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Journal of Current Pediatric Research | Volume: 23
March 14-15, 2019 | London, UK
International Conference on
Pediatrics & Neonatal Healthcare
P
oland 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).
e:
fadi.mhadeen@yahoo.comPoland syndrome: A case report
Fadi Almhadin
Burjeel Oasis Medical Center, Abu Dhabi
Curr Pediatr Res, Volume 23
DOI: 10.4066/0971-9032-C1-012