Hexapod Frame Correction of Proximal Tibial Recurvatum Caused Indirectly by a Femoral Aneurysmal Bone Cyst in an Adolescent: A Case Report and Systematic Review.

CASE: A 16-year-old boy presented with a recurrent distal femur aneurysmal bone cyst accompanied by a combined sagittal knee deformity (20° of femoral antecurvatum and 26.8° of tibial recurvatum) and limb shortening. After preoperative planning, the treatment involved new intralesional curettage, phenolization, and bone allograft filling. Additional procedures included distal extension femoral osteotomy with plate fixation, and proximal tibial osteotomy with, gradually corrected through a hexapod frame. At 2-year follow-up, lower limbs exhibited normoalignment and equal length. CONCLUSION: Complex knee deformities may occur with tumoral lesions around the knee but can be effectively addressed through double osteotomy and application of a hexapod frame.

Intraosseous injection of mesenchymal stem cells for the treatment of osteonecrosis of the immature femoral head and prevention of head deformity: A study in a pig model.

BACKGROUND: Cell therapy has been proposed as part of the therapeutic arsenal to assist bone formation and remodeling in the early stages of osteonecrosis of the femoral head. The purpose of this study is to determine the effects of intraosseous inoculation of mesenchymal stem cells on bone formation and remodeling in an established experimental model of osteonecrosis of the femoral head in immature pigs. METHODS: Thirty-one 4-week-old immature Yorkshire pigs were used. Experimental osteonecrosis of the femoral head was created in the right hip of all included animals (n = 31). The month after surgery, hip and pelvis radiographs were taken to confirm osteonecrosis of the femoral head. Four animals were excluded following surgery. Two groups were established: (A) mesenchymal stem cell-treated group (n = 13) and (B) saline-treated group (n = 14). One month after surgery the mesenchymal stem cell-group received an intraosseous injection of 10 × 106 mesenchymal stem cell (5 cc) and the saline-treated group of 5 cc of physiological saline solution. Osteonecrosis of the femoral head progression was assessed by monthly X-rays (1-, 2-, 3- and 4-months post-surgery). The animals were sacrificed 1 or 3 months following the intraosseous injection. Repair tissue and osteonecrosis of the femoral head were histologically evaluated immediately after sacrifice. RESULTS: At time of sacrifice, radiographic images showed evident osteonecrosis of the femoral head with associated severe femoral head deformity in 11 of the 14 animals (78%) in the saline group and in only 2 of the 13 animals (15%) in the mesenchymal stem cell group. Histologically, the mesenchymal stem cell group showed less osteonecrosis of the femoral head and less flattening. In the saline group, there was pronounced femoral head flattening and the damaged epiphyseal trabecular bone was largely replaced with fibrovascular tissue. CONCLUSION: Intraosseous mesenchymal stem cells inoculation improved bone healing and remodeling in our immature pig osteonecrosis of the femoral head model. This work supports further investigation to determine whether mesenchymal stem cells enhance the healing process in immature osteonecrosis of the femoral head.

Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review.

Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood inflammatory disease. SJIA accounts for approximately 5-15 per cent of all cases of JIA and has a high morbidity and mortality rate. In this disease, pulmonary complications (PC) other than pleuritis are much less frequent and not easily recognised by clinicians. Pulmonary hypertension, the most severe PC, is associated with uncontrolled disease and use of biologic therapies. We present a case of a school-age female with sJIA who died of acute cardiopulmonary instability secondary to pulmonary venous-occlusive disease demonstrated by necropsy. We describe her clinical evolution. We also undertook a narrative review of the literature about PC in sJIA to discuss the current state of the art regarding this complication. High disease activity and the use of multiple therapies include disease-modifying anti-rheumatic drugs should be a red flag for clinicians when discounting PC and pulmonary hypertension. The combination of chest X-ray, electrocardiogram and echocardiogram appear to be the best tests to achieve an early diagnosis.

Bizarre Parosteal Osteochondromatous Proliferation (Nora Lesion) in Pediatric Phalanges.

Bizarre parosteal osteochondromatous proliferation, or Nora tumor, is an uncommon lesion affecting the tubular bones of the hands and feet. Normally arising from the cortical surface and periosteum of these bones, these lesions histologically consist of a hypercellular cartilaginous cap covering a bony stalk that is surrounded by ossified areas and spindle cell stroma. The differential diagnosis includes conditions involving the periosteum such as chondrosarcoma, parosteal osteosarcoma, osteochondroma, turret exostosis, and florid reactive periostitis. The only effective treatment is wide surgical excision; nevertheless, local recurrence rates are extremely high and may necessitate revision surgery. In the present study, we report 3 cases of Nora lesion located in the hand in pediatric patients. The diagnosis in these cases was challenging owing to their presenting symptoms and radiographic findings. The diagnosis was made based on characteristic findings noted on the radiographic images and was confirmed by histological examination following excision.

Composite sarcoma of bone with focal rhabdomyosarcoma and lymph node metastasis in an adolescent.

Composite sarcoma of bone is a very rare entity that primarily affects adolescent and young adult patients. It usually combines areas of liposarcoma and osteosarcoma, and up to 60% of cases have metastatic disease at diagnosis. It is a highly aggressive pathology with intrinsic resistance to bone sarcoma conventional treatments. The prognosis is poor, with long-term survival rates not exceeding 30%. We present the case of an adolescent female diagnosed with an aggressive composite sarcoma of bone with rhabdomyosarcoma foci and loco-regional lymph node involvement.

Gorham-Stout disease and cerebrospinal fluid otorrhea.

OBJECTIVE AND IMPORTANCE: Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician. CLINICAL PRESENTATION: We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage. INTERVENTION: Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased. CONCLUSION: Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.