Poland syndrome

Named after Sir Alfred Poland, Poland anomaly (PA) is described as an underdevelopment or absence of the chest muscle (pectoralis) on one side of the body and webbing of the fingers (cutaneous syndactyly) of the hand on the same side (ipsilateral hand). Sometimes referred to as "Poland syndrome," it is an uncommon condition present at birth (congenital). For people born with PA, the breastbone portion (sternal) of the pectoralis is also missing. more...

Since the severity of Poland anomaly differs from person to person, it is not often diagnosed or reported. Sometimes, a person does not realize they have the condition until puberty, when lopsided (asymmetrical) growth makes it more obvious. The incidence, therefore, is difficult to determine. Current estimates are between one in 10,000 to one in 100,000 births. Poland anomaly is more common in boys than girls, and the right side is affected twice as often as the left. The reasons for these differences are not known.

Clinical Signs & Symptoms:

abnormal gastrointestinal tract (Very frequent sign)

absent pectoral muscles (Very frequent sign)

brachydactyly (Very frequent sign)

dextrocardia (Very frequent sign)

diaphragmatic hernia/defect (Very frequent sign)

humerus absent/abnormal (Very frequent sign)

liver/biliary tract anomalies (Very frequent sign)

maternal diabetes (Very frequent sign)

oligodactyly/missing fingers (Very frequent sign)

radius absent/abnormal (Very frequent sign)

rhizomelic micromelia (Very frequent sign)

syndactyly of fingers (Very frequent sign)

ulnar absent/abnormal (Very frequent sign)

upper limb asymmetry (Very frequent sign)

abnormal rib (Frequent sign)

hypoplastic/absent nipples (Frequent sign)

scapula anomaly (Frequent sign)

agenesis/hypoplasia of kidneys (Occasional sign)

encephalocele/exencephaly (Occasional sign)

hypothal.-hypoph. axis abn. morphol. (Occasional)

hypothal.hypoph. axis abn. function (Occasional)

microcephaly (Occasional sign)

preaxial polydactyly (Occasional sign)

ureteric anomalies(reflux/duplex syst.) (Occasional)

vertebral segmentation anomaly (Occasional sign)

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