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Case Reports
. 2024 Jan-Mar;14(1):67-69.
doi: 10.4103/ijabmr.ijabmr_376_23. Epub 2024 Feb 20.

Hybrid Pulmonary Sequestration, Cystic Pulmonary Adenomatoid Malformation, and Dextrocardia: A Triple Whammy

Jyoti Bajpai  1 , Shreya Verma  2 , Surya Kant  1 , Ajay Kumar Verma  1 , Darshan Bajaj  1 , Akshyaya Pradhan  3 , Shalini Bhalla  4
Affiliations
Case Reports

Hybrid Pulmonary Sequestration, Cystic Pulmonary Adenomatoid Malformation, and Dextrocardia: A Triple Whammy

Jyoti Bajpai et al. Int J Appl Basic Med Res. 2024 Jan-Mar.

Abstract

Pulmonary sequestration and cystic pulmonary adenomatoid malformation are rare congenital cystic disorders of the lungs. The presence of both the diseases in the same individual is therefore very uncommon. Pulmonary sequestration is a nonfunctional pulmonary tissue mass that derives its blood supply from systemic blood supply other than pulmonary circulation. Congenital cystic pulmonary adenomatoid malformation represents a mass consisting of abnormal bronchiolar air spaces and a deficiency of functional alveoli. This is the case report of a 9-year-old girl with intermittent fever, left-sided chest pain, and cough for the past 15 days along with recurrent coughs since childhood suggestive of hybrid pulmonary sequestration, congenital cystic adenomatoid malformation, and dextrocardia.

Keywords: Congenital anomaly; contrast-enhanced computed tomography thorax; dyspnea; lung mass; pulmonary sequestrations.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Chest X-ray shows a large intrathoracic mass on the left side with many tiny air-filled cystic spaces (Red Arrow)
Figure 2
Figure 2
Computed tomography scan thorax revealed a heterogeneous well-defined lesion 7.6 cm × 7.2 cm × 5.3 cm heterogeneous well-defined lesion with multiple cystic lesions in the posterior part of the left lower lobe (Blue Arrow)
Figure 3
Figure 3
Computed tomography pulmonary angiography shows the systemic arterial supply of the lesion from the descending aorta and venous drainage to the left inferior pulmonary vein (Red Arrow)
Figure 4
Figure 4
(a) Photomicrograph shows areas of chronic inflammation with surrounding fibrosis and cystic degeneration (H and E, ×40). (b) Photomicrograph shows an aberrant systemic artery with an elastic layer along with areas of fibrosis (H and E, ×40) (Blue Arrow)
See this image and copyright information in PMC

References

    1. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: Report of seven cases and review of 540 published cases. Thorax. 1979;34:96–101. - PMC - PubMed
    1. Wei Y, Li F. Pulmonary sequestration: A retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40:e39–42. - PubMed
    1. Sfakianaki AK, Copel JA. Congenital cystic lesions of the lung: Congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Rev Obstet Gynecol. 2012;5:85–93. - PMC - PubMed
    1. Huang CC, Ko SF, Chung MY, Shieh CS, Tiao MM, Lui CC, et al. Infradiaphragmatic pulmonary sequestration combined with cystic adenomatoid malformation: Unusual postnatal computed tomographic features. Abdom Imaging. 2004;29:439–42. - PubMed
    1. Kanavi JV, Thangaraj J, Thomas A. Congenital cystic adenomatoid malformation (CCAM): Antenatal and postnatal management'. [[Last accessed on 2022 Nov 17]];Int J Reprod Contracept Obstet Gynecol. 2017 6:2447. Available from: https://link.gale.com/apps/doc/A534838501/HRCA?u=anon~956256ad&sid=googl... .

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