RESUMEN
A 33-year-old woman with a history of high blood pressure since she was 8 years old, hypothyroidism, polycystic ovary syndrome, metabolic syndrome, multiple nevi, and a maternal family history of death at age 50 due to malignant high blood pressure and heart failure. Cushing's syndrome secondary to a secretory pituitary microadenoma was diagnosed, being the cause of secondary arterial hypertension, and ruling out other causes such as renal stenosis and coarctation of the aorta. A transthoracic and transesophageal echocardiogram was performed, which detected a left atrial myxoma. Given the presence of an atrial myxoma, Cushing's syndrome and polycystic ovary syndrome, a diagnosis of Carney Complex was made due to the presence of positive Stratakis criteria. The cardiac tumor was resected, and pathology confirmed that it was an atrial myxoma. She evolved clinically stable in outpatient controls in a 6-month follow-up. Resection of the pituitary microadenoma is planned as a curative treatment for Cushing's syndrome and arterial hypertension.
Mujer de 33 años, con antecedentes de hipertensión arterial desde los 8 años, hipotiroidismo, síndrome de ovario poliquístico, síndrome metabólico, nevos múltiples y antecedente familiar materno de muerte a los 50 años por hipertensión arterial maligna e insuficiencia cardiaca. Se diagnosticó síndrome de Cushing secundario a un microadenoma hipofisario secretor, siendo la causa de la hipertensión arterial secundaria, y descartándose otras causas como estenosis renal y coartación de aorta. Se realizó u n ecocardiograma transtorácico y transesofágico que detectaron un mixoma auricular izquierdo. Ante la presencia de un mixoma auricular, síndrome de Cushing y síndrome de ovario poliquístico se llegó al diagnóstico de Complejo de Carney por la presencia de criterios de Stratakis positivos. Se realizó la resección del tumor cardiaco, y la anatomía patológica confirmó que se trataba de un mixoma auricular. Evolucionó clínicamente estable en controles ambulatorios en un seguimiento de 6 meses, y se planifica la resección del microadenoma hipofisario como tratamiento curativo del síndrome de Cushing y la hipertensión arterial.
Asunto(s)
Fibrilación Atrial , Complejo de Carney , Síndrome de Cushing , Neoplasias Cardíacas , Hipertensión , Mixoma , Neoplasias Hipofisarias , Síndrome del Ovario Poliquístico , Femenino , Humanos , Persona de Mediana Edad , Adulto , Niño , Complejo de Carney/complicaciones , Complejo de Carney/diagnóstico , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/etiología , Síndrome de Cushing/cirugía , Síndrome del Ovario Poliquístico/complicaciones , Fibrilación Atrial/complicaciones , Mixoma/complicaciones , Mixoma/diagnóstico por imagen , Mixoma/cirugía , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Neoplasias Hipofisarias/complicaciones , Hipertensión/complicacionesRESUMEN
Resumen: Presentamos un caso de una paciente femenina de 27 años, con síndrome de Cushing ACTH dependiente con hipercortisolismo severo, causado por un macroadenoma hipofisario recurrente y resistente pese a dos cirugías transesfenoidales, radioterapia y terapia médica. Dada la falla en las diferentes terapias se realiza una adrenalectomía bilateral como tratamiento definitivo. La paciente fallece en el posoperatorio por causa no clara. Si bien la adrenalectomía bilateral ha sido reportada como un tratamiento efectivo en pacientes con enfermedad de Cushing, se ha relacionado con una mortalidad significativa vinculada con la severidad del hipercortisolismo y las comorbilidades presentes. En este caso la adrenalectomía izquierda se tuvo que convertir a cielo abierto, asociada con mayor morbimortalidad.
Abstract: The study presents the case of a 27-year-old female patient with adrenocorticotropic hormone (ACTH) dependent Cushing's disease and severe hypercortisolism caused by recurrent pituitary macroadenoma that was resistant to treatment despite two transsphenoidal surgeries, radiotherapy and medical treatment. Upon failure of the different therapies a bilateral adrenalectomy was performed as the final treatment. The patient died in after surgery although the case of death was not clear. Despite bilateral adrenalectomy having been reports as an effective treatment in patients with Cushing's disease, it has been related to significant mortality rates in connection with the severity of hypercortisolism and existing comorbilities. In this case the left adrenalectomy ended up being an open surgery, which is associated to a higher mortality rate.
Resumo: Apresentamos o caso de uma paciente de 27 anos com síndrome de Cushing ACTH-dependente com hipercortisolismo grave causado por macroadenoma hipofisário, recorrente e resistente, apesar de haver sido submetida a duas cirurgias transesfenoidal, radioterapia e terapia medicamentosa. Diante do fracasso das diferentes terapias, foi realizada adrenalectomia bilateral como tratamento definitivo. A paciente faleceu no pós-operatório por causa não esclarecida. Embora a adrenalectomia bilateral tenha sido relatada como tratamento eficaz em pacientes com doença de Cushing, ela tem sido associada a mortalidade significativa relacionada à gravidade do hipercortisolismo e às comorbidades presentes. Neste caso, a adrenalectomia esquerda teve que ser convertida para cirurgia aberta, associada a maior morbimortalidade.
Asunto(s)
Humanos , Femenino , Adulto , Adenoma/complicaciones , Síndrome de Cushing/complicaciones , Síndrome de Cushing/terapia , Adenoma Hipofisario Secretor de ACTH/complicaciones , Recurrencia , Enfermedad Catastrófica , Resultado Fatal , Adrenalectomía , Síndrome de Cushing/cirugíaRESUMEN
PURPOSE: Pregnancy is associated with the activation of the hypothalamus-pituitary-adrenal axis, which can cause a misdiagnosis of Cushing's syndrome. The aim of this study is to evaluate the impact of pregnancy after pituitary surgery on the recurrence rate in Cushing's disease (CD) patients. METHODS: This was a retrospective study in a tertiary center. Between 1990 and 2020, 355 CD patients underwent pituitary surgery. Of those, we included 113 female patients who were ≤ 45 years old (median age of 32 years, 14-45), PS remission, a follow-up of ≥6 months (median of 122 months, 6-402) and an available obstetric history. Recurrence was defined as the diagnosis of Cushing's syndrome via at least two altered first-line methods. The patients were divided into two subgroups according to pregnancy: no pregnancy or pregnancy prior to CD diagnosis (NP/PP) and pregnancy after CD pituitary surgery (PA). RESULTS: Overall, recurrence occurred in 43 out of 113 patients (38%). A higher recurrence rate was seen in the PA subgroup (11/22, 50%), but there was no significant difference between the NP/PP subgroup (32/91, 35%). No difference in survival-free recurrence (SFR) was found between NP/PP and PA subgroups. The lower SFR was related to a higher PS plasma ACTH and normal pituitary at pathological analyses. CONCLUSIONS: There was no difference in the recurrence rate in patients according to pregnancy history. Other studies with higher numbers of patients are needed to confirm these data.
Asunto(s)
Síndrome de Cushing , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT) , Neoplasias Hipofisarias , Humanos , Femenino , Adulto , Persona de Mediana Edad , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/cirugía , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/complicaciones , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/cirugía , Neoplasias Hipofisarias/cirugía , Estudios Retrospectivos , Hipófisis/cirugía , Recurrencia , HidrocortisonaRESUMEN
Introducción: desde el año 1997 se comenzó a realizar la adrenalectomía laparoscópica en nuestro centro. Objetivo: analizar los resultados de la adrenalectomía laparoscópica desde su implementación en el servicio de cirugía general del Hospital Clínico Quirúrgico Hermanos Ameijeiras. Métodos: Se realizó un estudio descriptivo de los resultados obtenidos en 160 pacientes a los que se les realizó adrenalectomía por vía laparoscópica, en el período comprendido desde noviembre de 1997 hasta septiembre del año 2017 en el servicio de cirugía general del Hospital Clínico Quirúrgico Universitario Hermanos Ameijeiras. Se empleó la técnica laparoscópica con abordaje lateral intraperitoneal en la mayoría de los casos y el abordaje en decúbito supino en 2 casos para la adrenalectomía bilateral. Resultados: se realizaron 162 adrenalectomías laparoscópicas en 160 pacientes, ya que a dos pacientes se les realizó la adrenalectomía bilateral en un tiempo. La edad promedio fue de 44,8 años. La indicación quirúrgica fue el incidentaloma en 68 pacientes (42,5 pr ciento), el síndrome de Cushing en 27 (16,8 por ciento), feocromocitoma en 26 (16,25 por ciento), la Enfermedad de Cushing con fracaso del tratamiento neuroquirúrgico en 12 (7,5 por ciento), mielolipomas en 7 (4,37 por ciento), tumor adrenal en 6 (3,75 por ciento), tumor metastásico en 5 (3,1 por ciento), quistes adrenales en 4 (2,5 por ciento), hiperaldosteronismo primario en 3 (1,87 por ciento), tumor virilizante adrenal en 1 paciente y 1 paciente con un Síndrome de secreción ectópica de ACTH que le provocaba un Síndrome de Cushing complicado. Predominaron las lesiones del lado izquierdo en 86 pacientes, 72 del lado derecho y 2 bilaterales. Fueron convertidos a cirugía convencional 2 pacientes (1,25 por ciento). El tiempo quirúrgico promedio fue de 82 minutos. La estadía posoperatoria promedio fue de 2.5 días. Conclusiones: la adrenalectomía laparoscópica es una técnica reproducible y segura con las ventajas inherentes...(AU)
Introduction: As far as 1997, the performing of laparoscopic adrenalectomy started in our center. Objective: To analyze the results of laparoscopic adrenalectomy since its implementation in the general surgery service of Hermanos Ameijeiras Clinical Surgical Hospital. Methods: A descriptive study was carried out with the results obtained from 160 patients who underwent laparoscopic adrenalectomy, in the period from November 1997 to September 2017, in the general surgery service of Hermanos Ameijeiras University Clinical Surgical Hospital. The laparoscopic technique with intraperitoneal lateral approach was used in the majority of cases and the supine approach was used in two cases for bilateral adrenalectomy. Results: 162 laparoscopic adrenalectomies were performed in 160 patients, since two patients underwent bilateral adrenalectomy at one time. The average age was 44.8 years. Surgical indication cases were incidentalomas, in 68 patients (42.5 percent), Cushing's syndrome in 27 (16.8 percent), pheochromocytomas in 26 (16.25 percent), Cushing's disease with neurosurgical treatment failure in 12 (7.5 percent), myelolipomas in 7 (4.37 percent), adrenal tumor in 6 (3.75 percent), metastatic tumor in 5 (3.1 percent), adrenal cysts in 4 (2.5 percent), primary hyperaldosteronism in 3 (1.87 percent), adrenal virilizing tumor in 1 patient, and 1 patient with an ectopic ACTH secretion syndrome that caused a complicated Cushing's syndrome. Lesions on the left side were predominant in 86 patients, 72 on the right side and two bilateral. Two patients (1.25 percent) were converted to conventional surgery. The average surgical time was 82 minutes. The average postoperative stay was 2.5 days. Conclusions: Laparoscopic adrenalectomy is a reproducible and safe technique with the advantages inherent to laparoscopic surgery(AU)
Asunto(s)
Humanos , Adulto , Laparoscopía , Médula Suprarrenal/lesiones , Adrenalectomía/métodos , Epidemiología Descriptiva , Síndrome de Cushing/cirugíaRESUMEN
Introducción: desde el año 1997 se comenzó a realizar la adrenalectomía laparoscópica en nuestro centro. Objetivo: analizar los resultados de la adrenalectomía laparoscópica desde su implementación en el servicio de cirugía general del Hospital Clínico Quirúrgico Hermanos Ameijeiras. Métodos: Se realizó un estudio descriptivo de los resultados obtenidos en 160 pacientes a los que se les realizó adrenalectomía por vía laparoscópica, en el período comprendido desde noviembre de 1997 hasta septiembre del año 2017 en el servicio de cirugía general del Hospital Clínico Quirúrgico Universitario Hermanos Ameijeiras. Se empleó la técnica laparoscópica con abordaje lateral intraperitoneal en la mayoría de los casos y el abordaje en decúbito supino en 2 casos para la adrenalectomía bilateral. Resultados: se realizaron 162 adrenalectomías laparoscópicas en 160 pacientes, ya que a dos pacientes se les realizó la adrenalectomía bilateral en un tiempo. La edad promedio fue de 44,8 años. La indicación quirúrgica fue el incidentaloma en 68 pacientes (42,5 pr ciento), el síndrome de Cushing en 27 (16,8 por ciento), feocromocitoma en 26 (16,25 por ciento), la Enfermedad de Cushing con fracaso del tratamiento neuroquirúrgico en 12 (7,5 por ciento), mielolipomas en 7 (4,37 por ciento), tumor adrenal en 6 (3,75 por ciento), tumor metastásico en 5 (3,1 por ciento), quistes adrenales en 4 (2,5 por ciento), hiperaldosteronismo primario en 3 (1,87 por ciento), tumor virilizante adrenal en 1 paciente y 1 paciente con un Síndrome de secreción ectópica de ACTH que le provocaba un Síndrome de Cushing complicado. Predominaron las lesiones del lado izquierdo en 86 pacientes, 72 del lado derecho y 2 bilaterales. Fueron convertidos a cirugía convencional 2 pacientes (1,25 por ciento). El tiempo quirúrgico promedio fue de 82 minutos. La estadía posoperatoria promedio fue de 2.5 días. Conclusiones: la adrenalectomía laparoscópica es una técnica reproducible y segura con las ventajas inherentes a la cirugía laparoscópica(AU)
Introduction: As far as 1997, the performing of laparoscopic adrenalectomy started in our center. Objective: To analyze the results of laparoscopic adrenalectomy since its implementation in the general surgery service of Hermanos Ameijeiras Clinical Surgical Hospital. Methods: A descriptive study was carried out with the results obtained from 160 patients who underwent laparoscopic adrenalectomy, in the period from November 1997 to September 2017, in the general surgery service of Hermanos Ameijeiras University Clinical Surgical Hospital. The laparoscopic technique with intraperitoneal lateral approach was used in the majority of cases and the supine approach was used in two cases for bilateral adrenalectomy. Results: 162 laparoscopic adrenalectomies were performed in 160 patients, since two patients underwent bilateral adrenalectomy at one time. The average age was 44.8 years. Surgical indication cases were incidentalomas, in 68 patients (42.5 percent), Cushing's syndrome in 27 (16.8 percent), pheochromocytomas in 26 (16.25 percent), Cushing's disease with neurosurgical treatment failure in 12 (7.5 percent), myelolipomas in 7 (4.37 percent), adrenal tumor in 6 (3.75 percent), metastatic tumor in 5 (3.1 percent), adrenal cysts in 4 (2.5 percent), primary hyperaldosteronism in 3 (1.87 percent), adrenal virilizing tumor in 1 patient, and 1 patient with an ectopic ACTH secretion syndrome that caused a complicated Cushing's syndrome. Lesions on the left side were predominant in 86 patients, 72 on the right side and two bilateral. Two patients (1.25 percent) were converted to conventional surgery. The average surgical time was 82 minutes. The average postoperative stay was 2.5 days. Conclusions: Laparoscopic adrenalectomy is a reproducible and safe technique with the advantages inherent to laparoscopic surgery(AU)
Asunto(s)
Humanos , Adulto , Laparoscopía/estadística & datos numéricos , Médula Suprarrenal/lesiones , Adrenalectomía/métodos , Epidemiología Descriptiva , Síndrome de Cushing/cirugíaRESUMEN
OBJECTIVES:: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD:: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS:: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION:: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.
Asunto(s)
Técnicas de Ablación/métodos , Hiperfunción de las Glándulas Suprarrenales/cirugía , Etanol/uso terapéutico , Tomografía Computarizada por Rayos X/métodos , Neoplasias de la Corteza Suprarrenal/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Adenoma Corticosuprarrenal/cirugía , Adulto , Anciano , Aldosterona/biosíntesis , Síndrome de Cushing/cirugía , Femenino , Humanos , Hiperplasia/cirugía , Masculino , Persona de Mediana Edad , Feocromocitoma/cirugía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Técnicas de Ablación/métodos , Hiperfunción de las Glándulas Suprarrenales/cirugía , Etanol/uso terapéutico , Tomografía Computarizada por Rayos X/métodos , Neoplasias de la Corteza Suprarrenal/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Adenoma Corticosuprarrenal/cirugía , Aldosterona/biosíntesis , Síndrome de Cushing/cirugía , Hiperplasia/cirugía , Feocromocitoma/cirugía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate the association between Cushing syndrome and hypercoagulability in children. STUDY DESIGN: A prospective, observational study was performed of 54 patients with Cushing syndrome, 15.1 ± 3.9 years, treated at the National Institutes of Health Clinical Center. Coagulation profiles were taken before and 6-12 months after surgery and compared with18 normocortisolemic children, 13.7 ± 3.6 years. RESULTS: At baseline, patients with Cushing syndrome had greater levels of the procoagulant factor VIII (FVIII) vs controls (145 IU/dL ± 84 vs 99 ± 47, P = .04); 6-12 months after surgery, FVIII levels decreased to 111 ± 47, P = .05. Patients with Cushing syndrome had greater levels of the antifibrinolytic α2-antiplasmin, 96 ± 17% vs 82 ± 26%, P = .015. After surgery, antifibrinolytic α2-antiplasmin levels decreased to 82 ± 24%, P < .001. Anticoagulants were greater in patients with Cushing syndrome vs controls at baseline, including protein C (138 ± 41% vs 84 ± 25%, P < .001), protein S (94 ± 19% vs 74 ± 19%, P = .001), and antithrombin III (96 ± 18% vs 77 ± 13%, P < .0001). The 24-hour urinary free cortisol levels correlated positively with FVIII levels, r = 0.43, P = .004. CONCLUSION: Children with Cushing syndrome had elevated procoagulants, antifibrinolytics, and anticoagulants at baseline compared with controls; normalization of coagulation measures was seen after surgical cure. Despite the increase in anticoagulants, hypercortisolemia is associated with a hypercoagulable state in children, as is the case in adults. This finding has potential implications for prevention of venous thromboembolism in children with Cushing syndrome. TRIAL REGISTRATION: ClinicalTrials.gov:NCT00001595.
Asunto(s)
Síndrome de Cushing/sangre , Síndrome de Cushing/complicaciones , Trombofilia/etiología , Adolescente , Síndrome de Cushing/cirugía , Femenino , Humanos , Masculino , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
OBJECTIVE: To assess skeletal maturity by measuring bone age (BA) in children with Cushing syndrome (CS) before and 1-year after transsphenoidal surgery or adrenalectomy, and to correlate BA with hormone levels and other measurements. STUDY DESIGN: This case series conducted at the National Institutes of Health Clinical Center included 93 children with Cushing disease (CD) (43 females; mean age, 12.3 ± 2.9 years) and 31 children with adrenocorticotropic hormone-independent CS (AICS) (22 females, mean age 10.3 ± 4.5 years). BA was obtained before surgery and at follow-up. Outcome measures were comparison of BA in CD vs AICS and analysis of the effects of hypercortisolism, insulin excess, body mass index, and androgen excess on BA. RESULTS: Twenty-six of the 124 children (21.0%) had advanced BA, compared with the expected general population prevalence of 2.5% (P < .0001). Only 4 of 124 (3.2%) had delayed BA. The majority of children (76%) had normal BA. The average BA z-score was similar in the children with CD and those with AICS (0.6 ± 1.4 vs 0.5 ± 1.8; P = .8865). Body mass index SDS and normalized values of dehydroepiandrosterone, dehydroepiandrosterone sulfate, androsteonedione, estradiol, and testosterone were all significantly higher in the children with advanced BA vs those with normal or delayed BA. Fifty-nine children who remained in remission from CD had follow-up BA 1.2 ± 0.3 years after transsphenoidal surgery, demonstrating decreased BA z-score (1.0 ± 1.6 vs 0.3 ± 1.4; P < .0001). CONCLUSION: Contrary to common belief, endogenous CS in children appears to be associated with normal or even advanced skeletal maturation. When present, BA advancement in CS is related to obesity, insulin resistance, and elevated adrenal androgen levels and aromatization. This finding may have significant implications for treatment decisions and final height predictions in these children.
Asunto(s)
Hormona Adrenocorticotrópica/fisiología , Determinación de la Edad por el Esqueleto , Desarrollo Óseo , Síndrome de Cushing/fisiopatología , Síndrome de Cushing/cirugía , Hormonas Esteroides Gonadales/fisiología , Obesidad/fisiopatología , Niño , Síndrome de Cushing/complicaciones , Femenino , Humanos , Masculino , Obesidad/complicaciones , Estudios Retrospectivos , Factores de TiempoRESUMEN
BACKGROUND: Laparoscopic approach has become the gold standard for the surgical treatment of suprarenal gland. Nevertheless there is still controversy about the laparoscopic treatment of adrenal carcinoma. MATERIAL AND METHODS: From April 2005 to April 2012, 37 laparoscopic adrenalectomies were performed. We describe and analyze retrospectively: age, sex, side, indication for surgery, tumor size, length of hospital stay, complications and conversion rate. RESULTS: 37 Patients, 19 male and 18 female, aged 51.72 ± 14.42 years, were operated on between 2005 and 2012. Twenty-two left-sided lesions (59.45%) and 15 right-sided lesions (40.54%) were operated on. The indications for surgery were non-functioning adenoma larger than 4 cm or rapid growth and hormone-secreting tumours. The diagnosis was confirmed in all the cases with computed tomography and or magnetic resonance imaging and also metaiodobenzylguanidine scintigraphy if pheochromocytoma was suspected. In all the cases we realized a complete pre-operative hormonal study. CONCLUSIONS: Laparoscopic adrenalectomy is a safe procedure and gold standard technique for suprarenal surgery. Our experience is very satisfactory, with comparable results to the reference standard open approach.
Antecedentes: la vía de acceso laparoscópico es la técnica de elección en el tratamiento quirúrgico de la glándula suprarrenal, excepto del carcinoma suprarrenal. Objetivo: revisar nuestra experiencia en suprarrenalectomías laparoscópicas por vía lateral transperitoneal efectuadas entre los años 2005 y 2012. Material y método: estudio descriptivo y retrospectivo efectuado mediante la revisión de historias clínicas de 37 pacientes con diagnóstico, al alta, de tumor adrenal y a quienes se hizo adrenalectomía laparoscópica entre abril de 2005 y abril de 2012. Se consideraron los siguientes datos: edad, sexo, lateralidad, indicación quirúrgica, resultados anatomopatológicos, tamaño de la lesión, estancia hospitalaria, tasa de conversión y complicaciones perioperatorias. Resultados: durante el periodo de estudio se intervinieron 37pacientes (19 varones y 18 mujeres) con edad media de 51.72 ± 14.42 años. Se realizaron 22adrenalectomías izquierdas (59.45%) y 15 derechas (40.54%). Las indicaciones de suprarrenalectomía fueron: incidentaloma mayor de 4 cm o con crecimiento rápido y tumores productores de hormonas. El diagnóstico se confirmó con tomografía computada, resonancia magnética, o ambas, y con gammagrafía metaiodobencilguanidina en el caso de sospecha de feocromocitoma y estudio hormonal completo en todos los pacientes. Conclusiones: la suprarrenalectomía laparoscópica sigue siendo la técnica de elección en el tratamiento de pacientes con afectación de la glándula suprarrenal porque ha demostrado ser segura y eficaz, como quedó confirmado en nuestra serie, que tuvo resultados similares a los de la bibliografía.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Laparoscopía/métodos , Adenoma/cirugía , Enfermedades de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Glándulas Suprarrenales/patología , Antagonistas Adrenérgicos alfa/administración & dosificación , Adulto , Anciano , Carcinoma/diagnóstico , Carcinoma/cirugía , Síndrome de Cushing/tratamiento farmacológico , Síndrome de Cushing/cirugía , Femenino , Hamartoma/cirugía , Humanos , Hidrocortisona/administración & dosificación , Hiperaldosteronismo/cirugía , Hiperplasia , Hallazgos Incidentales , Masculino , Persona de Mediana Edad , Mielolipoma/cirugía , Feocromocitoma/diagnóstico , Feocromocitoma/tratamiento farmacológico , Feocromocitoma/cirugía , Premedicación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: Successful surgery does not always resolve all the clinical consequences of hypercortisolism in patients with Cushing's disease (CD). Our purpose was to integrally evaluate a group of CD patients cured by pituitary surgery and look for the persistence of CD symptoms, signs, and comorbidities. METHODS: We performed clinical and biochemical evaluations of 29 CD patients (2 males) cured by pituitary surgery. All patients underwent early (median 12 months) and late (median 58 months) postoperative evaluations. We sought information regarding hypercortisolism-related symptoms and signs, as well as metabolic, cardiovascular, reproductive, and psychologic comorbidities. RESULTS: The prevalence of obesity dropped from 72.4% at diagnosis to 31% at early evaluation but increased again to 44.8% at the late evaluation. Diabetes was present in 14 patients (48.3%) at diagnosis and persisted in 9 at the late evaluation. Hypertriglyceridemia was present in 58.6% and 55.1% of patients at diagnosis and at the late follow-up, respectively. The prevalence of hypercholesterolemia was 79.3% at diagnosis, decreased to 55.1% at the early evaluation, and increased to 65.5% at the late evaluation. Menstrual abnormalities were originally present in 15 of 20 women, and 8 of the 15 had recovered normal periods when seen at the last evaluation. Among the 24 patients with depression at diagnosis, 11 and 6 still exhibited mood abnormalities at the early and late evaluations, respectively. CONCLUSIONS: In a variable proportion of patients, the cardiovascular, metabolic, and emotional comorbidities of CD persist after long-term remission, irrespective of the initial degree of hypercortisolism.
Asunto(s)
Síndrome de Cushing/cirugía , Depresión/epidemiología , Fatiga/epidemiología , Hirsutismo/prevención & control , Hipertensión/prevención & control , Hipófisis/cirugía , Acné Vulgar/epidemiología , Acné Vulgar/prevención & control , Adolescente , Adulto , Estudios de Cohortes , Comorbilidad , Síndrome de Cushing/epidemiología , Síndrome de Cushing/metabolismo , Síndrome de Cushing/fisiopatología , Depresión/prevención & control , Fatiga/prevención & control , Femenino , Estudios de Seguimiento , Hirsutismo/epidemiología , Hirsutismo/etiología , Humanos , Hidrocortisona/sangre , Hidrocortisona/metabolismo , Hidrocortisona/orina , Hipertensión/epidemiología , Hipertensión/etiología , Hipopotasemia/epidemiología , Hipopotasemia/prevención & control , Masculino , Trastornos de la Menstruación/epidemiología , Trastornos de la Menstruación/etiología , Trastornos de la Menstruación/prevención & control , México/epidemiología , Persona de Mediana Edad , Hipófisis/metabolismo , Prevalencia , Adulto JovenRESUMEN
It is known that hipercortisolism and overactivity of the Hypotalamus pituitary adrenal axis are associated to psiquiatric diseases presentation. In patients with Cushing syndrome, mood disorders are common, also psicosis cases also are present during the disease. But, the presence and mostly the onset with a episode of catatonic psicosis is unusual. The cortisol excess produce structural damage in the Central Nervous System, reversible and irreversible, specially in the hypocampus. We show the case of a young woman with previous diagnostic of Turner syndrome, karyotype 45 X0, who presented a psychiatric episode caracterized for depresive psicotic symptoms and posteriorly a catatonic state as an unusual form of Cushing disease onset. This patient presented health improvement after pituitary resection. There is not evidencia that Turner syndrome influes over this unusual form of presentation.
Se conoce que el hipercortisolismo y la sobreactividad del eje hipotálamo pituitario adrenal están asociados a la presentación de enfermedades psiquiátricas. En pacientes con síndrome de Cushing son frecuentes los trastornos del ánimo aunque también se presentan casos de psicosis durante el curso de la enfermedad. Sin embargo es inusual la presencia y más aún el debut con un cuadro de psicosis catatónica. El exceso de cortisol produce daño estructural en el sistema nervioso central tanto reversible como irreversible, especialmente en el hipocampo. Presentamos el caso de una paciente mujer joven con diagnóstico previo de síndrome de Turner, cariotipo 45 X0, quien presentó un cuadro psiquiátrico caracterizado por síntomas depresivos psicóticos y posteriormente catatonia como forma inusual de debut de enfermedad de Cushing y presentó mejoría posterior a la resección de la pituitaria. No se tiene evidencia que el síndrome de Turner influya sobre esta rara forma de presentación.
Asunto(s)
Humanos , Femenino , Adulto , Catatonia/etiología , Síndrome de Cushing/complicaciones , Trastornos Psicóticos/etiología , Hiperfunción de las Glándulas Suprarrenales/complicaciones , Imagen por Resonancia Magnética , Síndrome de Cushing/cirugía , Síndrome de Turner/complicacionesRESUMEN
The relapse of autoimmune diseases following resolution of Cushing syndrome (CS) has been established with frequency, especially for thyroid disease. We present the case of a 39-year-old woman with a history of psoriasis vulgaris, with no other comorbidities. She presented a one-year of weight gain, high blood pressure, hypermenorrhea, pre-diabetes and dyslipidemia. During this period of time, the complete remission of psoriatic lesions was confirmed. The laboratory analysis demonstrated elevated free urinary cortisol in two opportunities, and a confirmatory low-dose dexamethasone test. Low ACTH levels were established (12,3pg/ml), and the abdominal images where compatible with a right adrenal adenoma of 3,0 x 2,3 cm, sothe patient underwent a laparoscopic right adrenalectomy with no complications. Her blood pressure, glucose and cholesterol levels improved following surgery. One month after surgery the patient developed new, generalized psoriatic plaques. The excess of glucocorticoids produced by the adrenal tumor could have controlled the immunopathogeny of psoriasis. By removing the tumor, with the consequent reduction of cortisol levels, a more severe relapse of her cutaneous disease occurred.
Asunto(s)
Humanos , Adulto , Femenino , Adrenalectomía/efectos adversos , Psoriasis/patología , Síndrome de Cushing/cirugía , Laparoscopía/efectos adversos , RecurrenciaRESUMEN
A ocorrência de síndrome de Cushing (SC) durante a gravidez é rara, com menos de 150 casos reportados na literatura. Este relato demonstra a dificuldade diagnóstica e a gravidade da desordem adrenal específica durante a gestação, assim como a necessidade de diagnóstico e intervenção precoces. Este trabalho apresenta paciente portadora da SC com início das suas manifestações clínicas a partir do terceiro mês de gestação. Foi realizada durante a gestação a investigação bioquímico-laboratorial, que permitiu avaliação clínico-laboratorial e confirmação diagnóstica de SC. O método de imagem empregado foi a ressonância magnética do abdômen. Após a cirurgia por videolaparoscopia, houve redução do peso e dos níveis pressóricos e normalização dos níveis do cortisol plasmático e urinário, além do controle dos níveis glicêmicos.
The occurrence of Cushing Syndrome (CS) in pregnancy is rare, with less than 150 case reports in the literature. This report shows the diagnosis difficulty and the severity of the specific adrenal disorder during pregnancy, as well as the need for precocious diagnosis and intervention. This paper presents a patient with CS starting its physical manifestations from the third gestation month. The biochemical-laboratory survey was carried out during the pregnancy, which allowed the clinic-laboratory evaluation and the CS diagnosis confirmation. The used image method was abdomen magnetic resonance. After surgery by video laparoscopy, there was weight and pressure levels reduction and normalization of plasma cortisol and urinary levels, besides the glycemic levels control.
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Complicaciones del Embarazo , Síndrome de Cushing/cirugía , Síndrome de Cushing/diagnóstico , Hipertensión , LaparoscopíaRESUMEN
UNLABELLED: Many hypertensive patients affected by endogenous Cushing's syndrome (CS) persist with high blood pressure (HBP) despite good control of cortisol excess. We assessed the effect of preoperative ketoconazole administration and of definitive treatment of CS on arterial hypertension and analysed the factors involved in the persistence of hypertension. We assessed retrospectively 71 patients with CS and HBP (60 women, 11 men; 50 pituitary, 21 adrenal) successfully treated by surgery and/or radiotherapy; 19 of them received ketoconazole (KNZ) before surgery. After treatment, patients were divided into those with persistent high blood pressure (PHBP) and those with normal blood pressure (NBP). As possible predictive factors for PHBP we analysed age, duration and family history of HBP, pre-treatment 24 hour urinary free cortisol (24h-UFC) and body mass index (BMI). HBP normalized in 53 out of 71 patients (74.6%), regardless of the origin of Cushing's syndrome. PHBP patients were older (p=0.003), had longer duration (p=0.007) and higher systolic blood pressure before treatment (p=0.046) than NBP patients. Thirteen out of 19 patients (68.4%) treated with ketoconazole, normalized their hypertension and remained normotensive after successful surgery. Five patients became normotensive only after surgery. IN CONCLUSION: a) blood pressure levels normalized in most patients after remission of CS; b) ketoconazole was effective for the Control of HBP and seems to be a good indicator of post-surgical outcome, and c) higher age at presentation, longer duration of hypertension and higher systolic blood pressure figures before treatment negatively influence normalization of blood pressure after resolution of Cushing's syndrome.
Asunto(s)
Antihipertensivos/administración & dosificación , Síndrome de Cushing/tratamiento farmacológico , Hidrocortisona/sangre , Hipertensión/tratamiento farmacológico , Cetoconazol/administración & dosificación , Adolescente , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Glándulas Suprarrenales/cirugía , Adulto , Anciano , Presión Sanguínea/efectos de los fármacos , Índice de Masa Corporal , Síndrome de Cushing/cirugía , Femenino , Estudios de Seguimiento , Humanos , Hidrocortisona/orina , Hipertensión/diagnóstico , Hipertensión/cirugía , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Laparoscopic adrenalectomy is one of the most clinically important advances in the past 2 decades for the treatment of adrenal disorders. When compared to open adrenalectomy, laparoscopic adrenalectomy is equally safe, effective, and curative; it is more successful in shortening hospitalization and convalescence and has less long-term morbidity. The laparoscopic approach to the adrenal is the procedure of choice for the surgical management of cortisol-producing adenomas and for patients with corticotropin (ACTH) dependent Cushing's syndrome for whom surgery failed to remove the source of ACTH. The keys to successful laparoscopic adrenalectomy are appropriate patient selection, knowledge of anatomy, delicate tissue handling, meticulous hemostasis, and experience with the technique of laparoscopic adrenalectomy.
A adrenalectomia laparoscópica é um dos avanços clínicos mais importantes das últimas duas décadas para o tratamento de doenças adrenais. Quando comparada à adrenalectomia aberta (convencional), a adrenalectomia laparoscópica é igualmente segura, efetiva e curativa; ela tem maior sucesso em encurtar a hospitalização e a convalescença e apresenta menor morbidade a longo prazo. A abordagem laparoscópica da adrenal é o procedimento de escolha para ao manejo cirúrgico dos adenomas produtores de cortisol e para os pacientes com síndrome de Cushing ACTH-dependente nos quais a cirurgia hipofisária falhou em remover a fonte de ACTH. Os pontos críticos para o sucesso da adrenalectomia laparoscópica são: a seleção apropriada do paciente, o conhecimento da anatomia da região, o manuseio cuidadoso do tecido, a homeostase meticulosa e a experiência com essa técnica cirúrgica.
Asunto(s)
Humanos , Adrenalectomía/métodos , Síndrome de Cushing/cirugía , Laparoscopía/métodos , Adrenalectomía , Estudios de Factibilidad , LaparoscopíaRESUMEN
Cushing's syndrome (CS) is a chronic and systemic disease caused by endogenous or exogenous hypercortisolism, associated with an increase of mortality rate due to the clinical consequences of glucocorticoid excess, especially cardiovascular diseases. After cure, usually obtained by the surgical removal of the tumor responsible for the disease, the normalization of cortisol secretion is not constantly followed by the recovery of the clinical complications developed during the active disease, and it is often followed by the development of novel clinical manifestations induced by the fall of cortisol levels. These evidences were mostly documented in patients with pituitary-dependent CS, after surgical resection of the pituitary tumor. Indeed, despite an improvement of the mortality rate, metabolic syndrome and the consequent cardiovascular risk have been found to partially persist after disease remission, strictly correlated to the insulin resistance. Skeletal diseases, mainly osteoporosis, improve after normalization of cortisol levels but require a long period of time or the use of specific treatment, mainly bisphosphonates, to reach the normalization of bone mass. A relevant improvement or resolution of mental disturbances has been described in patients cured from CS, although in several cases, cognitive decline persisted and psychological or psychiatric improvement was erratic, delayed, or incomplete. On the other hand, development or exacerbation of autoimmune disorders, mainly thyroid autoimmune diseases, was documented in predisposed patients with CS after disease remission. The totality of these complications persisting or occurring after successful treatment contribute to the impairment of quality of life registered in patients with CS after disease cure.
A síndrome de Cushing (SC) é uma desordem sistêmica crônica causada por hipercortisolismo endógeno ou exógeno, associada a um aumento da taxa de mortalidade devido às conseqüências clínicas do excesso de glicocorticóides, especialmente a doença cardiovascular. Após a cura, usualmente obtida pela remoção cirúrgica do tumor responsável pela desordem, a normalização da secreção de cortisol não é sistematicamente seguida da recuperação das complicações clínicas desenvolvidas durante a fase ativa da doença, e é freqüentemente seguida pelo surgimento de novas manifestações clínicas induzidas pela queda dos níveis de cortisol. Estas evidências foram, na sua maioria, documentadas em pacientes com SC de origem hipofisária, após a ressecção cirúrgica do tumor na hipófise. Na verdade, a despeito de uma melhoria na taxa de mortalidade, a síndrome metabólica e seu conseqüente risco cardiovascular têm se mostrado parcialmente persistentes após a remissão da doença, em estrita relação com a resistência à insulina. Anormalidades esqueléticas, especialmente a osteoporose, melhoram após a normalização dos níveis de cortisol, mas requerem um longo tempo ou o uso de tratamento específico, principalmente bisfosfonatos, para se obter a normalização da massa óssea. Uma melhora significativa ou mesmo resolução dos distúrbios mentais têm sido descritos em pacientes curados da SC, embora em vários casos o declínio cognitivo persista e a melhora psicológica ou psiquiátrica tenham sido erráticas, demoradas ou incompletas. Por outro lado, o desenvolvimento ou exacerbação de processos autoimunes, em especial as doenças autoimunes da tiróide, foram documentadas em pacientes predispostos com SC, após a remissão da doença. A totalidade dessas complicações, persistentes ou ocorrendo após o tratamento bem sucedido, contribuem para um prejuízo da qualidade de vida registrado em pacientes com SC após a cura da doença.
Asunto(s)
Humanos , Síndrome de Cushing , Enfermedades Autoinmunes/etiología , Enfermedades Óseas/etiología , Enfermedades Cardiovasculares/etiología , Síndrome de Cushing/sangre , Síndrome de Cushing/complicaciones , Síndrome de Cushing/mortalidad , Síndrome de Cushing/cirugía , Hidrocortisona/sangre , Enfermedades Renales/etiología , Síndrome Metabólico/etiología , Inducción de Remisión , Resultado del TratamientoRESUMEN
OBJECTIVES: Cushing's syndrome is a rare disease. Most patients have a hypophyseal tumor and the treatment is neurosurgical. A small percentage of patients require bilateral adrenalectomy. This paper reports the cases of two patients undergoing bilateral simultaneous laparoscopic surgery with excellent results. METHODS: Under general anesthesia, with the patient in the lumbotomy position right adrenalectomy was performed first. 15 mm Hg Pneumoperitoneum was started with the Verres needle in the ipsilateral upper quadrant; four trocars were placed, two 10 mm and two 5 mm. In both cases the adrenal vein was divided between metallic clips. Once dissection was completed the gland was placed in a plastic bag and extracted through one of the trocar incisions. For the left side the position of the patient was changed, and the same procedure was performed using three trocars, two 10 mm and one 5 mm. No drainage was employed in either patient. RESULTS: The procedures were successfully performed without conversion. Surgical time was 150 minutes for the first patient and 210 for the second. Mean estimated blood loss was 125 ml. Mean hospital stay was 2.5 days. Both patients had a clinical reversion and they are managed with oral corticoid supplements. CONCLUSIONS: The results show that laparoscopic access to the adrenal gland is the approach of choice with very satisfactory outcomes for both patients and medical team.
Asunto(s)
Adrenalectomía/métodos , Síndrome de Cushing/cirugía , Laparoscopía , Adulto , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Many hypertensive patients affected by endogenous Cushing's syndrome (CS) persist with high blood pressure (HBP) despite good control of cortisol excess. We assessed the effect of preoperative ketoconazole administration and of definitive treatment of CS on arterial hypertension and analysed the factors involved in the persistence of hypertension. We assessed retrospectively 71 patients with CS and HBP (60 women, 11 men; 50 pituitary, 21 adrenal) successfully treated by surgery and/or radiotherapy; 19 of them received ketoconazole (KNZ) before surgery. After treatment, patients were divided into those with persistent high blood pressure (PHBP) and those with normal blood pressure (NBP). As possible predictive factors for PHBP we analysed age, duration and family history of HBP, pre-treatment 24 hour urinary free cortisol (24h-UFC) and body mass index (BMI). HBP normalized in 53 out of 71 patients (74.6%), regardless of the origin of Cushing's syndrome. PHBP patients were older (p=0.003), had longer duration (p=0.007) and higher systolic blood pressure before treatment (p=0.046) than NBP patients. Thirteen out of 19 patients (68.4%) treated with ketoconazole, normalized their hypertension and remained normotensive after successful surgery. Five patients became normotensive only after surgery. In conclusion: a) blood pressure levels normalized in most patients after remission of CS; b) ketoconazole was effective for the control of HBP, and seems to be a good indicator of post-surgical outcome, and c) higher age at presentation, longer duration of hypertension and higher systolic blood pressure figures before treatment negatively influence normalization of blood pressure after resolution of Cushing's syndrome.
Muchos pacientes con síndrome de Cushing (SC) permanecen hipertensos a pesar del control del exceso glucocorticoideo. Investigamos el efecto de la administración de ketoconazol (KNZ) y del tratamiento definitivo del SC sobre la hipertensión arterial (HTA), analizando su relación con diversos factores. Evaluamos 71 pacientes con SC e HTA (60 mujeres, 11 varones; 50 pituitarios, 21 adrenales) exitosamente tratados por cirugía y/o radioterapia; 19 de ellos recibieron KNZ antes de cirugía. Luego del tratamiento, fueron divididos en pacientes con HTA persistente (HTAP) y normal (HTAN). Como posibles factores predictivos de HTAP se analizaron edad, duración, historia familiar de HTA, cortisol libre urinario de 24 hs pre-tratamiento e índice de masa corporal. La HTA normalizó en 53/71 pacientes (74.6%) independientemente del origen del síndrome de Cushing. Los pacientes con HTAP fueron de mayor edad (p=0.003), con mayor duración previa (p=0.007) y valores mayores de presión arterial sistólica antes de tratamiento (p=0.046) que aquellos con HTAN. Trece de 19 pacientes (68.4 %) tratados con ketoconazol normalizaron su tensión arterial y se mantuvieron normotensos luego de cirugía exitosa. Cinco pacientes se tornaron normotensos solo después de cirugía. En conclusión: a) la HTA se normalizó en la mayoría de pacientes luego de remisión del SC, b) el ketoconazol fue efectivo para el control tensional y aparenta ser indicador de la evolución pos-quirúrgica, y c) mayor edad, duración más prolongada de la HTA y valores más altos de presión sistólica influencian negativamente la normalización de la presión arterial luego de resolución del síndrome de Cushing.