Dysplasia Epiphysealis Hemimelica (Trevor's Disease) in Children, Two New Cases: Diagnosis, Treatment, and Literature Review.

Dysplasia epiphysealis hemimelica (DEH), also known as Trevor's disease, is a rare nonhereditary skeletal disorder affecting one side of the epiphyses or the epiphyses-equivalents. It is often misdiagnosed for traumatic injuries, infections, or other tumors because of the nonspecific clinical features. The diagnosis is mostly based on radiographic involvement of one half of the epiphysis displaying an overgrowth; it is hard to distinguish between DEH and osteochondroma on the gross hystopathological exam. There are few immunohistochemical markers, as well as genetic tests, for EXT1 and EXT2 gene expression that can reveal a more accurate diagnosis. No evidence of malignant changes has been reported and no hereditary transmission or environmental factor has been incriminated as an etiological factor. The natural history of the disease is continuous growth of the lesions until skeletal maturity. Without treatment, the joint might suffer degenerative modification, and the patient can develop early onset osteoarthritis. In the present paper, we report two new cases of DEH of the ankle. The aim of this paper is to consider Trevor's disease when encountering tumoral masses in the epiphyses of pediatric patients and to present our treatment approach and results.

Negative pressure wound therapy in reconstructing extensive leg and foot soft tissue loss in a child: a case study.

Open fractures of the leg with large loss of tissue require extensive reconstructive methods that can injure the donor area. The use of negative pressure wound therapy (NPWT) may minimise the impact of these reconstructive methods because of its capacity to create granulation tissue that will form a wound bed for the skin graft, thus reducing the volume of soft tissue defect and saving the donor region. This case study describes the effectiveness of NPWT in the treatment and reconstruction of an open fracture of the leg, with massive loss of soft tissue, associated with elastic intramedullary nailing in a 10-year-old female patient, who was a victim of a car accident. Clinical examination revealed a Gustilo-Anderson IIIB open fracture of the left leg, with the avulsion of the fifth toe, disarticulation of the fifth metatarsal bone, extensively damaged skin and subcutaneous tissue in the medium and distal third of the left leg and left foot. The bone was exposed in the distal part of the leg, external malleolus and left calcaneus. Profuse lavage, reduction of the tibial fracture and elastic intramedullary nailing, amputation of the fifth left toe, necrectomy and debridement of devitalised tissue were performed. NPWT was started, with the dressing changed every five days. After 55 days of using NPWT, granulation tissue covered the soft tissue defect and created a wound bed for the skin graft. NPWT helped the management of this open wound, achieving a wound bed for the skin graft, avoiding the use of complex reconstructive methods.

Treatment of the aneurysmal bone cyst by percutaneous intracystic sclerotherapy using ethanol ninety five percent in children.

INTRODUCTION: Aneurysmal bone cyst (ABC) is a benign intraosseous lesion filled with blood that can determine a blowout distension of the bone. The purpose of this study is to evaluate the efficacy of sclerotherapy by percutaneous intralesional administration of ethanol 96% for the treatment of this pathology in paediatric patients. METHOD: The retrospective study includes 17 paediatric patients with ABC who were treated by repeated intracystic injection with ethanol 96%, 1 ml/kg, in our clinic between December 2015 and July 2017. Fluoroscopic guidance was used to inject the cyst with contrast agent. The mean follow-up period was 11 months. RESULTS AND DISCUSSION: All cysts are healed or are in the healing process. The mean age was 11 years old. Seven patients needed three repeated injections and ten patients needed two injections until healing. We observed a mean reduction in the size of the lesions, measured on plain X-rays, of 68%. The complications that were observed included the following: dizziness after injection, skin pigmentation at the injection site, local inflammatory reaction, and pain after injection. The current study approves the importance of this minimally invasive treatment with no recurrence after a follow-up of 19 months. The healing rate was 100%. A limitation of this study consists in the small number of patients. CONCLUSION: Sclerotherapy with ethanol 96% is a useful method for the treatment of ABC. It is a minimally invasive method, with no major complications, which lowers the risks of open surgical intervention and has a good rate of success.

Aggressive development of an aneurysmal bone cyst of the proximal femur in a paediatric patient: a case report.

We report development of an aneurysmal bone cyst (ABC) that was located in the proximal region of the femur in an 11-year-old girl. Over a period of 30 weeks, the ABC showed fulminant local progression, with destruction of the bone, which led to an abrupt loss of function of the left hip. The standard tumour treatment protocol was followed. We performed embolisation of the tumour followed by a biopsy, which confirmed the diagnosis of ABC. The outcome was negative with total destruction of the proximal third of the femur, despite repeating the embolisation. Because of the unfavourable local progression, a second biopsy was performed and we reconfirmed the initial diagnosis. The final decision regarding the therapeutic approach was total hip arthroplasty with femoral reconstruction with a prosthesis. Following this treatment, the patient's outcome was favourable, with complete recovery of function and no local relapse.

Outcome of pinning in patients with slipped capital femoral epiphysis: risk factors associated with avascular necrosis, chondrolysis, and femoral impingement.

Objective This study aimed to assess the principal risk factors that could lead to the most common long-term complications of slipped capital femoral epiphysis, such as avascular necrosis, chondrolysis, and hip impingement. Methods We conducted a single-centre, retrospective study and evaluated patients (70 patients, 81 hips) who were treated for slipped capital femoral epiphysis from 2010 to 2015 and who underwent pinning. We measured the severity of displacement radiologically using the Southwick angle. Postoperative radiographs were evaluated for the most frequent long-term complications of avascular necrosis (AVN), chondrolysis, and femoral acetabular impingement (FAI). Results We found seven cases of AVN, 14 cases of chondrolysis, and 31 hips had an α angle of 60°. Sex, ambulation, and symptoms did not affect development of these complications. Patients with a normal weight were almost two times more likely to develop FAI. Patients with moderate and severe slips had a similar percentage of AVN. In severe slips, 85.7% of patients had an α angle higher than 60°. Conclusions This study shows that severe slips have a higher risk of developing AVN and hip impingement. Every patient who suffers from SCFE (even the mildest forms) should be regularly checked for FAI.

Surgical Treatment of a Rare "Reverse" Madelung Deformity in 11 Years Female Patient.

Madelung deformity is an abnormality of the distal part of the forearm due to a growth arrest in the distal radial physis creating an increase of the radial tilt angle associated with a dorsal subluxation of the distal ulna in most cases. It is a rare condition which represents only 1.7% of hand deformities being characterized by the presence of an abnormal structure, Vickers ligament, that tethers the distal radius to the lunate bone. Although it is believed to be a congenital disorder, the symptoms are absent till late childhood. We present a case of a 11 years old girl patient, who came to our clinic for deformity of both forearms, which consisted of an anteriorly curved radius, volar proeminence of the distal ulna, partial limitation of supination and pain in the last 6 months, with and insidious onsed and aggravated lately. The mother of the patient, at the age of 13, was diagnosed with the same deformity which was surgically treated at that time. Furthermore, the patient has an older sister with no deformity of the forearms. X-rays revealed an increased radial tilt and anterior luxation of the distal ulna. Considering the deformity and the presence of pain we decided to excise the Vickers ligament and make an opening and derotation wedge osteotomy of the distal radius.

Total Hip Replacement in Congenital Hip Dislocation in 14 Years Female Patient.

INTRODUCTION: development dysplasia of the hip is one of the common disorders in pediatric orthopaedics. This requires an early orthopaedic treatment. Neglected cases can lead to hip dislocation, which has an incidence of 1.4 / mille new-borns, being bilateral in 70-80% of cases and prevalence of girls being 7 / 1-4 / 1 (girls / boys). Congenital hip dislocation requires a complex surgical and orthopaedic treatment. In most cases develop hip osteoarthritis in adulthood, requiring total hip replacement. In certain selected cases total hip replacement is required since the pediatric age. METHODS: We present the case of a 14 year old patient who was admitted to our clinic for limping with leg-length discrepancy, pain in the right groin. The disease has an insidious onset at the start of the walking age. The treatment was denied until the age of 9, when was performed open reduction of the hip, shortening osteotomy of the femur, right hip adductor tenotomy muscles. Despite treatment the disease has an poor outcome, patient was presenting at age of 14 a shortening of the right leg 7 cm, with the greater trochanter elevated, positive Trendelenburg sign, limiting of daily activities, Harris score 48. We performed transkeletal traction for one month, after that performing total hip replacement. The postoperative evolution was excellent, the patient having no pain, with a 2 cm leg-length discrepancy, starting to walk at 3 month after surgery. CONCLUSIONS: Although total hip replacement in congenital hip dislocation at pediatric age is an exceptional procedure, in some cases carefully selected it proves to be a viable alternative in the medium term.