Pathogeny and natural history of congenital dislocation of the hip.

Based on a review of the literature, the authors have made a critical study of several etiological factors. Endogenous factors such as acetabular dysplasia, increased anteversion of the femoral neck, and capsular laxity support the genetic theory but are neither constant nor necessary and are only facilitating factors. The major factor seems to be a mechanical one linked to the position in the uterus: hyperflexion with adduction and external rotation constituting the dislocating foetal posture combined with abnormal pressure on the greater trochanter and leading to expulsion of the head upward and backward. This theory can explain the natural history of C D H which is first, at birth a hip instability followed by two possible evolutions: either persistent luxation becoming irreducible or spontaneous stabilisation leading sometimes to complete healing or to residual abnormalities (subluxation or dysplasia). This concept suggests practical conclusions: the importance of an early diagnosis, the selection of the signs of the hip at risk, the pattern of prevention, the role for non-clinical investigations, the principles of the treatment based on postures, the indications for the different types of treatment.

Genetic characterization of the body attributed to the evangelist Luke.

Historical sources indicate that the evangelist Luke was born in Syria, died in Greece, and then his body was transferred to Constantinople, and from there to Padua, Italy. To understand whether there is any biological evidence supporting a Syrian origin of the Padua body traditionally attributed to Luke, or a replacement in Greece or Turkey, the mtDNA was extracted from two teeth and its control region was cloned and typed. The sequence determined in multiple clones is an uncommon variant of a set of alleles that are common in the Mediterranean region. We also collected and typed modern samples from Syria and Greece. By comparison with these population samples, and with samples from Anatolia that were already available in the literature, we could reject the hypothesis that the body belonged to a Greek, rather than a Syrian, individual. However, the probability of an origin in the area of modern Turkey was only insignificantly lower than the probability of a Syrian origin. The genetic evidence is therefore compatible with the possibility that the body comes from Syria, but also with its replacement in Constantinople.

[Fibrodysplasia ossificans progressiva. Apropos of a case]. / Myosite calcifiante progressive. A propos d'une observation.

PURPOSE OF THE STUDY: Fibro-dysplasia ossificans progressive is a rare inherited disease, presumably transmitted as an autosomal dominant defect. The high level of spontaneous mutations explains the sporadic cases. Pre symptomatic diagnosis could be actually evoked by the association of progressive ossification of soft tissues with congenital anomalies of bones (metacarpal and metatarsal). The purpose of this study is to report a clinical case with a very severe course. CASE REPORT: A 10-year-old boy developed progressive ossification of muscles and soft tissues in multiple sites neck, back, shoulders, elbows, hips and knees. The clinical course was severe due to the ankylosis of all the joints and the decrease of pulmonary reserve with fixation of the chest wall. A malformation of the great toes facilitated the diagnosis. DISCUSSION: This pattern of heterotopic ossification together with the congenital malformations of the great toes defines the developmental phenotype for fibrodysplasia ossificans. A slow course with successive thrusts occurs. Ossification progressively involves tendons, ligaments and the connective tissue of skeletal muscles. Excision of heterotopic bone is futile as the trauma of the operation can lead to the stimulation of new heterotopic ossification at the operative site. Surgery is only useful for biopsy and histological study reveals bone metaplasia. The mature heterotopic bone is histologically indistinguishable from mature skeletal bone. The non skeletic muscles are characteristically spared from ossification. Premature death often results from respiratory failure due to fixation of the thoracic cage. The pathogenesis and the treatment of the disorder are unknown.

[Pathogeny and natural history of congenital dislocation of the hip]. / Pathogénie et histoire naturelle de la luxation congénitale de la hanche.

Based on a review of the literature, the authors have made a critical study of several etiological factors. Endogenous factors such as acetabular dysplasia, increased anteversion of the femoral neck, capsular laxity support the genetic theory but they are neither constant nor necessary and are only facilitating factors. The major factor seems to be a mechanical one linked to the position in uterus: hyperflexion with adduction and external rotation causing an abnormal pressure on the greater trochanter leading to an expulsion of the head upward and backward. This theory can explain the natural history of C D H which is first, at birth a hip unstability followed by to possible evolutions: either a persistent luxation becoming irreducible or a spontaneous stabilisation leading sometimes to complete healing or to residual abnormalities (subluxation or dysplasia). This concept suggests practical conclusions: the importance of an early diagnosis, the selection of the signs of the hip at risk the pattern of prevention, the importance of non clinical investigations, the principles of the treatment based on postures, the indications for the different types of treatment.

[Congenital convexity of the feet. Anatomical study and practical conclusions]. / Le pied convexe congénital. Etude anatomique et déductions pratiques.

The authors have studied the pathological anatomy of two congenital pes convex, (C.P.C) coming from an 8 days boy suffering of arthrogryposis. One of the feet has been dissected by stage whilst the other one has been operated on according to a procedure of correction of the deformities. The two feet combined an irreducible dislocation of the os naviculare on the neck of the talus, a calcaneo cuboïdeum subluxation, a moderate equinus of the hind foot, an important contracture of the extensor tendons and an elongation of the structural soft tissues of the sole. The main abnormalities of the talus and of the calcaneus involved the upper direction of the anterior articular facet, in relation to the dorsal dislocation of the os naviculare and the dorsal subluxation of the cuboïdeum. The subtalaris joint was little altered. The study of dissections and the clinical reports published connected with the observations of the authors confirm the pre-eminence of the transversal dislocation of the tarsus and of its irreducibility. On the other hand the abnormalities of the subtalar joint have been, probably over estimated. The sometimes important increase of talo calcaneal angle on A.P. radiographies may be explained by the incidence itself. The verticality of the talus is not a reliable feature of C.P.C. and the deformities of the fore foot are variable (pronation, supination). This study leads to a new definition of the C.P.C.: Congenital deformity of the foot with tarsi-transversa dislocation or subluxation involving mainly the talo-navicular joint. The criterious of diagnosis are debated and a logical surgical procedure of correction is suggested.