Natural history study of arthrogryposis multiplex congenita, amyoplasia type

Arthrogryposis or Arthrogrypsosis Multiplex Congenita (AMC) are terms used to describe the clinical finding of multiple congenital contractures. There are more than 300 distinct disorders associated with arthrogryposis. Amyoplasia is the most common type of arthrogryposis and is often referred to as the “classic” type. There is no known cause of amyoplasia and no risk factors have been identified. Moreover, there is no established diagnostic criteria, which has led to inconsistency and confusion in the medical literature. The purpose of this study was to describe the natural history of amyoplasia, to determine if there are any identifiable risk factors and develop a list of diagnostic criteria. A retrospective chart review of 59 children with arthrogryposis ascertained at the Shriners Hospitals for Children in Houston, Texas was performed and included the following information: prenatal, birth, and family histories, and phenotypic descriptions. Forty-four children were identified with amyoplasia and 15 children with other multiple congenital contractures and other anomalies (MCC) were used as a comparison group. With the exception of abnormal amniotic fluid levels during pregnancy, there were no significant demographic or prenatal risk factors identified. However, we found common features that discriminate amyoplasia from other types of arthrogryposis and developed a diagnostic checklist. This checklist can be used as diagnostic criteria for discriminating amyoplasia from isolated and multiple contracture conditions.

Two Obstetrical Heresies

Dr. Silas F. Starley deplores what he considered errors generally taught and accepted in the late 19th century in Two Obstetrical Heresies . “The first is the part that membranes containing the amniotic fluid and the foetus play in effecting dilation of the os uteri in the first stage of labor.The second is the supposed necessity for waiting for their rupture and the escape of the waters before applying the forceps, in every case, without exception.” Silas F. Starley (1823-1887) was born in Alabama and moved to Texas with his family in 1837. He graduated from the University of Louisville School of Medicine in 1854 and spent his professional career in Texas, ending his career in Corsicana. He was President of the State Medical Association of Texas (Texas Medical Association) in 1883 and wrote articles in Texas medical journals on various topics including obstetrics, vascular tumor, and pneumonia. Texas State Historical Association, http://www.tshaonline.org/handbook/online/articles/sat05 , accessed 10/16/2012. Texas Physicians Historical Biographical Database, http://www4.utsouthwestern.edu/library/doctors/doctors.cfm?DoctorID=16809 , accessed 10/16/12.