Saturday, September 18, 2010

Maurice Klippel

Klippel-Feil Syndrome
Maurice Klippel (1858 - 1942) French physician, for whom two medical contions are named, namely Klippel-Feil syndrome and Klippel-Trenaunay-Weber syndrome. Kleppel-Feil syndrome is a heterogenous group of conditions, all characterized by the fusion of 2 or more of the 7 cervical vertebrae. It is caused by the failure of the segmentation of the cervical spine during fetal development. Most common signs of the disorder include a short neck, low hairline, and decreased range of cervical motion. A Sprengel's deformity (a codntion of abnormal fetal shoulder development where one scapula fails to descend and is thereby situated higher on the back than the other, an omovertebral bone also sometimes connects the affected scapula to the spine) is generally associated with this condition. Other associated anomalies are frequent - spina bifida, cleft palate, abnormalities of heart and kidney. The former English cricketer Gladstone Small suffers from this syndrome. Klippel-Trenaunay-Weber syndrome is a rare congenital anomaly where blood and lymph vessels fail to develop properly. When Klippel and Trenaunay first described the condition in 1900, they termed it Naevus Vasculoses Osteohypertrophicus. The syndrome is characterized by the presence of port-wine stains, varicose veins, hypertrophy of bone and soft tissue (leading to local gigantism or shrinking) & and improperly developed lymph tissues.