Radial osteotomy for the correction of forearm deformities in hereditary multiple osteochondroma.

Forearm deformities are often observed in patients with hereditary multiple osteochondroma, resulting in functional disability and cosmetic impairment. The aim of this study was to assess clinical and radiological outcomes after corrective osteotomy of the radius (COR). We performed a retrospective analysis of clinical and radiologic data from patients with forearm deformities who underwent COR combined with osteochondroma resection between 1978 and 2015. Seventeen patients (17 forearms) were included. The mean (range) age at surgery was 11.8 years (3.2-14.4), and the mean interval between surgery and last follow-up was 8.2 years (2-34.2). Range of motion was moderately increased and postoperative radiological assessments found significant improvements in ulnar variance, radial articular angle, bowing of the radius, and carpal slip. At last follow-up, a loss of ulnar variance correction was noted in 11 cases (mean loss: 4mm). The mean score on the Quick Disabilities of the Arm, Shoulder and Hand self-administered questionnaire was 13.9. Our results show that a forearm deformity in a patient with hereditary multiple osteochondroma is an appropriate indication for COR combined with osteochondroma resection and should be performed at the end of growth. This simple, safe technique corrects bowing of the radius and radius-ulna length discrepancy and could limit the risk of radial head dislocation. LEVEL OF EVIDENCE: IV.

Pamidronate treatment of children with moderate-to-severe osteogenesis imperfecta: a note of caution.

BACKGROUND/AIMS: Bisphosphonates have been reported to decrease fractures related to osteogenesis imperfecta (OI). We assessed the efficacy and long-term safety of pamidronate therapy in patients with moderate-to-severe OI. METHODS: We conducted an open-label uncontrolled study in 14 boys and 13 girls whose mean age was 6.8 years at baseline. Intravenous pamidronate, 1 mg/kg/day, was given for 3 consecutive days every 4 months for 2-6 years, with physical therapy and orthopedic surgery as appropriate. Mobility score, fracture rate, height, bone mineral density (BMD) and bone healing were evaluated throughout follow-up. RESULTS: In 24 (89%) patients, the fracture rate decreased to 6 months) occurred in 8 (29.6%) patients; their BMD gains, baseline age and treatment duration were not significantly different from those in the other patients. Tolerance was good. CONCLUSION: Pamidronate with physiotherapy and orthopedic management improved outcomes without delaying fracture healing in 19 (70%) of 27 patients. Delayed fracture healing occurred in 8/27 patients. Pamidronate should be reserved for severe OI with multiple fractures and/or flattened vertebras.

[Fibrous dysplasia of the proximal femur in children and teenagers: surgical results in 22 cases]. / La dysplasie fibreuse de l'extrémité proximale du fémur chez l'enfant et l'adolescent: résultats du traitement chirurgical dans 22 cas.

PURPOSE OF THE STUDY: Fibrous dysplasia is a rare benign bone tumor which occurs preferentially in the proximal femur. In children, there is a risk of repeated fractures and coxa vara deformity, particularly in the polyostotic form. The most common orthopedic problem is fibrous dysplasia of the proximal femur which generally requires surgical treatment. The purpose of this study was to analyze clinical and radiological outcome after surgical treatment. MATERIAL AND METHODS: All children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively. The study cohort included 22 children (11 boys and 11 girls). Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail. RESULTS: In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities. DISCUSSION: In light of our results and those reported in the literature, the prognosis of the monostotic form can be considered good after surgical treatment. For the polyostotic form, preventive fixation is necessary. Osteotomy with "humeralization" appears to correct the deformity and prevent coxa vara in certain cases. For the more severe forms, medical treatment with biphosphonates may be a useful complement to the surgical treatment.

Outcomes of orthopedic surgery in a cohort of 49 patients with X-linked hypophosphatemic rickets (XLHR).

BACKGROUND: X-linked hypophosphatemic rickets (XLHR) is due to mutations in PHEX leading to unregulated production of FGF23 and hypophosphatemia. XLHR is characterized by leg bowing of variable severity. Phosphate supplements and oral vitamin analogs, partially or, in some cases, fully restore the limb straightness. Surgery is the alternative for severe or residual limb deformities. OBJECTIVE: To retrospectively assess the results of surgical limb correction in XLHR (osteotomies and bone alignment except for 3 transient hemiepiphysiodesis). METHODS: We analyzed the incidence of recurrence and post-surgical complications in 49 XLHR patients (29F, 20M) (mean age at diagnosis 6.0 years (± 7.1)). RESULTS: At first surgery, the mean age was 13.4 years (± 5.0). Recurrence was observed in 14/49 (29%) patients. The number of additional operations significantly decreased with age (2.0 (± 0.9), 1.7 (± 1.0) and 1.2 (± 0.4) in children <11 years, between 11 and 15, and >15 years; P < 0.001). Incidence of recurrence seemed to be lower in patients with good metabolic control of the rickets (25% vs 33%). Complications were observed in 57% of patients. CONCLUSION: We report a large series of surgical procedures in XLHR. Our results confirm that phosphate supplements and vitamin D analog therapy is the first line of treatment to correct leg bowing. Surgery before puberty is associated with a high risk of recurrence of the limb deformity. Such procedures should only be recommended, following multidisciplinary discussions, in patients with severe distortion leading to mechanical joint and ligament complications, or for residual deformities once growth plates have fused.

Acromicric dysplasia: long term outcome and evidence of autosomal dominant inheritance.

Acromicric dysplasia is a rare bone dysplasia characterised by short stature, short hands and feet, normal intelligence, mild facial dysmorphism, and characteristic x ray abnormalities of the hands. Only a very small number of children with this condition have been reported so far. Here we report on a series of 22 patients including 10 boys and 12 girls with acromicric dysplasia. Length was normal at birth and height fell progressively off the centiles postnatally. The mean adult height was 130 cm (133 cm in males, 129 cm in females). The hands, feet, and limbs were short and OFC was normal. Intelligence was normal and mild dysmorphic features were noted. Other occasional features included well developed muscles, a hoarse voice, generalised joint limitation in some patients, frequent ear, tracheal, and respiratory complication, and spine abnormalities. Long term follow up showed that facial dysmorphism was less obvious in adults and that carpal tunnel syndrome was frequent in older patients. Apart from short metacarpals and phalanges, internal notch of the second metacarpal, external notch of the fifth metacarpal, and internal notch of the femoral heads, there were no major x ray abnormalities. No major complications, such as cardiac disease or major orthopaedic problems, occurred in the course of the disease. The condition appeared to be sporadic in 16 cases but the observation of vertical transmission in three families was consistent with an autosomal dominant mode of inheritance.

[Functional and orthopaedic aspects of dystrophinopathies]. / Aspects fonctionnels et orthopédiques des dystrophinopathies.

Although the clinical picture and the natural progression of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) differ, borderline forms exist. Classical orthopaedic treatment is based on self-rehabilitation (by the parents and the patient), physical therapy, posture alignment with orthotics, ergotherapy to set up technical aides, notably positioning in an electric wheelchair to provide more satisfactory autonomy. The functional aspect predominates over pure orthopaedics. Although surgical indications have evolved for the lower limbs, pelvic-spinal arthrodesis for treatment of scoliosis remains the reference treatment, but the methods have evolved since the advent of corticotherapy for DMD. Corticotherapy slows the progression of motor deficits, the age at which walking ability is lost is delayed (shifting from 10 years to 13-14 years depending on the studies), scoliosis progresses later, respiratory insufficiency is better controlled, and therefore survival is prolonged (between 20 and 40 years). However, although this functional aspect seems to respond better to the progress in overall treatment, it also results from a multidisciplinary approach to the disease. Nevertheless, assessment is required, not at a time t as reflected by the scales currently in use, but during daily activities as proposed by qualitative and quantitative monitoring seeking to model nyctohemeral functional motor skills. The principle is to characterize the type of activity (sitting, standing, lying down, walking), its duration, its intensity (walking speed), its frequency (number of activity changes, number of walking episodes), and their sequence (temporal sequence, organization of activity variation). The goal is to identify the variety of functional motor skills and their occurrence over time to determine whether treatment contributes a functional benefit and whether this benefit is put into practice daily.

Dysspondylochondromatosis.

We report on 3 unrelated patients with an unusual form of neonatal dwarfism with unequal limb length. Radiographs show multiple enchondromatosis of the tubular and the flat bones and severe segmentation abnormalities of the vertebral column. These observations differ from the hitherto described forms of multiple enchondromatosis with growth disorders of the spine, spondyloenchondroplasia, and others. Therefore we propose to delineate this disorder as a new entity.

Osteochondritis dissecans of the humeral capitellum in children.

We studied 13 cases of osteochondritis dissecans of capitellum humeri in 12 children, 11 boys and 1 girl, aged between 10 and 15 years. We distinguished this affection from Panner's disease which affects young children and resembles Legg-Perthes-Calvé disease of the hip. Operative treatment was performed in 7 out of 13 elbows for removal of loose bodies or excision of osteochondritis in situ with cartilage damage. In other cases, functional treatment was carried out. At long-term follow-up, ranging from 2 to 13 years, clinical examination demonstrated satisfactory results in 9 cases; in 3 cases limitation of movement was related to fracture of the radial head or to delay in operative treatment for too long. Roentgenographically, changes related to growth disturbance were constantly observed; they involved the radial head, the olecranon, the trochlea and the proximal end of the ulna.

Desepiphysiodesis--elimination of partial premature epiphyseal closure. Experience of 17 cases.

Between 1975 and 1990, 17 growth plates have been operated on by epiphyseal bridge resection. The children were from 4 years and 10 months to 13 years and 10 months old. The etiology of partial closure was traumatic (10 times), caused by therapeutic mistakes (3 times), septic osteomyelitis (1 case), purpura fulminans (1 case), unknown (2 cases). There was always length discrepancy or deformity of bone. The regions that have been subjected to treatment were distal femur, proximal tibia, distal tibia, distal radius. Evaluation of the bone bridge was made by tomoscintigraphies and recently by MR imaging and computed tomoscintigraphy. The bone bridge size was from 2.5% to 60% of the growth plate surface; surgical technique consists of resection of bone bridge connecting epiphysis and metaphysis which is replaced by methyl metacrylate. In 16 cases simultaneous corrective osteotomy was performed. Results are poor, there were only two good results and 8 failures; seven results were medium. The failures can all be explained by mistakes in technique or indication, except one. Indications are post-traumatic narrow bridges in young children. It would be useful to know the vitality of the residual growth plate.

[Atheromatous stenosis of the left coronary trunk. 28 surgical cases]. / Sténoses athéromateuses du tronc de la coronaire gauche. Vingt-deux cas opérés

22 patients with atheromatous narrowing of the left coronary trunk underwent surgery between 1969 and 1974. Most of these patients had severe and incapacitating angina pectoris. The clinical features are readily explained by the size of the diffuse anatomical lesions, which are to be found on the left coronary trunk as well as on the three coronary vessels themselves. This series has not confirmed the serious risk of surgery in such cases, as there were no operative deaths. Secondary mortality was low (9%), and the 20 survivors (average length of follow-up: 26 months) were mostly (18/20) in an excellent condition functionally. These facts have lead us to advise surgery whenever possible.

[Idiopathic juvenile osteoporosis (author's transl)]. / Ostéoporose juvénile idiopathique.

A young girl aged 13 years was hospitalized for metaphyseal fractures of both knees, and was found to have idiopathic juvenile osteoporosis which followed a favourable course over a period of one year. The authors recall the difficulties encountered in diagnosing this rare affection as no specific biological changes have been demonstrated. The criteria used are: the age of the patient, the disease usually becoming evident in the prepuberty period, the contrast between the very severe osteoporosis noted on radiological examination and the perfect general condtion of the patient, the negative biological tests results, and finally, the spontaneous repair of the osteoporosis in one to four years.

[Regeneration of a diaphyseal segment of the radius (author's transl)]. / La régénérescence diaphysaire d'un os long chez l'enfant.

The osteogenesis potential and the subsequent skeleton reconstruction are well known in young children. Our case illustrate these two features. A six years old patient had lost the entire radial diaphysis during an open trauma of the forearm ; after the debridement of the wound, the limb was simply immobilized in a cast leaving off the radial diaphysis. Six months later an antomical reconstruction of the radius was obtained with a very good function.

[Congenital pseudarthrosis of the tibia and fibula in children. Results of the treatment of 18 cases with nails and bone grafts]. / Pseudarthroses congénitales du tibia et du péroné chez l'enfant. Résultats du traitement de 18 cas par enclouage et greffe.

Eighteen cases of congenital pseudarthrosis of the leg in 17 children were treated by nailing and tibio-fibular graft with a mean follow-up of six years (from one to twelve years). Six patients had reached the end of growth. Primary union was obtained in 17 cases in a mean time of 6.5 months (from 3.5 to 18 months), but one case developed a new pseudarthrosis at the level of an associated upper tibial osteotomy. Only one case required a supplementary graft. Walking was usually achieved without any apparatus after five-and-a-half months, the tibia being protected by a nail which was left in place until the end of growth. In two cases, a fracture produced by trauma occurred in the limb long after consolidation. These two fractures healed in the normal time. Inequality of limb length was present in all the cases, but its progress varied. On three occasions it was treated by femoral lengthening. Stiffness of the ankle and subtaloid joints resulted from nailing of the hindfoot in nine cases and improved very little after freeing of the joints. The use of telescopic rods avoids the need for further operations to change the rod during growth. We consider that this is a reliable method but that, in certain cases, the effects on growth and on the foot can be diminished by appropriate modifications of technique.

[Fractures of the talus in children. Apropos of 23 cases]. / Les fractures de l'astragale de l'enfant. A propos de 23 cas.

The authors have reviewed 23 fractures of the talus in children. They propose a classification based on the radiological maturity of the bone and the specific type of fracture. Group I: Children younger than 6 years (6 cases). The fracture line separates the bone into two parts. The diagnosis was difficult, with displacement being underestimated in some cases. Associated lesions in the same limb were present in 5 cases. Five fractures of the neck of the talus were treated conservatively with excellent or fair results. One comminuted fracture was treated surgically with a poor result. In all cases treatment should aim to maintain a good tibio-tarsal joint. Group II: Children older than 6 years (17 cases). Eight cases were osteochondral fractures, two were partial fractures and seven were complete fractures. Two of these developed subsequent avascular necrosis. The relationship between osteochondral fractures and osteochondritis is discussed.

[Hemimelica epiphysialis dysplasia (author's transl)]. / Dysplasie épiphysaire hémimélique (tarsomégalie). Aspects cliniques, radiologiques et évolutifs, traitement chirurgical. A propos de huit observations.

Hemimelica Epiphysialis Dysplasia is a rare osteochondrodystrophy producing cartilaginous epiphysial hypertrophy with hemimelic topography involving preferentialy the lower limb. Lesions are generally medialy situated (medial condyle of the femur, medial malleolus talus, tarsal scaphoid and first metatarsal bone). 8 cases of patients are discussed with a follow up of 4 years. The evolution is to a massive ossification of the hypertrophic cartilaginous areas. The issue is a fast arthrosic degeneration of the involved joints (mainly the ankle, more rarely the knee). The surgical procedures are often unsuccessful.

[Progressive femoral lengthening in children. Apropos of 36 cases]. / L'allongement progressif du fémur chez l'enfant. A propos de 36 cas.

The authors have analysed the result of femoral lengthening in 36 cases, 24 of them congenital. The technique, which is fully described, was that of progressive lengthening using external distraction either by the Judet or Wagner method. The average lengthening obtained was 18,3 p. 100 of the initial length of the femur--that is 5.2 centimetres on average. The bony and articular complications are described. The authors believe that, thanks to modifications of their technique, one femur should normally be able to be lengthened by 20 to 25 p. 100 in 30 days and should be solid in six months.

[Expanding intramedullary rods in the treatment of osteogenesis imperfecta (author's transl)]. / L'enclouage centro-médullaire télescopique chez l'enfant atteint d'ostéogenèse imparfaite.

Eleven cases of osteogenesis imperfecta were treated by corrective osteotomies and fixation with expanding intramedullary rods. The authors have used the Bailey-Dubow nail. Despite a short follow-up (average one year) they think that the device will avoid future repeated changes of rods during the growing period. Several technical pitfalls are described. It is thought that the same technique could be applied to congenital pseudarthrosis of the tibia.

[Instability and misdiagnosed or neglected dislocations of the upper cervical spine in children. Apropos of 20 cases]. / Instabilités et luxations méconnues ou négligées du rachis cervical supérieur de l'enfant. A propos de 20 observations.

Neglected instabilities or luxations of the upper cervical spine in children are rare if one discards conditions such as chondrodysplasia, Down Syndrome or others, were the spine is known to be at high risk of instability. We have studied twenty cases of neglected luxations and the delay in diagnosis is explained either by the asymptomatic character of some of these lesions, or by the difficulty in diagnosis. At the occipito-atlantal level we have reviewed: an instability in translation which required an occipito-axial fusion; two compensatory counter occipito-atlantal luxation of an atlanto-axial rotatory fixation. The diagnosis was best made with computed tomography scan and the treatment was not much different from the isolated atlanto-axial rotatory luxation. An instability in flexion extension, which was merely followed at regular intervals. At the atlanto-axial level 9 cases of sagittal instabilities in kyphosis and translation with a distance between atlas and axis of more than 5 mm were observed. These instabilities were most often associated with a malformation of the cranio-cervical junction; their treatment was usually surgical by means of an atlanto-axial or occipito-axial arthrodesis depending on the case. 9 other cases of atlanto-axial rotatory luxations were either isolated (7 cases) or associated with a counter occipito-atlantal rotatory subluxation (2 cases). Their diagnosis was made on routine X-rays, but the complete or incomplete aspect of the luxation, as well as its fixed aspect, was best appreciated with dynamic CT scan. Their treatment was always started with collar neck or halo traction in order to obtain, reduction of the dislocation, or at least the disparition of the torticollis and the head straight up on shoulders. The stability of the spine was achieved with a minerva cast jacket, halo cast or spine fusion depending on the case.

[Caput valgum in children. Natural history and treatment of a series of 17 hips that reached skeletal maturation]. / Caput valgum chez l'enfant. Histoire naturelle et traitement d'une série de 17 hanches parvenues à maturation squelettique.

The growth disturbance of the superior end of the femur which is related to a superior lateral epiphysiodesis of the femoral neck is known as caput valgum (C.V.). Most often, it appears after the treatment of a congenital dislocation of the hip (C.D.H.) which can also produce many other growth disorders of the hip. Fifteen children (seventeen hips) presenting similar evolution and morphological abnormalities have been reviewed. We performed nine surgical procedures most of the time for painful hips because of excentration of the femoral head. When the bone maturity was acquired all hips except two (sequelae of infections) were asymptomatic with femoral heads well covered. When discovered or suspected, this disease needs of careful follow-up. When operative treatment is necessary, we think that it has to be a pelvic surgery. We performed 4 times a triple pelvic osteotomy; 3 times a Chiari osteotomy; and twice a hip shelf arthroplasty. Those operative treatments have always been done with good results in our review.

[Atlanto-axial instability in children with trisomy 21: atlanto-axial (C1-C2) or occipito-axial (O-C2) arthrodesis?]. / Instabilité atloïdo-axoïdienne de l'enfant trisomique 21: arthrodèse atloïdo-axoïdienne (C1-C2) ou occipito-axoïdienne (O-C2)?

A retrospective study involving seven cases of operated atlantoaxial (C1-C2) instability in patients with Down Syndrome prompted the authors to raise the following question: which kind of arthrodesis is to be performed for these patients? The possibility of an occipito-atlantal (O-C1) instability, is in fact relatively frequent in this condition, as the authors have observed themselves and in a literature survey; this is an argument for performing an occipito-axial (O-C2) arthrodesis. The difficulties met to reduce the C1-C2 luxation, the frequent pseudarthroses (3 cases in our series) and a tetraplegia following a re-operation for non-union are as many other arguments to perform a O-C2 arthrodesis rather than a C1-C2 arthrodesis.