1029 - Nerve-racking Asymmetry: Neuropathic Pain in Poland Syndrome

Poland syndrome with neuropathic pain

Case Description:

A 46-year-old female presented to clinic complaining of left neck, shoulder, axilla, and chest wall pain due to Poland Syndrome that started three years ago. She was diagnosed after noticing atrophy, hypoplasia, and tenderness of left breast and pinky, decreased range of motion, and paresthesias of left upper extremity. Of note, she had normal features before symptom onset without complications with childbearing and breastfeeding.

Physical exam was notable for hypoplastic left breast and fifth digit, along with tenderness of left chest wall and axilla. Initial workup with MRI cervical spine and chest x-ray were unremarkable. She was initially recommended physical therapy which showed some improvement of left shoulder movement without pain, but given persistent neuropathy, the patient was started on gabapentin 100mg three times a day for 30 days.

Discussions:

Poland syndrome is a constellation of abnormalities characterized by partial or complete absence of the pectoral muscles, aplasia or hypoplasia of other chest wall muscles, breast and nipple tissue, absence of costal cartilages, high riding scapula, and digital abnormalities. Defects are overwhelmingly unilateral.

The etiology is thought to arise from abnormalities relating to the subclavian arteries and branch vessels during embryogenesis, reducing perfusion to the affected ipsilateral side of the chest wall, usually due to a teratogen.

Most patients with Poland syndrome are asymptomatic and have few functional manifestations; however, surgical intervention can be indicated such as paradoxical movement of the chest wall, hypoplasia or aplasia of the female breast, and cosmetic indication for men and women with chest wall asymmetry.

Conclusions:

Poland Syndrome is an often overlooked condition that has wide-ranging effects; however, there are few articles highlighting neuropathy as a symptom. Thus, it is important to consider pain when evaluating patients for Poland syndrome or consider pain as a limiting factor when approaching conservative treatment.