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Simultaneous resection of bilateral anomalous systemic supply to the basal segments of the lungs: a case report
© Makino et al. 2015
Received: 24 May 2015
Accepted: 28 October 2015
Published: 31 October 2015
Anomalous systemic arterial supply to the normal basal lung segments is a sequestration spectrum variant (Pryce type 1) that is distinguished from pulmonary sequestration by normal bronchopulmonary and parenchymal tissues.
A 33-year-old Japanese man was referred to our hospital because of an abnormal pulmonary shadow. Computed tomography showed two aberrant arteries arising from the descending aorta and running into the lower lung lobes on each side, without any bronchial anomaly. He was diagnosed with bilateral anomalous systemic supply to the basal segments. A left thoracotomy was performed and the aberrant arteries were ligated and dissected at their origin followed by left basal segmentectomy. Simultaneous right S10 segmentectomy was performed under video-assisted thoracic surgery.
Although bilateral anomalous systemic arterial supply to the basal segments is extremely rare, knowledge of this anomaly should allow for a definitive diagnosis and appropriate therapy.
Anomalous systemic arterial supply to the normal basal lung segments  is a sequestration spectrum variant (Pryce type 1) that is distinguished from pulmonary sequestration by normal bronchopulmonary and parenchymal tissues [2, 3]. Surgery is indicated for this disease because of the potential risks including hemoptysis due to pulmonary hypertension and heart failure due to left-to-left shunt . Although bilateral anomalous systemic arterial supply to the basal segments is extremely rare , knowledge of this anomaly allows for a precise diagnosis and ligation of the aberrant arteries followed by simultaneous dissection of the lesions on both sides.
Anomalous systemic arterial supply to the normal basal lung segments is a rare congenital anomaly typically involving the left lower lobe, where pulmonary arteries of the affected segments are also absent. This anomaly also is known as Pryce type 1 sequestration.
Bilateral pulmonary sequestration is extremely rare. Moreover, we were able to perform bilateral segmentectomy to preserve pulmonary function. Savic et al.  reported that there were only two bilateral intralobar sequestration cases in their evaluation of 540 pulmonary sequestration cases. To date, seven cases of a synchronous bilateral resection have been reported, and two of them were treated with synchronous video-assisted resection of bilateral pulmonary sequestration [6, 7]. However, those reports did not describe the operative procedures in detail. In the present case, we decided to first approach the site of origin of the aberrant arteries via a left thoracotomy; then, left basal segmentectomy and simultaneous right S10 segmentectomy were performed under VATS. We thought the ligation of the aberrant arteries at their origin was important because of the potential risk of aneurismal formation later.
Previous treatment for this disease was lobectomy or basal segmentectomy with anastomosis of the divided anomalous systemic artery to the pulmonary artery [2–4], or simple ligation of the anomalous artery . Recently, coil embolization was reported in a few cases . Although ligation and coil embolization could cause pulmonary infarction when the pulmonary arterial supply is absent, in most previous cases it has not led to severe complication because of the abundant collateral circulation from the bronchial, intercostal, inferior phrenic and the other nearby arteries . In contrast, if collateral flow is not present, there is the potential risk of hemorrhage after simple ligation or embolization. In the present case, we decided to perform simultaneous resection of the bilateral anomalous systemic supply to the basal segments of the lungs because simple occlusion or ligation of the anomalous artery had a risk of pulmonary infarction and a risk of recurrent hemoptysis, although the long term data of the available therapeutic options have not been available until recently.
Evaluation of anomalous variations before selecting a therapeutic strategy and surgical procedure is essential. In the present case, we performed a left thoracotomy first according to the preoperative identification of two aberrant arteries on three-dimensional CT, which indicated that ligation of the two aberrant arteries could be performed from the left side.
Although bilateral anomalous systemic arterial supply to the basal segments is extremely rare, knowledge of this anomaly should result in the prompt, careful tracing of the course of these vessels on CT, which should allow for a definitive diagnosis and selection of appropriate therapy.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
This study was supported in part by a Grant-in-aid for Scientific Research (C) 15 K10272 from the Japanese Ministry of Education, Culture, Sports, Science and Technology and The Research Promotion Grant from Toho University Graduate School of Medicine (No.15-02 to A.I.).
The authors have no funding, financial relationships or conflicts of interest disclose.
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