Imaging Pearls ❯ Musculoskeletal ❯ Congenital Anamolies
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- What is Poland anomaly?
- “Named after Sir Alfred Poland, Poland anomaly (PA) is described as an underdevelopment or absence of the chest muscle (pectoralis) on one side of the body and webbing of the fingers (cutaneous syndactyly) of the hand on the same side (ipsilateral hand). Sometimes referred to as "Poland syndrome," it is an uncommon condition present at birth (congenital). For people born with PA, the breastbone portion (sternal) of the pectoralis is also missing.”
- National Human Genome Research Institute - What are the associated features of Poland anomaly?
- Absence (aplasia) of some of the chest (pectoralis) muscles.
- The end of the main chest muscle, where it attaches to the breastbone, is usually missing.
- The nipple, including the darkened area around it (areola) is underdeveloped or missing. And in females, this may extend to the breast and underlying tissues.
- Abnormally short, webbed fingers (syndactyly).
- Often, the armpit (axillary) hair is missing.
- The skin in the area is underdeveloped (hypoplastic) with a thinned subcutaneous fat layer.
- The upper rib cage can be underdeveloped or missing. Sometimes, such abnormal development extends to the shoulder blade or the bones of the arm.
- All these symptoms occur on one side of the body (unilateral) - How is Poland anomaly diagnosed and treated?
- “Poland anomaly can be detected as early as birth and as late as adolescence, depending on how severe it is. PA can be detected through thorough clinical evaluation and from a variety of specialized tests. Tests may include advanced magnetic resonance imaging (MRI) techniques, computerized tomography (CT) scans and X-Rays. CT scans help determine the extent to which the muscles may be affected, by showing cross-sectional images of particular structures within the body. X-Rays can help identify and characterize any specific abnormality in the hand, forearm, ribs and/or shoulder blades.” - How is Poland anomaly diagnosed and treated?
- The most viable treatment for PA is reconstructive surgery. Existing chest muscles are used to rebuild the chest. Muscle can be taken from other parts of the body, for instances where there is not enough chest muscle for rebuilding. For males with PA, reconstructive surgery can be done as early as age 13. For females, surgery may have to be postponed until breast development is complete. Reconstructive surgery can be helpful for the psychological development of teenagers, who are especially aware of physical differences. However, if surgery is performed too early, while the individual is growing, asymmetry can result or be made greater than before. For females, reconstructive surgery may be performed to correct the different sizes of the two breasts. (National Human Genome Research Institute) - “Cutaneous and subcutaneous malignancies represent the most common type of cancer and frequently present with overlapping imaging appearances. CT, MRI, and F-FDG PET/CT are commonly used for staging, preoperative planning, and posttreatment assessment. Knowledge of the multimodality imaging features can narrow down the differential diagnosis and elucidate their metastatic pattern.”
Malignant skin and subcutaneous neoplasms in adults: multimodality imaging with CT, MRI, and 18F-FDG PET/CT.
Juan YH et al.
AJR Am J Roentgenol. 2014 May;202(5):W422-38