Bilateral Tibial Fibrous Dysplasia in a Pediatric Patient treated with Intramedullary Nailing.

Fibrous dysplasia is a benign developmental disorder of bone in which fibrous connective tissue containing abnormal bone with irregular trabeculae replaces normal cancellous bone. It may affect 1 (monostotic) or multiple bones (polyostotic). Polyostotic disease is the less common of the 2, occurring in only 20 to 25% of fibrous dysplasia patients and tending to affect those who are younger than 10 years of age; patients having this form tend to experience bone enlargement beyond normal skeletal maturation, which can cause pain, progressive damage, and increased risk of pathological fracture. There are limited reports of bilateral involvement in fibrous dysplasia, with said involvement presenting most frequently in early childhood. We describe the rare case of an adolescent presenting with bilateral fibrous dysplasia in the tibial diaphysis (in the exact same location) that was successfully treated with bilateral intramedullary nailing of both tibias.

Bilateral Tibia Fibrous Dysplasia in a Pediatric Patient Treated with Intramedullary Nailing: A Case Report.

Fibrous dysplasia is benign developmental disorders of bone where normal lamellar cancellous bone is replaced with immature fibro-osseus tissue resulting in poorly formed trabeculae of immature woven bone. It may affect one (monostotic) or multiple bones (polyostotic). Polyostotic disease is less common, 20-25% of patients, mostly in children below 10 years old and tends to enlarge beyond bone maturation ration which can cause pain, progressive damage and higher tendency of pathological fractures.(1) We report a case of polyostotic fibrous dysplasia present on bilateral tibia diaphysis. Patient presented with bilateral leg pain on examination and imaging showed bilateral tibia lytic lesion. Management consisted of reaming and intramedullary nailing on bilateral tibia after which patient presented without pain and radiographs showing no evidence of lesion. Tibia x-rays demonstrated exuberant cortical intramedullary bone formation with disappearance of the scalloped osteolytic lesions on the inner wall of tibial cortex. Clinically the patient's symptoms disappeared giving the opportunity to return to contact sports without limitation.

Early-onset scoliosis: a narrative review.

Early-onset scoliosis (EOS) is defined as any spinal deformity that is present before 10 years old, regardless of etiology. Deformity must be evaluated based on the intercorrelation between the lungs, spine, and thorax. Curvatures of early-onset have increased risk of progression, cardiorespiratory problems, and increased morbidity and mortality. Progression of the deformity may produce thoracic insufficiency syndrome, where a distorted thorax is unable to support normal respiratory function or lung growth. Management and treatment of EOS should pursue a holistic approach in which the psychological impact and quality of life of the patient are also taken into consideration. Growth-friendly surgical techniques have not met the initial expectations of correcting scoliotic deformity, promoting thoracic growth, and improving pulmonary function.

Is the numerical rating scale an adequate predictor for assessing MRI underlying pathologies among painful adolescent idiopathic scoliosis patients?

The use of constant pain and night pain as clinical markers for predicting the presence of underlying pathology in painful adolescent idiopathic scoliosis (AIS) patients has been questioned. Pain intensity has been recognized as an important domain in pain assessment. The numerical rating scale (NRS) is one of the most commonly validated tools to assess pain intensity in children above 8 years of age. The aim of this study was to assess the NRS as a predictor of underlying pathologies found by MRI in painful AIS patients. A cross-sectional study comprising of all AIS patients with back pain lasting >4 weeks from April 2015 to April 2019 was performed. An MRI was performed on each AIS patient presenting with back pain after a nondiagnostic history, physical examination and spinal X-ray. The pain was graded using the NRS, ranging from 0 to 10. Patients were divided into three groups: NRS (1-3), NRS (4-6) and NRS (7-10). Variables, including gender, age, scoliosis magnitude and presence of underlying pathology, were compared between the groups. A total of 186 AIS patients were evaluated in the study, were 66/186 (35.5%) of them had underlying pathologies. The mean age of subjects was 14.7 years, 85% being female. An underlying pathology was identified in 4/11 (36.4%) with NRS (1-3), 28/82 (34.2%) with NRS (4-6) and 34/93 (36.6%) with NRS (7-10) (P = 0.94). The use of an NRS was not found to be an adequate predictor for identifying an underlying pathology via MRI in painful AIS patients.

Hypomagnesemia following posterior spinal fusion in adolescent idiopathic scoliosis.

Adolescent idiopathic scoliosis (AIS) patients undergoing posterior spinal fusion (PSF) usually require surveillance in the pediatric ICU (PICU). Some reports have documented evidence of hypomagnesemia following PSF at PICU. Little has been studied about relationship between AIS and postoperative serum magnesium (Mg+2) levels. The aim of this study is to determine the prevalence and risk factors of hypomagnesemia in AIS patients admitted to PICU after PSF. IRB approved the cross-sectional study of AIS patients admitted to PICU after PSF from January 2016 to May 2017. Serum electrolyte levels of phosphorous, calcium, sodium, potassium and hemoglobin were taken preoperative and 24 h postoperative and compared with postoperative magnesium levels. Blood volume reinfused from cell saver return (CSR), estimated blood loss (EBL), volume of fluids intraoperative and average of vertebra levels operated were charted and compared with postoperative magnesium levels. A total of 46 AIS patients undergoing PSF met inclusion criteria, with an average age of 15 years (78% female). Postoperatively, hypomagnesemia was reported in 24/46 patients (53%). No association was found between hypomagnesemia and serum electrolyte (phosphorous, calcium, sodium and potassium) or hemoglobin levels. In addition, no statistical association was found with age, operative time, vertebrae level fused, CSR, EBL and volume of fluids intraoperatively. Fifty-three percent of pediatric patients developed hypomagnesemia after surgical correction for AIS. Further investigation of this electrolyte disturbance will likely result in a useful clinical tool for physician in the management of AIS.

Escoliosis desarrollo temprano. Curso de instrucción / Early onset scoliosis. Instructional course

Introducción Escoliosis de desarrollo temprano, es aquella escoliosis que se presenta antes de los 10 años de edad secundaria a anomalías estructurales congénitas de la columna, enfermedades neuromusculares, síndromes, o idiopáticamente. Materiales & Métodos Revisión literaria narrativa de todo lo publicado sobre escoliosis de desarrollo temprano durante 2003 al 2018. Resultados Esta deformidad de la columna a temprana edad limitara el crecimiento pulmonar e incapacitara la función respiratoria apropiada, provocando el síndrome de insuficiencia torácica. Históricamente, la historia natural de esta condición puede ser letal y el daño ocasionado por la escoliosis de desarrollo temprano suele ser permanente a pesar de su restauración. Actualmente, la escoliosis de desarrollo temprano se clasifica utilizando la combinación de las variables: edad, etiología, magnitud de la escoliosis y cifosis. Los métodos de tratamiento más utilizados son: enyesado en serie, implantes de distracción costal longitudinal en aleación de titanio, barras de crecimiento dobles, técnica de Shilla™, y barras de crecimiento electromagnéticas. Discusión Indudablemente, es imperativo conocer sus manifestaciones y peculiaridades para poder clasificar, monitorear la severidad, personalizar el tratamiento, detener el deterioro de la columna lo más pronto posible, y prevenir la insuficiencia respiratoria Nivel de evidencia IV

Background Early onset scoliosis presents before the age of 10 years old due to congenital structural anomalies of the thorax, neuromuscular diseases, and syndromes, or is idiopathic. Methods A review of the literature from 2003 to 2018 on the early onset scoliosis. Result A deformity of the spine at an early age limits lung development and proper respiratory function, thereby provoking the onset of a thoracic insufficiency syndrome. Historically, the natural path of this condition can be lethal, and the damage caused by the early onset scoliosis tends to be permanent, regardless of its restoration after the age of 10. The early onset scoliosis classification is currently based on the age, aetiology, and the extent of the deformity. The treatment modalities most often used are: serial casts, vertical expandable prosthetic titanium ribs, double traditional growing rods, Shilla™ growing rods, and magnetically controlled growing rods. Discussion Undoubtedly, it is imperative to know its manifestations and peculiarities in order to classify the disease, as well as to monitor the disease. Treatment should be personalised, and the deterioration of the spine halted as soon as possible, as well as to prevent respiratory insufficiency Level of evidence IV

Ewing sarcoma superimposed on a previous osteochondroma in multiple osteochondromatosis.

It has been reported that patients with hereditary multiple exostoses (called multiple osteochondromatosis by the World Health Organization) are at increased risk for malignant transformation of osteochondromas to secondary chondrosarcomas. A review of the literature found 14 cases showing transformation of osteochondromas into osteosarcomas; however, Ewing sarcoma has never been reported superimposed on an osteochondroma. This article presents the case of a boy who underwent biopsy of a previously existent osteochondroma for which the pathology report showed cytologic and immunohistochemical properties consistent with Ewing sarcoma. A 13-year-old boy with hereditary multiple exostoses (multiple osteochondromatosis) presented to an orthopedic clinic because of waxing and waning pain superficial to a previous osteochondroma on the lateral aspect of the right leg, below the knee, of 1 month's duration. On examination, inflammation was noted over a bony mass associated with tenderness to palpation of the affected area. There was no evidence of penetrating injury or trauma, and the patient reported no constitutional symptoms, including fever. Radiographs showed marked osteolysis and signs of periosteal reaction. Magnetic resonance imaging showed evidence of cortical bone erosion and extension of the mass into soft tissue. Malignant transformation was suspected, and the patient underwent biopsy. The pathology findings were consistent with Ewing sarcoma. The highly uncommon presentation of this malignancy must serve as a red flag to other physicians who treat patients with hereditary multiple exostoses. Ewing sarcoma tends to be of higher grade and have a worse prognosis than other malignancies that are more commonly seen in these patients.