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DOI:10.2214/AJR.05.1236
AJR 2006; 187:W133-W134
© American Roentgen Ray Society

Bilateral Pulmonary Sequestration with Bridging Isthmus in a Boy with Williams Syndrome

Richard Kuo, Shin-Lin Shih and Jon-Kway Huang

Mackay Memorial Hospital Taipei, Taiwan
Mackay Memorial Hospital and Tapei Medical University Taipei, Taiwan

A 5-year-old boy presented with cough and intermittent fever for 1 week. Unusual facial appearances of flat nasal bridge, long philtrum, and wide mouth were noted during physical examination. A systolic heart murmur at the right upper sternal border was also found. Echocardiography revealed supravalvular aortic stenosis. Chest radiography showed a soft-tissue mass superimposed with cardiac shadow (Fig. 2A). A chest CT scan revealed a well-defined mass at the medial basal segments of the bilateral lower lobes with a midline bridging isthmus behind the heart (Fig. 2B). An MR angiogram showed that the blood supply of the mass was an aberrant feeding artery from the celiac trunk (Fig. 2C). A finding of supravalvular aortic stenosis was also obtained on MR images (Fig. 2D). Surgical intervention found a mass crossing the midline. Pathologic findings of the mass were consistent with pulmonary sequestration. The boy did well after the surgery. Fluorescence in situ hybridization (FISH) revealed deletion of the elastin gene located at chromosome 7. The result of the FISH was consistent with Williams syndrome.


Figure 1
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  		Fig. 2A —5-year-old boy with Williams syndrome. Chest radiograph showed ovoid soft-tissue mass superimposed with cardiac shadow (arrowheads).

 

Figure 2
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  		Fig. 2B —5-year-old boy with Williams syndrome. Contrast-enhanced chest CT scan revealed well-defined heterogeneous mass at medial basal segments of bilateral lower lobes with midline bridging isthmus posterior to heart.

 

Figure 3
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  		Fig. 2C —5-year-old boy with Williams syndrome. Gadolinium-enhanced MR angiogram in coronal section showed aberrant feeding artery from celiac trunk.

 

Figure 4
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  		Fig. 2D —5-year-old boy with Williams syndrome. Gadolinium-enhanced MR angiogram in sagittal section revealed hourglass deformity of ascending aorta just above sinus (arrowheads), compatible with supravalvular aortic stenosis.

 

Pulmonary sequestration is defined as a segment of lung parenchyma that lacks communication with the tracheobronchial tree and receives an aberrant systemic arterial blood supply. The presence of a bridging isthmus between bilateral pulmonary sequestrations is extremely unusual. To our knowledge, it has only been reported once before [1]. Bilateral pulmonary sequestrations with a bridging isthmus may mimic a horseshoe lung. Horseshoe lung is formed from the joining of bilateral lung bases by an isthmus of lung parenchyma behind the heart. The arterial supplies to the isthmic portion of a horseshoe lung are almost always the branches from the pulmonary artery [2]. Most of the reported cases have been associated with a certain degree of pulmonary hypoplasia [3]. The feeding artery of the mass in our case turned out to be an arterial branch from the celiac trunk. Although there are a few reported cases of horseshoe lung in which the isthmus is supplied by both pulmonary and aortic branches [2], the boy did not have the anomalies associated with hlorseshoe lung. So the diagnosis of bilateral pulmonary sequestrations with a bridging isthmus is more favored than horseshoe lung.

Williams syndrome is a rare genetic condition with clinical manifestations of distinctive elfin face, mental retardation, friendly behavior, and congenital cardiovascular anomalies. Deletion of chromosome band 7 that includes the elastin gene is identified as present in about 95% of individuals with Williams syndrome. The commonly associated anomalies include supravalvular aortic stenosis, pulmonary artery stenosis, coronary artery stenosis, and mitral valve prolapse [4]. To our knowledge, the association of Williams syndrome with bilateral pulmonary sequestration has not been described previously. But the association may just be coincidental.


References
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 References
 

  1. Cerruti MM, Marmolejos F, Cacciarelli T. Bilateral intralobar pulmonary sequestration with horseshoe lung. Ann Thorac Surg 1993; 55:509 -510[Abstract]
  2. Goo HW, Kim YH, Ko JK, Park IS, Yoon CH. Horseshoe lung: useful angiographic and bronchographic images using multidetector-row spiral CT in two infants. Pediatr Radiol 2002;32 : 529-532[CrossRef][Medline]
  3. Lutterman J, Jedeikin R, Cleveland DC. Horseshoe lung with left lung hypoplasia and critical pulmonary venous stenosis. Ann Thorac Surg 2004; 77:1085 -1087[Abstract/Free Full Text]
  4. Bruno E, Rossi N, Thuer O, Cordoba R, Alday LE. Cardiovascular findings, and clinical course, in patients with Williams syndrome. Cardiol Young 2003;13 : 532-536[Medline]

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