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.

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.

[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.

Children ankle valgus deformity treatment using a transphyseal medial malleolar screw.

INTRODUCTION: Ankle valgus deformity is rare in children. It generally leads to difficulties wearing shoes, walking instability and mechanical pain. No medical treatment is effective and the only option is surgical correction of the deformity. Two main options are available: supramalleolar osteotomy and medial malleolar epiphysiodesis. We report our experience with epiphysiodesis using a transphyseal medial malleolar screw. PATIENTS AND METHODS: This is a retrospective study of all children followed for ankle valgus and treated by transphyseal medial malleolar screw epiphysiodesis in our department. The study included 10 cases of ankle valgus deformity in seven children (four with multiple extostoses, two type 1 neurofibromatosis, one Larsen's syndrome) who completed skeletal maturity. At surgery, median bone age was 12 years (10 to 13 years and 6 months) and the median tibiotalar angle was 17.5° (10° to 30°). RESULTS: At skeletal maturity, preoperative valgus was corrected in six patients (9/10 ankles). The median tibiotalar angle was 5° (0° to 25°). Valgus was not corrected in one patient (30° to 25°). No postoperative complications occurred. DISCUSSION: Epiphysiodesis by transphyseal medial malleolar screw is a simple, efficient and safe procedure to correct a significant or symptomatic ankle valgus deformity in children before skeletal maturity. LEVEL OF EVIDENCE: Level IV, retrospective study.

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.

[Posterior spinal fusion for kypho-scoliosis associated with osteogenesis imperfecta: long-term results]. / Résultats des arthrodèses vertébrales postérieures dans les scolioses secondaires à une ostéogenèse imparfaite.

PURPOSE OF THE STUDY: A survey was conducted at the Necker-Enfants-Malades Hospital in Paris and the Roscoff Helio-Marin Center in Roscoff, France to document results of posterior spinal fusion for scoliosis associated with osteogenesis imperfecta. MATERIAL AND METHODS: The cases of 27 patients who underwent posterior spinal fusion and Harrington (n = 2) or CD (n = 23) instrumentation performed by one senior orthopedic surgeon were reviewed. Two patients had posterior arthrodesis without instrumentation and were left in halo-traction together with a plaster cast until bone healing was achieved. RESULTS: The series included eleven males and sixteen females. Mean age at fusion was 13 y 4 m. The average curvature before surgery was 77 degrees and the average correction was 37 degrees. Mean follow-up was six years. Minor complications due to halo pin infection or migration were observed in two patients and loss of correction with rod fracture in four. DISCUSSION: Because of the risk of physical and respiratory incapacity, we believe that spinal surgery and arthrodesis for spinal deformity should be considered after a preparation period by halo-traction with exceptional precautions to correct major curves and prevent complications that could occur in patients with osteogenesis imperfecta. Correction and stabilization of the scoliotic spine in osteogenesis imperecta has yet to be definitively described since we have noticed that the site of deformation is transferred to the sacroiliac region once spinal fusion is achieved.

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.

Operative treatment of severe scoliosis in osteogenesis imperfecta: results of 20 patients after halo traction and posterior spondylodesis with instrumentation.

Correction and stabilisation of the scoliotic spine in osteogenesis imperfecta is difficult. The optimal technique has yet to be determined, since no large series in which a single procedure has been carried out by a single surgeon using a single protocol has yet been described. The charts of 20 patients with osteogenesis imperfecta who had undergone halo gravity traction (HGT) and a posterior spondylodesis with Cotrel-Dubousset (n = 18) or Harrington (n = 2) instrumentation were reviewed. No correction was made at the time of the surgical spondylodesis. The average follow-up was 4.8 years (range 2-10.5 years). The preoperative traction improved the Cobb angle of the scoliosis by 32% (from a mean of 78.5 degrees to a mean of 53.3 degrees) and improved the kyphosis by 24% (from a mean of 56.0 degrees to mean of 42.5 degrees). This correction deteriorated slightly at final follow-up, for both the scoliosis and the kyphosis (mean 57.6 degrees and 44.4 degrees respectively). Few complications were encountered during the HGT period. In 16 cases no complications occurred during the follow-up period. Ambulation and functional ability were upgraded for 7 of 20 patients.

[Essential bone cysts in children. Value of systematic cystography. Apropos of a series of 42 cysts]. / Kystes osseux essentiels de l'enfant: intérêt de la kystographie systématique. A propos d'une série de 42 kystes.

INTRODUCTION: The treatment of children's essential bone cysts, is controversial. Intra focal injection of a corticoid, the Methylprednisolone, described by Scaglietti in 1974, given in most of the case serials, a rate of healing of more than 30 per cent. MATERIAL AND METHODS: The case serial we present include 42 essential bone cysts treated between 1975 and 1992 in the orthopaedic department of Necker Enfants Malades hospital. These children have been reviewed with a mean follow up of 4 years. A healing rate over 35 per cent has been noticed. However, some failures stayed completely an understanding even if nothing at the beginning let suppose a slower evolution. RESULTS: Attempting to explain those phenomenes, the authors realised in 70 per cent cases, an opaque cystography, before the Methylprednisolone injection. This simple radiological technique permitted to reveal abnormal aspect in 75 per cent cases. Most of the time, it shows massive-veinous licks in an abnormal veinous system or one or plurial separations of the cystic area. This type of picture could perfectly explain the defect of the corticoids action by a lick of the solution or by a partial unefficacity of the solution because of the separation in the cyst area. The hypothetic idea has been completed by the calcul of the duration of the evolution. Effectively, the cysts showing an abnormal cystography had a longer healing delay compared to the cyst whose cystography was normal. CONCLUSION: The opaque cystography is for us a necessary element in the treatment of essential bones cysts, once the diagnostic is certain and the indication of intra focal corticoid injection has been retained. The radiographic study of the cyst area permits to precise the treatment; for example multiplying the injections in the areas of the cysts when there is separations and overseing the evolution.

[Foot deformities in longitudinal ectromelia of the lower limbs]. / Les anomalies du pied dans les ectromélies longitudinales des membres inférieurs.

We have studied 204 feet in 181 children with congenital limb shortening. There were 17 short femurs in 11 children in whom it was impossible to lengthen the leg. Thirteen feet were abnormal. It was necessary to adapt the foot to fit the chosen prosthesis. In 34 children it was possible to correct the length of the short femur. In 21 the feet were normal. 13 had abnormal feet associated with fibular aplasia and deformities included equinovalgus, hind foot synostosis, and deficient rays. In 72 children with congenital hypoplasia or aplasia of the fibula, 76 feet were affected. The function of the foot depends on attaining a stable position beneath the tibia. In 34 children (35 feet), the feet were in a stable position. There were 22 ball and socket ankle joints. Before carrying out leg lengthening, operation was necessary on 9 feet, and a further 13 required operation after lengthening had been carried out. In 34 children (41 feet) there was fibular aplasia. The feet lay in equinovalgus and were often narrow. Only 5 did not have a degree of synostosis. Twenty-three feet in twenty two children were operated on with early soft tissue release, tendon transfer and tibiotalar arthrodesis. This is our treatment of choice. Twenty-seven children had tibial dysplasia which affected 37 feet. The feet lay in equinovarus and in 5 there was partial diplopodia. In the absence of a tibia (19 feet) disarticulation of the knee was carried out. In 12 there was proximal shortening of the tibia which in 9 cases was treated by tibiofibular union. It was possible to retain only 2 feet in a proper position in a lengthened leg. In 30 children (33 feet) both fibular and tibial hypoplasia was present. The feet functioned well. There were 22 ball and socket ankles, 28 narrow feet and numerous synostoses. In limbs with atypical dysplasia 7 abnormal feet were noted. If it is possible to preserve or lengthen the limb it is vital that the foot is retained in a stable position beneath the tibia in order that satisfactory function be obtained. If limb lengthening is impossible, the foot must be adapted to match the prosthesis.

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.

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.

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.

[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.

[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.

[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.

[Long-term outcome of pectus excavatum surgically treated in children]. / Le devenir à long terme du thorax en entonnoir opéré chez l'enfant.

The authors have studied the result of 34 pectus excavatum, corrected by two surgical methods, followed up between 3 and 20 years: half of cases were treated by Judet's procedure, making turn over of anterior wall chest, with 35 per cent of good result. The other cases were treated by sternochondroplasty and internal fixation, with 88 per cent of good result. Early failures related to sternal non union and to infection, occurred in 53 per cent of Judet's procedure; eight of the 13 failures were managed by sternochondroplasty (7 cases) and Judet's procedure (1 case); followed by good results. Failure at further time was related to the young age of the patients at treatment, to the deficiency of fixation and early removal of material. Consequently, durable correction at long term was obtained, when treatment was performed by sternochondroplasty procedure, over 14 years of age, when many pins were used for fixing the correction, left in place for more than one year. In such cases, we didn't observe regression of result. When small residual deformities subsisted they were often masked by the development of the muscles in man, and breast in woman, providing acceptable aspect of chest.

[Epidural chordoma without bone involvement. A propos of a case in a child. Review of the literature]. / Les chordomes épiduraux sans atteinte osseuse. A propos d'une observation pédiatrique. Revue de la littérature.

The authors report the case of a chordoma developed exclusively in the epidural space at the L1-L2 level in a 6 year old child. The tumor was easily removed in toto. No post-operative radiotherapy was given. The diagnosis of chordoma was based on the histological characteristics of the lesion and the results of immunohistological studies which were both typical of this lesion. This observation is exceptional because of the young age of the patient, the lumbar level of the tumor and, even more, the absence of any bone involvement. The 8 previously published cases of chordoma developed along the craniospinal axis but without bone involvement are reviewed and analysed: all were subdural; 7 were intracranial (6 prepontic, 1 suprasellar): only one was intraspinal. These lesions raise the problem of their diagnosis, of their treatment and of their embryogenesis. They could develop from ectopic notochordal remnants: the "ecchondrosis physaliphura".