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POLAND SYNDROME

Causes & Symptoms Diagnosis & Procedures FAQ

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 Procedures

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/Willine procedure

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.

Frequently Asked Questions

Q: Can Poland syndrome be fully corrected in a single surgery?

For female patients with Poland syndrome, surgical correction usually requires two separate operations. The initial operation addresses the bony deformity, including reshaping or reconstructing the depressed or absent bony structures of the chest wall. The subsequent operation focuses on the soft tissues, encompassing the reconstruction of chest wall muscles and breast molding to achieve a more natural and satisfactory aesthetic contour.

Q: Does the chest wall contour change gradually after surgery, or does it improve immediately?

In most cases, the deformity is significantly corrected right after the procedure, resulting in a chest wall that appears close to normal. Following this initial correction, the chest continues a process of slight, gradual remodeling over time. The new contour is typically well-established within about 3 months and fully stabilizes after 2 to 3 years.

Q: How much does Poland Syndrome surgery cost?

The total cost usually ranges from $10,000 to $15,000. The exact amount will be determined by factors such as the patient’s condition and the specific surgical plan.

Q: Will the surgery be very painful? How does the hospital manage postoperative pain?

It is common to experience significant pain in the initial postoperative period, particularly among adolescent and adult patients due to their more rigid skeletal structure. Our hospital employs a comprehensive, multi-modal analgesia protocol to ensure effective pain control. This integrated approach includes:

Intraoperative Intervention: Intercostal nerve blocks are administered to prevent pain signals from transmitting.
Postoperative Medication: Continuous pain management is delivered through a patient-controlled analgesia (PCA) pump, supplemented with scheduled intravenous analgesics.
Adjunctive Rehabilitation Therapy: Our dedicated rehabilitation team provides personalized physiotherapy, incorporating techniques such as acupuncture, therapeutic massage, electrical stimulation, and ultrasound therapy. These modalities are highly effective in alleviating localized pain and common discomforts like postoperative bloating.

Q: How long will I need to stay in the hospital after Poland Syndrome surgery?

Most patients stay in the hospital for 7 - 10 days, although the actual duration depends on individual recovery.

Q: Is there any risk of the bars displacing or deforming after surgery?

The risk is very low. The bars used in surgery are made of titanium alloy, which provides excellent rigidity and resistance to deformation. In addition, the Wang Technique, a cutting-edge bar fixation method, is utilized during surgery to rigidly stabilize the bars in position, effectively preventing movement. Long-term clinical data confirms that the vast majority of patients do not experience bar displacement or deformation. It is crucial to note that during the early postoperative period (within the first 3 months), patients should avoid vigorous exercise and be mindful in their daily lives to avoid significant impact or trauma to the chest, thereby reducing the likelihood of bar displacement.

Q: When can I resume my normal daily life after surgery?

Most patients can get out of bed and walk within 3–4 days after surgery, and resume daily activities around 10 days postoperatively. Patients can usually return to normal work or school (excluding heavy physical labor) around 1 month. Light exercise, such as jogging or hiking, can start within the first three months, with intensity gradually increased thereafter.

It is crucial to note that if you encounter any discomfort, such as chest pain or shortness of breath, during exercise, you should stop the activity immediately. If necessary, a chest X - ray or CT scan can be arranged for further examination.

Q: Do I need to sleep on my back after the surgery?

Yes, for the initial postoperative period, maintaining a specific sleeping position is important for healing. It is advised to sleep in a supine position (lying on your back) or a modified lateral position (partially reclining on one side) for the first month , adjusting as needed for comfort around the incision sites. Additionally, you need to avoid movements with a large range of motion, like chest expansion, bending over, and lifting heavy objects. After about a month, once your incisions have completely healed, you can gradually start sleeping on your side.

Q: When can I take a shower after surgery?

After the drainage tubes are removed (the removal time is determined by the drainage volume and follow - up examination results, generally within 1 - 2 weeks), you can take a shower with the wound covered by waterproof dressings. After finishing the shower, replace the dressings with breathable gauze to protect the wound. Around 3 weeks after surgery, once the incisions have completely healed, you can take a normal shower.

Q: What should I do if complications occur after returning to my country?

Our discharge protocol is designed to ensure your safety. Typically, patients can be safely discharged 10 - 14 days post-surgery. After continuing to observe for about one week post - discharge, the risk of the vast majority of complications can generally be excluded. If recovery progresses smoothly during the first three weeks post-surgery, the likelihood of later complications is extremely low. Additionally, the bars are firmly secured, and the surgical technique is specifically designed to prevent issues like bar displacement.

However, if you notice symptoms such as pneumothorax, pleural effusion, significant pain, or poor wound healing, please contact our doctors promptly. In case of an emergency, please seek immediate care at a local hospital.

Q: Is regular follow-up necessary? When can the bars be removed?

If your recovery goes well without any noticeable discomfort or abnormal conditions, regular follow-up is usually not required. However, if you develop symptoms such as persistent high fever (temperature >38.5°C), sudden chest tightness, shortness of breath, or difficulty breathing, please have a chest X-ray or CT scan locally and consult a thoracic surgeon or our doctor for further guidance.

Regarding bar removal, if the patient has a chest wall depression and was implanted with conventional bars (such as Nuss or Park bars), it is recommended to remove the bars 3 years after surgery. If the patient had chest wall defects and underwent reconstruction with MatrixRIB, then bar removal is not necessary.

Q：Can I take a flight after surgery?

Yes. Upon discharge, we will provide a discharge summary and a medical certificate. If airport security raises any concerns, presenting these medical documents will facilitate your passage.

In most cases, taking a flight does not cause discomfort, you can travel with confidence.

Q: Are there dietary restrictions after surgery? How should I supplement nutrition?

Optimal nutrition is crucial for healing. We recommend the following dietary plan, tailored to your recovery phase:

Initial Phase (during bed rest): Choose easily digestible semi-liquid foods, such as porridge, juice, or well-cooked noodles.

Recovery Phase (once able to get out of bed): Gradually resume a normal, balanced diet with an emphasis on high-quality protein sources such as fish, chicken, and eggs. Make sure to eat fresh fruits and vegetables daily to supplement vitamins and electrolytes.

Precautions: Strictly avoid spicy and greasy foods, and be cautious with foods that may trigger allergies, such as seafood or mangoes.