Dr.Akshay Kishore , Dr.Vinitha Prasad , Dr.C.Jayakumar , Amrita institute of medical sciences
Six year old male child presented with asymmetry of the anterior left chest with flat ill developed nipple
No history of left side weakness
Diagnosis considered is Poland syndrome.
On examination ,
There is absence of pectoralis major on the left side. Overlying soft tissues of the chest wall tends to be thinner than normal. Hypoplasia of nipple seen on the left side.
Apex beat palpable on the left side
Pectus excavatum present.
There is no cervical spine or craniofacial abnormality.
Chest Xray was normal.
Discussion :
Poland syndrome is a rare anatomical abnormality due to absence of sternocostal head of the pectoralis major muscle. Incidence is 1 in 50,000 newborns.
Etiology of Poland syndrome is not well understood and is thought to arise from abnormalities relating to subclavian arteries and branch vessels during embryogenesis, reducing the perfusion to the affected ipsilateral part of the chest wall. The physical evaluation for Poland syndrome includes an assessment of the anterior chest wall and associated anatomic limb abnormalities.Evaluation of the anterior chest wall includes palpation to assess the asymmetry of the chest wall musculature and the osseous and cartilaginous deformities of the rib cage. Evaluation for absence or hypoplasia of the costosternal pectoralis major muscle and the pectoralis minor muscle can usually be diagnosed with visual inspection and palpation. Overlying soft tissues of the chest wall also tend to be thinner than normal. Nipple absence or hypoplasia is frequently seen, and regional alopecia of the anterior chest wall and axilla can often be appreciated. Physical examination to evaluate the muscular caliber of the serratus, latissimus dorsi, and trapezius should be performed, as hypoplasia of these muscles can also be present. A cardiopulmonary examination to evaluate for dextroposition, especially in the setting of left-sided Poland syndrome, should also be performed. Chest wall reconstruction in the setting of severe rib hypoplasia and aplasia can also serve as an operative indication. Syndactyly release can usually be performed in a pediatric patient without significant complications and can be performed with or without a skin graft.
Take home message : Asymmetry of chest wall with pectus excavatum , always think of Poland syndrome.