[Arthrogryposis: clinical manifestations and management]. / L'arthrogrypose : manifestations cliniques et prise en charge.

INTRODUCTION: Arthrogryposis is a condition existing at birth, seen in different diseases that have in common the existence of multiple joint stiffness. The clinical presentation is diversified and the functional prognosis depends on the etiology, which makes therapeutic options different from one case to another. The objective of this study was to describe clinical manifestations observed in arthrogrypotic patients and show the different therapeutic methods and functional results. METHODS: This was a retrospective study over a period of 12 years from 2000 to 2012, based on clinical examinations of 23 patients with arthrogryposis. RESULTS: The mean age of the patients was 6.6 years. The four limbs were involved in 19 cases and only the lower limbs in four cases. The etiology was amyoplasia in nine patients, distal arthrogryposis in three, peripheral neuropathy in two, and muscular atrophy in one infantile patient. Foot deformities were observed in 22 cases, with 25 talipes equinovarus deformities (clubfoot), six convex feet, and five valgus feet. The Ponseti method was applied in 12 clubfeet with recurrence in eight cases. The other clubfeet had functional treatment and necessitated surgical release in 11 cases. Deformation of the knee was seen in 15 patients, flessum in 16 knees, restriction of flexion in eight cases, genu recurvatum in three, stiffness in extension in two, and agenesis of the patella in one case. Surgical treatment was carried out in only one case. Other cases of deformity of the knee were treated by rehabilitation with slight improvement. Deformation of the hip was seen in 15 patients, with 16 hips dislocated and four hips in flessum. The dislocation of the hip was treated orthopaedically in two cases and surgically in six cases, with reduction in all cases but persistence of stiffness. Involvement of the shoulders was observed in eight cases, the elbow in seven cases, the wrist in ten cases, and the fingers in 16 cases. The treatment was based on physical therapy and orthotics in all cases, and improvement was better in the wrist and fingers. CONCLUSION: Arthrogryposis has different clinical presentations. Successful treatment is not constant. Multidisciplinary care is necessary and should be early and continued in order to gain the maximum autonomy and facilitate patients' social integration.

[MR imaging features of tuberculosis of the sellar region]. / Aspects IRM de la tuberculose de la région sellaire.

PURPOSE: Tuberculosis of the sellar region, especially the pituitary gland, is rare. The purpose of this article is to demonstrate through a review of five clinical cases the value of imaging, especially MR imaging, in the evaluation of this pathology. PATIENTS AND METHODS: CT and MRI of the brain were obtained in all cases along with a chest radiograph. RESULTS: Four patterns were detected on MRI: pituitary tuberculoma mimicking adenoma; pituitary abscess, extending to the cavernous sinus in one case and associated with infundibulum thickening in another; hypophysitis with suprasellar extension in association with tuberculous meningoencephalitis; and infundibular thickening associated with tuberculous meningoencephalitis. Diagnosis was based on biopsy in two cases and combination of imaging and clinical data in three cases. Outcome was favorable with anti-tuberculosis drugs. CONCLUSION: Irrespective of the imaging features, a history of travel to an endemic region combined to other findings such as infundibular thickening should raise concern for the possibility of tuberculosis even in the absence of signs of systemic infection.

[Cleidocranial dysplasia: report of 2 cases and literature review]. / La dysplasie cleidocrânienne : rapport de 2 cas et revue de la littérature.

Cleidocranial dysplasia or dysostosis involves dental anomalies, bone abnormalities with membranous ossification (clavicles, cranium, face, pelvis), rarely of the spine and the remainder of the skeleton. We report 2 new cases and describe the different clinical aspects of this disorder and the orthopedic problems that it can pose. The clinical demonstrations of this disease are highly variable and inconsistent, which explains the diversity of circumstances of discovery. Abnormalities of the face and clavicles, as well as of pelvic ossification are most frequent and can be regarded as major signs. These clinical demonstrations do not require treatment in the majority of the cases. Dental anomalies, coxa vara and scoliosis require regular monitoring and treatment in the event of progressive aggravation. The incomplete penetrance of this autosomal dominant disease and its good tolerance explain the frequency of undiagnosed forms, whose clinical expression is discrete.

Hamatum osteoblastoma.

We report the case of an osteoblastoma of the hamate bone that was successfully treated by curettage. This tumor is very rare in a carpal bone and only nine cases have been reported in the literature. Pathological examination is mandatory before treatment due to the lack of distinctive clinical and radiological features. Osteoblastomas are benign, but potentially aggressive bone tumors. Treatment of the lesion may either be a conservative "intralesional resection" or radical "wide en bloc resection". The latter option, which has non-negligible functional consequences in the wrist, should be reserved for recurrence after curettage but may also be considered a primary immediate alternative for aggressive forms.

Results of surgical treatment of congenital convex pes valgus (ten nonidiopathic feet).

PURPOSE OF THE STUDY: Congenital pes valgus is a rare and complex deformity of the foot raising serious diagnostic and therapeutic challenges. The purpose of our work was to present the surgical procedures used in our series and to analyze outcome. MATERIAL AND METHODS: Ten feet presenting congenital convex pes valgus treated surgically over a six-year period using the same operative technique were reviewed at minimum five years follow-up. Idiopathic deformities were excluded from this series. Deformities were secondary to arthrogryposis in five feet, multiple malformative syndrome in four, and diastematomyelia in one. The surgical technique used two approaches: a posteromedial incision to release the dorsal flexors, disinsert the tibialis posterior, open the talonavicular joint, release the Achilles tendon and release the posterior tibiotalar capsule; a lateral incision to lengthen the fibular tendons and perform an osteotomy of the anterior process of the calcaneum. A talonavicular pin and a calcaneocuboid pin maintained the correction. The tibialis posterior tendon was reinserted on the anterior aspect of the talonavicular capsule after incision of the dislocation chamber. RESULTS: Outcome was considered good in five cases and fair in five. Outcome was fair in the arthrogryposis feet. Under correction was observed in two feet and valgus flatfoot in three. Talar necrosis occurred in one foot and navicular necrosis in two. DISCUSSION: Simultaneous correction of the different anomalies observed in the congenital convex foot was achieved in this series. The anatomic and functional results were satisfactory. We recommend avoiding overly extensive release in order to decrease the risk of talar and navicular necrosis. It is also important to check the reduction radiographically during the operation. Patients should use an orthesis for several months postoperatively to avoid recurrence.

[Treatment of bone loss in limbs by bone transport]. / Traitement des pertes de substance osseuse des membres par transport osseux segmentaire.

INTRODUCTION: Ilizarov's segmental bone transport technique is one of the treatments of bone defects. The aim of our investigation was to identify which technical specific features could ensure the success of this surgical option and to analyze the various difficulties and complications inherent in this method. MATERIAL AND METHODS: Our analysis covered a series of nine patients - five males and four females with a mean age of 20 years old - all treated by bone transport between 1996 and 2003. Bone loss was secondary to a traumatism in five cases, to the excision of a tibial congenital pseudoarthrosis in three cases and to an osteomyelitis in one case. Bone defect was reported to be located in the tibial distal metaphysis in seven cases, in the tibial diaphysis in one case and in the distal femoral methaphysis in another case. In most cases, bone transport was performed as revision surgery after previous unsuccessful surgical attempts. The mean length of the bone defect was 4.1cm (range: 2 to 8 cm). A shortening of the limb - ranging 1 to 6 cm - was associated with bone loss in six cases. The average follow-up period was 28 months (range 10 to 96 months). RESULTS: The reported difficulties and technical incidents encountered with this method included one incomplete corticotomy, one distraction which was performed in the wrong direction, and one case of cutaneous invagination and axial deviation of the lower limb. At the end of the distraction phase, union was achieved in all cases. At the end of transport, six patients required supplemental bone grafting of the docking site to improve healing. The average size of bone formation was 4.5 cm (range: 3 to 8 cm). The mean distraction index was 14 days per centimeter. The mean time between external fixation and healing was nine months for eight patients. The mean external fixation index was 57 days per centimeter. No infection at the non-union and distraction site was reported. An axial deviation was observed in four cases. Bony reconstruction was excellent in three cases, good in four cases and fair in two cases. All patients could return to normal activity at the end of the treatment. Functional outcomes were excellent in one case and good in eight cases. DISCUSSION: Ilizarov's segmental bone transport technique is a reliable option for the treatment of bone defects that other surgical procedures failed to manage. However, the different technical difficulties and complications inherent in this method require the need for a meticulous planning, adapted to each surgical case.

[Results of surgical treatment of congenital convex pes valgus (10 non-idiopathic feet)]. / Résultats du traitement chirurgical du pied convexe congénital.

PURPOSE OF THE STUDY: Congenital pes valgus is a rare and complex deformity of the foot raising serious diagnostic and therapeutic challenges. The purpose of our work was to present the surgical procedures used in our series and to analyze outcome. MATERIAL AND METHODS: Ten feet presenting congenital convex valgus treated surgically over a six-year period using the same operative technique were reviewed at minimum five years follow-up. Idiopathic deformities were excluded from this series. Deformities were secondary to arthrogryposis in five feet, a multiple malformative syndrome in four and diastematomyelia in one. The surgical technique used two approaches: a posteromedial incision to release the dorsal flexors, disinsert the tibialis posterior, open the talonavicular joint, release the Achilles tendon and release the posterior tibiotalar capsule; a lateral incision to lengthen the fibular tendons and perform an osteotomy of the anterior process of the calcaneum. A talonavicular pin and a calcaneocuboid pin maintained the correction. The tibialis posterior tendon was reinserted on the anterior aspect of the talonavicular capsule after incision of the dislocation chamber. RESULTS: Outcome was considered good in five cases and fair in five. Outcome was fair in the arthrogyrposis feet. Undercorrection was observed in two feet and valgus flatfoot in three. Talar necrosis occurred in one foot and navicular necrosis in two. DISCUSSION: Simultaneous correction of the different anomalies observed in the congenital convex foot was achieved in this series. The anatomic and functional results were satisfactory. We recommend avoiding overly extensive release in order to decrease the risk of talar and navicular necrosis. It is also important to check the reduction radiographically during the operation. Patients should use an orthesis several months postoperatively to avoid recurrence.

[Traumatic hip dislocations in children]. / Les luxations traumatiques de la hanche chez l'enfant.

PURPOSE OF THE STUDY: Traumatic hip dislocation is a rare event in children. Appropriate management remains a subject of debate. The purpose of this study was to investigate the epidemiological, therapeutic features of this situation and the long-term outcome after treatment. MATERIAL AND METHODS: This was a retrospective analysis of 15 traumatic hip dislocations collected over a period of 20 years in pediatric patients with at least two years follow-up. We searched for predisposing factors and factors affecting prognosis. RESULTS: The series included 11 boys and three girls, mean age eight years. Dislocation was posterior in 13 hips and anterior in two. Time to reduction was less than 3h in eight cases, 3-6h in five and greater than 6h in two. After reduction, traction was performed in nine children, for 20 days on average, followed in five cases by immobilization for 40 days on average. Five hips were immobilized directly after reduction. We identified two groups by age: group 1 with dislocations in children aged less than six years (seven children) were characterized by low-energy trauma. Dislocation was not associated with other lesions. Predisposing factors (overt ligament hyperlaxity, insufficient superolateral head cover, coax valga) were noted in six children. Reduction was simple. Later treatment consisted in immobilization with a pelvispedious cast for 30-45 days. Group 2 were dislocations in children aged over six years (seven children) victims of high-energy trauma. Associated injuries were frequent. Predisposing factors were not present. At mean 11 years follow-up, all hips are considered normal clinically. The radiograph was normal for 14 hips. In one case, there was a slight coax magna. In three patients, defective femoral head cover persisted. Coxa valga persisted in two patients. DISCUSSION: Traumatic dislocation of the hip joint is rare in very young children, but results from a minimally traumatic event. This suggests the presence of predisposing factors in this category of patients, particularly capsuloligamentary hyperlaxity. After reduction, immobilization can be recommended. Traumatic hip dislocations in children are different from the adult variety due to their rarity, the general absence of associated fractures, easy reduction and better prognosis. The epidemiological and therapeutic features in children older than six years are however similar to those in adults.

[Supracondylar fractures of the humerus in children]. / Les lésions nerveuses dans les fractures supra-condyliennes de l'humérus chez l'enfant.

PURPOSE OF THE STUDY: The most frequent type of elbow fracture in children is by far the supracondylar fracture. This type of fracture also raises the greatest risk of nerve injury. We wanted to study the clinical and prognostic features of nerve injuries in children presen-ting supracondylar fractures of the humerus. MATERIAL AND METHODS: We analyzed the files of 55 children with nerve lesions identified among 1 180 files on supracondylar fractures in children. Most presented Lagrange and Ribault stage IV fractures. Nerve injuries involved the radial nerve (n=28), medial nerve (n=20), and ulnar nerve (n=7). The nerve injury was diagnosed before treatment in 32 children, and after treatment in 23. When nerve injury was identified before treatment, closed reduction had been used for eleven fractures and open reduction for 21. Nerve injury identified after treatment was found in eleven fractures after closed reduction and in 12 fractures after open reduction. RESULTS: When nerve injury was recognized at the initial examination, spontaneous nerve recovery was achieved in all cases, irrespective of the treatment modality, within a maximum of four months. When nerve injury was recognized after treatment, spontaneous nerve recovery was obtained in twenty cases. Time to recovery was longer. The three other cases required nerve exploration with neurolysis for two and a nerve graft for one. DISCUSSION: Nerve injury discovered after treatment is either caused by or aggravated by the treatment. Prognosis is less favorable than for injuries discovered at the initial examination. This highlights the importance of carefully searching for nerve deficit, even partial deficiency, in all children presenting a supracondylar fracture of the humerus. It also emphasizes the importance of care in obtaining bone reduction if the initial examination did not reveal any nerve deficit.

[Osteoid osteoma of the olecranon]. / Ostéome ostéoïde de l'olécrane.

We report a case of osteoid osteoma of the olecranon which developed in the greater sigmoid cavity. The patient was a 22-year-old male who complained of arthritic-like symptoms limited to the elbow joint and progressing for three years. Rheumatoid arthritis then tuberculosis were entertained as possible diagnoses. The diagnosis of osteoid osteoma was not established until the disease had progressed for three years and had already become stiff due to epiphyseal remodeling. Resection of the nidus only alleviate pain and joint motion was not improved.

[Septic dislocation of the hip secondary to BCG vaccination]. / Luxation septique de la hanche secondaire à la vaccination par le BCG.

We report a case of septic dislocation of the hip in an eight-month-old infant secondary to BCG vaccination. The usual treatment of septic arthritis with surgical drainage and broad spectrum antibiotics was unsuccessful. Cure was achieved after institution of an anti-tuberculosis treatment and a second surgical drainage. This rare complication of BCG vaccination can develop several months after administration of the vaccine. Diagnosis is often difficult to establish due to the minimal clinical and non-specific clinical expression. Early radiological signs are also non-specific. Identification of the causal agent can be most difficult. Certain diagnosis is generally achieved after biopsy and pathology examination. Despite the attenuated virulence of the vaccine, anti-tuberculosis treatment is indispensable to achieve cure. Surgery drainage alone is insufficient.

[Extra-abdominal desmoid tumors: 7-year outcome after surgical treatment in 12 patients]. / Tumeurs desmoïdes extra-abdominales: résultats du traitement chirurgical. Douze cas avec un recul moyen de 7 ans.

PURPOSE OF THE STUDY: The purpose of this study was to report the clinical course and outcome in 12 patients with extra-abdominal desmoid tumors. MATERIAL AND METHODS: This retrospective series included 12 patients with extra-abdominal desmoid tumors treated between 1987 and 1996. Female gender predominated (8/12) and age ranged from 4 months to 60 years. The tumors were identified in the thoracic wall (n=2), the upper limb (n=4), and the lower limb (n=6) with a bony localization in the upper femoral metaphysis. Magnetic resonance imaging was highly contributive to the search for extension. Surgical treatment was given in all cases. All patients were living at last follow-up. RESULTS: Complete resection was achieved in three patients and incomplete resection in nine. Mean follow-up was seven years. There was one case of sarcomatous transformation and nine cases of recurrence. Mean time to recurrence was one year (range 6 months-4 years). Two tumors, in the forearm and the leg, did not recur. Secondary surgery was not attempted in four patients whose clinical situation has remained stable after recurrence. A second resection was performed for recurrence in four other patients who then remained recurrence-free to last follow-up. One tumor recurred three times after surgical resection. Functional outcome was good in nine patients. DISCUSSION: The poorly limited nature of extra-abdominal desmoid tumors makes resection a difficult task, particularly when located in the girdles or the most proximal part of the limbs. Wide first-intention surgical resection is recommended but mutilation must be avoided due to the benign nature of the tumor, its slow progression, and the possibility of regression or stability after recurrence. Nevertheless, surgical treatment of recurrent tumors is difficult and often insufficient, compromising functional outcome. Complementary treatment (radiotherapy) has been advocated for better control tumor growth.

[Multifocal bony hemangioendothelioma: a case report]. / Hémangioendothéliome osseux multicentrique.

Hemangioendothelioma is a rare tumor (0.5% of primary malignant bone tumors) generally found in long bones and tarsal bones. We report a case in a 67-year-old man who underwent surgery in 1987 for a tumor of the left ankle. He again consulted in 1998 for pain in the left knee. Standard x-rays of the left lower limb evidenced multiple partitioned bone defects with cortical lysis in the femoral condyle, the upper and lower portions of the tibia and the talus. Computed tomography showed a lytic image of the lower femoral and superior tibial epiphysis without cortical or soft tissue involvement. Laboratory findings were normal. Pathology examination of a biopsy specimen led to the diagnosis of grade I multifocal bony hemangioendothelioma of the medial femoral condyle and the medial upper tibial facet of the left knee. Search for extension was negative. Curettage followed by cement filling of all tumor sites and postoperative radiotherapy were successful. No recurrence has been observed 3 years later. The histological polymorphism of hemangioendotheliomas leads to varying therapeutic management schemes.

[Mediterranean lymphoma mimicking Crohn's disease]. / Lymphome méditerranéen simulant une maladie de Crohn.

We report an uncommon localization of mediterranean lymphoma of the terminal ileum in a 28 year-old male patient. Ultrasound and Computed Tomography showed moderate regular and symmetrical intestinal wall thickening simulating Crohn's disease. We highlight the role of computed tomography in the diagnosis, staging and detection of complications.

[Splenic nodules and sickle cell anemia]. / Nodules spléniques et drépanocytose.

OBJECTIVE: We report 4 patients with sickle cell anemia presenting with intra-splenic benign nodules corresponding to islands of preserved tissue within splenic ferro-calcinosis. MATERIAL AND METHODS: Ultrasound, CT and MRI findings were evaluated and compared to a follow-up study by ultrasound and CT done after 6 to 12 months. RESULTS: Ultrasound showed multiple well-defined rounded nodules appearing hypoechoic compared to the rest of the spleen that was hyperechoic. On CT, the nodules were homogenous, hypodense relative to the spleen, isodense to the liver in 3 cases and hypodense to the liver in 1 case. On MRI, the nodules appeared relatively hyperintense within low-signal-intensity spleens. The ultrasound and CT follow-up study demonstrated no remarkable change. CONCLUSION: In sickle cell patients, intra-splenic benign nodules corresponding to normal splenic tissue may be identified on imaging studies. The differential diagnosis is discussed.