Polands Syndrome Essays - Health, Muscular System, Medicine

Poland's Syndrome POLANDS SYNDROME There is a rare and perplexing congenital condition known today as Polands Syndrome. This extremely unusual disorder that is present at birth falls very near and dear to my heart. I will explain in later paragraphs. An anatomy student by the name of Alfred Poland made the first documented observation in 1841 at Guys Hospital in London. Alfred Poland discovered this extraordinary and unexplored abnormality while dissecting the cadaver of a convict that was hung for murder. The description Alfred Poland first provided was that of a congenital thoracic anomalies involving the pectoralis major and pectoralis minor with additional abnormalities on the affected side of the body (ipsilateral) consisting of webbed or fused phalanges (syndactyly). The term Polands Syndactyly was used to describe this disorder until 1967 when Baudinne renamed it, Polands Syndrome. It was felt that the condition would be more accurate if referred to as a syndrome because there were many anomalies then just syndactyly. Today, the laymens definition of Polands Syndrome consists of a developmental disorder that is present at birth. It is characterized by the absence or underdevelopment of chest muscles (pectoralis major, pectoralis minor) and abnormally short, webbed fingers or absence of middle phalanges. Additional finding might include underdeveloped or absence of one nipple or breast on the affected side. More extreme cases exhibit upper underdeveloped ribs and or an abnormally short arm with underdeveloped forearm bones. Unfortunately the limitation of Polands Syndrome deformity doesnt always cease here. The medical society has documented isolated cases over the past 100 years of chest wall disfigurement, lung herniation and spine curvature. Abnormalities involving the clavicle, scapula, kidney, testes, heart and associated occurrences of leukemia were also observed in patients with Polands Syndrome. The most recent medical studies given on this irregularity in the past 40-50 years has confirmed that Polands Syndrome begins within the thorax and is confined unilaterally. Any additional unilateral abnormalities affecting the extremities or nearby musculoskeletal components vary in severity. For an accurate diagnosis of Polands Syndrome to be made it must include the congenital partial or total absence of the pectoralis major muscle with or without the loss of the pectoralis minor muscle. The very little research and studies that have been done on this infrequent condition within the medical community have agreed that the ratio of affected males to females is 3 to 1. In accession, the studies revealed that the congenital deformity has a 75% chance of being prominent on the right side. The origin of Polands Syndrome is still unknown. Over the years there have been many theories as to the cause of this sporadic mutation which prays on approximately 1 in 30,000. Many have hypothesized that Polands Syndrome is heredity in nature. Even though a number of cases have been reported involving multiple family members, there isnt enough information within the studies to prove a genetic hypothesis. The most sensible theory that has been established relates to fetal development during the sixth week of gestation. It is widely known among the medical profession that the right side of the developing vascular tree suffers more anomalies then the left. Because of this known fact, Bouwes-Bavnick and Weaver made a possible correlation implicating the developing vascular tree as the culprit. They stated that the cause of Polands Syndrome could be explained by an isolated defect in the development of the vascular tree. They stated that the key time period for the development of the vascular is the sixth week and that also happens to be one of the most sensitive points of fetal growth. It is the sixth week of gestation that is the point of vascular differentia tion from the sixth vascular arches to the matured vascular pattern. It is at that time that the vertebral and subclavian arterial branches become distinct entities. They hypothesized the fetal phenomenon to a specific location within the maturing vascular tree, which will result in a predictable malformation. I know this vascular tree theory doesnt sound all that convincing and that is why, when a child is diagnosed with Polands Syndrome the medical doctors still describe the cause as unknown. Even though Polands Syndrome can effect up to five of the bodies systems (muscular, skeletal, integumentary,