Poland Syndrome

Introduction

Poland Syndrome is a relatively uncommon condition that involves various deformities. It is characterized by the congenital underdevelopment or absence of the bony and soft tissue structures of the chest wall, including the absence of the pectoralis major, pectoralis minor, and ribs. This can lead to abnormalities in the upper limb on the affected side, such as finger absence, syndactyly (fused fingers), brachydactyly (short fingers), and carpal hypoplasia, as well as in the breast and other areas.

Furthermore, Poland syndrome is more commonly seen on the right side, less frequently on the left side, with occasional cases involving both sides.

Causes

The exact cause of Poland syndrome remains unknown. It might be related to abnormal growth of the pectoral muscle bud during fetal development, disrupted blood supply to the chest wall during embryonic development, genetic factor, or other influences.

Symptoms

The typical symptoms include pectoral muscle deficiency, syndactyly, rib absence, breast defects, etc. Other symptoms like breathing difficulty and pectus excavatum may also develop. Without prompt treatment, the condition could lead to complications such as scoliosis, ventricular septal defect, and renal dysplasia.

Diagnostic Methods

In addition to analyzing detailed medical history and physical examinations, imaging examinations such as CT scan, MRI, and chest X-ray, along with laboratory tests, are also required to further clarify the lesions of the chest wall, upper limbs, and suspicious organs, in order to provide a basis for treatment planning.

Surgical Treatment

The surgical treatment for Poland syndrome at ICWS involves two main components: correction for bone structure and reconstruction for soft tissues. Due to the diverse and complex lesions associated with Poland syndrome, surgical strategies are customized based on each patient’s own condition.

Wang procedure

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Willine procedure

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These two procedures are suitable for patients with bone structure indentation.

Chest wall reconstruction surgery:

This procedure is suitable for patients with bone structure defect, involving the use of autologous bone or artificial material(MatrixRIB) to reconstruct the deficient chest wall.

Soft tissue reconstruction surgery:

This procedure is suitable for patients with underdeveloped or absent soft tissues. It utilizes techniques such as muscle transplantation, autologous fat grafting, and implant filling to reconstruct the chest muscles and breasts.