Berry syndrome: A rare myriad of cardiopulmonary vascular anomalies
Year: 2025
Type: article
Abstract: Dear Editor, A 3-week-old term infant with complex cardiac and bony anomalies on antenatal ultrasound presented with respiratory distress to the pediatric emergency. Radiographs showed multiple skeletal abnormalities in the form of butterfly vertebrae and an absent 12th rib on the left side. Computed tomography angiography (CTA) of the chest was done, which showed an intact interventricular septum [Figure 1a], a type B interrupted aortic arch [Figure 1b], a type II aortopulmonary window [Figure 1c and d], the aortic origin of the right pulmonary artery (AORPA) [Figure 1d], and aberrant right subclavian artery [Figure 1e-1f]. Patent ductus arteriosus (PDA) was reforming the descending thoracic aorta. A final diagnosis of berry syndrome was suggested.Figure 1: Axial computed tomography angiography (CTA) image (a) shows an intact interventricular septum (arrow). Coronal maximum intensity projection (MIP) CT image, (b) shows an interrupted aortic arch (white arrow) beyond the origin of the left common carotid artery (black arrow), suggestive of type B interrupted aortic arch. Axial CTA images, (c-d) show the distal type of aortopulmonary defect (white arrows), suggestive of type II aortopulmonary window. The aortic origin of the right pulmonary artery is seen (black arrow in d), Coronal (e) and oblique axial (f) MIP images show an aberrant right subclavian artery (black arrows in e and f) having a retrotracheal and retroesophageal courseThe association of aortopulmonary septal defect with an intact ventricular septum, AORPA, PDA, and aortic arch interruption was first described by Berry et al. The patient had a distal type of aortopulmonary defect, which indicates a partially persisting common arterial trunk, as opposed to the conventional proximal aortopulmonary septal defect oriented in a sagittal plane. The RPA may be partially or displaced into the aorta because of the bifurcation of the main pulmonary artery communicating to this intact truncal segment. This anomalous origin of the RPA may result in hypoplasia of the aortic arch and steal from the aortic flow during embryogenesis.[1,2] CTA helps delineate the complex cardiac and associated vascular anomalies, which helps in surgical planning and prognostication. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest The authors declare that there is no conflict of interest.
Source: Lung India
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4409956794