The diagnosis of Marfan syndrome (MFS) is based on evaluating a large number of clinical criteria. We have observed that many persons presenting in specialized centers for "Marfan-like" features do not have MFS, but exhibit a large spectrum of other syndromes. The spectrum of these syndromes and the distribution of "Marfan-like" features remain to be characterized. Thus, we prospectively evaluated 279 consecutive patients with suspected MFS (144 men and 135 women at a mean age of 34+/-13 years) for presence of 27 clinical criteria considered characteristic of MFS. The most frequent reasons to refer individuals for suspected MFS were skeletal features (31%), a family history of MFS, or aortic complications (29%), aortic dissection or aneurysm (19%), and eye manifestations (9%). Using established criteria, we confirmed MFS in 138 individuals (group 1) and diagnosed other connective tissue diseases, both with vascular involvement in 30 (group 2) and without vascular involvement in 39 (group 3), and excluded any distinct disease in 72 individuals (group 4). Clinical manifestations of MFS were present in all four patient groups and there was no single clinical criterion that exhibited positive and negative likelihood ratios that were per se sufficient to confirm or rule out MFS. We conclude that "Marfan-like" features are not exclusively indicative of MFS but also of numerous, alternative inherited diseases with many of them carrying a hitherto poorly defined cardiovascular risk. These alternative diseases require future study to characterize their responses to therapy and long-term prognosis.
We report the cases of 28 patients with bacterial infections of the shoulder treated between 1979 and 1991. There were ten cases of septic arthritis, ten cases of simple osteomyelitis of the proximal humerus, four cases of septic arthritis and concomitant osteomyelitis of the proximal humerus and four cases of periarticular soft-tissue infection. The infections, except for the cases of osteomyelitis, were staged by a "Classification of Exogenic Bacterial Infections" (CEBI). In septic arthritis and in periarticular soft-tissue infection, the time between the initial symptoms of infection and diagnosis was about 20 days. In the cases with osteomyelitis, there was an average delay of 9 months, which was partly due to the slow evolution of plasmacellular osteomyelitis. Treatment was based on operative debridement and arthrotomy, the insertion of drains, the implantation of gentamicin-polymethylmethacrylate beads and the application of high-dose parenteral antibiotics. In the postoperative period physiotherapy with early active and/or passive range-of-motion exercises favoured the draining of secretions and therefore gave better results than complete immobilisation. Treatment was evaluated using a modification of the shoulder score of Wülker et al. [17]. This study demonstrated that favourable results could only be obtained if the diagnosis was made early. This is particularly true for infections with Staphylococcus aureus (found in 19 patients). The overall result of the treatment of osteomyelitis and periarticular soft-tissue infection was good or satisfactory, while unsatisfactory results were noted for the patients with septic arthritis, particularly those with both septic arthritis and osteomyelitis.
The language spoken by orthopaedic surgeons is filled with eponyms. In orthopaedics, perhaps more than any other medical specialty, we speak cryptically to one another using code words and other secret language. Certain hyphenated eponyms are of particular interest because they pique one's curiosity as to how these people came to be partners in orthopaedic history. In this paper, we offer some bits of orthopaedic hyphenated-history, outlining the pertinent work of Theodor Kocher and Bernhard von Langenbeck, as well as associated background information. These two surgeons are linked through a common hyphenated eponym in orthopaedic surgery: the Kocher-Langenbeck surgical approach, a well-known posterior approach to the proximal femur and acetabulum.
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