Correction of knee and ankle valgus in hereditary multiple exostoses using the Ilizarov apparatus

Background Hereditary multiple exostoses (HME) is a genetic disorder that causes limb deformities due to disturbance at the growth plates. Materials and methods Six adolescents, whith symptomatic valgus deformity at the ankle and knee (seven affected legs) underwent correction procedures using the Ilizarov apparatus. In 5 legs, a bifocal Ilizarov apparatus was used, whereas in 2 legs the use of a monofocal apparatus was sufficient. Results Correction of the mechanical axis was achieved in all cases, and limb length discrepancy was equalized in the 3 cases that underwent limb elogation. The average knee and ankle corrections were 15° and 18°, respectively. The average time from application to removal of the Ilizarove apparatus was 4.6 months. No major complication occurred. Conclusions The use of the Ilizarov method in adolescents with HME enables successful simultaneous correction of multiplanar, multifocal complex limb deformities.     

Correction of ankle valgus deformity secondary to multiple hereditary osteochondral exostoses with Ilizarov

The following case report highlights basic aspects of Multiple Hereditary Osteochondral Exostoses (MHOCE) and discusses the successful treatment of an adult with ankle pain secondary to growth arrest and foreshortening of the fibula. Two salient features include the age of the patient at presentation and the success of the procedure. Symptomatic valgus deformities of the ankle secondary to MHOCE are normally corrected during adolescence, prior to physeal closure. Reducing the ankle mortise by distally displacing the fibula and correcting rotational and angular ankle deformities with Ilizarov external fixation improved this patient's ankle function and relieved his pain.     

Hemiepiphyseal stapling for ankle valgus in multiple hereditary exostoses

If left uncorrected, valgus ankle deformity in multiple hereditary exostoses can cause significant disability in skeletally immature children and in adults. Various management methods have been described, including hemiepiphyseal stapling, transphyseal screw placement, fibular-Achilles tenodesis, distal tibial osteotomy, and ablative epiphyseodesis. In this article, we report the cases of 3 skeletally immature children who had undergone hemiepiphyseal stapling of the medial distal tibial epiphysis for correction of valgus ankle deformity in multiple hereditary exostoses. Correction of the tibiotalar axis, in relation to chronological and bone age, was evaluated. Hemiepiphyseal stapling of the medial distal tibial epiphysis provides ipsilateral corrective potential while allowing staple removal for reversal of growth retardation. This procedure is useful in the management of ankle valgus in multiple hereditary exostoses.     

Incidence of brachydactyly and hand exostosis in hereditary multiple exostosis

Forty-two radiographs and charts of twenty-two patients with a diagnosis of hereditary multiple exostosis and hand involvement were examined to determine the incidence of hand exostoses and association with brachydactyly. An average of 11.6 exostoses were found per hand. The proximal phalanges and metacarpals are affected in the majority of patients and the thumb and distal phalanges are rarely involved. Most exostoses were located in the juxtaepiphyseal region (61.8%) and typically involved less than 50% of the bone diameter. Brachydactyly can be seen in patients with hereditary multiple exostosis when no exostoses is present; however, the presence of an exostosis results in even more shortening. The location and size of the exostosis had no relationship to increased bone shortening. Operative treatment was required in four of twenty-two patients for debulking and impingement.     

Development of hip dysplasia in hereditary multiple exostosis

In approximately 25% of patients with hereditary multiple exostosis, there is an abnormal osteochondral formation localized in the femoral proximal metaphysis. This formation often causes a mechanically progressive insufficiency of the acetabular cavity, a true developmental hip dysplasia, that together with a coxa valga deformity, which is also present, causes a gradual deterioration in the relations of this joint. This malformation has a poor prognosis and is difficult to manage. Although this entity is rather frequent and quite severe, it is rarely found in the medical literature. The author describes six private cases, taken from a total of 24,000 patients (0.25/1000) as examples of this entity, and provides a review of the literature.     

Risk factors for ankle valgus in children with hereditary multiple exostoses: a retrospective cross-sectional study

PurposeThe aim of this study was to identify risk factors for ankle valgus in children with hereditary multiple exostoses (HME).MethodsWe retrospectively reviewed the medical records of patients with HME who were examined at our hospital between 2010 and 2020. Patients’ age and sex were recorded along with radiographic variables including mechanical axis deviation (MAD), mechanical lateral distal tibia angle (LDTA), fibula/tibia length ratio (F/T); distal fibula station according to Malhotra’s classification, location of exostoses at the ankle joint and fibular neck/physis width (N/P) ratio, which were measured from radiographs. Binary logistic regression analysis was performed to identify significant independent risk factors for ankle valgus.ResultsThere were 61 children (20 girls and 41 boys; 122 ankles) who met the inclusion criteria. The mean age was 10.4 years (sd 3.4) and mean LDTA was 83° (sd 7°). Ankle valgus was found in 64 ankles (52%). In addition to younger age, exostoses involving the lateral aspects of the distal tibial and the medial aspect of the distal fibula (odds ratio (OR) = 4.091; 95% confidence interval (CI) 1.065 to 15.712; p = 0.040), F/T ratio < 0.96 (OR = 4.457; 95% CI 1.498 to 13.261; p = 0.007) and N/P ratio > 1.6 (OR = 2.855; 95% CI 1.031 to 7.907; p = 0.043) were associated with an increased risk of developing ankle valgus, while sex and MAD were unrelated to its occurrence.ConclusionYoung age, exostoses involving both the distal tibia and fibula, the F/T ratio < 0.96 and fibular N/P width ratio > 1.6 seemed to be risk factors of developing ankle valgus.Levels of evidencePrognostic studies, IV     

Fractured patella: operative treatment using the tension band principle

The tension band principle was applied in the operative treatment of fractures of the patella in 139 patients, of which 100 were reviewed retrospectively; 61 per cent were males and 70 per cent were aged between 50 and 70. The fractures were treated soon after admission and movement of the knee was started as soon as possible after the operation. Sixty-eight patients were reviewed and 72 per cent were happy with the resulting knee function. Objectively, 81.3 per cent had an excellent or good result. Minor troubles from the operations were common but it was concluded that the wiring of fractured patellas using the tension band principle is a safe and effective technique.     

Progressive subluxation of the hip joint in a child with hereditary multiple exostosis

We describe the treatment and follow-up of a case of hereditary multiple exostosis in a 16-year-old girl, who had intraarticular and extraarticular osteochondromas in the right hip joint which caused hip subluxation. These osteochondromas were excised, the femoral head was located concentrically, and the patient was put in a spica cast for 6 weeks. Six months later, hip magnetic resonance imaging showed no evidence of avascular necrosis. Three years after the operation the patient walked freely, without pain, and there was satisfying coverage of the femoral head. The authors emphasize that careful attention should be given to the surgical technique prior to extraarticular and intraarticular acetabular osteochondroma excision in order to avoid the development of avascular necrosis.     

The treatment of hereditary multiple exostosis of the upper extremity

Thirty of 50 patients with hereditary multiple exostosis developed significant deformities of the arm in one extremity. The degree of deformity is dependent on the location of the osteochondroma. If the osteochondroma is on the radius, deformity will usually be only minimal. If the osteochondroma is at the distal end of the ulna, the epiphysis usually stops growing. We believe that the ulnar collateral ligament then acts as a tether, very similar to that seen in the ulnar clubhand. The radius then has to either bow or dislocate at the elbow, and the wrist displaces ulnarly. We performed operations on 10 patients that consisted of cutting the ulnar collateral ligament, lengthening the ulna, osteotomizing the radius, and removing any osteochondromas. In the young, growing child, staples are placed across the lateral side of the distal radial epiphysis. The cosmetic results of the surgery were very gratifying. Nine patients also had osteochondromas removed from the hands and the forearms.     

Low energy dislocation of the hip joint in an adult with hereditary multiple exostosis

This article will describe the incidence of a low-energy hip dislocation, due to a fall from a standing height, in a 64-year-old female with hereditary multiple exostosis (HME). The dislocated left hip was reduced under spinal anesthesia, and the patient was restricted to 3 weeks of bed rest with skeletal traction of the affected limb. The post-reduction CT scan revealed a tangential fracture in the apex of the exostosis on the posteroinferior aspect of the femoral neck. The exostosis was assumed to impinge on the ischium and that the lever mechanism led to the dislocation. The 3 month follow-up MRI revealed no evidence of subluxation or avascular necrosis of the femoral head.     

遗传性多发性外生骨疣伴颈髓压迫1例

<正>患者,男,29岁,因左侧肢体无力7年,加重1个月于2016年8月26日来我院脊柱外科就诊。7年前无明显诱因出现左侧肢体无力,以下肢为重,起步及迈步时自觉下肢沉重,手部精细活动受限,同时伴有右侧肢体麻木及胸腹部束带感,无大小便障碍,就诊于当地医院后行X线检查被诊断为股骨和肱骨骨软骨瘤,曾于2007年接受左股骨和左肘截     

Management of ankle deformities in multiple hereditary osteochondromata

Nine patients with multiple hereditary osteochondromata underwent ankle surgery for valgus deformity. The indications for operation included pain from trauma of the prominent masses, pain in the ankle joint associated with the deformity, limited ankle motion, and undesirable cosmesis. The procedures included excision of osteochondromata, fibular lengthening, and medial tibial hemiepiphyseal retardation by inserting staples. Excision of osteochondromata as an isolated procedure relieved pain and improved cosmesis but did not alter the tibiofibular length discrepancy or the ankle valgus. Lengthening of the fibula and medial tibial hemiepiphyseal stapling, alone or in combination, corrected the valgus deformity of the ankle. At final follow-up (mean 43 months), no patient had pain or functional impairment.     

Cervical cord compression by hereditary multiple exostosis: case report and review of literature

Background

Hereditary multiple exostosis (HME) is an hereditary disease, characterized by the presence of multiple osteochondromas; 7% of patients with HME have a spinal disease. Through this observation, the authors discuss the diagnostic and therapeutic aspects of this rare lesion.

Case report

A 45-year-old woman, operated 10 years ago for an exostosis of the right fibula and left femur. She has since few years paresthesia of all four limbs with distal predominance, walking fatigability kind of spinal claudication. The MRI shows a voluminous osteoma at the second cervical vertebra compressing the spinal cord. The patient was operated with macroscopically complete resection of the exostosis and C2 laminectomy.

Conclusion

The cervical exostosis associated with HME is a rare disease. The clinical symptomatology is dominated by spinal cord compression and surgical treatment allows excellent clinical results.     

髌骨骨折治疗中对张力带原理的误解

克氏针张力带是治疗髌骨骨折的首选治疗方法,但张力带原理却一直被错误解读。传统张力带原理认为张力带通过功能锻炼使骨折块的分离力转化压应力。但偏心固定和关节面开裂是其最大缺点,在静息和运动时都会使骨块分离,形成没有压力的裂隙。功能锻炼并不能使骨折块的分离力转化为压应力。     

Thoracic vertebral body exostosis as a cause of myelopathy in a patient with hereditary multiple exostoses

The posterior thoracic vertebral body appears to be a novel origin for an exostosis causing myelopathy. A patient with hereditary multiple exostoses and myelopathy caused by an exostosis originating from the posterior aspect of the T5 vertebral body was treated with a staged anterior decompression/corpectomy and posterior spinal fusion. The patient had near-complete resolution of his myelopathy immediately after undergoing removal of the exostosis through a right-sided lateral thoracotomy approach. This was a unique origin for an exostosis causing spinal cord compression in a patient with hereditary multiple exostoses. The delivery of the exostosis was performed en bloc during the anterior decompression and corpectomy portion of the surgery. This resulted in the expected favorable outcome.     

Treatment of cervical cord compression, caused by hereditary multiple exostosis, with laminoplasty: a case report

STUDY DESIGN: Case report. OBJECTIVES: Successful excision of the exostosis within the spinal canal. SUMMARY OF BACKGROUND DATA: Myelopathy caused by exostosis within the spinal canal developed in a 13-year-old boy with hereditary multiple exostosis. METHODS: Spinous process-splitting laminoplasty with an ultrasonic knife was performed to remove the mass and minimize the possibility of postlaminectomy kyphosis. RESULTS: The spinal canal exostosis with cervical cord compression was excised successfully with laminoplasty. After surgery there has been no recurrence of tumor, and the stability of the cervical spine has been preserved. CONCLUSION: This is the first report of laminoplasty as a useful surgical approach for intraspinal exostosis to prevent postoperative cervical instability.