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1.
Laryngoscope Investig Otolaryngol ; 6(2): 325-331, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33869765

RESUMEN

OBJECTIVES: Insertion of tympanostomy tubes (TT) is generally accomplished in children in the operating room under general anesthesia. We report on 229 children treated in-office with a novel device. METHODS: Investigators participated in an IRB-approved, prospective, single arm, multisite investigation of in-office TT placement in awake children. Topical anesthetic was applied, and protective restraint was used. TT placement was performed with a single-pass TT insertion device. Safety was assessed by monitoring procedural events. RESULTS: Four hundred and forty-four ears were treated in 229 children at 10 sites. Children were in age groups 6-24 months (n = 211, mean = 13 months) and 5-12 years (n = 18, mean = 8.3 years). Two hundred and fifteen children received bilateral TT placement, and 14 received unilateral placement. Overall, 226/229 (98.7%) children had successful TT placement in the office (209/211 in 6-24 months and 17/18 in 5-12 years). Three children were rescheduled for the operating room due to anatomical challenges or patient movement. Median procedure time for bilateral cases in both age groups was 4:53. Two minor adverse events (AEs) were reported in one patient. Per independent assessment of 30 procedure videos by clinicians, TT placement was tolerated acceptably by all children. CONCLUSION: In-office TT placement in awake young children using topical anesthetic, enabled by a single pass delivery device, was safe, successful and well tolerated. The American Academy of Otolaryngology (AAO) recently released a Position Statement supporting in-office TT placement in appropriate children. These results affirm an in-office alternative for clinicians and parents who have concerns with the risk, inconvenience and cost of surgery in an operating room under general anesthesia.Level of Evidence: 2c.Clinical Trials Registration Number: NCT03544138.

2.
Otolaryngol Head Neck Surg ; 157(3): 533-535, 2017 09.
Artículo en Inglés | MEDLINE | ID: mdl-28585495

RESUMEN

Insertion of tympanostomy tubes is a common elective pediatric surgical procedure and is typically performed under general anesthesia. The potential to reduce general anesthetic requirements for young children has led to increased interest in alternatives for tympanostomy tube placement. A tympanostomy tube system, developed to enable tympanostomy tube placement in a single pass on conscious patients under moderate sedation, was evaluated. A prospective study on 128 children and 253 tympanostomy tube placements conducted at 4 centers in the United States demonstrated an 88.3% success rate in performing the procedure under moderate sedation with adverse events within normal rates reported in the literature. The feasibility of completing tympanostomy tube placement under moderate sedation enables avoidance of general anesthesia and provides additional choices to physicians and parents.


Asunto(s)
Sedación Consciente , Ventilación del Oído Medio/instrumentación , Ventilación del Oído Medio/métodos , Adolescente , Niño , Preescolar , Diseño de Equipo , Humanos , Lactante , Estudios Prospectivos
3.
Otolaryngol Head Neck Surg ; 148(2): 249-52, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23151833

RESUMEN

OBJECTIVE: To analyze the incidence of postparotidectomy facial nerve dysfunction in pediatric patients and to evaluate the association between patient demographics or underlying pathology and functional outcomes. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care pediatric hospital. SUBJECTS AND METHODS: We reviewed charts of patients younger than 18 years who underwent parotidectomy performed by a pediatric otolaryngologist between 1999 and 2011 at a tertiary pediatric center. Distributions of postoperative facial nerve dysfunction and timing of recovery were noted. Chi-square and t-test analyses were conducted to determine the association between demographics or pathology and functional outcomes. RESULTS: Forty-three patients met inclusion criteria: 41 underwent superficial parotidectomy, and 2 underwent total parotidectomy. Leading indications for surgery were atypical mycobacterial infection (16/43 [37.2%]) and first branchial cleft anomaly (8/43 [18.6]). The facial nerve was sacrificed in 1 (2%) patient with Ewing's sarcoma involving the nerve. The incidence of immediate facial nerve paresis was 21% (9/43), involving the marginal mandibular nerve (n = 7), buccal branch (n = 1), and both marginal mandibular and frontal branches (n = 1). Full recovery of nerve function in patients with paresis occurred within 1 month (n = 2), 2 months (n = 1), 6 months (n = 3), or 10 months (n = 2). CONCLUSION: Postparotidectomy paresis of distal branches of the facial nerve is a common occurrence in the pediatric population and should be discussed during preoperative evaluation. The risk is comparable to that of the adult population. Age, gender, and pathologic diagnosis were not predictive of postoperative nerve dysfunction.


Asunto(s)
Traumatismos del Nervio Facial/etiología , Traumatismos del Nervio Facial/fisiopatología , Parálisis Facial/etiología , Parálisis Facial/fisiopatología , Enfermedades de las Parótidas/cirugía , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/fisiopatología , Adolescente , Distribución de Chi-Cuadrado , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino
4.
Ann Otol Rhinol Laryngol ; 121(7): 478-84, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22844868

RESUMEN

OBJECTIVES: We examined surgical outcomes in children with cervicofacial nontuberculous mycobacterial lymphadenitis and attempted to identify predictors of complications. METHODS: A retrospective chart review from 2 tertiary pediatric centers was used to identify .11 presentation or operative variables (age at surgery, gender, symptom duration, pain, violaceous skin changes, skin breakdown, fluctuance, purified protein derivative positivity, operative procedure, use of nerve integrity monitoring, and use of skin flap advancement) and to compare these to 5 postoperative complications (facial nerve dysfunction [paresis or paralysis], poor scarring, recurrence, wound infection, and wound dehiscence without infection). RESULTS: The 45 patients analyzed for presentation or operative variables (28 female, 17 male; average age, 31.2 months) typically presented with painless masses averaging 8.2 weeks in duration, along with violaceous skin changes in 29 of the 45 cases (64%) and skin breakdown in 9 cases (20%). The surgical procedures included parotidectomy with or without selective lymphadenectomy in 38 of the 45 cases (84%) and lymphadenectomy alone in 7 cases (16%). Skin resection and cervicofacial advancement flap reconstruction was performed in 20 cases (44%). Nerve integrity monitoring was utilized in 32 cases (71%). In the 44 patients analyzed for postoperative complications, we found facial nerve paresis in 14 (31.8%), poor scarring in 9 (20.5%), wound infection in 6 (13.6%), recurrence in 4 (9.1%), and facial nerve paralysis in 2 (4.5%). Nine of the 14 cases (64.3%) of initial facial nerve paresis resolved. At final follow-up, facial nerve paresis persisted in 5 of the 14 children (35.7%) with initial postoperative paresis and in 1 of the 2 children (50.0%) with initial postoperative paralysis. Facial nerve paralysis persisted in the other child with initial postoperative paralysis. Overall, 6 of these 7 patients (85.7%) with persistent facial nerve dysfunction had follow-up of less than 1 month. All transient and permanent facial nerve dysfunction was in the distribution of the marginal mandibular nerve only. No statistically significant predictors of complications were identified. CONCLUSIONS: We report acceptable but not insignificant rates of marginal mandibular distribution facial nerve injury, poor scarring, wound infection, and recurrence following resection of cervicofacial nontuberculous mycobacterial lymphadenitis in children that must be discussed with patients and parents before operation. No presentation or operative variables predicted the complications.


Asunto(s)
Linfadenitis/microbiología , Linfadenitis/cirugía , Infecciones por Mycobacterium no Tuberculosas/cirugía , Complicaciones Posoperatorias , Preescolar , Cicatriz/etiología , Procedimientos Quirúrgicos Dermatologicos , Parálisis Facial/etiología , Femenino , Humanos , Escisión del Ganglio Linfático , Masculino , Glándula Parótida/cirugía , Recurrencia , Estudios Retrospectivos , Factores Sexuales , Colgajos Quirúrgicos
5.
Otolaryngol Head Neck Surg ; 147(3): 557-62, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22517014

RESUMEN

OBJECTIVE: Functional endoscopic sinus surgery (FESS) provides symptomatic relief of sinus disease in patients with cystic fibrosis (CF), but it is unclear whether it has beneficial effects on lung disease in this population. This study assessed the effect of FESS on the respiratory status of adult patients with CF. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary medical center. SUBJECTS: Thirty-two adult patients with CF who underwent 45 operative cases. METHODS: Clinical information retrieved for the 12-month periods preceding and following to determine the effect of FESS on the rate of decline in lung function, as well as intravenous antibiotic use and hospitalization for pulmonary exacerbation. RESULTS: The rate of decline in forced expiratory volume in 1 second and forced vital capacity was not significantly different in the 12 months before and after FESS. Functional endoscopic sinus surgery did not reduce days hospitalized or days on intravenous antibiotics for a respiratory exacerbation in the pre- vs postoperative period. Limiting the analysis to the 30 surgeries that were performed in patients with concomitant respiratory symptoms (ie, excluding the 15 surgeries performed for sinus symptoms alone) did not significantly alter the results. Covariates of importance in CF, including CFTR genotype, gender, or microbiology, did not affect the study results. CONCLUSION: These results did not demonstrate an effect of FESS on progression of lung disease in patients with CF, but further research is needed because low statistical power has made some of the negative findings inconclusive.


Asunto(s)
Fibrosis Quística/cirugía , Endoscopía , Volumen Espiratorio Forzado/fisiología , Senos Paranasales/cirugía , Complicaciones Posoperatorias/fisiopatología , Espirometría , Capacidad Vital/fisiología , Adulto , Fibrosis Quística/diagnóstico , Femenino , Humanos , Estudios Longitudinales , Masculino , Senos Paranasales/fisiopatología , Adulto Joven
6.
Otolaryngol Head Neck Surg ; 146(5): 782-7, 2012 May.
Artículo en Inglés | MEDLINE | ID: mdl-22261488

RESUMEN

OBJECTIVE: Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. RESULTS: A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. CONCLUSIONS: There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.


Asunto(s)
Implantes Cocleares , Complicaciones Posoperatorias/epidemiología , Falla de Prótesis , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Humanos , Lactante , Persona de Mediana Edad , Factores de Riesgo , Factores de Tiempo
7.
Am J Rhinol Allergy ; 24(1): e54-6, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-20109327

RESUMEN

BACKGROUND: Balloon catheter sinuplasty (BCS) for chronic rhinosinusitis (CRS) was found to be successful in adults. The safety and feasibility of BCS in children has been recently established. The purpose of this study was to study the outcome of this technology in CRS in children. METHODS: A prospective, multicenter, nonrandomized evaluation was performed on patients with CRS. Thirty-two children between the ages of 2 and 11 years were enrolled. Safety was assessed by rate of adverse events. Effectiveness was assessed using the Sino-Nasal (SN)-5 quality-of-life questionnaire for children with CRS at 52 weeks follow-up. RESULTS: Thirty-two children were enrolled of which 24 completed their 52 weeks follow-up. No adverse events were reported because of the procedure. SN-5 score improved from a mean of 4.9 at baseline to a mean of 2.95 at 52 weeks (p < 0.0001). Twelve (50%) children had a significant improvement of their SN-5 (>-1.5), 7 (29%) had moderate improvement (>-1.0 and <-1.5), 2 (8%) had mild improvement (>-0.5 and <-1.0), 1 (4%) remained the same, and 2 (8%) had worsening scores. CONCLUSION: Balloon catheter dilation of the sinus ostia in children was safe and a significant number showed improvement of their SN-5 at 1 year follow-up. Additional studies comparing balloon sinuplasty to other modalities of treatment in children are needed to determine its efficacy in the treatment of CRS in children.


Asunto(s)
Cateterismo , Rinitis/terapia , Sinusitis/terapia , Cateterismo/efectos adversos , Niño , Preescolar , Enfermedad Crónica , Estudios de Seguimiento , Humanos , Masculino , Obstrucción Nasal , Senos Paranasales/patología , Senos Paranasales/fisiopatología , Calidad de Vida , Rinitis/patología , Rinitis/fisiopatología , Sinusitis/patología , Sinusitis/fisiopatología , Encuestas y Cuestionarios , Resultado del Tratamiento
8.
Allergy Rhinol (Providence) ; 1(2): 11, 2010 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-28569233

RESUMEN

BACKGROUND: Balloon catheter sinuplasty (BCS) for chronic rhinosinusitis (CRS) was found to be successful in adults. The safety and feasibility of BCS in children has been recently established. The purpose of this study was to study the outcome of this technology in CRS in children. METHODS: A prospective, multicenter, nonrandomized evaluation was performed on patients with CRS. Thirty-two children between the ages of 2 and 11 years were enrolled. Safety was assessed by rate of adverse events. Effectiveness was assessed using the Sino-Nasal (SN)-5 quality-of-life questionnaire for children with CRS at 52 weeks follow-up. RESULTS: Thirty-two children were enrolled of which 24 completed their 52 weeks follow-up. No adverse events were reported because of the procedure. SN-5 score improved from a mean of 4.9 at baseline to a mean of 2.95 at 52 weeks (p < 0.0001). Twelve (50%) children had a significant improvement of their SN-5 (>-1.5), 7 (29%) had moderate improvement (>-1.0 and <-1.5), 2 (8%) had mild improvement (>-0.5 and <-1.0), 1 (4%) remained the same, and 2 (8%) had worsening scores. CONCLUSION: Balloon catheter dilation of the sinus ostia in children was safe and a significant number showed improvement of their SN-5 at 1 year follow-up. Additional studies comparing balloon sinuplasty to other modalities of treatment in children are needed to determine its efficacy in the treatment of CRS in children.

9.
Am J Rhinol ; 22(4): 395-8, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18702904

RESUMEN

BACKGROUND: Topical delivery of nebulized antibiotics to the paranasal sinuses has been shown to improve clinical outcomes in patients with chronic sinus disease after functional endoscopic sinus surgery (FESS). The most efficient method for delivering nebulized particles to the sinuses, however, has not been established. This study investigates how the size of nebulized particles influences the efficiency of deposition in the maxillary sinus of human cadavers after FESS. METHODS: Endoscopic maxillary antrostomy was performed on eight sides in four cadavers. Each cadaver's nasal vault was nebulized with technetium99m-labeled sulfur colloid particles of three size ranges. Anterior-posterior and left lateral static gamma-camera images of the head were captured with an acquisition cutoff limit of 30,000 gamma-count. Regions of interest were defined for the left and right maxillary sinus and gamma-photon counts were recorded. Analysis of variance (ANOVA) for repeated measures and paired t-test were used to determine statistical significance. RESULTS: Mean diameter of particles generated was 6, 0.99, and 0.67 microm. There was a statistically significant difference in deposition between the largest particle size and the two smaller sizes, with a mean gamma-photon count of 254 for 6-microm particles versus 811 for 0.99-microm particles and 835 for 0.67-microm particles (ANOVA, p = 0.002). CONCLUSION: Particles in the 0.67- to 0.99-microm range had improved efficiency of deposition in the maxillary sinus compared with larger particles after maxillary antrostomy. Larger particles appeared to deposit directly in the nasal vault while smaller particles were more likely to reach the maxillary sinus.


Asunto(s)
Aerosoles/administración & dosificación , Seno Maxilar/metabolismo , Sinusitis Maxilar/tratamiento farmacológico , Tecnecio/farmacocinética , Administración Intranasal , Aerosoles/farmacocinética , Cadáver , Enfermedad Crónica , Endoscopía , Humanos , Seno Maxilar/diagnóstico por imagen , Sinusitis Maxilar/diagnóstico por imagen , Sinusitis Maxilar/metabolismo , Nebulizadores y Vaporizadores , Procedimientos Quirúrgicos Otorrinolaringológicos/métodos , Cintigrafía
10.
Int J Pediatr Otorhinolaryngol ; 72(6): 767-73, 2008 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-18403026

RESUMEN

OBJECTIVE: Infants are diagnosed with severe to profound hearing loss at an earlier age due to the advent of universal newborn hearing screening. This offers the opportunity to provide intervention in the form of cochlear implantation at an earlier age than was previously possible. The purpose of this investigation is to evaluate the risk of cochlear implant surgery in children less than 12 months of age. DESIGN: Retrospective review of children who underwent cochlear implantation before 12 months of age. SETTING: Patients were identified from a database of pediatric cochlear implant patients at a tertiary care center. All patients were diagnosed with severe to profound hearing loss by otoacoustic emission and auditory brainstem response. Follow-up ranged from 2 months to 5 years. RESULTS: Fourteen of 15 patients had full insertions of the electrode hardware. Less than full insertion and post-operative CSF otorrhea occurred in one patient with severe cochlear abnormalities. There were no other perioperative surgical complications. The average speech detection threshold was 27.6 dB (20-45 dB) at approximately 1-3 months post-stimulation and 25 dB (15-30 dB) at approximately 5-7 months. CONCLUSION: In our experience, we feel cochlear implantation is safe for infants as young as 6 months of age. The current standard at our institution is to implant by 7 months of age for prelingual deafness as opposed to waiting additional time until 12 months of age before the brain is presented with speech.


Asunto(s)
Implantación Coclear , Pérdida Auditiva/cirugía , Audiometría de Tonos Puros , Umbral Auditivo , Femenino , Pérdida Auditiva/etiología , Humanos , Lactante , Masculino , Estudios Retrospectivos , Percepción del Habla
11.
Laryngoscope ; 117(10): 1825-9, 2007 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-17690611

RESUMEN

OBJECTIVES: Infectious complications may cause significant delay in cochlear implant device initiation and programming and be a source of additional morbidity. We reviewed our experience with infectious complications in the pediatric age group to determine specific sources that may not be seen in adults. STUDY DESIGN: A retrospective analysis from a single implant center. METHODS: Cases of pediatric cochlear implants were reviewed for data on infectious complications. Complications were identified as "major" or "minor," "early" or "delayed." Information was gathered regarding any comorbid, chronic health condition. Data related to the causative organism(s) were collected. RESULTS: Two hundred sixty-eight cases of pediatric implants were reviewed. Twenty-two cases were identified (an infection rate of 8.2%), all classified as "major." The majority, 12, were classified as "delayed" complications. Twenty-one cases required explantation with 14 successfully reimplanted. Five cases (in 4 patients) or 23% were associated with a specific chronic pediatric condition including two children with tracheostomies. Among implanted children who had chronic health conditions, 42% developed implant-related infections. Among otherwise healthy implanted children, only 6.6% developed implant-related infections. Resistant bacterial infections were not identified. CONCLUSIONS: Health conditions in the pediatric age group were associated with 23% of our complications, a risk factor not previously identified in the literature. These children, demonstrating seven times the infection rate of healthy children, should be carefully observed postoperatively. Overall, cochlear implantation in children continues to be associated with a low risk of infectious complications.


Asunto(s)
Implantes Cocleares/microbiología , Implantes Cocleares/estadística & datos numéricos , Complicaciones Posoperatorias/epidemiología , Infecciones Relacionadas con Prótesis/epidemiología , Infecciones Relacionadas con Prótesis/microbiología , Infecciones por Pseudomonas/epidemiología , Infecciones por Pseudomonas/microbiología , Infecciones Estafilocócicas/epidemiología , Infecciones Estafilocócicas/microbiología , Niño , Preescolar , Enfermedad Crónica/epidemiología , Sordera/congénito , Sordera/epidemiología , Femenino , Estado de Salud , Humanos , Incidencia , Lactante , Masculino , Prevalencia , Pseudomonas aeruginosa/aislamiento & purificación , Estudios Retrospectivos , Staphylococcus aureus/aislamiento & purificación
12.
Am J Rhinol ; 21(2): 196-7, 2007.
Artículo en Inglés | MEDLINE | ID: mdl-17424879

RESUMEN

BACKGROUND: Recent studies suggest that topical therapy is beneficial in many conditions underlying chronic sinusitis. Current literature has documented low aerosolized particle deposition efficiency into the paranasal sinuses. Mathematical modeling suggests that three factors influence the deposition efficiency: particle size, pressure gradient, and size of the sinus ostium. Ostium size is the most dominant factor. Therefore, we sought to determine if maxillary antrostomy and ethmoidectomy would increase the deposition efficiency. METHODS: Five cadavers underwent pre- and postoperative scintigraphy after administration of aerosolized Tc-99M. Images were obtained with a gamma-camera and regions of interest (ROIs) were drawn around the maxillary sinuses. Counts per minute in the pre- and postoperative ROIs were then compared using the paired t-test. RESULTS: Results indicated a significant increase in deposition of radioactivity in the maxillary sinuses in the postoperative state (p < 0.01). CONCLUSION: Topical therapy for chronic sinusitis may be more feasible in the postoperative population.


Asunto(s)
Aerosoles/farmacocinética , Endoscopía , Seno Maxilar , Senos Paranasales/cirugía , Cadáver , Humanos , Seno Maxilar/diagnóstico por imagen , Nebulizadores y Vaporizadores , Cintigrafía , Tecnecio
13.
Arch Otolaryngol Head Neck Surg ; 130(6): 741-5, 2004 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-15210556

RESUMEN

OBJECTIVE: To determine the effectiveness of distraction osteogenesis of the mandible for relief of airway obstruction in neurologically impaired children. DESIGN: Prospective pilot study. SETTING: Tertiary care children's hospital. PATIENTS: Five children with upper airway obstruction secondary to hypotonia were identified by airway endoscopy, pulse oximetry, and polysomnography. Four children were tracheotomy-dependent because of upper airway obstruction, and 1 was being considered for tracheotomy because of progressive airway obstruction. INTERVENTIONS: All patients underwent distraction osteogenesis of the mandible for relief of their airway obstruction. MAIN OUTCOME MEASURES: Treatment success was determined by endoscopy, continuous pulse oximetry, and polysomnography. RESULTS: Four of the 5 children underwent distraction osteogenesis of the mandible with successful resolution of airway obstruction or tracheotomy decannulation. One child did not show adequate improvement of upper airway obstruction and remained tracheotomy-dependent. Follow-up was 2 to 40 months. CONCLUSIONS: Children with cerebral palsy and hypotonia of the upper airway may achieve relief of their chronic airway obstruction following distraction osteogenesis of the mandible. Appropriate selection criteria must be adhered to, including demonstration of tongue base obstruction on flexible laryngoscopy.


Asunto(s)
Obstrucción de las Vías Aéreas/cirugía , Parálisis Cerebral/complicaciones , Mandíbula/cirugía , Osteogénesis por Distracción , Obstrucción de las Vías Aéreas/etiología , Preescolar , Remoción de Dispositivos , Síndrome de Down/complicaciones , Femenino , Estudios de Seguimiento , Humanos , Lactante , Intubación Intratraqueal , Masculino , Hipotonía Muscular/complicaciones , Estudios Prospectivos , Traqueotomía
14.
J Pediatr ; 144(5): 569-73, 2004 May.
Artículo en Inglés | MEDLINE | ID: mdl-15126988

RESUMEN

OBJECTIVES: To study the efficacy of hematopoietic stem cell transplantation (HCT) for ameliorating the clinical manifestations of alpha-mannosidosis. STUDY DESIGN: Four patients with alpha-mannosidosis underwent allogeneic HCT at the University of Minnesota. Diagnosis was established by assay of leukocyte alpha-mannosidase activity level. Physical features, donor engraftment, leukocyte alpha-mannosidase activity, neuropsychologic function, and hearing were monitored before and after transplantation, with follow-up ranging from 1 to 6 years. RESULTS: All 4 patients showed slowing of their neurocognitive development and sensorineural hearing loss before HCT. All patients are alive, with normalization of leukocyte enzyme activity after HCT. Intellectual function has stabilized, with improvement in adaptive skills and verbal memory function in 3 of 4 patients. Hearing has improved to normal or near normal for speech frequencies in 3 patients. No new skeletal abnormalities have developed. CONCLUSIONS: HCT can halt the progressive cognitive loss in patients with alpha-mannosidosis. Early diagnosis and treatment with HCT is critical for optimal results.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , alfa-Manosidosis/terapia , Adulto , Factores de Edad , Niño , Preescolar , Discapacidades del Desarrollo/etiología , Discapacidades del Desarrollo/prevención & control , Femenino , Estudios de Seguimiento , Pérdida Auditiva Sensorineural/etiología , Pérdida Auditiva Sensorineural/prevención & control , Humanos , Masculino , alfa-Manosidosis/complicaciones
15.
J Assoc Res Otolaryngol ; 4(3): 384-93, 2003 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-14690056

RESUMEN

A hallmark of mucoid otitis media (MOM, i.e., chronic otitis media with mucoid effusion) is mucus accumulation in the middle ear cavity, a condition that impairs transduction of sounds in the ear and causes hearing loss. The mucin identities of mucus and the underlying mechanism for the production of mucins in MOM are poorly understood. In this study, we demonstrated that the MUC5B and MUC4 were major mucins in MOM that formed distinct treelike polymers (mucus strands). The MUC5B and MUC4 mRNAs in the middle ear mucosa with MOM were up regulated 5-fold and 6-fold, compared with the controls. This upregulation was accompanied by the extensive proliferation of the MUC5B- and MUC4-producing cells in the middle ear epithelium. Further study indicated that the mucin hyperproduction was significantly linked to CD4+ and CD8+ T cells and/or CD68+ monocyte macrophages. It suggests that MUC5B and MUC4 expression may be regulated by the products of these cells.


Asunto(s)
Mucinas/genética , Otitis Media/inmunología , Otitis Media/fisiopatología , Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD8-positivos/inmunología , División Celular , Oído Medio/inmunología , Oído Medio/patología , Oído Medio/fisiopatología , Expresión Génica/inmunología , Células Caliciformes/fisiología , Humanos , Macrófagos/inmunología , Metaplasia , Monocitos/inmunología , Mucina 4 , Mucina 5B , Membrana Mucosa/inmunología , Membrana Mucosa/patología , Membrana Mucosa/fisiopatología , Otitis Media/patología , ARN Mensajero/análisis
16.
Otolaryngol Head Neck Surg ; 129(6): 622-9, 2003 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-14663426

RESUMEN

OBJECTIVE: We sought to review the current and proposed management, as well as bring about discussion, of managing the patient with distal tracheal and pulmonary parenchymal involvement by recurrent respiratory papillomatosis (RRP). DESIGN, SETTING, AND PATIENTS: We conducted a review of 6 patients with pulmonary metastasis from RRP at 3 academic tertiary care hospitals. Interventions included surgical and medical management with antiviral, chemotherapeutic, and/or immune-modulating agents. RESULTS: Although treatment with alpha-2-beta interferon, isotretinoin, and methotrexate have not proved to eradicate pulmonary involvement by RRP, possible epithelial stabilization and slowing of disease progression are noted. CONCLUSIONS: The rates of distal tracheal and pulmonary metastasis as seen in our cohort were higher than previously reported. Approximately 12% of our patients with RRP have distal tracheal spread and as many as 7% of all patients with RRP at our institutions have pulmonary dissemination. Also, high suspicion for malignant conversion to squamous carcinoma in the patient with pulmonary spread should be maintained. In addition, aggressive treatment, although not proved to eradicate the pulmonary disease, should be undertaken due to the high morbidity and mortality associated with pulmonary dissemination of RRP in our cohort.


Asunto(s)
Neoplasias Pulmonares/secundario , Recurrencia Local de Neoplasia/patología , Papiloma/patología , Neoplasias del Sistema Respiratorio/patología , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Masculino , Papiloma/terapia , Neoplasias del Sistema Respiratorio/terapia
17.
Arch Otolaryngol Head Neck Surg ; 129(5): 563-9, 2003 May.
Artículo en Inglés | MEDLINE | ID: mdl-12759271

RESUMEN

OBJECTIVE: To evaluate the effects of exogenous interferon gamma treatment in patients with chronic rhinosinusitis and evidence of aberrant production of interferon gamma (IFN-gamma) and its regulatory cytokines. METHODS: Ten patients with treatment-resistant chronic rhinosinusitis (4 males and 6 females) treated with exogenous interferon gamma (50 micro g/m2) were retrospectively evaluated by assessing clinical outcomes compared with clinical and laboratory findings before interferon gamma treatment. RESULTS: Dysregulated IFN-gamma production was suspected to be characterized by (1) decreased interleukin 12 production (n = 1), (2) defects in interleukin 12 receptor signaling (n = 4), (3) intrinsic defects in interleukin 12 (n = 4), and (4) decreased IFN-gamma production. Eight patients had a history of chronic otitis media with positive bacterial cultures of sinus lavage samples. Adverse skin reactions to various antibiotics were reported in 7 patients. Asthma was reported in 4 patients. Along with sinusitis symptoms, these conditions were better controlled in all 9 patients who received exogenous interferon gamma for longer than 3 months. In 1 patient, interferon gamma treatment was discontinued after 3 weeks secondary to "presumed" tremor that was later diagnosed as a tic. Repeated surgical procedures and hospitalizations were reported in 2 patients after interferon gamma treatment secondary to recurrent chronic otitis media/mastoiditis/catheter infection and G-tube leakage. Interferon gamma treatment was discontinued in 1 of these patients because of a concern about neutropenia that occurred after catheter infection. Adverse effects of using exogenous interferon gamma were generally limited to local skin reactions. CONCLUSION: Exogenous interferon gamma may be a therapeutic option in a subset of patients with treatment-resistant chronic rhinosinusitis and evidence of dysregulated IFN-gamma production.


Asunto(s)
Interferón gamma/uso terapéutico , Rinitis/tratamiento farmacológico , Sinusitis/tratamiento farmacológico , Adolescente , Adulto , Niño , Preescolar , Enfermedad Crónica , Femenino , Humanos , Masculino , Neutrófilos/fisiología , Proyectos Piloto , Rinitis/fisiopatología , Sinusitis/fisiopatología
18.
Laryngoscope ; 112(10): 1737-41, 2002 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-12368606

RESUMEN

OBJECTIVES: High-resolution MRI (MRI) of human inner ear structures provides several advantages over other imaging modalities. High-resolution visualization of inner ear ultrastructure in a noninvasive manner may provide important information about inner ear disease that is not obtainable in other ways. The study was performed to demonstrate the capabilities of MRI at high resolution on the human cochlea, vestibular structures, and facial nerve. Comparative analyses of MRI anatomy with that seen on histological dissection were made. The aim of the study was to define the anatomy of human cadaveric cochlea using a 9.4-Tesla magnetic resonance scanner, currently the most powerful magnetic resonance magnet available. STUDY DESIGN: Experimental pilot study of cadaveric human cochleae. METHODS: Serial scanning using a 9.4-Tesla magnetic resonance imager on normal preserved and fresh cadaveric inner ears was performed in different planes. RESULTS: The images revealed detailed anatomy of the modiolus, utricle, saccule, semicircular canals, and facial nerve. Specifically, identifiable structures within the cochlea included the osseous spiral lamina, Reissner's membrane, membranous spiral lamina, spiral ligament, and others. CONCLUSIONS: Data established through the acquisition of images from cadaver cochlea, facial nerve, and vestibular complex provide a foundation for developing steps for testing temporal bones and, eventually, patients with Meniere's disease and other inner ear disease. The present ongoing project will provide information on baseline images of the inner ear using high-resolution MRI.


Asunto(s)
Cóclea/anatomía & histología , Imagen por Resonancia Magnética , Humanos , Aumento de la Imagen
19.
Laryngoscope ; 112(8 Pt 1): 1387-93, 2002 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-12172250

RESUMEN

OBJECTIVES/HYPOTHESIS: Spontaneous rupture of the trachea or subglottis as a complication of difficult delivery has not been reported in the United States literature. There have been a few cases reported in the European literature. The present report describes a series of newborns with this complication and discusses the signs and treatment options of this difficult, life-threatening problem. STUDY DESIGN: Retrospective review. METHODS: Newborns born between 1996 and 2001 who were treated for spontaneous subglottic or tracheal rupture at a tertiary care children's hospital neonatal intensive care unit were reviewed. RESULTS: Four cases of spontaneous subglottic rupture were seen at the hospital. In three of the four cases the tracheas were intubated on an emergency basis after subcutaneous air was noted in the anterior aspect of the neck. In the fourth patient the trachea was not intubated until the subglottic tear was visualized intraoperatively. Two of the four patients died. One died without securing of an airway; the other died of complications of prolonged hypoxia. Eight cases from European literature of spontaneous neonatal subglottic and tracheal tears are reviewed and are compared with the cases presented in the current report. CONCLUSIONS: Early detection of airway rupture by flexible endoscopy is essential for timely diagnosis and appropriate treatment. Standard endotracheal intubation can exacerbate the problem and should be deferred if possible until direct airway visualization can be accomplished. Signs associated with tracheal tears include subcutaneous emphysema, respiratory distress, pneumothorax, and pneumomediastinum. These should lead to emergent consultation with otolaryngologists for examination and securing of the airway.


Asunto(s)
Glotis , Enfermedades de la Laringe , Enfermedades de la Tráquea , Femenino , Humanos , Recién Nacido , Enfermedades de la Laringe/diagnóstico , Enfermedades de la Laringe/terapia , Masculino , Estudios Retrospectivos , Rotura Espontánea , Enfermedades de la Tráquea/diagnóstico , Enfermedades de la Tráquea/terapia
20.
Int J Pediatr Otorhinolaryngol ; 65(1): 27-33, 2002 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-12127219

RESUMEN

BACKGROUND: The contribution of an 'aberrant innominate artery' to respiratory distress syndromes has been a matter of debate nearly since the introduction of this concept. Recent advances in dynamic imaging are proving to be of value in assessing tracheal function in patients with respiratory distress. We therefore evaluated patients with innominate artery compression syndrome using the cine magnetic resonance imaging (CMRI) modality. OBJECTIVES: To apply the CMRI modality to evaluate patients with respiratory distress who exhibited tracheal compression at the level of the innominate artery. METHODS: A cohort of three patients in respiratory distress underwent bronchoscopy, followed by CMRI using a Siemens 1.5T Vision system. RESULTS: These three patients exhibited tracheal compression at the level of the innominate artery in agreement with their findings during bronchoscopy. All three exhibited dynamic tracheal compression that varied with the respiratory cycle. The degree of tracheal compromise was readily appreciated using the dynamic, real-time CMRI modality. Due to the severity of symptoms, the two children underwent innominate arteriopexy with complete resolution of their symptoms. CONCLUSIONS: CMRI provides extremely rapid acquisition of images, as well as integrated information regarding relationships of mediastinal structures. By providing functional imaging of tracheal patency during the respiratory cycle, CMRI may provide additional insight into innominate artery compression syndrome as more patients are evaluated.


Asunto(s)
Tronco Braquiocefálico/anomalías , Imagen por Resonancia Cinemagnética/métodos , Estenosis Traqueal/diagnóstico , Adolescente , Adulto , Obstrucción de las Vías Aéreas/diagnóstico , Tronco Braquiocefálico/cirugía , Broncoscopía , Descompresión Quirúrgica/métodos , Estudios de Seguimiento , Humanos , Lactante , Masculino , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
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