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Poland's Syndrome

Poland's syndrome (also called Poland syndrome) is a rare congenital anomaly of chest wall formation, originally described by a 19 year old London, England medical student named Alfred Poland. The syndrome, which may affect only about 1 in 30,000 people, consists of unilateral (one-sided) absence of some portions of the front of the chest. Less than 150 children a year are born with this problem in the United States. The male:female ratio is about 3 to 1, with the right side affected more commonly than the left.

Types of Poland's Syndrome

Poland's syndrome may be considered mild (with absence of the nipple or parts of the breast), moderate (to include absence of much of the pectoral muscles), or severe (with inclusion of parts of the underlying ribcage bones). It may also be associated, in some patients, with other chest wall deformities, including pectus excavatum, pectus carinatum, and Currarino-Silverman syndrome (also called pectus arcuatum).

Traditional Treatment for Poland's Syndrome

Reconstruction techniques have traditionally concentrated on the more superficial aspects of the condition, with muscle flaps and/or implants to try to match the contour of the opposite chest. These are usually done by plastic surgeons. Unfortunately, if patients have an underlying rib or breastbone defect, the muscle flaps and/or implants tend to "sink" into the chest, causing partial compression of the underlying lung, worsening any pre-existing respiratory difficulties.

Optimal Treatment for Poland's Syndrome

At the WakeMed Chest Wall Deformity Center, we have discovered that the optimal treatment for affected patients should involve a multidisciplinary team for the evaluation, surgical planning, and appropriate correction strategy. We collaborate with pediatric radiologists to perform special 2-D and 3-D chest CT scans, with SSD (shaded surface display) technology, for a thorough understanding of the deformity. We collaborate with pediatric pulmonologists and cardiologists, if those organs are affected. We collaborate with several adult cardiothoracic surgeons, for coordinated care in affected young adults and/or those who have previously undergone cardiac surgical procedures or other chest wall surgery. WakeMed anesthesiologists, with special fellowship training in pediatric anesthesiology and cardiac anesthesiology, work with us to optimize intra-operative monitoring and anesthesia care. Most patients don't require intensive care unit stays. However, if they do, our pediatric intensive care unit (PICU) has pediatric intensivist staffing 24 hours a day, 7 days a week, to assist with perioperative recovery. Unlike most PICU's, at WakeMed parents are able to stay with their children (in the same private PICU room) 24/7. PICU rooms have pull-out beds for a parent and private bathrooms for parent use.

An exciting new development for patients with Poland's syndrome is the use of custom-made titanium implants. After many years of lobbying from surgeons in various professional societies, such as the Surgical Section of the American Academy of Pediatrics and the American College of Surgeons, the Custom Device Exemption law was finally modified by the US Congress in 2012, with the addition of the new FDASIA (Food and Drug Administration Safety and Innovation Act) law. With FDASIA, for the first time ever, the FDA allows the production of specially-made custom implants to treat rare and unusual conditions, like Poland's syndrome. We have collaborated with design engineers (3D Systems in Colorado) and development engineers (Zimmer/Biomet in Florida). Using state-of-the-art 3-D design technology and 3-D printers capable of making "new ribs" from titanium, we are able to design and construct custom-made unique-to-the-patient "replacement ribs" (and breastbones) to match the bones on the contralateral "normal" side of the chest. Following successful reconstruction of the skeleton, we can then work with our plastic surgery colleagues to complete any additional skin or soft tissue procedures, including breast implant placement in affected young women.