Poland's anomaly

A seven year old male child presented to the Department of Pediatrics at Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India, in February 2013 with asymmetry of chest wall and drooping of left shoulder since birth (Fig. 1A). Chest X-ray revealed hyperlucent right lung field with hypoplasia of second, third ribs in the form of non-visualised anterior ends as compared to the left side and grade 1 Sprengel's deformity of the right shoulder (Fig. 1B). Sprengel's deformity is congenital elevation of shoulder due to failure of the normal descent of scapula from initial mid-cervical position to final thoracic position. On conventional radiography, there is elevation of the affected scapula with the inferior angle rotated medially; pointing to spine. Grade 1 Sprengel's deformity is very mild. The shoulders are almost at the same level and the deformity cannot be noticed with the clothes on. On magnetic resonance imaging (MRI) absent right sided pectoral muscles were identified (Fig. 2A). Normal imaging of pectoral muscles is depicted (Fig. 2B). The clinical and radiological findings were consistent with Poland's anomaly, a birth defect which is characterized by underdevelopment or absence of pectoralis muscle on one side of the body and (but not always) webbing of the fingers (syndactyly) of the hand. Additional signs on the affected side may include hypoplastic forearm, herniated lungs, strabismus, epicanthus, Simian crease of affected extremity, dextrocardia, diaphragmatic hernia, history of maternal diabetes, microcephaly, encephalocele, vertebral, hepatobiliary and renal anomalies. Currently, the patient is advised physiotherapy exercises, will be followed up till the child reaches 12 years of age and is planned for reconstruction surgery.

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Fig. 1

(A). Clinical photograph of the child showing asymmetry of chest wall and drooping of left shoulder. (B). Chest radiograph shows hyperlucent right lung field, hypoplasia of second and third ribs (thin white arrows) as compared to left side ribs and grade 1 Sprengel's deformity (thick white arrows) of right shoulder.

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Fig. 2

(A). Axial MR image confirming absent right sided pectoralis muscle (thin white arrow) and normal pectoralis muscle on the left side (thick white arrow). (B). Axial MR of normal child showing normal pectoral muscles (thick white arrow).

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