Left pulmonary vein anatomical variation in Turner syndrome. Benefits of three-dimensional visualization for surgical planning

Abstract

A 41 year-old female with a medical history of Turner syndrome underwent a chest computed tomography (CT) scan which revealed a varicose left pulmonary vein and an endobronchial tumor of the left lower lobe. As venous drainage of each lobe seemed to be respected, surgical resection was considered. During surgical exploration, the absence of fissure and a unique venous trunk was observed. Surgical resection was aborted as only pneumonectomy was possible in this context. Endobronchial resection was performed. To better understand this particular anatomy, a three-dimensional (3D) reconstruction was performed a posteriori. This technique is already commonly used in the preoperative planning of pulmonary segmentectomy. Here, we have shown its interest in a lung malformative context.

Keywords: anomalous pulmonary vein connection; carcinoid tumor; turner syndrome; varicose pulmonary veins.

FIGURE 1

Preoperative investigations. (a, b) Computed tomography (CT) scan of the chest (axial view). An anomaly of the left pulmonary vein is diagnosed which appears varicose and has an unusual course. (c) Vascular reconstruction of the chest CT scan. A varicose vein of 9.8 mm diameter is visualized in the left upper lobe. (d) CT scan of the chest (coronal view). An endobronchial lesion is incidentally diagnosed in a left basal bronchus (white arrow). (e) A left pulmonary angiography showing a varicose superior pulmonary vein with an abnormal course appearing to drain into a superior venous trunk. The star shows the pulmonary veinous trunk. The upper arrow shows the abnormal varicose superior pulmonary vein. The lower arrow shows the inferior pulmonary vein.

FIGURE 2

(a) Per operative view. Noninflated lung with no fissure. (b) Per operative view after fissure dissection. The black star indicates the heart, white star shows the varicose vein passing through the lung and the black triangle indicates what is supposed to be the left lower lobe. The white triangle shows what is supposed to be the left upper lobe. The blue surgical drape indicates the back of the patient. F, front of patient; H, head of patient; LCW, lateral chest wall. (c) Three‐dimensional (3D) modeling of the bronchovascular tree. The white star indicates the pulmonary artery, and the black star shows a unique left pulmonary vein draining the whole lung. (d) 3D modeling of the bronchovascular tree and lung parenchyma. The black line indicates the theoretical fissure. The superior pulmonary vein crosses the lung to drain into a unique inferior vein. There is no “fissural” pulmonary artery.