+44 1400 530055Poland syndrome: A case report
International Conference on Pediatrics & Neonatal Healthcare
March 14-15, 2019 | London, UK
Fadi Almhadin
Burjeel Oasis Medical Center, Abu Dhabi
Posters & Accepted Abstracts : Curr Pediatr Res
Abstract:
Poland syndrome (PS) is a rare chest wall developmental anomaly characterized by ipsilateral agenesis/ hypoplasia of the sternocostal head of pectoralis major, hypoplasia of nipple or breast, absence of subcutaneous fat, multiple rib abnormalities, elevated and rotated scapula (Sprengel deformity) and ipsilateral digit abnormalities (brachydactyly, syndactyly). These findings vary and all are rarely found in a single individual. Poland Syndrome also (Poland’s syndactyly, Poland sequence, and Poland’s anomaly) was first described by Sir Alfred Poland in 1840. Etiology of PS remains unknown. It is rarely genetically inherited and is regarded as a sporadic event. It has been suggested that during the sixth week of gestation, the injury occurs due to regional vascular defect of the subclavian artery 1. It is a period associated with splitting of the two heads of pectoralis major and the development of tissues between the digits. The incidence of Poland’s syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births, with higher frequency among males (ratio: 2:1-3:1). In75% of the cases, it is located on the right hemithorax in the unilateral form. Treatment is primarily reconstructive surgery depending on the severity of the malformation, gender and patient preference. Our reported case is A 4-year-old boy with no medical history who presented for evaluation of urinary symptoms. There were no breathing or cardiac complaints on review of systems and Uneventful pregnancy and delivery, with negative family history for same problem. Physical examination showed chest asymmetry with right anterior chest wall depression and flattening of the right pectoral region with displaced nipple. Abduction of the shoulders showed absence of the sternocostal head of pectoralis major. Hand examination showed Ipsilateral short and webbed fingers (symbrachydactyly).
Biography:
E-mail:
fadi.mhadeen@yahoo.comPDF HTML