Neumotórax espontaneo en paciente gestante / Spontaneous Pneumothorax in a Pregnant Woman

RESUMEN Introducción: El neumotórax es una de las enfermedades pleurales más frecuentes en la práctica médica, siendo excepcional su asociación con el embarazo, existiendo pocos casos reportados en la literatura mundial. La linfangioleiomiomatosis es una enfermedad multisistémica poco frecuente, que afecta predominante al sexo femenino en edad fértil y puede verse exacerbada por el embarazo. Objetivo: Realizar el reporte de un caso y la revisión del tema que permitan un diagnóstico precoz y una conducta de forma temprana. Caso clínico: Paciente gestante de 15 semanas, de 36 años de edad, con antecedentes de salud anterior, que debuta súbitamente con disnea a los medianos esfuerzos, tos, dolor torácico y neumotórax derecho espontáneo, recurrente en su evolución y posteriormente bilateral. Su curso fue tórpido requiriendo tratamiento en Unidad de Cuidados Intensivos por fallo respiratorio agudo, falleciendo luego de 3 meses del inicio del cuadro. Se le realizó, Radiografía de tórax: patrón reticular de tipo panal de abejas, tomografía de tórax: imágenes quísticas múltiples en todo parénquima pulmonar de predominio basal. Biopsia pulmonar compatible con linfangioleiomiomatosis. Conclusiones: La aparición de disnea súbita, dolor pleurítico y neumotórax en una gestante, deben ser siempre suficientes para tener en cuenta la presencia de una linfangioleiomiomatosis. Su inespecificidad sintomática inicial se traduce en un diagnóstico tardío, lo que empobrece su pronóstico(AU)

ABSTRACT Introduction: Pneumothorax is one of the pleural diseases most frequent in medical practice. Its association with pregnancy is essential. Few cases are reported in worldwide medical literature. Lymphangioleiomyomatosis is a rare multisystem disease that predominantly affects individuals of the female sex and at fertile age; it can be aggravated by pregnancy. Objective: To present a case report and a topic review that allow early diagnosis and early management. Clinical case: A 15-week-pregnant patient aged 36 years and with a previous health history suddenly presented dyspnea for average efforts, cough, chest pain, and spontaneous right pneumothorax, recurrent in its evolution and, later, bilateral. Its evolution was slow, a reason why it required intensive care for acute respiratory failure. The patient died three months after the onset of symptoms. The patient was performed chest x-ray, which showed honeycomb-type reticular pattern; and chest tomography, which showed multiple cystic images throughout pulmonary parenchyma, predominantly at baseline. Lung biopsy consistent with lymphangioleiomyomatosis was performed. Conclusions: Onset of sudden dyspnea, pleuritic pain and pneumothorax in a pregnant woman should always be sufficiently indicative of lymphangioleiomyomatosis. Its initial symptomatic non-specificity is determined by late diagnosis, which impoverishes prognosis(AU)

Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report

ABSTRACT CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung. Treatment with everolimus was started. The disease stabilized in the third month of treatment, according to the response evaluation criteria in solid tumors. CONCLUSION: PEComas are tumors with unpredictable behavior. Therefore, these patients require long-term follow-up, even in cases of correct diagnosis and benign PEComa.

[Lymphangioleiomyomatosis and tuberous sclerosis association]

The Lymphangioleiomyomatosis is a rare lung disease encountered almost exclusively in women of childbearing age. The combination with tuberous sclerosis is even more rare. Lung affection is characterized by thin walled multiple pulmonary cysts, recurrent pneumothorax, an obstructive ventilatory trouble, and an evolation to chronic respiratory failure within an average of 10 years. A patient aged 51 years old with a history of generalized seizures, hospitalized in the department for exertional dyspnea of NYHA stage III of gradual onset since 5 years, combined with a dry cough and some episodes of minimal hemoptysis leading to weakness of the general condition. Clinical examination found diffuse bilateral crepitational rale. The chest radiograph showed diffuse bilateral interstitial syndrome. The cardiovascular examination with ECG and echocardiography showed a mild PAH of 42 mmHg. The chest CT revealed multiple diffuse thin-walled cystic formations highly suggestive of lymphangioléimyomatose. Abdominal and kidney ultrasound showed no appearance of renal angiomyolipoma or uterine fibroids. Furthermore, cerebral CT showed encephalic calcifications objectified for a Tuberous sclerosis of Bourneville. Plethysmography showed a mixed ventilatory disorder predominantly obstructive. The diagnosis of pulmonary Lymphangioleiomyomatosis associated with Tuberous sclerosis of Bourneville has been retained. The patient was put on a long term oxygen therapy with good evolution. The combination Lymphangioleiomyomatosis and tuberous sclerosis of Bourneville is rare, interesting almost exclusively young women. International diagnostic criteria have been proposed for the diagnosis of these disorders. The prognosis remains reserved