Background: Airway management in patients with Moebius syndrome can be difficult due to the presence of orofacial malformations. This paper aims to present our institution's experience in the evaluation and management of the upper airway in patients with Moebius syndrome Methodology: The authors performed a retrospective study including every patient with Moebius syndrome submitted to surgery between 2012 and 2017. Difficult airway was defined as one requiring more than 2 attempts to achieve endotracheal tube placement, a Mallampati score equal or above III, a Cormack score equal or above III, or need of a fiberscope during intubation. Results: Fifty-one patients were included, (39.3% males) requiring 172 procedures. Each patient required an average of 3.37 ± 1.94 anesthetic events. Average Body Mass Index (BMI) was 20.07 ± 6.06, 45.1% were overweight or obese. Four patients (7%) were considered as having a potentially difficult airway. Endotracheal intubation was achieved in all patients; 38 patients were intubated in a single attempt, while the rest were successfully intubated on a second try. Fiberscope was not necessary. Univariate analysis showed that overweight/obese patients were more likely to be considered as having a difficult airway (P = 0.03). Conclusion: Intubation can be difficult in patients with Moebius syndrome, but failure is rare. Overweight or obese patients are at risk of presenting a difficult airway. An extensive preoperative evaluation and adequate communication between the members of the multidisciplinary team in charge of these patients is paramount.
Carpometacarpal joint dislocations are uncommon hand injuries. These dislocations are usually misdiagnosed due to their non-specific clinical signs and tend to be difficult to identify in simple X-rays. We report our experience in the management of carpometacarpal bone dislocations at a specialized hand surgery center. Patients with carpometacarpal dislocations seen at the emergency department between 2013 and 2017 were included. All patients were treated with either closed reduction and percutaneous pinning (CRPP) or open reduction and internal fixation (ORIF). Postoperative functional assessment was performed at 6 and 18 months using the Disabilities of the Arm, Shoulder, and Hand (DASH) score. Eleven patients were included, 8 (72%) were managed with CRPP and the rest required ORIF (28%). For the functional outcome, we found an average DASH score of 27.9 at the 6 months threshold and of 1.5 at the 18 months threshold. Carpometacarpal dislocations are uncommon and easy to misdiagnose; the hand surgeon should have a high clinical suspicion in patients who sustained high-energy trauma, and imaging studies should be thoroughly evaluated. Closed reduction and percutaneous pinning is a safe and effective treatment option, with long-term good functional results.
ResumenObjetivo: Presentar nuestra experiencia en el diagnóstico y el tratamiento de 42 pacientes con síndrome de Goldenhar. Método: Se realizó un estudio descriptivo, observacional y retrospectivo usando el registro médico y fotográfico de todos los pacientes diagnosticados con síndrome de Goldenhar tratados por la unidad de cirugía craneofacial del departamento de cirugía plástica y reconstructiva del hospital Dr. Manuel Gea González entre 2010 y 2018. Resultados: Se obtuvieron 42 pacientes, el 54% varones, con predominio de menores de 10 años (57%), de los cuales todos se sometieron al menos a un procedimiento. Los procedimientos quirúrgicos se dividieron en: 14 auriculares (20%), 17 mandibulares (24%), 2 Lefort (4%), 10 volumen (14%), 9 macrostoma (13%) y 16 otros (21%). En total se realizaron 71 procedimientos. Conclusión: El síndrome de Goldenhar es una enfermedad poco frecuente que afecta diversas estructuras y se presenta predominantemente en varones. Es necesario un diagnóstico precoz y un manejo individualizado llevado a cabo por un equipo multidisciplinario encabezado por cirujanos plásticos.
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