RESUMEN
We present the case of a 7-month-old female infant with a history of recurrent respiratory infections and symptoms of respiratory distress during feeding. Echocardiography isolated revealed supracardiac-type total anomalous pulmonary venous connection with a large ostium secundum atrial septal defect and severe pulmonary hypertension. Computed tomographic angiography confirmed the diagnosis and identified stenosis at the level of the venous confluence. The surgical intervention involved a novel approach using dual anastomoses between the pulmonary venous confluence and the left atrium, alongside atrial septal defect repair with a bovine pericardial patch. Postoperative recovery was uneventful, with successful weaning from mechanical ventilation on Day 9 and discharge on Day 12. The patient showed optimal venous drainage and hemodynamic stability, indicating a successful surgical outcome. This case highlights the importance of early surgical intervention in total anomalous pulmonary venous connection with complex anatomical presentations.
RESUMEN
RATIONALE: Desmoid-type fibromatosis (DTF), also known as aggressive fibromatosis, is a rare neoplasm originating from the fascial or musculoaponeurotic tissues. While benign and characterized by slow growth, it exhibits local aggressiveness and lacks specific clinical characteristics. However, in a considerable percentage of patients, it could be asymptomatic and discovered by accident during routine clinical examinations. Only a few cases of DTF arising from the psoas major muscle have been reported in the medical literature. PATIENT CONCERNS: A 24-year-old male, asymptomatic and without significant personal or family medical history, was diagnosed with grade 2 hydronephrosis by abdominal ultrasonography during a routine physical examination. This diagnosis was made 15 days after undergoing uncomplicated open-heart surgery to repair an atrial septal defect. DIAGNOSIS: Intravenous pyelogram revealed hydronephrosis with dilation of the pelvicalyceal system. Ureteroscopy ruled out any intrinsic lesions of the ureter. Contrast-enhanced computed tomography identified a 3.5â ×â 2â ×â 5.2 cm mass in the retroperitoneum, closely associated with the psoas muscle and enveloping the ureter adjacent to the iliac artery. Postoperative pathological analysis confirmed a definitive diagnosis of sporadic DTF. INTERVENTIONS: The patient underwent exploratory abdominal surgery, during which the tumor was resected without any intraoperative complications. RESULTS: After close monitoring over a 5-year follow-up period, which included periodic physical examinations, magnetic resonance imaging, and ultrasonography, no local recurrence was detected. LESSONS: Achieving an accurate preoperative diagnosis presents a challenge in cases involving retroperitoneal tumors originating from the psoas major muscle and encasing the ureter. However, the insertion of a double J stent is deemed a crucial step in the surgical process, facilitating the dissection and isolation of the ureter from the tumor while preserving kidney function.