital Universitario 12 de Octubre.

Primary hyperoxaluria (PH) is a metabolic liver disease with an autosomal recessive inheritance that results in oxalate overproduction that cannot be metabolized by the liver. Urinary excretion of oxalate results in lithiasis and nephrocalcinosis leading to a progressive loss of renal function that often requires renal replacement therapy despite medical treatment. Type 1 PH is the most common form and is due to a deficiency in the alanine-glycolate aminotransferase enzyme found in hepatic peroxisomes. Therefore, a liver-kidney simultaneous transplant (LKST) is the definitive treatment for end-stage renal disease (ESRD) patients. However, some studies suggest that the morbidity and mortality rates are greater when this procedure is performed instead of only a kidney transplant (IKT). Herein, we report five patients with PH and a mean glomerular filtration rate of 20.2 ± 1.3 ml/min/1.73 m2 who received a LKST between 1999 and 2015 at the Hospital Universitario 12 de Octubre. Recurrence and liver or kidney graft loss was not observed during the postoperative period and only one case of late acute rejection without graft loss was diagnosed. The recipient survival rate was 100% with a median follow up of 84 months. As LKST is a curative and safe procedure with a low mortality and high survival rate, it must be considered as the treatment of choice in adults with HP and ESRD.

Simultaneous pancreas-kidney transplantation. Experience of the Doce de Octubre Hospital. / Trasplante de páncreas-riñón simultáneo. Experiencia del Hospital Doce de Octubre.

INTRODUCTION: Simultaneous pancreas-kidney transplantation (SPKT) constitutes the therapy of choice for diabetes type1 or type2 associated with end-stage renal disease, because is the only proven method to restore normo-glicemic control in the diabetic patient. METHODS: Retrospective and descriptive study of a series of 175 patients who underwent SPKT from March 1995 to April 2016. We analyze donor and recipient characteristics, perioperative variables and immunosuppression, post-transplant morbi-mortality, patient and graft survival, and risk factors related with patient and graft survival. RESULTS: Median age of the donors was 28years and mean age of recipients was 38.8±7.3years, being 103 males and 72 females. Enteric drainage of the exocrine pancreas was performed in 113 patients and bladder drainage in 62. Regarding post-transplant complications, the overall rate of infections was 70.3%; graft pancreatitis 26.3%; intraabdominal bleeding 17.7%; graft thrombosis 12.6%; and overall pancreas graft rejection 10.9%. The causes of mortality were mainly cardiovascular and infectious complications. Patient survival at 1, 3 and 5-year were 95.4%, 93% and 92.4%, respectively, and pancreas graft survival at 1, 3 and 5-year were 81.6%, 77.9% y 72.3%, respectively. CONCLUSIONS: In our 20-year experience of simultaneous pancreas-kidney transplantation, the morbidity rate, and 5-year patient and pancreas graft survivals were similar to those previously reported from the international pancreas transplant registries.

Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment. / Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico.

INTRODUCTION: Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors. METHODS: A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014. RESULTS: Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer. CONCLUSIONS: Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery.

Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico / Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment

Introducción: El tumor mucinoso de apéndice tiene una incidencia inferior al 0,5% entre todos los tumores digestivos. Suele presentarse en la sexta década de la vida, con una clínica parecida a la de la apendicitis aguda. El objetivo de este estudio es describir los aspectos demográficos, clínicos, diagnósticos y terapéuticos de estos tumores. Además, se analiza la asociación entre tumores mucinosos con pseudomixoma peritoneal (PP) y la relación que presentan con los tumores de ovario y colorrectales. Métodos: Se realizó un estudio retrospectivo de todas las apendicectomías practicadas en nuestro centro desde diciembre de 2003 hasta diciembre de 2014. Resultados: Entre 7.717 apendicectomías diagnosticamos un tumor mucinoso apendicular en 72 pacientes, lo que representa una incidencia de 0,9%. La edad media era de 64 años; eran mujeres el 62% y hombres, el 38%. El diagnóstico fue incidental en el 43% de los casos. El PP se presentó en 16 casos (22%), con una asociación estadísticamente significativa entre este tumor y el tumor de bajo potencial maligno. La cirugía programada se realizó en 42 casos y la urgente en 30. De los 72 tumores mucinosos del apéndice, 22 (30,5%) también presentaron cáncer de colon sincrónico o metacrónico. Conclusiones: Los tumores mucinosos de apéndice son con frecuencia hallazgos incidentales. El PP se asocia con un tumor mucinoso de bajo potencial maligno y el tratamiento puede comprender desde una apendicectomía hasta una cirugía citorreductiva, dependiendo del grado histológico del tumor y de la diseminación peritoneal (AU)

Introduction: Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors. Methods: A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014. Results: Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer. Conclusions: Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery (AU)

Candidiasis intraabdominal masiva secundaria a cirugías digestivas en pacientes inmunocompetentes. A propósito de 2 casos / Massive intra-abdominal candida infection secondary to digestive surgery in inmunocompetent patients: A report on 2 cases

La perforación asociada a infección intraabdominal difusa por Candida spp. es excepcional. Suele asociarse a pacientes inmunodeprimidos o con enfermedad tumoral avanzada. Presentamos 2 casos de perforación digestiva secundaria a candidiasis invasiva. En el primer caso, una mujer de 68 años con una perforación duodenal secundaria a Candida spp., se realiza laparotomía exploradora y reparación de la perforación duodenal. Sin embargo, la paciente requiere más de 2 intervenciones, observándose Candida spp. macroscópica diseminada por toda la cavidad abdominal. El segundo caso es el de un varón de 60 años que presenta un postoperatorio complicado de una hemicolectomía derecha, que se asocia con pancretitis, y con posterior diseminación fúngica abdominal secundaria a Candida parapsilopsis, con múltiples complicaciones infecciosas. En ambos casos se intentó un tratamiento basado en resección quirúrgica y cambio de anti-fúngicos, sin éxito. El tratamiento antifúngico precoz evita la diseminación hematógena y el shock séptico, disminuyendo la morbimortalidad de estos pacientes.

Candida spp. as cause of diffuse intraabdominal infection is very rare. Often associated with immunocompromised or patients with advanced tumor disease. We are reporting 2 cases of gastrointestinal perforation secondary to invasive candidiasis. The first case, a 68 years old female with a Candida spp. duodenal perforation. An emergency exploratory laparotomy was performed and a duodenal perforation repair was done. However, the patient required 2 more reoperation due to Candida spp. macroscopic intra-abdominal disemination. The second case, is presented in the context of a postoperative period of a right hemicolectomy, pancreatitis associating abdominal spread and subsequent secondary fungal Candida parapsilopsis with multiple infectious complications. In both cases there were unsuccessful surgical resection and antifungal change. The early antifungal treatment prevents hematogenous dissemination and septic shock, reducing the morbidity and mortality of these patients.

Fibromatosis mesentérica con trombosis completa de la aorta como indicación de trasplante intestinal / Mesenteric fibromatosis with complete aortic thrombosis as an indication for intestinal transplant

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Incidence, risk factors and outcome of de novo tumors in liver transplant recipients focusing on alcoholic cirrhosis.

Orthotopic liver transplantation (OLT) is an established life-saving procedure for alcoholic cirrhotic (AC) patients, but the incidence of de novo tumors ranges between 2.6% and 15.7% and is significantly increased in comparison with patients who undergo OLT for other etiologies. Tobacco, a known carcinogen, has been reported to be between 52% and 83.3% in AC patients before OLT. Other risk factors that contribute to the development of malignancies are dose-dependent immunosuppression, advanced age, viral infections, sun exposure, and premalignant lesions (inflammatory bowel disease, Barrett's esophagus). A significantly more frequent incidence of upper aerodigestive (UAD) tract, lung, skin, and kidney-bladder tumors has been found in OLT recipients for AC in comparison with other etiologies. Liver transplant recipients who develop de novo non-skin tumors have a decreased long-term survival rate compared with controls. This significantly lower survival rate is more evident in AC recipients who develop UAD tract or lung tumors after OLT mainly because the diagnosis is usually performed at an advanced stage. All transplant candidates, especially AC patients, should be encouraged to cease smoking and alcohol consumption in the pre- and post-OLT periods, use skin protection, avoid sun exposure and over-immunosuppression, and have a yearly otopharyngolaryngeal exploration and chest computed tomography scan in order to prevent or reduce the incidence of de novo malignancies. Although still under investigation, substitution of calcineurin inhibitors for sirolimus or everolimus may reduce the incidence of de novo tumors after OLT.

Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años / Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years

Fundamento y objetivo: La polineuropatía amiloidótica familiar (PAF) constituye el tipo más prevalente de amiloidosis sistémica hereditaria. Es una enfermedad autosómica dominante que se caracteriza por el depósito de una variante anómala de la transtiretina. Tiene una distribución mundial, con zonas endémicas localizadas en Portugal, Suecia y Japón. En España hay un foco endémico, localizado en Mallorca. El trasplante hepático es la única opción curativa para los pacientes con PAF. El objetivo de este estudio fue describir las características clínicas y demográficas de los pacientes trasplantados con diagnóstico de PAF. Material y método: Se evaluaron 6 pacientes trasplantados por PAF entre abril de 1986 y diciembre de 2012. Resultados: La edad media fue de 57,7 + 16 años, los pacientes de origen español eran mayores de 60 años. Todos los pacientes presentaban síntomas progresivos en forma de polineuropatía mixta. En 2 pacientes se realizó un doble trasplante hepatocardiaco secuencial, efectuándose en primer lugar el trasplante hepático. La supervivencia del paciente y del injerto fue del 80% a los uno, 3 y 5 años. Conclusiones: El único tratamiento etiológico eficaz para la PAF es el trasplante hepático. Una detección temprana es la clave para el tratamiento y el control, evitándose el daño orgánico irreversible (AU)

Background and objective: Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. Material and method: Six patients with PAF underwent liver transplantation between April 1986 and December 2012. Results: The mean age was 57.7 + 16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart-liver transplants sequentially were performed. Patient survival and graft was 80% at one, 3 and 5 years. Conclusions: The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage (AU)

Presentación clínica de adenocarcinoma de ductos anales como estenosis anal benigna / Clinical presentation of anal duct adenocarcinoma as benign anal stenosis

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[Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years]. / Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años.

BACKGROUND AND OBJECTIVE: Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. MATERIAL AND METHOD: Six patients with PAF underwent liver transplantation between April 1986 and December 2012. RESULTS: The mean age was 57.7+16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart-liver transplants sequentially were performed. Patient survival and graft was 80% at one, 3 and 5 years. CONCLUSIONS: The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage.