Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 22
Filtrar
1.
Laryngoscope ; 133(5): 1099-1102, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-36106862

RESUMEN

We present in this paper a novel approach to perform endoscopic chondrolaryngoplasty without any external visible scars. The technique involves utilizing the endoscopic transoral vestibular approach. We have modified the technique by using the placement of a suture immediately above the anterior commissure, which provides a marker for the inferior extent of cartilage resection. Laryngoscope, 133:1099-1102, 2023.


Asunto(s)
Cirugía Endoscópica por Orificios Naturales , Tiroidectomía , Masculino , Humanos , Tiroidectomía/métodos , Feminización/cirugía , Cuello/cirugía , Endoscopía , Cicatriz/cirugía , Cirugía Endoscópica por Orificios Naturales/métodos
2.
Allergy Asthma Proc ; 43(2): 96-105, 2022 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-35317886

RESUMEN

Background: The coronavirus disease 2019 (COVID-19) pandemic has been associated with a dramatic increase in postviral olfactory dysfunction (PVOD) among patients who are infected. A contemporary evidence-based review of current treatment options for PVOD is both timely and relevant to improve patient care. Objective: This review seeks to impact patient care by qualitatively reviewing available evidence in support of medical and procedural treatment options for PVOD. Systematic evaluation of data quality and of the level of evidence was completed to generate current treatment recommendations. Methods: A systematic review was conducted to identify primary studies that evaluated treatment outcomes for PVOD. A number of medical literature data bases were queried from January 1998 to May 2020, with completion of subsequent reference searches of retrieved articles to identify all relevant studies. Validated tools for the assessment of bias among both interventional and observational studies were used to complete quality assessment. The summary level of evidence and associated outcomes were used to generate treatment recommendations. Results: Twenty-two publications were identified for qualitative review. Outcomes of alpha-lipoic acid, intranasal and systemic corticosteroids, minocycline, zinc sulfate, vitamin A, sodium citrate, caroverine, intranasal insulin, theophylline, and Gingko biloba are reported. In addition, outcomes of traditional Chinese acupuncture and olfactory training are reviewed. Conclusion: Several medical and procedural treatments may expedite the return of olfactory function after PVOD. Current evidence supports olfactory training as a first-line intervention. Additional study is required to define specific treatment recommendations and expected outcomes for PVOD in the setting of COVID-19.


Asunto(s)
COVID-19 , Trastornos del Olfato , COVID-19/complicaciones , COVID-19/terapia , Humanos , Trastornos del Olfato/etiología , Trastornos del Olfato/terapia , Olfato , Resultado del Tratamiento
3.
Allergy Rhinol (Providence) ; 12: 21526567211010736, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34158983

RESUMEN

OBJECTIVE: Demonstrate feasibility, safety and outcome metrics of geriatric sinus surgery (GESS). STUDY DESIGN: Retrospective review of patients undergoing sinus surgery for indication of chronic rhinosinusitis with and without nasal polyposis. SETTING: Tertiary referral center. PARTICIPANTS: Patients who underwent FESS from 2008-2017; excluding skull base, craniofacial, or oncologic surgery. Primary study group were patients aged 65 years and older. Patients aged 40-64 years of age were included for comparison.Main Outcomes and Measures: Multivariate analysis was performed to identify independently associated patient characteristics and perioperative variables. Preoperative medical and treatment history, revision and primary surgery, preoperative and post-operative SNOT-22 and NOSE scores, Lund-McKay scores were recorded when available. Post-operative data was assessed at a minimum of two months after the index procedure. Post-operative complications were included. RESULTS: Ninety-one (91) patients met criteria. 21.2% of the geriatric patients were taking systemic anticoagulation prior to surgery, and underwent treatment with nasal steroids (25.0%), oral antibiotics (67.7%), nasal irrigations (48.4%), and systemic steroids (37.5%) over an average of 7.3 months prior to surgery. There was an average post-operative reduction of 15.0 points (p < 0.0001) and 42.5 points (p = 0.0008) for SNOT-22 and NOSE scores, respectively. Average operative time was 117.4 minutes in geriatric patients compared to 183.4 minutes in younger patients (p = 0.004), with an average estimated blood loss of 55.6 milliliters (mL) compared to younger patients (111.8 mL) (p = 0.04). Linear regression identified revision surgery as associated with reductions in Sinonasal Outcome Test (SNOT-22) scores (p = 0.011). Geriatric patients had a shorter operative time (p = 0.011) while male sex was associated with a longer operative time (p = 0.014). Patients over 65 had fewer minor complications (p = 0.01), and there were no major complications in either group. CONCLUSIONS AND RELEVANCE: Geriatric sinus surgery is effective and safe in this cohort of patients.

4.
Head Neck ; 43(1): 278-287, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32996247

RESUMEN

BACKGROUND: A minimal access technique for the management of nasopharyngeal tumors extending below the palatal plane and laterally beyond the pterygoid musculature is yet to be developed. In this study we demonstrate the feasibility of endoscopic transoral nasopharyngectomy (ETON) for resection of large nasopharyngeal lesions as a natural orifice alternative to traditional approaches. METHODS: ETON was completed in three latex-injected specimens. Surgical freedom (SF) and angles of attack (AoA) were calculated along the internal carotid artery (ICA). RESULTS: An endoscopic transoral approach was successfully used to identify the parapharyngeal ICA and subsequently perform a complete nasopharyngeal resection. SF and AoA (sagittal) were found to be the greatest at the anterior genu of the ICA. CONCLUSIONS: ETON is feasible. It provides wide exposure of the skull base and proximal control of the ICA. It may be indicated for the management of nasopharyngeal tumors with inferolateral extension, involving the ICA.


Asunto(s)
Endoscopía , Procedimientos Quírurgicos Nasales , Cadáver , Humanos , Nasofaringe/cirugía , Faringectomía , Base del Cráneo
5.
Oper Neurosurg (Hagerstown) ; 19(3): 271-280, 2020 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-32472685

RESUMEN

BACKGROUND: COVID-19 poses a risk to the endoscopic skull base surgeon. Significant efforts to improving safety have been employed, including the use of personal protective equipment, preoperative COVID-19 testing, and recently the use of a modified surgical mask barrier. OBJECTIVE: To reduce the risks of pathogen transmission during endoscopic skull base surgery. METHODS: This study was exempt from Institutional Review Board approval. Our study utilizes a 3-dimensional (3D)-printed mask with an anterior aperture fitted with a surgical glove with ports designed to allow for surgical instrumentation and side ports to accommodate suction ventilation and an endotracheal tube. As an alternative, a modified laparoscopic surgery trocar served as a port for instruments, and, on the contralateral side, rubber tubing was used over the endoscrub endosheath to create an airtight seal. Surgical freedom and aerosolization were tested in both modalities. RESULTS: The ventilated mask allowed for excellent surgical maneuverability and freedom. The trocar system was effective for posterior surgical procedures, allowing access to critical paramedian structures, and afforded a superior surgical seal, but was limited in terms of visualization and maneuverability during anterior approaches. Aerosolization was reduced using both the mask and nasal trocar. CONCLUSION: The ventilated upper airway endoscopic procedure mask allows for a sealed surgical barrier during endoscopic skull base surgery and may play a critical role in advancing skull base surgery in the COVID-19 era. The nasal trocar may be a useful alternative in instances where 3D printing is not available. Additional studies are needed to validate these preliminary findings.


Asunto(s)
Betacoronavirus , Infecciones por Coronavirus/prevención & control , Máscaras/normas , Cavidad Nasal/cirugía , Neuroendoscopía/normas , Pandemias/prevención & control , Equipo de Protección Personal/normas , Neumonía Viral/prevención & control , COVID-19 , Humanos , Cavidad Nasal/diagnóstico por imagen , Neuroendoscopía/instrumentación , Impresión Tridimensional/normas , SARS-CoV-2 , Cirujanos/normas
6.
Immunol Allergy Clin North Am ; 40(2): 201-214, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32278445

RESUMEN

This literature review collates and summarizes recent literature to explore the relationship between chronic rhinosinusitis (CRS) and allergy. The relationship between CRS and allergy is not fully understood. However, current evidence suggests a relationship between allergy and specific endotypes of CRS with nasal polyposis, including allergic fungal rhinosinusitis and central compartment atopic disease. Specific endotypes of CRS with nasal polyps seem to have an association with allergy. More evidence is necessary to better characterize this relationship. Level of evidence: 5.


Asunto(s)
Pólipos Nasales/inmunología , Rinitis Alérgica/inmunología , Rinitis/inmunología , Sinusitis/inmunología , Células Th2/inmunología , Animales , Enfermedad Crónica , Citocinas/metabolismo , Humanos , Hipersensibilidad Inmediata , Pólipos Nasales/complicaciones , Rinitis/complicaciones , Rinitis Alérgica/complicaciones , Sinusitis/complicaciones
8.
Head Neck ; 40(11): 2409-2415, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-30307661

RESUMEN

BACKGROUND: There is little population-level data evaluating risk factors for postoperative complications after total laryngectomy. METHODS: We conducted a retrospective review of the American College of Surgeons National Quality Improvement Program identifying patients who underwent total laryngectomy as a primary procedure from 2005 to 2014. Multivariate analysis was performed to identify variables that were independently associated with overall and major complications. RESULTS: Eight hundred seventy-one cases met inclusion criteria. Three hundred twenty-eight patients (37.7%) had complications, with operative time (hours; P < .0001), class III (P < .001) wound status, and patient age (decade; P = .003) associated with overall complications. Two hundred one patients had major complications that were associated with steroid use (P = .01) and class III (P = .0083) wound classification. Preoperative hematocrit was correlated with a reduction of all and major complications on multivariate analysis (P < .0001 and P = .036). CONCLUSION: Identifying and optimizing risk factors may improve outcomes in total laryngectomy.


Asunto(s)
Pérdida de Sangre Quirúrgica/fisiopatología , Neoplasias Laríngeas/cirugía , Laringectomía/efectos adversos , Readmisión del Paciente/estadística & datos numéricos , Esteroides/efectos adversos , Adulto , Factores de Edad , Anciano , Bases de Datos Factuales , Femenino , Colgajos Tisulares Libres/trasplante , Humanos , Neoplasias Laríngeas/mortalidad , Neoplasias Laríngeas/patología , Laringectomía/métodos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Tempo Operativo , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/fisiopatología , Pronóstico , Procedimientos de Cirugía Plástica/métodos , Estudios Retrospectivos , Medición de Riesgo , Esteroides/uso terapéutico , Resultado del Tratamiento , Estados Unidos
9.
Ann Otol Rhinol Laryngol ; 127(4): 223-228, 2018 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-29338292

RESUMEN

OBJECTIVE: Sulcus vocalis and vocal fold scar involve derangement of the superficial lamina propria of the vocal fold, which results in significant dysphonia. Many options exist for treatment, most of which have unsatisfactory and unpredictable outcomes. Autologous transplantation of temporalis fascia into the vocal fold (ATFV) has the potential to be a better treatment option, but long-term outcomes have not been well studied. METHODS: Retrospective chart review and patient survey. Twenty-one patients diagnosed with vocal fold scar or sulcus vocalis and treated with ATFV with at least 1-year follow-up were included. Voice Handicap Index 10 (VHI-10) questionnaires were collected preoperatively and 6 months postoperatively. Patients were reached at the time of the study to complete another VHI-10 and a Likert scale survey. RESULTS: The mean decrease in VHI-10 scores between preoperation and 6 months postoperation was 8.35 ( P < .001). From preoperation to the time of the study (average 44 months; range, 12-72 months), the VHI decreased 13.53 ( P < .001). Eighty-eight percent of patients reported they would recommend this surgery to others with the same diagnosis. Only 1 minor self-limited complication occurred. CONCLUSION: Autologous transplantation of temporalis fascia into the vocal fold for the treatment of vocal fold scar and sulcus vocalis is a safe surgery with good long-term outcomes and high patient satisfaction.


Asunto(s)
Disfonía/cirugía , Fascia/trasplante , Efectos Adversos a Largo Plazo , Procedimientos Quirúrgicos Otorrinolaringológicos , Pliegues Vocales , Adulto , Cicatriz/diagnóstico , Cicatriz/fisiopatología , Cicatriz/cirugía , Disfonía/diagnóstico , Disfonía/etiología , Disfonía/fisiopatología , Femenino , Humanos , Efectos Adversos a Largo Plazo/diagnóstico , Efectos Adversos a Largo Plazo/fisiopatología , Masculino , Persona de Mediana Edad , Membrana Mucosa/patología , Procedimientos Quirúrgicos Otorrinolaringológicos/efectos adversos , Procedimientos Quirúrgicos Otorrinolaringológicos/métodos , Procedimientos Quirúrgicos Otorrinolaringológicos/psicología , Medición de Resultados Informados por el Paciente , Satisfacción del Paciente , Encuestas y Cuestionarios , Músculo Temporal/patología , Trasplante Autólogo , Pliegues Vocales/patología , Pliegues Vocales/fisiopatología , Calidad de la Voz
12.
Am J Otolaryngol ; 38(3): 337-350, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28366512

RESUMEN

OBJECTIVE: To elucidate the role of surgery in the management of anaplastic thyroid cancer. METHODS: Ovid MEDLINE, Cochrane Library, and Google Scholar databases were searched for publications from December 2000 to July 2016. Selection criterion was a focus on the management of anaplastic thyroid cancer in adults. Studies addressing only nonsurgical management and review articles were excluded. Data extraction was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Kaplan-Meier analysis was performed on a subset of patients. RESULTS: 40 publications were included in the study. Approaches to unresectability and interpretations of resection varied widely. For patients undergoing primary surgery, the median survival was 6.6months. The median survival for non-surgical patients was 2.1months. In the subgroup analysis, the median survival time for patients undergoing surgery was significantly longer in Stage IVB (p=0.022) but not IVC disease. Negative margins did not afford a statistically significant survival benefit. CONCLUSION: Surgery is a mainstay of treatment for Stage IVA and IVB disease. For Stage IVC cancer, distant metastasis was not a strict criterion against surgical candidacy among surgeons. The extent of resection and the definition of resectability remain controversial. Negative margins did not significantly increase survival.


Asunto(s)
Estadificación de Neoplasias , Carcinoma Anaplásico de Tiroides/cirugía , Neoplasias de la Tiroides/cirugía , Tiroidectomía , Humanos , Pronóstico , Carcinoma Anaplásico de Tiroides/diagnóstico , Neoplasias de la Tiroides/diagnóstico
14.
Laryngoscope ; 127(5): 1116-1118, 2017 05.
Artículo en Inglés | MEDLINE | ID: mdl-27435457

RESUMEN

OBJECTIVES/HYPOTHESIS: Many laryngopharyngeal disorders are effectively managed in the office. Herein, an in-office method utilizing the 532-nm potassium-titanyl-phosphate (KTP) laser for the treatment of benign laryngeal and vallecular mucoceles is described. STUDY DESIGN: Retrospective case review. METHODS: A review was performed of patients who had their vallecular or laryngeal mucoceles marsupialized in the office using KTP laser. RESULTS: Outcomes included long-term follow-up laryngoscopy for evidence of recurrence, significant intraprocedural complications, tolerance of the procedure, final pathology of the specimen, and resolution of symptoms. CONCLUSIONS: In-office KTP marsupialization of laryngeal and vallecular mucoceles is effective and well tolerated, with no episodes of recurrence to date. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1116-1118, 2017.


Asunto(s)
Procedimientos Quirúrgicos Ambulatorios , Enfermedades de la Laringe/cirugía , Láseres de Estado Sólido/uso terapéutico , Mucocele/cirugía , Anciano , Femenino , Humanos , Laringoscopía , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
15.
Ann Otol Rhinol Laryngol ; 126(2): 159-162, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27927778

RESUMEN

OBJECTIVE: Posterior glottal insufficiency manifests as a rough, breathy voice with poor vocal projection, air hunger, and decreased phonatory efficiency. There are limited medical and surgical therapies. This study reports the use of insetting a buccal mucosa graft into the posterior interarytenoid space to close the posterior glottic gap for phonatory rehabilitation. METHODS: Our study is a retrospective case series of 2 female patients undergoing buccal mucosa graft inset to repair acquired posterior glottic insufficiency as a result of prolonged intubation and laryngeal tuberculosis infection. Patients selected for this procedure had posterior glottal insufficiency due to erosion of posterior glottal tissue. They also had dysphonia refractory to voice therapy and injection augmentation. CONCLUSIONS: Interarytenoid buccal graft for posterior glottal insufficiency is effective with excellent vocal rehabilitation and minimal donor site morbidity.


Asunto(s)
Disfonía/cirugía , Glotis/cirugía , Enfermedades de la Laringe/cirugía , Mucosa Bucal/trasplante , Cartílago Aritenoides/patología , Disfonía/etiología , Femenino , Humanos , Enfermedades de la Laringe/complicaciones , Persona de Mediana Edad , Necrosis , Estudios Retrospectivos , Estroboscopía , Adulto Joven
16.
Laryngoscope ; 127(6): 1339-1344, 2017 06.
Artículo en Inglés | MEDLINE | ID: mdl-28000237

RESUMEN

OBJECTIVES/HYPOTHESIS: To identify relevant patient and surgical risk factors associated with prolonged length of stay, return to the operating room, and readmission within 30 days following total laryngectomy using the American College of Surgeons National Quality Improvement Program (ACS-NSQIP) STUDY DESIGN: Retrospective database study. Patients undergoing total laryngectomy alone for laryngeal cancer were identified from the ACS-NSQIP database from 2005 to 2014. METHODS: Multivariate logistic regression was used to identify independent predictors for prolonged length of stay, readmissions, and unplanned reoperations within 30 days. RESULTS: Among 871 patients meeting inclusion and exclusion criteria, the median length of stay was 8.0 days (range, 0-130 days). Totally dependent functional status (P < .01; odds ratio [OR]: 32.62), Black or African American race (P = .029; OR: 1.75), and operative time (P < .0001; OR: 1.15) were associated with prolonged length of stay. The overall rate of return to the operating room within 30 days was 12.4%. Contaminated wound status (P = .025; OR: 3.53), operative time (P = .015; OR: 1.10), steroid use (P < .01; OR: 2.92), and smoking (P = .05; OR: 1.60) were significantly associated with return to the operating room. Unplanned readmission rate was 11.9%, and 47.37% of readmissions were due to wound infection/pharyngocutaneous fistula. Dirty/contaminated wound classification (P = .05; OR: 22.5) was associated with readmission on multivariate analysis. CONCLUSIONS: This is the first population-level analysis to be performed on length of stay, readmission, and reoperation for total laryngectomy. Assessing and identifying modifiable risk factors on quality metrics may reduce overall cost and the burden on limited hospital resources. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1339-1344, 2017.


Asunto(s)
Neoplasias Laríngeas/cirugía , Laringectomía/efectos adversos , Tiempo de Internación/estadística & datos numéricos , Readmisión del Paciente/estadística & datos numéricos , Complicaciones Posoperatorias/etiología , Reoperación/estadística & datos numéricos , Anciano , Bases de Datos Factuales , Femenino , Humanos , Laringectomía/estadística & datos numéricos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Tempo Operativo , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento
17.
Arch Pathol Lab Med ; 139(11): 1389-97, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26288397

RESUMEN

CONTEXT: The incidence of oropharyngeal squamous cell carcinoma has increased during the past decade and is related primarily to the human papillomavirus. This change in etiology, from tobacco and alcohol to human papillomavirus, has resulted in improved survival for the disease. In the United States, open resection had largely been replaced by concurrent chemotherapy and/or radiotherapy by the early 2000s. The advent of transoral surgery has led to an increase in surgery as the primary treatment for both early- and advanced-stage oropharyngeal squamous cell carcinoma because it has potential advantages over open surgery and nonsurgical modalities. OBJECTIVE: To provide an overview of transoral robotic surgery for oropharyngeal squamous cell carcinoma and contrast it with other surgical and nonsurgical modalities. DATA SOURCES: Articles from 2000 to 2014 were accessioned on PubMed and reviewed for utility by the primary authors. CONCLUSIONS: Transoral surgery has become more commonly used as a minimally invasive approach to treat oropharyngeal tumors. Other strategies, including radiation, chemotherapy with radiation, and open surgery, are still important treatment approaches. The treatment options for an individual patient rely on multiple factors, including the tumor location and size, features of the tumor, and patient comorbidities. The continued study of these techniques is important to match the patient with the most appropriate treatment.


Asunto(s)
Carcinoma de Células Escamosas/cirugía , Neoplasias Orofaríngeas/cirugía , Orofaringe/cirugía , Procedimientos Quirúrgicos Robotizados/métodos , Humanos , Orofaringe/patología , Calidad de Vida , Resultado del Tratamiento
18.
Oral Maxillofac Surg ; 19(2): 109-16, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25663568

RESUMEN

OBJECTIVES: The molecular underpinnings of Moebius syndrome (MBS) are diverse. This article provides a comprehensive summation of the genetic and etiologic literature underlying this disorder. Elucidating the genetic causes of the disorder can aid in earlier detection and treatment planning. DESIGN: Articles from 1880-2013 were selected and reviewed by six researchers to understand all of the molecular theories and chronicity of advancements in the literature. RESULTS: Mutations in the MBS1, MBS2, and MBS3 gene loci all have contributed to the development of MBS through various pathways. HOX family genes coding for homeobox domains, also, have been implicated in the abnormal development of the human brain. These are among the numerous genes that have been linked to the development of MBS. CONCLUSION: Our study codified nascent findings of the molecular determinants of MBS. These findings add to a growing database of MBS-associated mutations and can be used to diagnose MBS and clarify pathogenesis.


Asunto(s)
Síndrome de Mobius/genética , Análisis Mutacional de ADN , Sitios Genéticos/genética , Proteínas de Homeodominio/genética , Síndrome de Mobius/diagnóstico
19.
J Plast Reconstr Aesthet Surg ; 68(1): 87-92, 2015 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-25455294

RESUMEN

Rhinoplasty is considered one of the most commonly performed and most challenging operations in facial plastic surgery. The modern facial plastic surgeon utilizes many instruments in performing this procedure. Many of these instruments take their names from the pioneers who invented or designed them. Although meritorious, the recent trend in medicine to move away from eponyms in lieu of descriptive names should not come at the price of forgetting history. Although this communication does not seek to provide a comprehensive history of rhinoplasty or of its pioneers, our aim is to mention several of the salient contributing figures upon whose shoulders we stand and whose eponymous instruments we use commonly in rhinoplasty.


Asunto(s)
Rinoplastia/instrumentación , Cirujanos/historia , Instrumentos Quirúrgicos/historia , Diseño de Equipo , Seguridad de Equipos , Estética , Historia del Siglo XIX , Historia del Siglo XX , Humanos , Rinoplastia/historia , Instrumentos Quirúrgicos/clasificación , Resultado del Tratamiento
20.
Oral Maxillofac Surg ; 18(4): 373-9, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25245177

RESUMEN

PURPOSE: Crouzon syndrome is a complex craniosynostosis of autosomal dominant transfer, with a highly variable phenotypic appearance. Some patients are afflicted with a mild form of the disease and able to live a fully functional lifestyle, whereas many patients suffer from a severe form of the disease causing a significant impact on their quality of life. Although several case reports and genetic studies have been performed, there has been no recent review of the literature to collate the information on this disorder. In this paper, we seek to unify the findings of this disorder to provide the pediatric provider with a succinct, but complete discussion of this disease. METHODS: Articles from 1994 to 2014 were queried on PubMed and reviewed for utility by all three researchers involved in the project. Further literature review was done on relevant articles found within references of selected articles. RESULTS: Crouzon syndrome, although of variable penetrance, is thought to be caused in part by a mutation in the fibroblast growth factor receptor-2 (FGFR2) on chromosome 10. Aside from craniofacial malformations, the disease can also cause hearing loss and airway challenges due to malformations in the nasal cavity and nasopharyngeal airway. Management options from the perspective of the otorhinolaryngologist are diverse and revolve around craniosynostectomy, offsetting midfacial hypoplasia, addressing obstructive sleep apnea and a compromised airway, psychosocial and esthetic interventions, and ultimately requiring a multidisciplinary team approach. CONCLUSION: Mutations in the FGFR2 are responsible for 50 % of mutations within this multifaceted syndrome. Crouzon syndrome is an autosomal dominant disorder with a number of distinguishing characteristics, including craniosynostosis, maxillary hypoplasia, exophthalmos, and multiple other features. Early intervention, both medically and surgically, as well as disciplined follow-up with the pediatric provider are crucial to the management of this disorder. In particular, management should address cranial suture release, midfacial advancement, evaluation for hearing deficits, and obstructive sleep apnea, with expedient intervention for airway compromise and increased intracranial pressure.


Asunto(s)
Disostosis Craneofacial/cirugía , Obstrucción de las Vías Aéreas/cirugía , Suturas Craneales/cirugía , Disostosis Craneofacial/genética , Disostosis Craneofacial/patología , Huesos Faciales/cirugía , Humanos , Grupo de Atención al Paciente , Receptor Tipo 2 de Factor de Crecimiento de Fibroblastos/genética , Procedimientos de Cirugía Plástica/métodos , Apnea Obstructiva del Sueño/cirugía
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA