Surgical management for mentosternal contractures by flap procedures

Mentosternal contractures still represent a surgical challenge due to their prominent position. They require early operative treatment for both function and aesthetic reasons. Careful clinical examination of scar position and traction forces, both at rest and when functioning, in addition to proper evaluation of the surrounding soft tissue are mandatory for precise preoperative planning of the required reconstruction. In general, the technically most feasible operation is favored if the functional and aesthetic results are good and postoperative risk for recurrence is low. Between 1987 and 1994, 21 patients with cervical, mentosternal, and mentothoracic contractures were operated upon. Eight patients were reconstructed with local flaps and 13 with microvascular free flaps.     

Effect of temperature variation (22˚C- 44˚C) on halothane and caffeine contracture testing in normal humans

Background: Malignant hyperthermia (MH) susceptibility is diagnosed using halothane-caffeine contracture testing of a muscle sample maintained at 37˚C. However, there has not been a systematic study that examines the effect of different temperatures on the response of normal muscle to halothane and caffeine. We hypothesized that altering bath temperature would modify the contracture responses.
Methods: We obtained muscle samples from 20 patients undergoing surgical procedures of the lower extremities. The samples were dissected into 245 bundles and the bundles were exposed to halothane 3% or incremental caffeine, according to the North American MH group protocol. Several bundles from each patient were simultaneously studied at four different temperatures (22˚C, 30˚C, 37˚C and 44˚C). Each bundle was studied at only one temperature, the muscle samples of 3 patients were simultaneously studied at all four temperatures for halothane and caffeine.
Results: Maximum contracture to caffeine (32 mM) was highest at 37˚C; however, at lower caffeine concentrations (2–4 mM), there was no consistent effect of temperature on contracture response. Likewise, temperature did not alter contracture responses to halothane. The extremes of temperature (22˚C and 44˚C) were associated with lack of twitch in response to electrical stimulation. For the bundles exposed to halothane at 22"C, the absence of a twitch was associated with the presence of a contracture, although these were never above the diagnostic threshold.
Conclusions: We conclude that temperature has little effect on responses of normal muscle to halothane and caffeine.     

Congenital contractures,ectodermal dysplasia,cleft lip/palate,and developmental impairment: A distinct syndrome

Brother were affected with severe congenital contractures, multiple cutaneous manifestations of ectodermal dysplasia, cleft lip/palate, and psychomotor and growth impairment. High resolution prometaphase chromosomes were normal. Molecular studies of DNA markers, closely flanking the X-linked hypohidrotic ectodermal dysplasia locus, did not show evidence of a submicroscopic deletion from the Xq12-q13 region. The parents and a normal sister exhibited none of these findings. This constellation of anomalies appears to represent a unique AR or XLR syndrome. © 1993 Wiley-Liss, Inc.     

Autosomal dominant inheritance of Klein–Waardenburg syndrome

We report on 2 sibs with the Klein–Waardenburg syndrome; they had dystopia canthorum, blepharophimosis, and bilateral flexion contractures of the fingers. The children's father and paternal aunt are also affected. This report confirms that the Klein–Waardenburg syndrome is an autosomal dominant syndrome.