Reviewed by Dr. Yang Liu

Dr. Liu is a chest wall surgeon specializing in complex chest wall deformities. He has participated in over 6,000 chest wall surgeries and published extensively in this field.

Is Poland syndrome a relatively uncommon condition? Yes, it is. Poland syndrome is regarded as a rare and relatively complex condition. However, with the advancement of medical science and increasing awareness of Poland syndrome, more patients are being accurately diagnosed and treated, which has led to a gradual rise in the reported incidence of this condition.

This article delves into the nature of Poland syndrome, its prevalence, and the Poland syndrome treatment options available today.

What is Poland Syndrome?

Poland syndrome is a complex congenital anomaly first described by Alfred Poland, a British medical student, in 1841.

The causes of Poland syndrome remain unclear. Several hypotheses exist, including abnormal growth of the pectoral muscle bud during fetal development, disrupted blood supply to the chest wall during embryonic development, genetic factors, or other potential influences.

The classic symptoms of Poland syndrome include abnormalities of the bony and soft tissue structures of the chest wall, as well as abnormalities of the ipsilateral upper limb^[1,2]. These conditions are usually unilateral, with the right side being more common, accounting for 60% to 70% of cases; the left side is less common, and bilateral involvement is occasionally observed^[3].

In some cases, patients may develop a depression in the chest wall. The indentation can compress the heart and lungs, ultimately leading to respiratory symptoms such as chest tightness, palpitations, and shortness of breath.

The primary symptoms of Poland syndrome include:

• Chest wall abnormalities: Bony structure abnormalities involve the absence and deformation of ribs and costal cartilages, which can manifest as chest wall depression. Soft tissue abnormalities involve developmental anomalies of the pectoralis major, pectoralis minor, and breast tissue, which can present as chest wall asymmetry and breast hypoplasia.

  
  

• Upper limb abnormalities: Syndactyly (fused fingers), brachydactyly (short fingers), absent fingers, and Carpal Coalition.

  
  

• Specific associated conditions: These may include respiratory symptoms and scoliosis.

Due to the wide range of symptoms, treatment for Poland syndrome requires a multidisciplinary approach, covering the correction of chest wall bony structures, soft tissue reconstruction, and functional restoration of the upper limb.

How Many People Have Poland Syndrome?

(1) Prevalence

Poland syndrome occurs in about 1 in 30,000 live births and is more often seen in males than females (3:1)^[4].

However, due to the wide variation in clinical manifestations, especially mild or atypical cases that may go unnoticed, the true prevalence of Poland syndrome may be underestimated.

(2) Notable Cases

Thanks to several public cases of celebrities with Poland syndrome, awareness of this condition has increased.

Female Celebrities with Poland Syndrome:

Hailey Dawson–An American girl born in 2010 who was born without her right pectoral muscle and three fingers on her right hand. She became widely recognized for throwing the ceremonial first pitch at all 30 Major League Baseball parks using a custom 3D-printed robotic hand designed by engineers at the University of Nevada, Las Vegas.

(Currently, Hailey Dawson is the most publicly recognized female case; most other notable cases reported are male.)

Male Celebrities with Poland Syndrome:

• Fernando Alonso–Formula One World Champion, missing his right pectoral muscle. He is one of the most famous athletes with Poland syndrome.

• Ted Danson–American actor best known for starring in the TV show Cheers. He revealed his condition publicly in 2000, sharing that he was bullied as a child because of it.

• Jeremy Beadle–British TV presenter, whose right hand was disproportionately small due to Poland syndrome.

• Jérôme Thomas–Olympic boxer from France, whose left arm and hand are significantly shorter and smaller than his right. He also lacks a left pectoral muscle.

• Bryce Molder–A professional golfer on the PGA Tour. He was born without a left pectoral muscle and has an underdeveloped left hand. He underwent several childhood surgeries to repair syndactyly.

  
  

Surgical Treatment for Poland Syndrome at ICWS

ICWS is the world's first independent surgical institution dedicated to chest wall disorders and is also the world's largest center for correcting chest wall deformities. The ICWS team specializes in the diagnosis and treatment of a wide range of chest wall conditions, including deformity, trauma, defects, infections, and tumors.

Based on the complex and variable nature of Poland syndrome, ICWS develops individualized surgical plans based on each patient's specific condition. The surgical treatment for Poland syndrome at ICWS primarily involves two aspects: the correction of bony structures and the reconstruction of soft tissues.

(1) Bony Structure Correction

1. For patients with chest wall indentation, minimally invasive procedures such as the Wang procedure and the Willine procedure can be used for correction.

2. If a patient has absent or underdeveloped ribs and costal cartilage, chest wall reconstruction surgery is performed using either autologous bones or MatrixRIB, an artificial material that does not need to be removed.

(2) Soft Tissue Reconstruction

Soft tissue reconstruction involves rebuilding and shaping the chest muscles and mammary glands. Chest muscle reconstruction can be achieved through muscle flap transfer or volume restoration using autologous fat or synthetic implants. For mammary gland issues, implants can also be used to achieve a better aesthetic outcome.

Case Studies on Poland Syndrome Treatment at ICWS

1. Wang Procedure for a 12-Year-Old Patient with Severe Poland Syndrome

A 12-year-old boy suffered from severe Poland syndrome, which caused a right-sided chest wall depression and multiple underdeveloped costal cartilages.

MatrixRIBs were first used to reconstruct the absent costal cartilages on his right side. Subsequently, the Wang procedure was performed, and a normal appearance of the chest wall was ultimately restored.

2. Chest Wall Reconstruction Surgery for a 3-Year-Old Patient with Severe Poland Syndrome

A 3-year-old patient was found to have an asymmetrical chest wall with the partial absence of ribs and costal cartilages on the right side due to severe Poland syndrome.

A chest wall reconstruction surgery was performed using MatrixRIBs. The surgery was successfully completed in 50 minutes, with the patient's chest wall appearance substantially restored to normal.

Conclusion

Although Poland syndrome is rare, patients can achieve significant functional improvements and appearance enhancements through early diagnosis and a multidisciplinary approach.

With the continuous advancement of surgical techniques, such as the Wang procedure, the Willine procedure, and chest wall reconstruction surgery, treatment outcomes are becoming increasingly ideal, bringing more hope to patients. The treatment for Poland syndrome requires a personalized plan tailored to each patient's unique situation.

If you or a family member is seeking treatment options for Poland syndrome, please get in touch with ICWS for an evaluation.

References:

[1] URSCHEL H C. Poland syndrome. Semin thorac cardiovasc surg, 2009, 221 (1) : 89 - 94.

[2] BALDELLI I, BACCARANI A, BARONE C, et al. Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence). Orphanet J rare dis, 2020, 15 (1) : 201.

[3] 张伟, 李健挺, 田文. Poland综合征. 实用手外科杂志, 2008, 22 (3) : 161 - 163.

[4] Hashim EAA, Quek BH, Chandran S. A narrative review of Poland's syndrome: theories of its genesis, evolution and its diagnosis and treatment. Transl Pediatr. 2021;10(4):1008-1019. doi:10.21037/tp-20-320