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Year : 2012  |  Volume : 7  |  Issue : 3  |  Page : 165-167
Congenital pulmonary venolobar syndrome: Value of multidetector computed tomography in preoperative assessment

Department of Cardiothoracic Surgery, University of Tanta, Egypt

Date of Submission18-Aug-2011
Date of Acceptance10-Oct-2011
Date of Web Publication21-Jul-2012

Correspondence Address:
Abdel-Mohsen Mahmoud Hamad
Department of Cardiothoracic Surgery, Tanta University Hospital, Tanta- 31111
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1737.98852

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A 6-month old baby referred to our department because of recurrent attacks of respiratory distress with chest infection. Chest radiology revealed reduction of the right hemithorax with mediastinal shift to the right. Multidetector computed tomography showed hypoplasia of the right lung and right pulmonary artery, systemic arterial supply to the lung from the abdominal aorta, and and absence of right venous drainage to the left atrium. This picture is consistent with congenital pulmonary venolobar syndrome. The patient underwent right pneumonectomy; the postoperative course was uneventful.

Keywords: Congenital anomaly, lung, pulmonary sequestration, scimitar syndrome

How to cite this article:
Hamad AMM. Congenital pulmonary venolobar syndrome: Value of multidetector computed tomography in preoperative assessment. Ann Thorac Med 2012;7:165-7

How to cite this URL:
Hamad AMM. Congenital pulmonary venolobar syndrome: Value of multidetector computed tomography in preoperative assessment. Ann Thorac Med [serial online] 2012 [cited 2023 Jan 28];7:165-7. Available from:

Congenital pulmonary venolobar syndrome (CPVLS) refers to a wide spectrum of pulmonary developmental anomalies that involve abnormal connections of the pulmonary parenchyma, the pulmonary and systemic vasculature, and, rarely, the gastrointestinal tract. Any of the anomalies described in this syndrome may appear singly or in combination.

   Case Report Top

We present a case of a 6-month-old baby who came to our attention because of repeated attacks of respiratory distress with chest infections. Chest radiograph showed reduction of the volume of the right lung with shift of the mediastinum to the right side. Chest computed tomogram (CT) confirmed the above mentioned data in addition to demonstration of abnormal vascularization of the right lung [Figure 1]a. Bronchoscopy showed bifurcation of the right main stem bronchus, a pattern similar to what is usually seen in the left tracheobronchial tree. The echocardiogram demonstrated dextrocardia, marked hypoplastic right pulmonary artery, but no intracardiac anomalies. Multidetector computed tomography (MDCT) of the chest demonstrated hypoplastic right lung and tiny right pulmonary artery [Figure 1]b, abnormal systemic blood supply arising directly from the aorta below the diaphragm [Figure 1]c, and an absence of right pulmonary venous drainage to the left atrium [Figure 1]d. Persistent left superior vena cava was also observed.
Figure 1: (a) Chest CT shows hypoplasia of the right lung, mediastinal shift and abnormal vascularization of the right lung; (b) MDCT chest showing small right pulmonary artery (white arrow); (c) MDCT chest showing systemic arterial supply of the right lung; (d) MDCT chest showing absence of right pulmonary venous drainage to left atrium

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The patient underwent right pneumonectomy through a right posterolateral thoracotomy. At surgery, the lung was hypoplastic with only one incomplete oblique fissure. The right pulmonary artery was very small [Figure 2]a, the main arterial supply was through multiple branches coming through the diaphragm and entering the lung through the diaphragmatic surface [Figure 2]c. The venous drainage was through a single large vein draining to the inferior vena cava [Figure 2]b. The postoperative course was uneventful. Eight months after surgery, the patient is doing well.
Figure 2: (a) Operative view showing very small right pulmonary artery; (b) showing the scimitar vein; (c) showing the resected right lung, black arrows points to the stumps of the systemic arteries entering the lung through the diaphragmatic surface

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   Discussion Top

This patient's findings are consistent with several features of a syndrome described by Felson as CPVLS. Major components of CPVLS include hypogenetic lung, partial anomalous pulmonary venous return, absence of a pulmonary artery, pulmonary sequestration, systemic arterialization of the lung, absence of the inferior vena cava, and accessory diaphragm. Minor components of CPVLS include tracheal trifurcation, eventration and partial absence of the diaphragm, phrenic cyst, horseshoe lung, esophageal and gastric lung, anomalous superior vena cava, and absence of the pericardium. [1] The primary feature of CPVLS is an abnormal drainage of a right pulmonary vein into the vena cava. This abnormal drainage course of the vein can often be seen on plain chest radiograph as a curvilinear density along the right heart border. Because this radiological sign resembles the Turkish sward, the anomaly was subsequently named the "scimitar syndrome." [2]

Traditionally, angiography has been used for imaging of pulmonary vascular anomalies, especially when pulmonary sequestration or scimitar syndrome is suspected. [3],[4] The objective is to identify the anomalous vessels (arteries and veins) in order to plan surgery and avoid accidental division or ligation. With advancement of CT technology, several studies have suggested the value of CT angiography as a noninvasive alternative in cases of pulmonary sequestration or CPVLS. [5],[6],[7] More recently, the introduction of MDCT allows improved delineation of the complex and variable anatomic abnormalities seen in patients with CPVLS obviating the use of angiography or magnetic resonance angiography for diagnosis. [8]

The clinical presentation of CPVLS is variable and depends mainly on the presence of associated cardiac anomalies and the potential development of pulmonary hypertension due to a large left to right shunt through the systemic arterial supply to the right lung. Some patients are symptomatic in the newborn period presenting with respiratory insufficiency and/or cardiac failure. Surgery is indicated for such patients and the success depends on the nature and severity of the underlying abnormalities. [9] This case underwent pneumonectomy. On the contrary, some surgeons may recommend coil embolization (or surgical ligation) of the systemic arterial supply and re-routing of the anomalous vein to the left atrium. The aim is to conserve lung tissue and avoid the long-term issues of pneumonectomy in a young infant. However, in this infant, the right pulmonary artery was very tiny supplying only part of the upper lobe, and the main blood supply was from the systemic artery. So, the consequences of embolization or ligation of the systemic artery was unpredictable.

Another group of patients presents during young adulthood with recurrent pulmonary infections. The infections usually affect the right lower lobe which is most likely to be the site of pulmonary sequestration. These patients may require lobectomy or occasionally right pneumonectomy to prevent recurrent infections. [10]

It is not uncommon to diagnose CPVLS as incidental finding in a chest radiograph. In such cases, due to absence of symptoms, these patients can be managed conservatively.

In summary, the term CPVLS is an umbrella to a group of pulmonary parenchymal and vascular anomalies that may present singly or in combination. The presence of any of these anomalies mandates search for the others. MDCT is an excellent diagnostic tool for delineation of the components of this syndrome.

   References Top

1.Woodring JH, Howard TA, Knaga JF. Congenital pulmonary venolobar syundrome revisited. Radiograhics 1994;14:349-69.  Back to cited text no. 1
2.Canter CE, Martin TC, Spray TL, Weldon CS, Stuass AW. Scimitar syndrome in childhood. Am J Cardiol 1986;58:652-4.  Back to cited text no. 2
3.Ellis K. Developmental abnormalities in the systemic blood supply to the lungs. AJR Am J Roentgenol 1991;156:669-79.  Back to cited text no. 3
4.Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994;41:453-72.   Back to cited text no. 4
5.Amitai M, Konen E, Rozenman J, Gerniak A. Preoperative evaluation of pulmonary sequestration by helical CT angiography. AJR Am J Roentgenol 1996;167:1069-70.  Back to cited text no. 5
6.Franco J, Aliaga R, Domingo ML, Plaza P. Diagnosis of pulmonary sequestration by spiral CT angiography. Thorax 1998;53:1089-92.  Back to cited text no. 6
7.Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: Radiologic-pathologic correlation. Radiographics 2002;22:S25-43.  Back to cited text no. 7
8.Konen E, Raviv-Zilka L, Cohen RA, Epelman M, Boger-Megiddo I, Bar-Ziv J, et al. Congenital pulmonary venolobar syndrome: Spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics 2003;23:1175-84.   Back to cited text no. 8
9.Schramel FM, Westermann CJ, Knaepen PJ, van den Bosch JM. The scimitar syndrome: Clinical spectrum and surgical treatment. Eur Respir J 1995;8:196-201.  Back to cited text no. 9
10.Thibault C, Perrault L, Delisle G, Cartier P, Cloutier A, Houde C, et al. Lobectomy in the treatment of the scimitar syndrome. Ann Thorac Surg 1995;59:220-1.  Back to cited text no. 10


  [Figure 1], [Figure 2]

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