European Journal of Radiology, 13 (1991) 138-142

138

EURRAD

0 1991 Elsevier Science Publishers

B.V. All rights reserved.

0720-048X/91/$03.50

00181

CT diagnosis of isolated systemic supply to the lung: a congenital broncho-pulmonary vascular malformation Josep M. Mata ‘, Jo& Cheres ‘Servicio de Radiodiagnhtico



and Xavier Lucaya2

de1 Hospital de la Santa Creu i Sam Pau J. and 2Servicio de Radiodiagnhtico Vail d’tlebrh,

(Received

Give&tat

16 October

Authoma

1990; accepted

Key words: Thorax, CT; Thorax, anomalies;

de Barcelona,

Barcelona,

after revision

19 February

Thorax, vascular malformation;

Introduction Isolated systemic supply to the normal lung (ISSNL) is a rare congenital anomaly, first described by Hurber in 1777 [ 11. Many cases have since been published, diagnoses being made by necropsy, surgery or more recently by angiography [l-15]. However, only one case in the literature has been studied by CT and the description of the findings were schematic [ 151. We present the CT findings in two patients with this rare malformation. In both cases CT provided sufficient data for the diagnosis. Case 1 A 5-year-old boy presented with a 2-year history of continuous murmur in the lower right hemithorax. A chest radiography (Fig. la) showed several tubular shadows in the right lower lobe, partially hidden by the diaphragm. Contrast-enhanced CT (Fig. lb and c) showed vascular structures arising from the aorta, perfusing the posterior segments of the right lower lung which were aerated and without any signs of disease. Aortography (Fig. Id) confirmed the existence of an anomalous artery originating in the abdominal aorta

Address for reprints: J.M. Mata, M.D., Servicio de Radiodiagnostico, Hospital de la Santa Creu i Sant Pau, Avda. S. Antoni M. Claret, 167, 08025 - Barcelona, Spain.

de la Clinica Infant2 de la C.S.

Spain

1991)

Computed

tomography,

lung

and perfusing the right basal segments. Venous return was by the right lower pulmonary vein. Pulmonary arteriography demonstrated a lack of perfusion of the segments supplied by the systemic artery. With the diagnosis of IS SNL a right thoracotomy was performed and the anomalous vessel was ligated. Ischemia during surgery motivated partial resection of the right lower lobe. The removed pulmonary parenchyma was normal. Case 2 A 28-year-old woman presented with a murmur in the left hemithorax first detected at 15 years of age. History disclosed hemoptysis at 7 years of age. Hemoptysis appeared again 5 months before the first visit to our centre. Alveolar condensation was detected in the left lower lobe at that time. Two months later she had a third episode of hemoptysis. On admission, the chest radiograph showed a nodular shadow in the left lower lobe. Bronchoscopy was normal. Contrastenhanced CT (Fig. 2a and b) revealed an anomalous artery arising from the aorta coursing into the pulmonary ligament and perfusing the basal segments of the left lower lobe. The pulmonary parenchyma appeared totally aerated and free of disease. Aortography (Fig. 2c) confirmed these findings. Venous return was via the left inferior pulmonary vein. Pulmonary arteriography (Fig. 2d) demonstrated lack of perfusion of the area supplied by the systemic artery. The patient refused surgery.

a

b

Fig. 1. (a) Multiple tubular images (arrows) in the right lower lobe converging towards the lowest portion of the cardiophrenic angle. (b) The anomalous vessel (arrow) is visible in CT crossing the mediastinum and entering the right lower lobe. (c) Lung window shows no signs of disease in the pulmonary parenchyma. The branches of the anomalous artery (arrow) are visible. (d) Aortography confirms the presence of an anomalous artery (arrows) arising from the abdominal aorta.

Fig. 2. (a) CT at the level of the pulmonary ligament shows a thick artery (arrows) arising from the descending aorta and penetrating into the pulmonary ligament. (b) Lung window shows the branches of the systemic vessels. There are no signs of pulmonary disease. The frontier between the segment irrigated by the systemic artery and by the pulmonary artery (arrows) is clearly visible. (c) Aortography confirms the presence of a large systemic artery (arrows) irrigating part of the left lower lobe. (d) The pulmonary arteriogram demonstrates lack of vascularization of the segments irrigated by the systemic artery and a decrease in the calibre of the left lower lobar artery.

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Discussion Aberrant systemic arterial supply to a lobe of the lung can occur as an isolated finding or be associated with anomalous pulmonary tissue [ 91. Pryce [ 1,161 was the first to classify pulmonary malformations with systemic perfusion and to employ the term “sequestration”. He classified systemic vascularization of the lung into three types according to whether it perfused normal lung, abnormal lung with no connection to the bronchial tree, or both. In the seventies, the term ‘sequestration spectrum’ [ 17-191 was used to encompass all pulmonary malformations either isolated or combining cystic pulmonary disease and anomalies of arterial or venous vascularization. In 1987 Clements and Warner proposed a new approach to pulmonary congenital malformations [ 201. They introduced the term ‘pulmonary malinosculation’ to describe a congenital abnormal connection or opening of one or more components of the bronchopulmonary-vascular complex. The various combinations of these components give rise to a wide spectrum of abnormalities. The lesions thus formed can then be classified simply according to their abnormal anatomical components. The classification is constructed in three steps. The first step describes the abnormality of the bronchopulmonary airway or arterial blood supply, or both. The possible combinations of these two components form a spectrum of lesions. At one end of the spectrum is absence or abnormality of bronchopulmonary airway with normal blood supply (bronchial pulmonary malinosculation). At the other end of the spectrum is an anomalous arterial supply to an area of normal lung (arterial pulmonary malinosculation). In between are all the combinations of abnormal bronchial and arterial connections (bronchoarterial pulmonary malinosculation). Heitzman [21] proposes a similar classification, with a normal lung perfused by systemic arteries at one end of the sequestration spectrum. At the opposite end there is anomalous pulmonary tissue similar to that found in sequestration, but without systemic perfusion. Between the two there are six pathological processes (congenital lobar emphysema, bronchogenic cyst, congenital cystic adenomatoid malformation, hypogenetic lung syndrome, bronchopulmonary sequestration and pulmonary arteriovenous malformation) with a high degree of overlapping between them, rendering classification difficult and in some cases making it impossible to group the pathologic findings into a definite entity. The prototype of these anomalies is pulmonary sequestration, defined as a congenital pulmonary malformation in which there is a mass of nonfunctioning pulmonary tissue, unattached to the bronchial tree, per-

fused by systemic arteries. If the anomalous arteries perfuse normal pulmonary tissue, it is considered a different malformation, called isolated systemic supply to normal lung (ISSNL) [ 121. In ISSNL, patients are usually asymptomatic, although there may exist a continuous murmur on the thoracic wall or left ventricular growth and heart failure secondary to left-to-left shunt [ 111. Hemoptysis has occasionally been described, as in our second case [ 1.51. Associated diaphragmatic defects may occasionally be seen [2,9]. Chest radiography shows increased density in the affected lung. It is sometimes possible to recognize well-defined tubular or rounded images produced by the anomalous vessels. The pulmonary parenchyma does not present any other changes, apart from the exceptional cases of bleeding. Bronchoscopy and bronchography are normal. Aortography shows the systemic vessel arising from the aorta and perfusing the affected lung without direct communication between the systemic artery and the pulmonary vein. Pulmonary angiography shows a decrease in the calibre or absence of the pulmonary branches perfusing the anomalous zone. CT has been used successfully in the study of congenital pulmonary malformations [ 221. The only case of ISSNL studied by CT in the literature [ 151 did not include a detailed description of the findings. In our two cases, CT enabled a definitive diagnosis revealing the characteristic signs of ISSNL, a systemic artery and absence of pathology of the underlying lung. In other entities with systemic vascularization, namely pulmonary sequestration, the anomalous artery is identified by CT in only 40% of cases [22], perhaps due to the fact that pulmonary disease hampers visualization of the artery. Visualization of the anomalous vessel and its intrapulmonary pathway in ISSNL is probably facilitated by the absence of pulmonary involvement. The majority of cases of IS SNL appear to have a single artery with a thick calibre, probably yet another factor enabling easy identification by CT. Differential diagnosis with sequestration is possible as pulmonary involvement is always seen by CT in true sequestration [ 22-261. Differential diagnosis with other vascular malformations without parenchymatous involvement is not required as the AV malformation comes from the pulmonary artery, has an anomalous venous return and does not perfuse the pulmonary parenchyma. Although our findings need confirmation in further studies, we believe CT should be the initial method of study when ISSNL is suspected as it provides rapid, non-invasive identification of the systemic vascularization and demonstrates the absence of pulmonary involvement, both characteristic signs of this entity.

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CT diagnosis of isolated systemic supply to the lung: a congenital broncho-pulmonary vascular malformation.

European Journal of Radiology, 13 (1991) 138-142 138 EURRAD 0 1991 Elsevier Science Publishers B.V. All rights reserved. 0720-048X/91/$03.50 001...
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