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.

Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review / Enfermedad venooclusiva fatal y artritis idiopática juvenil sistémica: caso clínico y revisión de la literatura

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.(AU)

La artritis idiopática juvenil sistémica (AIJs) es una enfermedad juvenil crónica que representa aproximadamente del 5 al 15% de todos los casos de AIJ y tiene una elevada tasa de morbimortalidad. En esta enfermedad, las complicaciones pulmonares (CP) distintas a pleuritis son mucho menos frecuentes, y no fácilmente reconocibles por los clínicos. La hipertensión pulmonar, la CP más grave, está asociada a la enfermedad incontrolada y el uso de terapias biológicas. Presentamos el caso de una mujer en edad escolar con AIJs que falleció debido a inestabilidad cardiopulmonar aguda secundaria a enfermedad venosooclusiva confirmada en la necropsia. Describimos su evolución clínica, y también realizamos una revisión narrativa de la literatura relativa a CP en AIJs, para debatir los avances más recientes sobre esta complicación. La elevada actividad de la enfermedad y el uso de terapias múltiples que incluyen fármacos antirreumáticos modificadores de la enfermedad deberían servir de signo de alarma a los clínicos para descartar CP e hipertensión pulmonar. La combinación de placas de tórax, electrocardiograma y ecocardiograma parece ser el mejor conjunto de pruebas para lograr un diagnóstico precoz.(AU)

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.

Hematuria asintomática como manifestación del tumor de Wilms botrioide / Asymptomatic haematuria as a sign of a botyroid Wilms tumour

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Fibroadenoma gigante de mama en adolescentes: procedimientos diagnóstico-terapéuticos / Giant breast fibroadenomas in adolescents: Diagnostic and therapeutic procedures

No disponible

Hiperplasia estromal seudoangiomatosa mamaria bilateral / Bilateral pseudoangiomatous stromal hyperplasia of the breast

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Dos casos de quiste de intestino anterior en cavidad oral / Two cases of duplication foregut cyst in oral cavity

Antecedentes y Objetivo. El quiste de intestino anterior es una entidad congénita poco frecuente. Cuando aparece, su localización más frecuente es el íleon y, aunque pueden aparecer a lo largo de todo el tracto digestivo, es rara su ubicación en la cavidad oral. A menudo se caracteriza por tres hallazgos principales: presencia de una capa de músculo liso bien desarrollada, una capa epitelial típica de alguna porción del tracto alimentario, y la unión íntima a alguna porción del tracto gastrointestinal. Las lesiones quísticas del suelo de boca en la edad infantil más frecuentes son los quistes salivares y las malformaciones linfáticas, ambas englobables en el término ránula. Sin embargo hay otras posibilidades, y la duplicación de intestino anterior, como aparece en los casos que presentamos, es una de ellas. Material y Método. Presentamos 2 pacientes en edad pediátrica con lesión lingual o de suelo de boca, que tras el estudio de imagen adecuado fueron operados por vía intraoral para la extirpación de la tumoración. En uno de los casos se usó abordaje cervical para extirpación de un segundo locus quístico. Resultados. El estudio anatomopatológico mostró en uno de los casos epitelio respiratorio, y en el segundo mucosa gástrica y epitelio respiratorio. Conclusiones. La importancia del diagnóstico y la extirpación completa en este tipo de lesiones viene dada por el riesgo de degeneración maligna en la edad adulta (AU)

Background and Objective. The foregut cyst is a rare congenital disease. However when it appears its location is, most of the times, in the ileum and although it can be present throughout entire digestive tract, it is rare in the oral cavity. Usually, it is characterized by three major features: presence of a well developed smooth muscle layer, a typical epithelial layer of some portion of the alimentary tract, and an intimate join to any part of the gastrointestinal tract. The most common mouth floor cysts in childhood are salivary cysts and lymphatic malformations. Both of them designed within the term ranula. Although there are other possibilities, and foregut duplication is one of them, as we present in our clinical cases. Methods. We present 2 patients in pediatric age with tongue or mouth floor masses, who after respective radiological study, were operated by transoral approach to remove the tumor. In one of the cases transcervical approach was used to remove a second cyst locus. Results. The pathological study showed respiratory epithelium in one case, and gastric mucosa and respiratory epithelium in the second case. Conclusions. The importance of diagnosis and complete surgical removal in this clinical condition is supported by the risk of malignant degeneration in adult age (AU)

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.