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A Rare Case of Right-Sided Extralobar Pulmonary Sequestration and Bronchogenic Cyst Carolyn Hanna1, Priya G

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A Rare Case of Right-Sided Extralobar Pulmonary Sequestration and Bronchogenic Cyst Carolyn Hanna1, Priya G Hanna et al. Egyptian Journal of Radiology and Nuclear Medicine (2021) 52:54 Egyptian Journal of Radiology https://doi.org/10.1186/s43055-021-00440-1 and Nuclear Medicine CASE REPORT Open Access Concurrent bronchopulmonary foregut malformations: a rare case of right-sided extralobar pulmonary sequestration and bronchogenic cyst Carolyn Hanna1, Priya G. Sharma1, Moiz M. Mustafa2, Jennifer Reppucci3, Archana Shenoy3 and Dhanashree Rajderkar1* Abstract Background: Bronchopulmonary foregut malformations are rare congenital malformations. It is extremely rare to have malformations that occur simultaneously. There is literature to show that extralobar sequestration is associated with other congenital anomalies, most commonly diaphragmatic hernias, and also with other bronchopulmonary foregut malformations (e.g., extralobar sequestration and congenital pulmonary airway malformations). However, very few case reports were found that reported extralobar sequestration and foregut duplication cysts and only one report of a right-sided complex foregut malformation with pulmonary sequestration. Case presentation: We present a case of a 3-month-old male infant with a prenatal diagnosis of a cystic lung lesion who, after developing symptoms of respiratory distress, was found to have concurrent right-sided extralobar pulmonary sequestration and a mediastinal bronchogenic cyst. Conclusions: The concurrent occurrence of these malformations in one patient could help support the theory that these malformations result from an early error in development during the time when both the lung buds and foregut are developing simultaneously. Keywords: Bronchopulmonary foregut malformation, Pulmonary sequestration, CPAM, Bronchogenic cyst, Foregut duplication cyst Background cases demonstrating an association between extralobar Bronchopulmonary foregut malformations are rare pulmonary sequestration and foregut duplication cysts. In congenital malformations resulting from anomalous this report, we present a unique case of a 3-month-old budding of the tracheobronchial tree and primitive male infant who was prenatally diagnosed with a cystic foregut. These lesions can be diagnosed in the antenatal lung lesion and developed respiratory distress postnatally period using prenatal ultrasound. Postnatal presentation is with the discovery of right-sided extralobar sequestration varied and can include cough, respiratory distress, and dif- and concurrent bronchogenic cyst. ficulty with feeding [1]. Surgical resection is the preferred method of treatment. The concurrent occurrence of mul- Case presentation tiple malformations has been documented, with several A 3-month-old male infant with a prenatal ultrasound diagnosis of a right cystic lung lesion presented for * Correspondence: [email protected] repeated episodes of respiratory distress, especially 1Department of Radiology, University of Florida College of Medicine, 1600 SW Archer RD, Gainesville, FL 32608, USA during feeding, with a prior bronchoscopy suggestive of Full list of author information is available at the end of the article tracheomalacia. He demonstrated appropriate weight © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Hanna et al. Egyptian Journal of Radiology and Nuclear Medicine (2021) 52:54 Page 2 of 5 gain and was meeting developmental milestones. On Follow-up was delayed related to COVID-19 circum- physical examination, the lungs were rhonchorous to stances. The patient is due for a short-interval follow-up auscultation, and there was evidence of bilateral subcos- CT examination of the chest. tal retractions and supra-sternal retractions after eating. A CT examination of the chest with IV contrast was performed which revealed a 4-cm mediastinal cyst caus- Discussion ing tracheal deviation and compression, suspicious for a Bronchopulmonary foregut malformations (BPFMs) are thymic epithelial cyst versus a bronchogenic cyst. In the a wide-encompassing term referring to several types of right upper lobe, there was an enhancing consolidation anomalies of pulmonary development resulting from ab- with anomalous venous drainage into the distal superior normal budding of the embryonic foregut and tracheo- vena cava (SVC), suspicious for pulmonary sequestration bronchial tree. BPFMs include foregut duplication cysts, (Fig. 1). pulmonary sequestrations, and congenital pulmonary The patient was admitted for these findings in the airway malformations (CPAMs) [2]. setting of increased work of breathing, and an Foregut duplication cysts arise from abnormal budding ultrasound-guided drainage of the cystic mediastinal of the embryonic foregut and tracheobronchial tree and mass was performed (Fig. 2) with the placement of a can be classified into three groups: bronchogenic, neu- drainage catheter. Cytology from the cyst fluid was not roenteric, and enteric cysts. Bronchogenic cysts arise concerning for malignancy. Respiratory status improved, from abnormal budding of the bronchial tree around 4– and a follow-up CT examination showed near-complete 6 weeks gestation and are lined by secretory respiratory resolution of the cystic mass with significant improve- epithelium [3]. Esophageal duplication cysts, a type of ment in mass effect on the adjacent thymus, great enteric cyst, arise from the posterior division of the em- vessels, and trachea (Fig. 3). bryonic foregut at 3–4 weeks gestation and are lined by Subsequently, the patient underwent right thoraco- gastric epithelium [2]. scopy and resection of the cystic mediastinal mass and Pulmonary sequestration is defined as the aberrant for- the right upper thoracic mass with a pedicle that ex- mation of segmental lung tissue that has no connection tended towards the SVC/azygos junction (Fig. 4). Gross with the bronchial tree or pulmonary arteries [4]. It re- pathology demonstrated a 3.0 × 2.5 × 1.1 cm segment of ceives systemic arterial blood supply, usually from the lung invested by visceral pleura consistent with extralo- thoracic or abdominal aorta. Venous return can either be bar pulmonary sequestration. Microscopic examination pulmonic or systemic. There are two types of sequestra- revealed focal cystic lung parenchyma with extravasated tion: extralobar pulmonary sequestration which has its mucin and interspersed thick-walled blood vessels. A own distinct pleural covering and complete separation second specimen demonstrated a collapsed cystic from adjacent lung tissue, and intralobar pulmonary se- structure which on histology showed a lining of ciliated questration which is embedded in the normal lung [4, 5]. columnar respiratory type epithelium and the cyst wall There is a predilection for the posterior inferior chest, including bronchial glands and cartilage, consistent with with 90% of extralobar sequestration occurring on the left a bronchogenic cyst (Fig. 5). The patient was extubated side. This contrasts with our patient who was found to 1 day after the operation and discharged after 2 days. have extralobar sequestration in the right superior chest. Fig. 1 Axial (a) contrast-enhanced CT of the chest of a 3-month-old male infant showing a median cystic mass (arrows) causing tracheal deviation and compression, as well as a mass effect on the more anterior thymus, was pathologically demonstrated to be a bronchogenic cyst. A posterior right upper lobe hyperdense consolidation (arrowhead) with anomalous venous drainage to the distal SVC (block arrow), consistent with pulmonary sequestration, is better demonstrated on the coronal image (b) Hanna et al. Egyptian Journal of Radiology and Nuclear Medicine (2021) 52:54 Page 3 of 5 Fig. 2 Gray-scale sonographic images of the upper mediastinum in the same patient show a large anechoic cystic structure (arrow) with no internal flow on color Doppler (not shown) (a). Approximately 12 ml of clear mucinous fluid was aspirated from the lesion (b) Embryology could help to explain the occurrence of Foregut duplication cysts arise during the same period some of these congenital anomalies simultaneously. Be- of embryogenesis as that of the development of the lung tween 3 and 5 weeks of gestation, the tracheobronchial buds. While pulmonary sequestrations are already a very tree is formed by a median bud that forms on the ventral rare malformation with an estimated incidence of 0.15– wall of the pharynx and grows caudally to form the right 6.4% [6], extralobar sequestration is less common than and left lung buds [4, 5]. As these lung buds elongate, intralobar sequestration comprising about 25% of all se- lateral ridges form between the lung buds and dorsal questrations. They have a greater prevalence to be associ- foregut to create the tracheoesophageal septum which ated with other congenital malformations
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