Poland Syndrome – Chest Wall Asymmetry

0
Poland syndrome
Arrow on the chest CT image showing absent right pectoralis major muscle.

A rare congenital disease in a 20-year-old male with asymmetric chest and limb defects

A 20-year-old man presented to the hospital with an asymmetrical appearance of the right chest wall. On physical examination, the right chest wall was asymmetrical, with a hypoplastic right nipple. His right-hand examination revealed syndactyly between third and fourth fingers. The doctors suspected Poland syndrome.

They performed a computed tomography scan of the chest, demonstrating the absence of the right pectoralis major muscle. However, the CT scan revealed no bone anomalies.

Poland syndrome

Poland syndrome is a rare congenital disorder characterized by unilateral absence (aplasia) of chest wall muscles (pectoralis muscles). Moreover, the affected individuals also have ipsilateral limb defects, including abnormal short, webbed fingers (symbrachydactyly) of the hand on the same side (ipsilateral). Affected individuals may also suffer from mammary hypoplasia and other muscle and bone abnormalities

The affected individuals have underdeveloped or absent muscles on one side of the body. The syndrome may affect the chest, shoulder, arm, and hand. However, the severity and extent of the abnormalities differ from person to person.

The exact cause of PS is unknown; however, there some probable theories regarding the aetiology, including disrupted development of the subclavian artery, defective migration of fetal mesodermal tissue, and intrauterine insults.

Affected patients with PS may present at birth if there is severe disconfiguration of the thoracic cavity and upper extremity may be detected at birth. In contrast, mild asymmetry may present later on in life, usually around puberty, as it mildly affects the thoracic wall and the breasts. However, the asymmetry becomes more evident at puberty, hence leads to the diagnosis.

It is imperative to further evaluate the affected individuals for other internal abnormalities because, in rare cases of severe Poland syndrome, individuals may also have anomalies of the internal organs including dextrocardia, and other anomalies involving the lungs or kidneys.

References:

Erdogan H, Oncu F, Arslan FZ, Durmaz MS, Arslan S, et al. (2018) Pectoralis Muscle Aplasia in Poland Syndrome. Clin Med Img Lib 4:091. doi.org/10.23937/2474-3682/1510091

Poland syndrome: https://www.annalsthoracicsurgery.org/article/S0003-4975(02)04161-9/pdf

World Hepatitis Day 2021
Previous articleMan Ruptures Throat By Holding A Sneeze
Next articleA Man Drank Racing Formula, A Car Fuel Additive
Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.

LEAVE A REPLY

Please enter your comment!
Please enter your name here