RESUMO
Truncus arteriosus, interrupted aortic arch and amniotic band syndrome are rare conditions. We report a case of a 38-year-old pregnant woman who was diagnosed on a routine morphological ultrasound scan with a Van Praagh type A4 persistent truncus arteriosus with an aortic arch interruption type B and abnormal limbs (oedematous left hand, hypoplastic fingers on the right hand and right big toe amputation). Elective termination of the pregnancy was carried out and the pathological examination confirmed all the sonographic findings. Furthermore, an amniotic band parallel to the umbilical cord undetected during the ultrasound scans was revealed, and was entangled around the right hand, left wrist and the umbilical cord, causing strangulation. We present the unusual association of these independent pathologies and emphasise the usefulness of fetal autopsy in all cases of pregnancy termination and abnormal ultrasound findings to make the complete diagnosis.
Assuntos
Anormalidades Múltiplas , Síndrome de Bandas Amnióticas/diagnóstico , Diagnóstico Pré-Natal/métodos , Doenças Raras , Persistência do Tronco Arterial/diagnóstico , Adulto , Amniocentese/métodos , Síndrome de Bandas Amnióticas/complicações , Diagnóstico Diferencial , Ecocardiografia/métodos , Feminino , Humanos , Gravidez , Persistência do Tronco Arterial/etiologiaRESUMO
La púrpura trombocitopénica trombótica es una enfermedad multisistémica de etiología desconocida. Se han observado diversas circunstancias que pueden actuar como factores predisponentes: la gestación, un proceso infeccioso, los anticonceptivos orales y la cirugía. Las mujeres embarazadas o en el período posparto representan un 10% de los casos de púrpura trombocitopénica trombótica, con una incidencia aproximada de un caso de cada 25.000 partos. Presentamos un caso clínico de una mujer que desarrolló púrpura trombocitopénica trombótica a los 6 días de habérsele realizado una cesárea, sin otros hallazgos significativos, enfatizando la importancia de reconocer esta afección en vista de la similitud con otras enfermedades durante el embarazo (AU)
Thrombotic thrombocytopenic purpura is a multisystem disease of unknown etiology. There are various predisposing factors, such as pregnancy, an infectious process, oral contraceptives, and surgery. Pregnant or postpartum women represent 10% of cases of thrombotic thrombocytopenic purpura, with an approximate incidence of one in 25,000 births. We report the case of a woman who developed thrombotic thrombocytopenic purpura 6 days after undergoing a caesarean section without other significant findings, emphasizing the importance of distinguishing this disease from other, highly similar illnesses during pregnancy (AU)