Scimitar syndrome: case report

Abstract

This clinical case is abouta 5-month-old asculin, born full-term, with weight and size suitable for gestational age, APGAR 9/9, no suffering, no resuscitation,no pathological or surgical history known. Consults the emergency department of the National Children's Hospital after one week of respiratory symptomatology, with frank clinical data of respiratorydistress : intercostal retractions, tachypnea, nasal flutter. Required the ingress use of high oxygen flow cannula to stabilize respiratory distress; in addition to negative response to therapeutic tests with salbutamol. Chest xray, among other findings, documents a slight erasure of the cardiac silhouette and dextrocardia; ultrasound can document the presence of pleural effusion, as well as right atelectasis data observed in bronchogram; as part of the initial management plan it is decided to internthe patient for hospital management. During internment, dextrocardia is confirmed by echocardiogram, in addition to right pulmonary vessel hypoplasia, computed axial tomography of cardiac vessels is performed describing it as a result: right pulmonary hypoplasia, abnormal venous drainage of the right lung to the lower vena cava, right intralobar pulmonary abduction, hypoplasia of the right pulmonary artery,thusallowing to document the presence of a Chimitarra syndrome. Management is given by the cardiology service, where the findings are confirmed and the list of problems is reorganized, in this way it is established as problem one to the diagnosis of Chimitarra Syndrome. The case is presented in clinical session to define surgical management of the same.