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Case Reports
. 2023 Dec;57(4):316-320.
doi: 10.4314/gmj.v57i4.9.

Scimitar syndrome in a four-month-old infant

Frank Owusu-Sekyere  1 , Victoria M Adabayeri  1 , Efua Otoo  1 , Claudia Adja-Sai  1 , Akosua M Boateng  2
Affiliations
Case Reports

Scimitar syndrome in a four-month-old infant

Frank Owusu-Sekyere et al. Ghana Med J. 2023 Dec.

Abstract

The Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable congenital anomaly of cardiopulmonary development characterised by an abnormal right-sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. These serious anomalies notwithstanding, their presentation is varied, ranging from asymptomatic to severe symptoms, particularly in the neonatal and early infantile period. Some symptomatic cases are misdiagnosed as recurrent lower respiratory tract infections or as heart failure, missing the underlying congenital anomaly. Considering associated serious complications such as pulmonary hypertension and a high mortality rate, accurate and timely diagnosis is mandatory. This requires a high index of suspicion, not only by clinicians but also by radiologists who encounter suggestive chest radiographs in asymptomatic children. Still, the classic radiological finding for which the condition is named is seen in only about 50% of all cases and only 10% in affected infants. We highlight this case to heighten clinicians' and radiologists' suspicions about Scimitar syndrome.

Funding: None declared.

Keywords: Ghana; Scimitar syndrome; infant; venolobar.

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Conflict of interest statement

Conflict of interest: None declared

Figures

Figure 1
Figure 1
Shows dextroposition with mediastinum shift to the right, significant reduction of right lung volume, obliteration of right heart silhouette and right hilum and consolidation of the right upper and middle lung lobes with bronchial wall thickening and interstitial shadowing on the left
Figure 2
Figure 2
a firm left lung and hypoplastic right lung
Figure 3
Figure 3
Partial anomalous pulmonary venous drainage
Figure 4
Figure 4
Fibrous bands representing atretic right pulmonary veins
See this image and copyright information in PMC

References

    1. Cooper G. Case of malformation of the thoracic viscera consisting of imperfect development of the right lung and transposition of the heart. London Med Gaz. 1836 Jul 16;18:600–601.
    1. Neill CA. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage,” Scimitar syndrome”. Bull. Johns Hopkins. Hosp. 1960;107:1–20. - PubMed
    1. Odenthal C, Sarikwal A. Anomalous unilateral single pulmonary vein versus scimitar syndrome: Comparison of two paediatric cases and a review of the literature. J Med Imaging Radiat Oncol. 2012;56:247–254. - PubMed
    1. Gudjonsson U, Brown JW. Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;9:56–62. - PubMed
    1. Wang CC, Wu ET, Chen SJ, Lu F, Huang SC, Wang JK, Chang CI, Wu MH. Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 2008;167:155–160. - PubMed

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