A brief note on Poland syndrome and its main points

This is a presentation of Poland syndrome It contains a brief summary of the main points, like: Definition, Incidence, Etiology, Clinical picture, Investigations and management, including surgical and non-surgical This is just a summary describing some points only

1. POLAND SYNDROME

2. Definition It is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, shoulder, arm, and hand.

3. Incidence It has been estimated to occur in 1 in 20,000 newborns It occurs more than twice as often in males as in females Analysis of the gender and sidedness indicates that males are more likely to have a right-sided anomaly, whereas females have an approximately equal distribution of left- and right-sided anomalies

4. Etiology The cause of Poland syndrome is unknown Researchers have suggested that it may result from a disruption of blood flow during development before birth. This disruption is thought to occur at about the sixth week of embryonic development and affect blood vessels that will become the subclavian and vertebral arteries on each side of the body. The arteries normally supply blood to embryonic tissues that give rise to the chest wall and hand on their respective sides. Abnormality of an embryonic structure called the apical ectodermal ridge , which helps direct early limb development, may also be involved in this disorder Rare cases of Poland syndrome are thought to be caused by a genetic change that can be passed down in families, but no related genes have been identified

5. Clinical features Missing or underdeveloped chest and shoulder muscles Abnormal ribs that sometimes can lead to breathing problems Breast or nipple abnormalities Missing or abnormally placed underarm hair Hand abnormalities such as short or fused fingers Shortened forearm bones Heart, lung, or kidney problems

6. Differential diagnosis Isolated breast and chest wall anomalies Hand and limb abnormalities without muscle involvement Congenital syndromes that can present with similar symptoms, such as Amazone syndrome Moebius syndrome Klippel-Feil syndrome

7. Investigations Blood tests Chest X-ray Computed tomography (CT) scan Electrocardiogram (ECG)  Pulmonary function tests Genetic testing :for related syndromes

8. Management Non-surgical  Physical and occupational therapy to improve function Psychological support to address the emotional impact of physical differences, especially during adolescence

9. Management Surgical The surgical treatment of Poland anomaly is usually divided into 2 categories: surgical management of the thoracic anomaly and surgical management of the hand anomaly. Surgical treatment options include reconstruction of the chest wall with myocutaneous flap coverage and breast augmentation procedure.  For patients with simple syndactyly, intervention should be limited to syndactyly reconstruction of the affected digits, aiming to improve pinch and grasp. Digital separation is recommended in all but the mildest incomplete syndactyly  Non-vascularized phalangeal toe transfers, vascularized toe transfers, and distraction osteogenesis are all viable surgical options with good functional results and acceptable patient satisfaction

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