High prevalence of venous thrombotic events in Cushing's syndrome: data from ERCUSYN and details in relation to surgery.

OBJECTIVE: The aim of this study was to evaluate the prevalence of venous thromboembolism (VTE) in patients included in the European Registry on Cushing's syndrome (ERCUSYN), compare their clinical characteristics with those who did not develop VTE and identify risk factors for VTE. DESIGN: A retrospective observational cohort study. METHODS: Data extraction from the registry was taken on February, 7, 2022. At the time there were 2174 patients diagnosed with Cushing's syndrome (CS) and 95 VTEs were reported in the database. RESULTS: Of 95 VTE events 70 (74%) were in pituitary-dependent CS patients, 12 (12.5%) in adrenal-dependant CS, 10 (10.5%) in ectopic CS, and 3 (3%) in CS due to other causes. Sex, 24-hour urinary free cortisol (UFC) value at diagnosis, as well as the number of operations remained statistically significant predictors of VTE. Of patients who were treated with at least one surgery, 12 (13%) VTE occurred before and 80 (87%) after the surgery. Nearly half of these VTEs occurred within six months since the operation (36; 45%). Over half of the centers that reported VTE did not routinely anticoagulate CS patients. Anticoagulation schemes varied widely. CONCLUSION: Patients with CS have an elevated risk of developing VTE for an extended period of time. From ERCUSYN cohort patients have higher risk for VTE if they need multiple surgeries to treat CS, are males and have high UFC values at the diagnosis of CS. Since there is no agreement on thromboprohpylaxis, a protocol for VTE prevention that is widely adopted appears to be necessary for patients with CS.

Endoscopic surgery in the skull base unit: experience in the first 72 cases.

INTRODUCTION: A multidisciplinary team is essential to develop and expand the indications in endonasal endoscopic skull base surgery. The aim of this study was to present our experience in a group of patients with skull base lesions treated using endonasal endoscopic approach. METHODS: From January 2008 to January 2012, 72 patients with skull base involvement were diagnosed and treated in our centre. RESULTS: The mean patient age was 53 years. The different pathologies included 36 pituitary adenomas, 10 cerebrospinal fluid leaks and 5 inverted papillomas as the most frequent pathologies. We performed a transsphenoidal transellar approach in 45 cases, a transmaxillary transpterygoid approach in 4 cases and a transnasal expanded approach in 6 cases. We performed an ethmoidal/sphenoidal approach in 12 patients and a Draf IIb/III procedure in four cases. Total resection was achieved in 61% of patients with pituitary adenomas, subtotal in 22% and partial in 17%. Successful repair was achieved in 86% of CSF leaks. No recurrences were observed in patients with inverted papilloma. Complications were observed in 21 patients (29%), 6 being major complications. CONCLUSIONS: Our centre stresses the importance of multidisciplinary collaboration in endoscopic endonasal skull base surgery.

Guía para el diagnóstico y tratamiento de la insuficiencia suprarrenal en el adulto / Guidelines for the diagnosis and treatment of adrenal insufficiency in the adult

La insuficiencia suprarrenal (IA) es un trastorno que se caracteriza por un déficit de glucocorticoides, al que se asocia en ocasiones un déficit de mineralocorticoides y/o andrógenos adrenales. Puede ser consecuencia de enfermedades intrínsecas del córtex adrenal (IA primaria), de procesos hipofisarios que afecten a la secreción de corticotropina (IA secundaria) o de trastornos hipotalámicos que afecten la secreción de la hormona liberadora de corticotropina (IA terciaria). Se trata de una entidad de baja prevalencia pero con elevado impacto sobre la salud individual, dado que entraña riesgo vital en ausencia de tratamiento y efectos deletéreos para la salud en caso de tratamiento inadecuado. En la actualidad no hay ninguna guía de práctica clínica para el manejo de esta enfermedad, por este motivo, a partir de una propuesta de la junta directiva de la Sociedad Española de Endocrinología y Nutrición (SEEN), se constituyó un grupo de trabajo dependiente del Área de Conocimiento de Neuroendocrinología de la SEEN, al que se encomendó la tarea de actualizar el diagnóstico y tratamiento de la IA del adulto. En cumplimiento de esta labor, el grupo de trabajo ha elaborado la presente guía, que, basándose en una revisión exhaustiva de la bibliografía, pretende dar respuesta a los interrogantes que se platean en el manejo de esta enfermedad. Se trata, por tanto, de un documento de carácter eminentemente práctico, cuya intención principal es servir de guía a los profesionales que se dedican al cuidado de los pacientes con IA

Adrenal insufficiency (AI) is a disease characterized by a deficient production or action of glucocorticoids, with or without deficiency in mineralcorticoids and/or adrenal androgens. It can result from disease intrinsic to the adrenal cortex (primary AI), from pituitary diseases that hamper the release of corticotropin (secondary AI) or from hypothalamic disorders that impair the secretion of the corticotropin-releasing hormone (tertiary AI). It is a disease with a low prevalence but its impact on the affected individual is very high as it can be life-threathening if not treated or lead to health problems if inadequately treated. However, currently there are no specific guidelines for the management of this disease. Therefore, at the proposal of the Spanish Society of Endocrinology and Nutrition (SEEN) board, a task-force under the Neuroendocrinology Knowledge Area of the SEEN was established, with the mandate of updating the diagnosis and treatment of AI. In fulfilment of this mandate the task-force has elaborated the present guide that, based on a comprehensive review of literature, is intended to provide an answer to questions related to the management of this disease. It is, therefore, an essentially practical document, mainly aimed at guiding the health professionals involved in the care of IA patients

Integración de la cirugía endoscópica en una unidad de base de cráneo: experiencia en los primeros 72 casos / Endoscopic surgery in the skull base unit: Experience in the first 72 cases

Introducción: La formación de un equipo multidisciplinar es imprescindible para desarrollar y ampliar las indicaciones en la cirugía endonasal endoscópica de la base de cráneo. El objetivo de este trabajo es presentar nuestra experiencia en el grupo de pacientes con afectación de la base de cráneo intervenidos con un abordaje endonasal endoscópico. Métodos: De enero de 2008 a enero de 2012, 72 pacientes con afectación de la base de cráneo fueron diagnosticados y tratados en nuestro centro. Resultados: La edad media de los pacientes en el momento del diagnóstico fue de 53 años. Las diferentes patologías incluyeron 36 adenomas de hipófisis, 10 fístulas de líquido cefalorraquídeo y 5 papilomas invertidos como las más frecuentes. En 45 casos se llevó a cabo un abordaje transesfenoidal transellar, en 4 casos un abordaje transmaxilar transpterigoideo y en 6 casos un abordaje transnasal ampliado. En 12 pacientes se realizó un abordaje a través del etmoides y/o del seno esfenoidal y en 4 casos se utilizó un abordaje frontal tipo Draf IIb/III. En el 61% de los adenomas se consiguió una resección total, en el 22% subtotal y en el 17% parcial. El 86% de las fístulas de líquido cefalorraquídeo se repararon con éxito. No se evidenció ninguna recidiva en los papilomas invertidos intervenidos. En 21 pacientes (29%) ocurrió algún tipo de complicación, apareciendo 6 complicaciones de orden mayor. Conclusiones: Nuestro centro apuesta por la colaboración multidisciplinar en la cirugía endoscópica de la base de cráneo como línea de excelencia (AU)

Introduction: A multidisciplinary team is essential to develop and expand the indications in endonasal endoscopic skull base surgery. The aim of this study was to present our experience in a group of patients with skull base lesions treated using endonasal endoscopic approach. Methods: From January 2008 to January 2012, 72 patients with skull base involvement were diagnosed and treated in our centre. Results: The mean patient age was 53 years. The different pathologies included 36 pituitary adenomas, 10 cerebrospinal fluid leaks and 5 inverted papillomas as the most frequent pathologies. We performed a transsphenoidal transellar approach in 45 cases, a transmaxillary transpterygoid approach in 4 cases and a transnasal expanded approach in 6 cases. We performed an ethmoidal/sphenoidal approach in 12 patients and a Draf IIb/III procedure in four cases. Total resection was achieved in 61% of patients with pituitary adenomas, subtotal in 22% and partial in 17%. Successful repair was achieved in 86% of CSF leaks. No recurrences were observed in patients with inverted papilloma. Complications were observed in 21 patients (29%), 6 being major complications. Conclusions: Our centre stresses the importance of multidisciplinary collaboration in endoscopic endonasal skull base surgery (AU)