Intralobar Pulmonary Sequestration with Anomalous Artery Arising from the Celiac Trunk

Alexandr E Mashkov,Vyacheslav V Slesarev,Johannes M Mayr,Andrei V Bobylev

doi:10.3390/surgeries2010009

Alexandr E Mashkov, Vyacheslav V Slesarev + Show 2 more

Open Access

https://doi.org/10.3390/surgeries2010009

Copy DOI

Journal: Surgeries	Publication Date: Feb 16, 2021
License type: CC BY 4.0

Affiliation: University Children’s Hospital Basel

Abstract

Pulmonary saequestration is a rare congenital malformation characterized by a dysplastic portion of lung parenchyma supplied by an anomalous artery originating from the aorta or its branches. The worldwide incidence of pulmonary sequestration among all congenital lung malformations in children ranges from 1.5% to 6.4%. There are two main types of pulmonary sequestration according to the localization of the malformation, i.e., intrapulmonary sequestration (dysplastic tissue located inside a lobe of the normal lung) and extrapulmonary sequestration. Our case presentation aims to make physicians aware of this rare anomaly which may be difficult to diagnose because of its oligosymptomatic course prior to first presentation. We present the case of a 10-year-old girl who suffered from a second episode of prolonged pneumonia of the left lower lobe. Contrast-enhanced-computed-tomography (CT) scan of the thoraco-abdominal segment of the aorta and its branches revealed intrapulmonary sequestration localized at the left lower lobe of the lung. The intrapulmonary sequester was perfused by a large artery arising from the celiac trunk. The girl underwent open surgery with ligation of the anomalous feeding artery and atypical pulmonary resection of the affected area of the left lower lobe. Postoperatively, the child recovered without any complications.

Highlights

Pulmonary sequestration is a rare congenital abnormality where a dysplastic portion of the lung parenchyma is supplied by an anomalous systemic artery originating from the aorta or its branches [1,2,3]
There are two main types of pulmonary sequestration depending on the localization of the malformation, i.e., intrapulmonary sequestration and extrapulmonary sequestration [5,6,7,8]
Surgical removal of the area of the lung affected by extrapulmonary or intrapulmonary sequestration or ectopic sequestration with ligation of the feeding artery is the method of choice for treating this congenital malformation [12,14]

Summary

Introduction

Pulmonary sequestration is a rare congenital abnormality where a dysplastic portion of the lung parenchyma is supplied by an anomalous systemic artery originating from the aorta or its branches [1,2,3]. The majority of intrapulmonary sequestrations diagnosed prenatally tend to decrease in size after 28 weeks of gestation [4]. This congenital malformation is characterized by autonomic arterial blood supply (often by an anomalous artery of large diameter), abnormal bronchial development, and impaired development of lung parenchyma. Surgical removal of the area of the lung affected by extrapulmonary or intrapulmonary sequestration or ectopic sequestration with ligation of the feeding artery is the method of choice for treating this congenital malformation [12,14]. We obtained written informed consent from the patient’s parents to publish this case report

Case Presentation

Differential Diagnoses

Investigations

Discussion

Cystic Type with Formation of Abscess

Pseudotumor Type

Bronchiectatic Type