A newborn with Poland anomaly and liver ectopy: an unusual association with important prognostic implications.
Poland syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand deformity and can include a spectrum of anomalies from a complete absence of the pectoralis major and minor, amastia, severe rib abnormalities, and absence of the hand, to minor hand abnormalities and isolated pectoralis minor aplasia. The etiology is unknown, but speculation includes hypoplasia of the subclavian artery or its branches between the 6th and 7th week of gestation. We report a newborn girl with Poland syndrome and an unusual associated anomaly consisting of ectopic liver tissue forming a chest wall mass. This association carries significant implications for oncologic awareness, prognosis, and counseling.
Chalak
Lina F
LF
Department of Pediatrics, University of Arkansas for Medical Sciences, Arkansas Children's Hospital, 800 Marshall Street, Slot 512 5-B, Little Rock, AR 72202, USA. chalaklinaf@uams.edu
Parham
David M
DM
D001706:Biopsy; D044383:Black People; D005260:Female; D005500:Follow-Up Studies; D006228:Hand Deformities, Congenital / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D010369:Pectoralis Muscles / Q000002:abnormalities; D011045:Poland Syndrome / Q000175:diagnosis* / Q000473:pathology; D011247:Pregnancy; D011379:Prognosis; D013902:Radiography, Thoracic; D013577:Syndrome; D013909:Thorax / Q000002:abnormalities; D016896:Treatment Outcome