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NEUROIMAGES
Year : 2019  |  Volume : 67  |  Issue : 4  |  Page : 1167

Poland Syndrome with Amastia-athelia


Department of General Medicine, Grant Medical College and Sir JJ Hospital, Mumbai, Maharashtra, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Bharat Rathod
Department of General Medicine, Grant Medical College and Sir JJ Hospital, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.266297

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How to cite this article:
Chatterjee R, Patel K, Pandey D, Rathod B. Poland Syndrome with Amastia-athelia. Neurol India 2019;67:1167

How to cite this URL:
Chatterjee R, Patel K, Pandey D, Rathod B. Poland Syndrome with Amastia-athelia. Neurol India [serial online] 2019 [cited 2019 Sep 23];67:1167. Available from: http://www.neurologyindia.com/text.asp?2019/67/4/1167/266297




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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  References Top

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.  Back to cited text no. 1
    
2.
Kennedy KR, Wang AL. Poland Syndrome. N Engl J Med 2018;378:72.  Back to cited text no. 2
    


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