0
Pediatrics |

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

Annalee de Leon-Manalo, MD
Author and Funding Information

Philippine Heart Center, Quezon City, Philippines


Chest. 2015;148(4_MeetingAbstracts):777A. doi:10.1378/chest.2269819
Text Size: A A A
Published online

Abstract

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

No Product/Research Disclosure Information


Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543