Craniofacial Fibrous Dysplasia: Surgical Management and Long-Term Outcomes at a Referral Center in Mexico City.

Background Craniofacial fibrous dysplasia (CFD) is an uncommon benign condition in which a bone is replaced by fibrous tissue. An adequate clinical characterization considering the number of affected bones and functional impairment is important to determine the most effective surgical intervention for its management. This study aims to present our institution's experience in the evaluation and management of CFD. Methods This was a retrospective study that included patients with CFD managed at our institution. Data included demographic characteristics, afflicted bones, surgical procedures performed, and recurrence. Results are presented as mean and percentages. Recurrence-free years and association between the type of surgery and recurrence was evaluated. Results Eighteen patients were included (11 females, 61%). The zygomatic, maxillary, and frontal bones were the most commonly affected with eight (18%) cases each. The most common procedure was bone burring, with 36 procedures. Recurrence was more prevalent after burring (58.3%) and occurred earlier than in the bone resection group (13 vs. 15 years, p > 0.05). Conclusion Surgery continues to be the cornerstone of CFD treatment. Bone burring is effective for debulking and contouring but increases the risk for recurrence. An individualized approach should be tailored according to the anatomical location of the disease, type of CFD, behavior of the lesion, and accompanying clinical complaints.

"Sigma Volvere" acerca del vólvulo de sigmoides, diagnóstico y manejo quirúrgico, reporte de serie de casos / "Sigma Volvere" About Sigmoid Volvulus, Diagnosis and Surgical Management. Case Series Report

Resumen Presentar la experiencia en el diagnóstico clínico-radiológico y tratamiento de vólvulo sigmoideo. Se incluyeron casos con clínica sugestiva de obstrucción intestinal secundaria a vólvulo de sigmoides. Los factores de riesgo fueron: sexo masculino, mayor estancia hospitalaria, edad mayor a 70 años y la tríada clásica. Los estudios de imagen resultaron concluyentes, siendo la radiografía de abdomen y la tomografía simple de abdomen, los de elección para el abordaje diagnóstico. El vólvulo de sigmoides tiene un patrón clínico-radiológico predecible, un examen físico minucioso y estudios de gabinete de primera línea pueden ser concluyentes. La opción terapéutica para estos pacientes fue la cirugía.

Abstract To present the experience in clinical/radiological diagnosis and treatment of sigmoid volvulus. Cases with symptoms suggestive of intestinal obstruction secondary to sigmoid volvulus were included. The risk factors were: male sex, longer hospital stay, age over 70 years and the classic triad. Imaging studies were conclusive, with abdominal radiography and simple abdominal tomography being the tests of choice for the diagnostic approach. Sigmoid volvulus has a predictable clinical-radiological pattern, a thorough physical examination and first-line cabinet studies can be conclusive. The therapeutic option for these patients was surgery.

Demencia rápidamente progresiva, sobre el reporte de un caso de Creutzfeldt-Jakob / Rapidly Progressive Dementia, on a Creutzfeldt-Jakob Case Report

Resumen La enfermedad de Creutzfeldt-Jakob (ECJ) es una entidad neurodegenerativa, neuroselectiva y fatal con casi nulo reporte en México. Se presenta el caso de un paciente del sexo masculino de 40 años que inició de padecimiento con alteraciones de la memoria a corto plazo, episodios depresivos y labilidad emocional con tendencia a la irritabilidad, posteriormente se agregó desorientación espacial y disminución de fuerza del hemicuerpo izquierdo, lateropulsión en la marcha ipsilateral e insomnio, por lo cual fue ingresado al hospital por 40 días para abordaje diagnóstico. Durante su estancia hospitalaria se le realizaron diversos estudios siendo los más relevantes para el diagnóstico: resonancia magnética, la cual presentó "cintas corticales" e hiperintensidades en los núcleos de la base, ambos hallazgos altamente sugerentes de la patología, así como proteína 14-3-3 positiva, lo cual reafirmó el diagnóstico. Tras 15 meses del inicio de los síntomas neurológicos presentó un cuadro de neumonía adquirida en la comunidad, por lo cual fue admitido al hospital donde se diagnosticó absceso pulmonar y demencia rápidamente progresiva, finalmente el paciente falleció en el nosocomio por una sepsis de origen pulmonar, 18 meses después del inicio de los síntomas, no se realizó necropsia, esto de acuerdo con los estándares actuales del manejo de la enfermedad.

Abstract The Creutzfeldt-Jakob disease is a neurodegenerative, neuroselective and fatal entity, that is not usually reported in Mexico. We present a 40-year-old male patient who presents the onset of this illness, with short-term memory disorder, depressive episodes and emotional lability with a tendency to irritability. He also presents space disorientation, decreased strength of the left and lateral hemibody drive in the ipsilateral walk, and insomnia, for which he is admitted to the hospital during 40 days for diagnostic approach. Several studies were carried out during his hospital stay, the most relevant for the diagnosis: a magnetic resonance which presented "cortical ribboning" and hyperintensities in the nuclei of the base, both diagnosis highly suggested the pathology. The positive results to protein 14-3-3 reaffirmed the diagnosis. After 15 months of the onset of neurological symptoms, the patient presented symptoms of pneumonia, which lead to the hospitalization. During his stay, he presented a pulmonary abscess and rapid progressive dementia. The patient died in the hospital by a pulmonary sepsis 18 months after the onset of symptoms. No necropsy was performed, following the current standards for the disease management.

Carpometacarpal Joint Dislocations: Management and Long-Term Outcomes at a Specialized Hand Surgery Center in Latin America.

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.

Les dislocations des articulations carpométacarpiennes sont des blessures peu courantes de la main. Elles sont généralement mal diagnostiquées en raison de leurs signes cliniques non spécifiques et ont tendance à être difficiles à dépister au moyen d'une simple radiographie. Les auteurs rendent compte de leur expérience dans la prise en charge des dislocations des os carpométacarpiens dans un centre spécialisé de chirurgie de la main. Les patients atteints d'une dislocation carpométacarpienne vus à l'urgence entre 2013 et 2017 étaient inclus dans l'étude. Tous les patients ont subi une réduction fermée et un embrochage percutané (RFEP) ou une réduction ouverte et une fixation interne (ROFI). L'évaluation fonctionnelle postopératoire a eu lieu au bout de six et 18 mois au moyen du score d'incapacité du bras, de l'épaule et de la main (DASH). Onze patients ont participé : huit (72 %) ont subi une RFEP et les autres ont dû subir une ROFI (28 %). Le score DASH moyen du résultat fonctionnel s'établissait à 27,9 au seuil de six mois et à 1,5 à celui de 18 mois. Puisque les dislocations carpométacarpiennes sont rares et faciles à mal diagnostiquer, le chirurgien de la main devrait en présumer la possibilité chez les patients victimes d'un traumatisme de forte énergie, et les études d'imagerie devraient faire l'objet d'une évaluation approfondie. La RFEP est une possibilité de traitement sécuritaire et efficace qui donne de bons résultats fonctionnels à long terme.

Difficult Airway Identification and Management in Patients With Moebius Syndrome.

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 ±â€Š1.94 anesthetic events. Average Body Mass Index (BMI) was 20.07 ±â€Š6.06, 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.

Hernia interna estrangulada por plastrón en el tercio distal del apéndice cecal. Reporte de un caso / Internal Hernia Strangulated by Plastron in the Distal Third of the Cecal Appendix. Case Report

Resumen Las hernias internas son causa infrecuente de obstrucción intestinal con una incidencia del 0.2-0.9%, por lo que su diagnóstico temprano representa un reto. El órgano más frecuentemente herniado es el intestino delgado, lo que confiere un gran espectro de síntomas, desde dolor abdominal ligero hasta datos de abdomen agudo1,2. Se presenta el caso de una paciente de 8 años de edad con sintomatología digestiva inespecífica; se realizó diagnóstico transoperatorio en el que se encontró hernia interna estrangulada por plastrón en el tercio distal del apéndice. Se realizó apendicectomía y a los cuatro días se dio de alta sin complicaciones.

Abstract Internal hernias are an infrequent cause of intestinal obstruction with an incidence of 0.2-0.9%, therefore their early diagnosis represents a challenge. The most frequently herniated organ is the small bowel, which results in a wide spectrum of symptoms, varying from mild abdominal pain to acute abdomen1,2. We present the case of an eight-year old patient with nonspecific digestive symptoms, a transoperative diagnosis was made in which an internal hernia was found strangulated by plastron in the distal third of the appendix. Appendectomy was performed and four days later the patient was discharged without complications.

Goldenhar syndrome: surgical management protocol in a reference center.

OBJECTIVE: To present our experience treating 42 patients with Goldenhar syndrome. METHOD: A descriptive, observational, retrospective study was carried out using the medical and photographic record of all patients diagnosed with Goldenhar syndrome treated by the craniofacial surgery unit of the plastic and reconstructive surgery department of the Dr. Manuel Gea González hospital between 2010 and 2018. RESULTS: A total of 42 patients were obtained,54% male of which all underwent at least one procedure. The majority of patients were of the first decade of age (57%). Surgical procedures could be divided mainly into 14 auricular (20%), 17 mandibular (24%), 2 Lefort (4%), 10 volume (14%), 9 macrostoma (13%) and 16 other (21%). A total of 71 procedures were performed. CONCLUSION: Goldenhar syndrome is a rare entity that affects various structures, which is why an early diagnosis and multidisciplinary management headed by a team of plastic surgeons is necessary.

OBJETIVO: 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.

Enfermedad de Lyme en la Ciudad de México / Lyme disease in Mexico City

Resumen: Se comunica el caso de una paciente de 29 años de edad, originaria y residente de la Ciudad de México, que inició su padecimiento con dolor en el segundo dedo de la mano izquierda posterior a una visita al bosque de Aragón, que progresó con náusea y diarrea, así como artralgias incapacitantes con predominio en las manos y los pies sin eritema multiforme en la mano. A su ingreso al hospital tuvo marcadores de inflamación de 19 x 103/μL leucocitos y proteína C reactiva de 28 mg/dL, que se elevaron durante su estancia hospitalaria; ante la falta de respuesta al tratamiento médico y múltiples pruebas negativas se decidió realizar Western Blot para ricketsias con lo que se obtuvo resultado positivo para Borrelia burgdorferi, con lo que se estableció el diagnóstico definitivo de borreliosis de Lyme; la paciente recibió tratamiento adecuado y tuvo mejoría clara. La enfermedad de Lyme es una zoonosis transmitida por las garrapatas del género Ixodes causadas por la espiroqueta Borrelia burgdorferi. La Ciudad de México no se ha reportado como zona endémica por lo que comunicamos este caso.

Abstract: This paper reports the case of a 29-year-old female patient, who was originally from Mexico City and began her condition with pain in the second finger of left hand after a visit to the Aragon forest in Mexico City, progressing with nausea and diarrhea, as well as incapacitating arthralgias with predominance in hands and feet without erythema multiforme in hand. Upon admission, she had inflammation markers of 19 x 103/μL leukocytes and C-reactive protein of 28 mg/dL, which were elevated during her hospital stay; due to the lack of response to medical treatment and multiple negative tests, a Western Blot test for ricketsias was done obtaining a positive result for Borrelia burgdorferi, establishing the definitive diagnosis of Lyme borreliosis, receiving adequate treatment and presenting clear improvement. Lyme disease is a zoonosis transmitted by the ticks of the genus Ixodes caused by the spirochete Borrelia burgdorferi. Mexico City has not been reported as an endemic area, which is why we report this case.