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INTRODUCTION: Endometriosis is a female disease that affects 5-10% of women of childbearing age, with predominantly pelvic manifestations. It is currently declared as a public health priority in France. Thoracic endometriosis syndrome (TES) is the most common extra-pelvic manifestation. OBJECTIVE: The objective of this study was to describe the epidemiological and clinical characteristics, and outcomes of patients with TES in Martinique. PATIENTS AND METHODS: We performed a descriptive, retrospective study including all patients managed at the University Hospital of Martinique for TES between 1 January 2004 and 31 December 2020. RESULTS: During the study period, we identified 479 cases of pneumothorax, of which 212 were women (44%). Sixty-three patients (30% of all female pneumothorax) were catamenial pneumothorax (CP) including 49 pneumothoraxes alone (78% of catamenial pneumothorax) and 14 hemopneumothorax (22% of catamenial pneumothorax). There were 71 cases of TES, including 49 pneumothoraxes (69%), 14 hemopneumothoraxes (20%) and 8 hemothorax (11%). The annual incidence of TES was 1.1 cases/100,000 inhabitants. The prevalence of TES was 1.2/1000 women aged from 15 to 45 years and the annual incidence of TES for this group was 6.9/100,000. The annual incidence of CP was 1 case/100,000 inhabitants. The average age at diagnosis was 36 ± 6 years. Eight patients (11%) had no prior diagnosis of pelvic endometriosis (PE). The mean age at pelvic endometriosis diagnosis was 29 ± 6 years. The mean time from symptom onset to diagnosis was 24 ± 50 weeks, and 53 ± 123 days from diagnosis to surgery. Thirty-two patients (47%) had prior abdominopelvic surgery. Seventeen patients (24%) presented other extra-pelvic localizations. When it came to management, 69/71 patients (97%) underwent surgery. Diaphragmatic nodules or perforations were found in 68/69 patients (98.5%). Histological confirmation was obtained in 55/65 patients who underwent resection (84.6%). Forty-four patients (62%) experienced recurrence. The mean time from the initial treatment to recurrence was 20 ± 33 months. The recurrence rate was 16/19 (84.2%) in patients who received medical therapy only, 11/17 (64.7%) in patients treated by surgery alone, and 17/31 (51.8%) in patients treated with surgery and medical therapy (p = 0.03). CONCLUSIONS: We observed a very high incidence of TES in Martinique. The factors associated with this high incidence in this specific geographical area remain to be elucidated. The frequency of recurrence was lower in patients who received both hormone therapy and surgery.
RESUMO
Resumen ANTECEDENTES: El tórax es la localización extrapélvica más frecuente de la endometriosis; esto sin que su incidencia se haya determinado con estudios suficientes. Tampoco se ha establecido del todo su fisiopatología, pero aun así se han planteado diversas teorías. El neumotórax catamenial es una de las posibles manifestaciones que hacen complejo establecer el diagnóstico e indicar un tratamiento. CASO CLÍNICO: Paciente de 31 años, con antecedente de neumotórax espontáneo a repetición, que acudió a consulta debido a disnea y dolor torácico de un mes de evolución. La radiografía de tórax evidenció el neumotórax del 50% en la parte derecha y la necesidad de la toracostomía. Puesto que los episodios de neumotórax ocurrían en fase catamenial, pero sin poder establecer una causa clara del cuadro clínico, se procedió a la toracoscopia diagnóstica con toma de muestra para estudio histopatológico. Durante el procedimiento se detectaron múltiples lesiones pseudonodulares, de aspecto inflamatorio crónico a nivel pleural y del parénquima pulmonar. El reporte histopatológico fue compatible con endometriosis pleuropulmonar; se le indicó terapia hormonal. Ante la evolución clínica satisfactoria y posoperatorio sin complicaciones la paciente fue dada de alta del hospital. CONCLUSIÓN: El neumotórax catamenial, como consecuencia de una endometriosis pleuropulmonar, es un diagnóstico realmente excepcional y su sospecha debe vincularse con el ciclo menstrual. La atención médica de las pacientes con este diagnóstico debe ser interdisciplinaria, no solo por las estrategias diagnósticas sino por la complejidad del tratamiento y su seguimiento. El tratamiento de elección suele requerir intervenciones quirúrgicas, sumadas a la indicación de la medicación hormonal para prevenir recurrencias, con tasas de éxito favorables. Están pendientes los estudios que establezcan la respuesta del tejido endometrial ectópico torácico al tratamiento médico.
Abstract BACKGROUND: The thorax is the most frequent extrapelvic location of endometriosis, although its incidence has not been sufficiently studied. Its pathophysiology has not been fully established, but several theories have been put forward. Catamenial pneumothorax is one of the possible manifestations that make it difficult to establish the diagnosis and indicate treatment. CLINICAL CASE: A 31-year-old female patient, with a history of repeated spontaneous pneumothorax, presented for consultation due to dyspnea and chest pain of one month of evolution. Chest X-ray showed a 50% pneumothorax on the right side and the need for thoracostomy. Since the episodes of pneumothorax occurred in catamenial phase, but without being able to establish a clear cause of the clinical picture, diagnostic thoracoscopy with sampling for histopathological study was performed. During the procedure multiple pseudonodular lesions of chronic inflammatory aspect were detected at pleural and lung parenchyma level. The histopathological report was compatible with pleuropulmonary endometriosis; hormonal therapy was indicated. Given the satisfactory clinical evolution and postoperative course without complications, the patient was discharged from the hospital. CONCLUSION: Catamenial pneumothorax, as a consequence of pleuropulmonary endometriosis, is a truly exceptional diagnosis and its suspicion should be linked to the menstrual cycle. The medical care of patients with this diagnosis should be interdisciplinary, not only because of the diagnostic strategies but also because of the complexity of the treatment and its follow-up. The treatment of choice usually requires surgical interventions, added to the indication of hormonal medication to prevent recurrences, with favorable success rates. Studies establishing the response of thoracic ectopic endometrial tissue to medical treatment are pending.
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Resumen El síndrome de endometriosis torácica (TES) es un trastorno poco común caracterizado por la presencia de tejido endometrial ectópico en la cavidad torácica. La manifestación clínica típica es un neumotórax espontáneo, que generalmente se presenta con dolor torácico, disnea y/o tos. El diagnóstico requiere un alto nivel de sospecha clínica junto con una historia ginecológica completa. Los estudios de imáge nes pueden ayudar con el diagnóstico, pero el gold standard es la cirugía toracoscópica videoasistida (VATS). Se ha demostrado que el tratamiento quirúrgico en combinación con al menos 6 meses de tratamiento médico hormonal mejora el pronóstico y reduce la recurrencia de esta entidad. Presentamos el caso de una paciente de 40 años con antecedentes de endometriosis pélvica y múltiples episodios de neumotórax, que consultó en nuestra institución por un nuevo episodio de neumotórax espontáneo. Se realizó una VATS donde se identificaron nódulos en la pleura parietal y orificios diafragmáticos. En el postoperatorio continuó con tratamiento hormonal. A los 6 meses de seguimiento refirió mejoría del dolor y no presentó nuevos episodios de neumotórax.
Abstract. Thoracic endometriosis syndrome (TES) is a rare disorder characterized by the presence of ectopic endometrial tissue in the chest cavity. The typical clinical manifestation is a spontaneous pneumothorax, which usually presents with chest pain, dyspnea, and/or cough. The diagnosis requires a high level of clinical suspicion and a complete gynecological history. Imaging studies can help with the diagnosis, although the gold standard is video-assisted thoracoscopic surgery (VATS). Surgical treatment in combination with at least 6 months of hormonal medical treatment has been shown to improve the prognosis and reduce the recurrence of this entity. We present the case of a 40-year-old patient with a history of pelvic endometriosis and multiple episodes of pneumothorax, who consulted at our institution for a new episode of spon taneous pneumothorax. A VATS was performed where nodules in the parietal pleura and diaphragmatic orifices were identified. In the postoperative period, she continued with hormonal treatment. At 6 months of follow-up, she reported improvement in pain and did not present new episodes of pneumothorax.
RESUMO
Thoracic endometriosis syndrome (TES) is a rare disorder characterized by the presence of ectopic endometrial tissue in the chest cavity. The typical clinical manifestation is a spontaneous pneumothorax, which usually presents with chest pain, dyspnea, and/or cough. The diagnosis requires a high level of clinical suspicion and a complete gynecological history. Imaging studies can help with the diagnosis, although the gold standard is video-assisted thoracoscopic surgery (VATS). Surgical treatment in combination with at least 6 months of hormonal medical treatment has been shown to improve the prognosis and reduce the recurrence of this entity. We present the case of a 40-year-old patient with a history of pelvic endometriosis and multiple episodes of pneumothorax, who consulted at our institution for a new episode of spontaneous pneumothorax. A VATS was performed where nodules in the parietal pleura and diaphragmatic orifices were identified. In the postoperative period, she continued with hormonal treatment. At 6 months of follow-up, she reported improvement in pain and did not present new episodes of pneumothorax.
El síndrome de endometriosis torácica (TES) es un trastorno poco común caracterizado por la presencia de tejido endometrial ectópico en la cavidad torácica. La manifestación clínica típica es un neumotórax espontáneo, que generalmente se presenta con dolor torácico, disnea y/o tos. El diagnóstico requiere un alto nivel de sospecha clínica junto con una historia ginecológica completa. Los estudios de imágenes pueden ayudar con el diagnóstico, pero el gold standard es la cirugía toracoscópica videoasistida (VATS). Se ha demostrado que el tratamiento quirúrgico en combinación con al menos 6 meses de tratamiento médico hormonal mejora el pronóstico y reduce la recurrencia de esta entidad. Presentamos el caso de una paciente de 40 años con antecedentes de endometriosis pélvica y múltiples episodios de neumotórax, que consultó en nuestra institución por un nuevo episodio de neumotórax espontáneo. Se realizó una VATS donde se identificaron nódulos en la pleura parietal y orificios diafragmáticos. En el postoperatorio continuó con tratamiento hormonal. A los 6 meses de seguimiento refirió mejoría del dolor y no presentó nuevos episodios de neumotórax.
Assuntos
Endometriose , Pneumotórax , Adulto , Diafragma , Endometriose/diagnóstico , Endometriose/diagnóstico por imagem , Feminino , Humanos , Pneumotórax/diagnóstico por imagem , Pneumotórax/etiologia , Pneumotórax/cirurgia , Cirurgia Torácica VídeoassistidaRESUMO
Resumen El neumotórax catamenial es una condición clínica rara y compleja que debe ser considerada como causa de neumotórax espontáneo y recurrente que a menudo es diagnosticado en forma errónea. Es una patología pulmonar comúnmente asociada a la menstruación, ya que ocurre dentro de las 72 horas antes o después del inicio del sangrado menstrual. La etiología y el mecanismo subyacente exacto no han sido identificados, pero podría ser una forma rara de endometriosis extrapélvica caracterizada por la presencia de tejido endometrial funcional en pleura, parénquima pulmonar y vías respiratorias. El diagnóstico es un desafío, por lo que puede resultar en recurrencias. Debe sospecharse en mujeres jóvenes en edad fértil. La primera línea de tratamiento es médica, mientras que el tratamiento quirúrgico es necesario para evitar la recurrencia. Se presenta un caso de neumotórax catamenial recurrente.
Abstract Catamenial pneumothorax is a rare and complex clinical condition that should be considered as a cause of spontaneous and recurrent pneumothorax that is often misdiagnosed. It is a pulmonary pathology commonly associated with menstruation since it occurs within 72 hours before or after the onset of menstrual bleeding. The etiology and the exact underlying mechanism have not been identified, but it could be a rare form of extra-pelvic endometriosis characterized by the presence of functional endometrial tissue in the pleura, pulmonary parenchyma, and respiratory tract. Diagnosis is a challenge, so it can result in recurrences. It should be suspected in young women of childbearing age. The first line of treatment is medical, while surgical treatment is necessary to avoid recurrence. A case of recurrent catamenial pneumothorax is presented.
RESUMO
El neumotórax catamenial se define como un neumotórax espontáneo recurrente que tiene relación con la menstruación. Su incidencia es baja, constituyendo el 3-6 por ciento de los casos de neumotórax espontáneo en la mujer en edad fértil. Puede relacionarse con la presencia de endometriosis torácica y fenestraciones diafragmáticas. PRESENTACIÓN DEL CASO: Mujer de 36 años con antecedente de neumotórax derecho hace 9 años. Presentó cuadro de neumotórax espontáneo derecho el cual fue tratado con pleurotomía. Estudio con tomografía computada de tórax mostró bulas apicales en pulmón derecho. Se realizó resección de vértice pulmonar y pleurodesis mecánica por videotoracoscopía. Evolucionó con múltiples recidivas del neumotórax durante los meses siguientes. Las características dela paciente hicieron posible plantear la sospecha de neumotórax catamenial, encontrándose correlación directa entre los episodios de neumotórax y el inicio de la menstruación. Se decidió reintervención quirúrgica por videotoracoscopía. Se observaron lesiones sospechosas de implantes endometriósicos y fenestraciones diafragmáticas. El estudio histopatológico del tejido resecado fue sugerente de tejido endometrial. Se inició además tratamiento hormonal con anovulatorios. Presentó nueva recidiva durante el mes siguiente, la cual fue tratada con pleurotomía. Actualmente se encuentra asintomática a 6 meses de seguimiento. DISCUSIÓN: El neumotórax catamenial es un tipo infrecuente de neumotórax espontáneo secundario. Se plantean múltiples hipótesis para explicar su etiopatogenia. El antecedente de neumotórax espontáneo recidivado en una mujer en edad fértil, obliga a sospecharlo evaluando la relación con los ciclos menstruales...
Catamenial pneumothorax is defined as a recurrent spontaneous pneumothorax related with menstruation. Its incidence is low, being 3-6 percent of cases of spontaneous pneumothorax in women of reproductive age. It has been related with thoracic endometriosis and diaphragmatic fenestrations. Multiple hypotheses are proposed to explain its pathogenesis. Case report: A 36 year old woman with a history of right pneumothorax9 years ago. She presented a right spontaneous pneumothorax which was treated with pleurotomy. Chest computed tomography showed apical bullae in the right lung. Resection of lung apex and mechanical pleurodesis by videothoracoscopywas performed. During the following months, she presented multiple recurrences of pneumothorax. Patient characteristics made possible the suspicion of catamenial pneumothorax. Direct correlation between episodes of pneumothorax and the onset of menstruation was found. Reoperation by videothoracoscopy was decided. During the surgery, lesions suspected of endometriosis implants and diaphragmatic fenestrations were observed. Histopathological study of the resected tissue was suspected of endometrial tissue. Hormonal treatment with oral contraceptive pills was also initiated. The following month, shewas submitted with a new pneumothorax episode which was treated with a chest tube. Currently the patient is asymptomatic 6 months follow up. Discussion: Catamenial pneumothorax is an uncommon cause of secondary spontaneous pneumothorax. Multiple hypotheses are proposed to explain its pathogenesis. A history of recurrent spontaneous pneumothorax in a woman of reproductive age requires the evaluation of the relationship with menstrual cycles...