Fracture in Humeroradial Synostosis: Description of Two Clinical Cases.

Synostosis is a generic term to indicate the union of two originally separated bones. At the elbow, humeroradial or longitudinal synostosis causes significant disability, which varies depending on hand function, elbow positioning, adjacent joints mobility and contralateral limb function. It is estimated that, to date, a little more than 150 patients have been described with this deformity, which is more common in subjects with deficient ulnar formation or affected by conditions such as Antley-Bixler and Hermann syndromes. The lack of the elbow joint, with the formation of a longer bone due to humerus-radius fusion, results in stiffness. As such, it is assumed that fractures in this topography are not uncommon. However, since synostosis is rare, this lesion was only described twice in the literature. We report two patients with a fracture of the single bone formed by a humeroradial synostosis and Bayne type-IV ulnar formation failure. Both patients were treated surgically with success. We emphasize the need for adequate treatment to not compromise the daily activities of patients who are adapted to their deformity, thus avoiding worsening the function of a previously affected limb.

Fracture in Humeroradial Synostosis: Description of Two Clinical Cases / Fratura em sinostose rádio-umeral: Descrição de dois casos clínicos*

Abstract Synostosis is a generic term to indicate the union of two originally separated bones. At the elbow, humeroradial or longitudinal synostosis causes significant disability, which varies depending on hand function, elbow positioning, adjacent joints mobility and contralateral limb function. It is estimated that, to date, a little more than 150 patients have been described with this deformity, which is more common in subjects with deficient ulnar formation or affected by conditions such as Antley-Bixler and Hermann syndromes. The lack of the elbow joint, with the formation of a longer bone due to humerus-radius fusion, results in stiffness. As such, it is assumed that fractures in this topography are not uncommon. However, since synostosis is rare, this lesion was only described twice in the literature. We report two patients with a fracture of the single bone formed by a humeroradial synostosis and Bayne type-IV ulnar formation failure. Both patients were treated surgically with success. We emphasize the need for adequate treatment to not compromise the daily activities of patients who are adapted to their deformity, thus avoiding worsening the function of a previously affected limb.

Resumo Sinostose é um termo genérico utilizado para indicar a união de dois ossos originalmente separados. No cotovelo, a sinostose rádio-umeral ou longitudinal causa importante incapacidade, que varia a depender da função da mão, da posição do cotovelo, da mobilidade das articulações adjacentes e da função do membro contralateral. Estima-se que um pouco mais de 150 pacientes foram descritos até hoje com essa deformidade, sendo mais frequente em portadores de deficiência de formação ulnar ou podendo fazer parte de síndromes como de Antley-Bixler e de Hermann. Devidoàrigidezcausadapelaausênciadaarticulaçãodocotovelo,queresultana formação de um osso mais longo com a fusão do úmero no rádio, presume-se que fratura nessa topografia não seja incomum. No entanto, pela raridade dessa patologia, tal lesão apresenta apenas duas descrições prévias na literatura. Relatamos os casos de dois pacientes com fratura do osso único formado pela sinostose entre o úmero e o rádio portadores de falha de formação ulnar do tipo IV de Bayne. Ambos os pacientes foram tratados de forma cirúrgica e evoluíram bem. Salientamos a necessidade do tratamento adequado para não comprometer as atividades da vida de um paciente já adaptado à deformidade, evitando piorar a função de um membro já alterado.

Association of postoperative furosemide use with a reduced blood transfusion rate in sagittal craniosynostosis surgery.

OBJECT A major challenge in sagittal craniosynostosis surgery is the high transfusion rate (50%-100%) related to blood loss in small pediatric patients. Several approaches have been proposed to prevent packed red blood cell (PRBC) transfusion, including endoscopic surgery, erythropoietin ortranexamic acid administration, and preoperative hemodilution. The authors hypothesized that a significant proportion of postoperative anemia observed in pediatric patients is actually dilutional. Consequently, since 2005, at CHU Sainte-Justine, furosemide has been administered to correct the volemic status and prevent PRBC transfusion. The purpose of this study was to evaluate the impact of postoperative furosemide administration on PRBC transfusion rates. METHODS This was a retrospective study of 96 consecutive patients with sagittal synostosis who underwent surgery at CHU Sainte-Justine between January 2000 and May 2012. The mean age at surgery was 4.9 ± 1.5 months (range 2.8-8.7 months). Patients who had surgery before 2005 constituted the control group. Those who had surgery in 2005 or 2006 were considered part of an implementation phase because furosemide administration was not routine. Patients who had surgery after 2006 were part of the experimental (or furosemide) group. Transfusion rates among the 3 groups were compared. The impact of furosemide administration on transfusion requirement was also measured while accounting for other variables of interest in a multiple logistic regression model. RESULTS The total transfusion rate was significantly reduced in the furosemide group compared with the control group (31.3% vs 62.5%, respectively; p = 0.009), mirroring the decrease in the postoperative transfusion rate between the groups (18.3% vs 50.0%, respectively; p = 0.003). The postoperative transfusion threshold remained similar throughout the study (mean hemoglobin 56.0 g/dl vs 60.9 g/dl for control and furosemide groups, respectively; p = 0.085). The proportion of nontransfused patients with recorded hemoglobin below 70 g/dl did not differ between the control and furosemide groups (41.7% vs 28.6%, respectively; p = 0.489). Surgical procedure, preoperative hemoglobin level, estimated blood loss, and furosemide administration significantly affected the risk of receiving a postoperative PRBC transfusion. When these variables were analyzed in a multiple logistic regression model, furosemide administration remained strongly associated with a reduced risk of being exposed to a blood transfusion (OR 0.196, p = 0.005). There were no complications related to furosemide administration. CONCLUSIONS A significant part of the postoperative anemia observed in patients who underwent sagittal craniosynostosis surgery was due to hypervolemic hemodilution. Correction of the volemic status with furosemide administration significantly reduces postoperative PRBC transfusion requirements in these patients.