Case contribution: Dr Radhiana Hassan
Clinical:
- A 23 years old man
- Investigated for loss of appetite and weight
- Known case of Poland syndrome, diagnosed since childhood.
Chest radiograph findings:
- Hypoplasia of second and third ribs on the left side (yellow arrow)
- It caused slight deformity of thoracic cage
- Otherwise no significant asymmetrical lucency of lung
- No other significant finding
CT scan findings:
- Smaller left thoracic cavity (white arrows)
- Absence of left pectoralis major and minor muscles
- Hypoplastic left 2nd and 3rd ribs
- No lung mass or nodule
Diagnosis: Poland syndrome
Discussion:
- Poland syndrome is a congenital unilateral absence of pectoralis muscles major and minor.
- Spectrum of abnormalities include underdeveloped or absence of chest muscle on one side of the body, absence of sternal part of the clavicle, aplasia or hypoplasia of ipsilateral 2-5th ribs, aplasia or hypoplasia of breast and nipple, simian crease of affected extremity and webbing of fingers on the same side
- The estimated incidence of this condition is 1 in 30,000.
- The cause is unknown
- This syndrome is almost always sporadic
- It tends to occur on the right side and more common in boys
- Although rare, there is an association with Mobius syndrome as well as ‘morning glory’ and Pierre-Robin syndrome.
- The severity differs from person to person
- Management may include surgical correction especially in female patients; breast and chest wall reconstruction can be done.
- In males the reconstruction may not be necessary.
