Poland syndrome

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 PA erect view

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
Contrast-enhanced CT thorax in axial plane soft tissue window

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
A 3D volume rendered image showing flat left chest compared to the other side.

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.
Author: radhianahassan