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| NEUROIMAGES |
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| Year : 2019 | Volume
: 67
| Issue : 4 | Page : 1167 |
Poland Syndrome with Amastia-athelia
Rudrarpan Chatterjee, Kruti Patel, Deepika Pandey, Bharat Rathod
Department of General Medicine, Grant Medical College and Sir JJ Hospital, Mumbai, Maharashtra, India
| Date of Web Publication | 10-Sep-2019 |
Correspondence Address:
Dr. Bharat Rathod
Department of General Medicine, Grant Medical College and Sir JJ Hospital, Mumbai - 400 008, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.266297
How to cite this article:
Chatterjee R, Patel K, Pandey D, Rathod B. Poland Syndrome with Amastia-athelia. Neurol India 2019;67:1167 |
A 14-year-old boy was admitted in view of malarial fever. On examination, it was noticed that there was asymmetry of the chest wall along with absence of the left nipple [Figure 1] and [Figure 2]. On palpation, the pectoralis major muscle was found to be absent on the left, along with an absent nipple and breast tissue. He did not report any weakness or inability to carry out his daily activities. On neurological examination, power was 4/5 for flexion of the left shoulder. He was noticed to compensate by simultaneously partially abducting the shoulder in actions involving flexion of the shoulder. There was winging of the scapula on the left [Figure 3]. A diagnosis of Poland's syndrome with amastia and athelia was made. Local ultrasound confirmed the absence of the left pectoralis major, left serratus anterior, and any glandular tissue in the left breast. Poland's syndrome is a rare congenital syndrome with an as yet undefined etiology. It is hypothesized to be due to lack of embryological development of the left arm and associated structures due to decreased blood supply through the subclavian artery.[1] Associations include ipsilateral athelia, amastia, kidney hypoplasia, lack of axillary hair, hypoplasia of the rib cage, syndactyly, polydactyly and hypoplasia of the bones of the arm, forearm and hands.[2] Surgical reconstruction may be indicated depending on the functional severity or for cosmetic purposes. No further treatment was advised for our case in view of good functional status. | Figure 1: Asymmetry of the chest with absent nipple, breast tissue and pectoralis muscle on the left
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 | Figure 2: Prominence of the ribs laterally due to absence of the serratus anterior
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 | Figure 3: Winging of the left scapula on abduction of the arm due to absence of the serratus anterior
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Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| » References | |  |
| 1. | Bavinck JN, Weaver DD. Subclavian artery supply disruption sequence: Hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies. Am J Med Genet 1986;23:903-18.  |
| 2. | Kennedy KR, Wang AL. Poland Syndrome. N Engl J Med 2018;378:72. |
[Figure 1], [Figure 2], [Figure 3]
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