[Anesthesia in a child presenting a anhydrotic ectodermic dysplasia associated with a multiminicore myopathy]. / Anesthésie d'une enfant presentant une dysplasie anhydrotique ectodermique associée à une myopathie multiminicore.

PURPOSE: To report the perioperative management of anesthesia and analgesia in a child presenting with the association of multiminicore myopathy (MMM) and anhydrotic ectodermic dysplasia (AED). CLINICAL FEATURES: An eight-year-old girl was admitted for elective orthopedic surgery of the lower limbs. AED is a congenital dermatosis characterized by the absence of sweating and subsequent problems with thermoregulation; in addition, maxillary hypoplasia and abnormal teeth can render intubation difficult. MMM is a rare congenital myopathy characterized by proximal muscle weakness, stable in time or with a slow and progressive evolution. It can involve respiratory muscles and be associated with severe cardiomyopathy. Moreover, MMM shares some characteristics with Central Core Disease which is known to be associated with malignant hyperthermia. Since depolarizing muscle relaxants and halogenated agents could not be used, a combined propofol-based intravenous anesthesia with lumbar epidural analgesia was chosen. This combination provided stable anesthesia, smooth recovery and excellent analgesia during and after the operation, without complications. Temperature was monitored closely during surgery and in the postoperative period. CONCLUSIONS: The association of MMM and AED requires rapid distinction between hyperthermia secondary to anhydrosis and malignant hyperthermia. The management should provide a "trigger-free" anesthetic and optimal postoperative analgesia without sedation. If appropriate for the surgical procedure, a combination of general with regional anesthesia is particularly attractive in achieving these objectives.

Growth disturbance of the ilium after splitting the iliac apophysis and iliac crest bone harvesting in children: a retrospective study at the end of growth following unilateral Salter innominate osteotomy in 21 children.

The morphology of the iliac bone was assessed at the end of growth on AP x-rays of the pelvis in 21 children who had previously undergone unilateral pelvic osteotomy. The nonoperated side was used as a reference. There were 13 girls and 8 boys. Age at operation varied from 12 months to 12 years with a mean of 3 years and 10 months. The patients were distributed in 2 groups depending on their age at operation: before age 5 (group A, 16 cases) or after age 5 (group B, 5 cases). The mean age at follow-up was 15 years and 2 months (range 11-19 years). The end of pelvic growth was established by Risser stage IV. Distinct hypoplasia of the ilium due to premature growth arrest was observed in 16 cases: 12 in group A and 4 in group B. Other changes in the morphology of the ilium were noted, e.a. increased height of the ilium which was noted in 12 cases. The cosmetic prejudice was however minor, as compared with the radiological changes. In the authors' opinion, the alar hypoplasia was related to growth disturbances due to repeated splitting of the iliac apophysis. To prevent this complication the authors recommend avoiding the use of an electrocautery to incise the iliac apophysis and cutting the Kirschner wires so that their proximal ends lie within the subcutaneous fat, in order to avoid repeated splitting of the apophysis at the time of hardware removal.

[Contribution of electromyographic analysis of the walking habits of children with spastic foot in cerebral palsy: a preliminary study]. / L'apport de l'analyse électromyographique de la marche dans l'étude du pied spastique de l'infirme moteur d'origine cérébrale: étude préliminaire.

PURPOSE OF THE STUDY: This preliminary study describes the methodology and the results of gait analysis in cases of equinus and equino-varus deformity of the foot in cerebral palsy children. The ultimate goal was to establish an aid to decision in spastic foot surgical management. MATERIAL: A prospective series of 12 walking children (16 feet) with cerebral palsy has been evaluated prior to surgical correction of equinus or equino-varus deformity of the foot. The mean age was 8 years (range 4 to 11 years of age). METHOD: The pattern of muscle activity during gait cycle has been recorded by surface electrodes for the tibialis anterior, the triceps and the peroneae and by implanted electrodes for the tibialis posterior. Foot switches have been used to differentiate swing and stance gait phases. The results were compared with these of a series of normal children previously published. RESULTS: In four cases, the dynamic equinus was due to an extended or continuous contraction of the triceps surae. The varus deformity appeared to originate from two muscles: the tibialis posterior in 9 cases and the tibialis anterior in 1 case. Both muscles were responsible for the deformity in 2 cases. Two muscular activation patterns were observed in the tibialis posterior: inverted (2 feet) or permanent (9 feet). From the main muscle which was responsible for deformity, we have determined the surgical technique which was most appropriate to restore the muscular balance. DISCUSSION: Our observations confirm Perry's hypothesis, namely that selective and phasic control during the walking cycle does not occur for patients suffering from cerebral palsy. Different surgical procedures were chosen according to the literature on this subject. In the treatment of equinus deformity, lengthening of the Achilles tendon is a satisfactory technique for hemiplegic patients. But we prefer gastrocnemius recession described by Vulpius in spastic diplegia in order to avoid over-lengthening or calcanal gait. If it can be shown that posterior tibial muscle overactivity is the cause of equinovarus, we perform a posterior tibial tendon lengthening, as proposed by Ruda and Frost, or a split posterior tibial tendon transfer as developed by Green. If the posterior tibial muscle is active only during the swing phase, we accomplish a split posterior tibial tendon transfer through the interosseus membrane as advocated by Saji. If the anterior tibial muscle is continuously active, a split anterior tibial tendon transfer to the cuboid described by Hoffer is performed. If the activity is continuous in both the tibial posterior and the tibial anterior muscles, we add a posterior tibial myotendinous lengthening to the split anterior tibial tendon transfer. CONCLUSION: Since 1992, we have developed in our institution a gait analysis laboratory in order to bring objective data in the process of decision making for tendon transfer surgery. With 4 years experience, this objective support now seems to us compulsory in decision of type of transfer. The goal of this preliminary study was to explain how we use the data and match these to our experience and literature.

[Upper tibial osteotomy for gonarthrosis in genu varum. Apropos of a series of 193 cases reviewed 6 to 10 years later]. / Ostéotomie tibiale haute de valgisation pour gonarthrose sur genu varum. A propos d'une série de 193 cas revus après 6 à 10 ans de recul.

The authors have analyzed the results of 193 tibial osteotomies with varus deviation reviewed after an average 8 year follow-up. There were 71 per cent of good results. 14 patients (7.2 per cent) were reoperated for unsuccessful osteotomy. The optimal postoperative tibio-femoral axial alignment was obtained between 3 degrees and 5 degrees valgus. The analysis of the series illustrates clearly the criteria of good results thus providing a guide to surgical indication. It is a question of the preoperative height of medial tibio-femoral space being higher or equal to 50 per cent of the normal, a constitutional tibial varus over 5 degrees, a preoperative mechanical axis of less or equal to a 10 degrees varus, the absence of subluxation of the tibial eminences. Statistically the most important factor (p = 0.0004) was the height of the medial tibio-femoral space of which the decrease illustrates the stage of development of A.O. Finally, in the case of arthritic genu varum, the painful patello-femoral syndrome progresses favorably in the case of good surgical indication on the medial tibio-femoral compartment.

[Surgical treatment of isthmic spondylolisthesis. A comparative study of 3 types of arthrodesis]. / Traitement chirurgical du spondylolisthésis isthmique. Etude comparative de 3 types d'arthrodèse.

The authors present a series of 186 cases of spondylolisthesis treated using 3 different types of arthrodesis: 84 by anterior arthrodesis, 35 by postero-lateral arthrodesis and 67 by combined arthrodesis. Slippage reduction was sought only in combined arthrodesis. The elimination of pain or a significant improvement were noted in 95 per cent of cases treated by anterior arthrodesis, in 74 per cent of those treated by postero-lateral arthrodesis and in 97 per cent of those treated by combined arthrodesis. The work resumption or professional rehabilitation rate was 89 per cent for anterior arthrodesis, 46 per cent for postero-lateral arthrodesis and 90 per cent for combined arthrodesis. Analysis of the clinical results according to the criteria of Stauffer and Coventry shows that good results were noted in 69 per cent of cases treated by anterior arthrodesis, in 43 per cent of those treated by postero-lateral arthrodesis and in 74 per cent of those treated by combined arthrodesis. A fusion rate of 86 per cent was achieved after anterior arthrodesis, 69 per cent after postero-lateral arthrodesis and 95 per cent after combined arthrodesis. Reduction had no effect on either clinical results or spinal statics in spondylolisthesis of less than 50 per cent. In the reduction of spondylolisthesis of more than 50 per cent, better results were obtained with the R. Louis technique than the Harrington technique in the restoration of spinal statics and maintenance of long-term clinical results.

[Dysplasia and spontaneous bilateral dissection of the internal carotid arteries: apropos of a case. Value of surgical treatment]. / Dysplasie et dissection spontanée bilatérale des carotides internes: à propos d'un cas intérêt du traitement chirurgical.

The authors report a case of spontaneous internal carotid artery dissection with a fibromuscular dysplasia of the heterolateral artery. Etiopathogeny, anatomo-pathology, different arteriographic forms and treatment are discussed. The interest of this case is to be found in two aspects: The dysplasia seems to be at the origin of the dissection. It shows that the treatment hereby applied (extra-intracranial bypass) will be of good interest in such situation. The patient became and remain totally asymptomatic 45 months after the second operation.

Teratologic dislocation of the hip: review of a series of 17 cases.

Teratologic dislocation of the hip is defined as a congenital dislocation which is irreducible by gentle manipulation at birth. It is often associated with other congenital defects, the most common of which is arthrogryposis. In a series of 25 teratologic dislocations, we studied 17 dislocations that were treated and followed up for an average of 4 years and 6 months (minimum 1 year, maximum 10 years). Clinical and radiological results confirm that the treatment--mainly surgical--can give these children, even arthrogrypotic ones, a congruent and functional hip. This treatment may be recommended for bilateral as well as for unilateral teratologic hip dislocation.

[Natural history of congenital hip dislocation during the first year of life]. / Histoire naturelle de la maladie luxante au cours de la première année.

It seems obvious that congenital hip dislocation is determined by perinatal factors that are all the more "pathogenic" that the baby presents laxity (female sex or family laxity of the joints) and the hip is less mature (hereditary factor?). In our population, at the time of birth less than one child out of 1000 presents a dislocated hip that can be reduced (typical standard Ortolani sign). One or two percent present unstable hips that stabilize spontaneously in most cases. This stabilization can be expedited by correct diapering in abduction or may be hindered by such practices as wrapping infants from birth with their legs stretched and adducted, strapping the baby to a cradle-board, or perhaps even by putting the child to bed on its side. Stabilization may even be hindered by spontaneous asymmetrical postures that maintain the hip of the concave side of the baby in adduction, as in the pelvic obliquity syndrome of the newborn. The remaining children have a so-called stable hip which presents a variable degree of laxity on clinical examination and a variable degree of maturity on ultrasonography. The observation of established congenital dislocations in children who have been followed since birth (and such cases escape the most meticulous screening) suggests that in some hips there is no clear-cut dividing line between instability and laxity. Hips either stabilize or evolve into a fixed dislocation within the first three months, whereas it is only during the fourth month that X-ray examination may confirm the normality of such at-risk hips. Nevertheless, at that age, normality criteria are not very accurate.(ABSTRACT TRUNCATED AT 250 WORDS)