Pediatrics |

Intralobar and Extralobar Pulmonary Sequestration With Scimitar Syndrome: A Complex Variant FREE TO VIEW

Annalee de Leon-Manalo, MD
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Philippine Heart Center, Quezon City, Philippines

Chest. 2015;148(4_MeetingAbstracts):777A. doi:10.1378/chest.2269819
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SESSION TITLE: Pediatrics and Congenital Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Tuesday, October 27, 2015 at 07:30 AM - 08:30 AM

INTRODUCTION: Pulmonary sequestration constitutes 0.15 - 6.4% of all congenital pulmonary malformations. There are two types of pulmonary sequestration, intralobar and extralobar. Coexistence of both is extremely rare.

CASE PRESENTATION: This is a case of a 16-year old female adolescent, presented with recurrent hemoptysis. She was initially managed as pneumonia, tuberculosis and later on as bronchiectasis. On physical examination, there was symmetrical chest expansion, decreased vocal and tactile fremiti, dullness of percussion and decreased breath sound on the right lower lung. Findings on chest ct-scan and angiography showed that the patient had intralobar and extralobar pulmonary sequestration with scimitar syndrome. Pulmonary function test was normal. 2D-echocardiography showed partial anomalous pulmonary venous return with normal pulmonary artery pressure. The patient underwent lobectomy of the right lower lobe with ligation of anomalous vessels and was eventually discharged improved. Histopathologic result was consistent with pulmonary sequestration.

DISCUSSION: Pulmonary sequestration is a dysplastic lung tissue which lacks normal communication with the tracheobronchial tree and receives blood supply from the systemic arteries. Most patients are asymptomatic and diagnosed incidentally through radiologic imaging. Our patient had presented with recurrent hemoptysis and was diagnosed to have unilateral intralobar and extralobar pulmonary sequestration with scimitar syndrome. Sequestration spectrum is the collection of abnormalities of the lung parenchyma and of its blood supply with other associated anomalies. Literatures stated that there are many variants to the pulmonary sequestration spectrum and that may include scimitar syndrome as seen in our patient. The treatment approach is controversial.

CONCLUSIONS: Our patient had presented a unique coexistence of intralobar and extralobar sequestration associated with scimitar syndrome. This is a rare developmental anomaly of the lung, described as a variant of pulmonary sequestration spectrum.

Reference #1: Jiangfeng Ou, Xiaoyong Lei, Zhou Iu, Ying Huang, Enmei Liu, et. al. Pulmonary sequestration in children: A clinical analysis of 48 cases. Int J Clin Exp Med. 2014; 7(5); 1355-1356

Reference #2: Shibli, MD, Cannery, MD, Shapiro, MD. Intralobar and extralobar bronchopulmonary sequestration complicated by Nocardia asteroides infection. South Med J. 2013; 96(1)

Reference #3: Sade, MD, Clouse, MD, Ellis Jr, MD. The spectrum of pulmonary sequestration. The Annals of Thoracic Surgery. 1974; 18(6); 644-658

DISCLOSURE: The following authors have nothing to disclose: Annalee de Leon-Manalo

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