Poland Syndrome Syndactyly Surgery

What is Poland syndrome?

Poland syndrome is a constellation of anatomical anomalies, featuring the absence of the sternal head of the pectoralis major muscle. It also commonly presents with underdevelopment or absence of the pectoralis minor muscle and syndactyly among other digital anomalies. Additional abnormalities include unilateral absence or hypoplasia of the breast, funnel chest deformity, and rib cage malformations. The condition predominantly affects one side, with the right side being more frequently involved. Although exceedingly rare, bilateral manifestations have been documented. There are numerous variations of this syndrome, ranging from mild hypoplasia of the pectoralis major to severe chest wall deficiencies. Upper limb defects vary as well, from mild syndactyly to severe brachysyndactyly.

What are the symptoms of Poland syndrome?

Muscle Abnormalities

• Pectoralis major: The pectoralis major muscle is attached to the sternum and is often missing or underdeveloped in people with Poland syndrome. This can cause a deformed chest wall.
• Pectoralis minor: The pectoralis minor muscle is also often affected, showing signs of underdevelopment or complete absence.
• Skeletal Abnormalities
• Chest Deformity: People may have missing ribs, especially on the affected side. This can cause a funnel-shaped chest, known as pectus excavatum deformity.
• Brachial Plexus Abnormalities: There may be abnormalities in the brachial plexus, which is a network of nerves that innervate the upper limbs.

Finger Abnormalities

• Syndactyly: One of the most common features of Poland syndrome is syndactyly, which is when two or more fingers are fused together. This condition can range from mild to severe.
• Brachidactyly: In some cases, the fingers may be shorter than usual, a condition called brachydactyly.

Gynecomastia and Other Breast Abnormalities

• Gynecomastia: In men with Poland syndrome, breast tissue may be missing or underdeveloped on the affected side. This can cause an asymmetrical chest.
• Breast Tissue Extension: In women, breast tissue on the affected side may extend into the surrounding area, causing a less defined outline.

Hair and Skin Abnormalities

• Hair Loss: You may lose hair on the affected side of your body, especially in the armpit area.
• Thinning Skin: The skin on the affected side may be thinner than usual.

Muscle and Bone Abnormalities

• Upper Arm Muscles: The upper arm muscles on the affected side may be underdeveloped or weaker than the muscles on the unaffected side.

Difficulty Breathing

• Breathing Symptoms: While most people with Poland syndrome don't have any breathing symptoms due to missing or underdeveloped ribs, some may have trouble breathing, especially with exercise. This may be due to a condition called paradoxical breathing, in which the abdomen moves inward when you breathe in and outward when you breathe out.

Treatment for Poland Syndrome

CAD-Designed Custom Implants

• Complete or Partial Muscle Deficiency: One of the most notable features of Poland syndrome is the complete or partial absence of the pectoralis major muscle. This can be corrected through the use of custom implants designed using computer-aided design (CAD).
• 3D Reconstruction: The implants are created based on medical scans and are designed to perfectly match the patient's anatomy. They are made from medical-grade silicone and are purely for cosmetic purposes, not to restore muscle balance.

Surgical Treatment

• Surgical Procedure: Surgery is performed under general anesthesia and typically takes less than an hour. The surgeon makes an 8-centimeter (3.1-inch) incision in the腋窝 and prepares the area for the implant. The implant is then inserted beneath the skin and the incision is closed in two layers.
• Aesthetic Enhancement: The implant can replace the missing pectoralis major muscle, resulting in a symmetrical chest appearance, especially in females. As needed, particularly in females, a second surgery may be required to incorporate breast implants and/or fat grafting.

Fat Grafting

• Progressive Advancement: Fat grafting has gradually become a preferred method for correcting breast and chest wall deformities in Poland syndrome. This technique seems to be a significant advancement in severe cases, as it allows for a higher quality of reconstruction while minimizing scarring.
• Minimal Scarring: One of the benefits of fat grafting is that it can achieve reconstruction results that were previously impossible, while minimizing scarring. This makes it a promising option for patients with severe forms of Poland syndrome.

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