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BACKGROUND: Surgical correction of tracheobronchomalacia (TBM) has evolved greatly over the past decade, with select pediatric institutions establishing dedicated surgery and anesthesia teams to navigate the complexities and challenges of surgical airway repairs. Although anesthetic techniques have evolved internally over many years to improve patient safety and outcomes, many of these methods remain undescribed in literature. TECHNIQUE: In this article, we describe the intraoperative negative pressure suction test. This simulates the negative pressure seen in awake and spontaneously breathing patients, including the higher pressures seen during coughing which induce airway collapse in patients with TBM. Also known as the Munoz maneuver in surgical literature, this test has been performed on over 300 patients since 2015. DISCUSSION: The negative pressure suction test allows for controlled intraoperative assessment of surgical airway repairs, replaces the need for risky intraoperative wake-up tests, increases the chances of a successful surgical repair, and improves anesthetic management for emergence and extubation. We provide a guide on how to perform the test and videos demonstrating its efficacy in intraoperative airway evaluation. CONCLUSIONS: As surgeries to repair TBM become more prevalent in other pediatric institutions, we believe that pediatric patients and anesthesia providers will benefit from the insights and methods described here.
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Anestésicos , Traqueobroncomalacia , Humanos , Niño , Succión , Traqueobroncomalacia/cirugía , Respiración , Extubación TraquealRESUMEN
Bronchomalacia, the weakening of bronchial cartilage, is a common cause of cough in dogs. The gold standard for diagnosis of bronchomalacia is bronchoscopy; however, fluoroscopy is often used as a preliminary diagnostic test due to its noninvasiveness. The normal amount of bronchial collapse in healthy dogs using fluoroscopy has not been previously established, with some authors citing <25%. The aims of this prospective and retrospective analytical observational study were to determine the normal amount of airway collapse in healthy animals, the amount of airway collapse in dogs with chronic cough and presumed bronchomalacia, and cut-off values of airway collapse during expiration and cough to distinguish between the two groups. Thoracic fluoroscopy was performed in right and left lateral recumbency during inspiration, expiration, and cough to evaluate the percentage collapse of the trachea and lobar bronchi in 45 healthy and 76 chronically coughing dogs. The bronchi of chronically coughing dogs exhibited a significantly greater amount of collapse during both expiration and cough compared with healthy dogs. Chronically coughing dogs exhibited up to 31.7 ± 26.6% collapse in expiration and 87.5 ± 23.7% collapse during cough, whereas healthy dogs exhibited 14.9 ± 6.1% collapse in expiration and 35.0 ± 13.7% collapse during cough. Based on ROC curves, the authors suggest using cut-off values of 30% during expiration and 60% during cough to distinguish between healthy dogs and those with presumed bronchomalacia. Findings suggest that though healthy dogs can exhibit a greater degree of airway collapse than previously thought, fluoroscopy can be used to distinguish between healthy and presumed bronchomalacic dogs.
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Tos , Enfermedades de los Perros , Animales , Perros , Tos/veterinaria , Fluoroscopía/veterinaria , Enfermedades de los Perros/diagnóstico por imagen , Enfermedades de los Perros/diagnóstico , Masculino , Femenino , Estudios Prospectivos , Estudios Retrospectivos , Broncomalacia/veterinaria , Broncomalacia/diagnóstico por imagen , Bronquios/diagnóstico por imagen , Enfermedad Crónica/veterinaria , Valores de ReferenciaRESUMEN
BACKGROUND: Relapsing polychondritis (RP) is a chronic and recurrent inflammatory disease of the cartilage tissues in the body. The cause of RP is unknown, and since it is a rare disease with symptoms that affect multiple organs, diagnosis is often delayed. CASE PRESENTATION: A 62-year-old woman with no smoking history visited our institution complaining of fever, cough, and dyspnoea. Chest CT showed a stenosis from the left main bronchus to the left lower lobe branch. Bronchoscopy visualised intense erythema and oedema at the left main bronchus, with airway narrowing. Biopsy of the ear revealed degenerative vitreous cartilage and fibrous connective tissue with a mild inflammatory cell infiltrate. She was subsequently diagnosed with RP and administered systemic corticosteroid therapy. Her symptoms improved rapidly, and post-treatment bronchoscopy revealed that although mild erythema of the airway epithelium remained, oedema markedly improved, and the airway stenosis was resolved. CONCLUSIONS: We report a case where pre-treatment bronchoscopy was able to visually confirm RP at the acute stage. Since RP is difficult to diagnose, severe airway narrowing can occur prior to diagnosis. Therefore, to determine the stage of the disease, it is helpful to perform bronchoscopic observation before treatment. However, bronchoscopic observation before treatment should be performed by experienced bronchoscopists due to the risk of airway obstruction.
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Obstrucción de las Vías Aéreas , Policondritis Recurrente , Humanos , Femenino , Persona de Mediana Edad , Constricción Patológica/complicaciones , Tráquea , Obstrucción de las Vías Aéreas/diagnóstico , Pulmón , Disnea/complicaciones , Policondritis Recurrente/complicaciones , Policondritis Recurrente/diagnóstico , Policondritis Recurrente/tratamiento farmacológicoRESUMEN
Absent pulmonary valve syndrome and double-outlet left ventricle are rare congenital anomalies, with, to the best of our knowledge, no cases reported to date. We present the treatment course in a patient with an absent pulmonary valve, double-outlet left ventricle, dextrocardia, hypoplastic right ventricle, valvular aortic stenosis, and bronchomalacia.
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Ventrículo Derecho con Doble Salida , Estenosis de la Válvula Pulmonar , Válvula Pulmonar , Transposición de los Grandes Vasos , Humanos , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Válvula Pulmonar/anomalías , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Estenosis de la Válvula Pulmonar/diagnóstico , Estenosis de la Válvula Pulmonar/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/cirugíaRESUMEN
Neonates and pediatric populations are vulnerable subjects in terms of health. Proper screening and early optimal treatment would reduce infant and child mortality, improving the quality of life. Researchers and clinicians all over the world are in pursuit of innovations to improve the medical care delivery system. Infant morphometrics changes drastically due to the rapid somatic growth in infancy and childhood, demanding for patient-specific customization of treatment intervention accordingly. 3D printing is a radical technology that allows the generation of physical 3D products from digital images and addresses the patient-specific requirement. The combination of cost-effective and on-demand customization offers a boundless opportunity for the enhancement of neonates and pediatric health.Conclusion: The advanced technology of 3D printing proposes a pioneering breakthrough in bringing physiologically and anatomically appropriate treatment strategies addressing the unmet needs of child health problems. What is Known: ⢠The potential application of 3D printing is observed across a multitude of fields within medicine and surgery. ⢠The unprecedented effect of this technology on pediatric healthcare is still very much a work in progress. What is New: ⢠The recent clinical applications of 3D printing provide better treatment modalities to infants and children. ⢠The review provides an overview of the comparison between conventional treatment methods and 3DP regarding specific applications.
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Pediatría , Calidad de Vida , Niño , Humanos , Recién Nacido , Medicina de Precisión , Impresión TridimensionalRESUMEN
Tracheobronchomalacia is a condition of dynamic collapse of the trachea and mainstem bronchi. The clinical significance of tracheobronchomalacia depends on its severity. Mild cases may be medically managed with limited symptomology, while severe cases require advanced therapies, lengthy hospital stays, and carry significant morbidity and mortality. Current therapies for severe tracheobronchomalacia include tracheostomy with prolonged mechanical ventilation, aortopexy, tracheobronchopexy, and intraluminal metallic, silicone, or bioresorbable stents. Three-dimensional (3D)-printed, patient-specific, bioresorbable airway splinting is a novel treatment option that is undergoing investigation in a cohort of critically ill children with severe tracheobronchomalacia. At the time of our last review of our data, 29 splints had been implanted in 15 children with intrathoracic tracheobronchomalacia. The median follow-up was 8.5 months. There were 12 long-term survivors, and all but one lived at home. This article discusses the details of our institution's development and use of 3D-printed, patient-specific, bioresorbable splints for treatment of severe tracheobronchomalacia in the pediatric population.
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Férulas (Fijadores) , Traqueobroncomalacia , Implantes Absorbibles , Niño , Humanos , Impresión Tridimensional , Tráquea , Traqueobroncomalacia/terapiaRESUMEN
Hand hyperphalangism leading to shortened index fingers with ulnar deviation, hallux valgus, mild facial dysmorphism and respiratory compromise requiring assisted ventilation are the key features of Chitayat syndrome. This condition results from the recurrent heterozygous missense variant NM_006494.2:c.266A>G; p.(Tyr89Cys) in ERF on chromosome 19q13.2, encoding the ETS2 repressor factor (ERF) protein. The pathomechanism of Chitayat syndrome is unknown. To date, seven individuals with Chitayat syndrome and the recurrent pathogenic ERF variant have been reported in the literature. Here, we describe six additional individuals, among them only one presenting with a history of assisted ventilation, and the remaining presenting with variable pulmonary phenotypes, including one individual without any obvious pulmonary manifestations. Our findings widen the phenotype spectrum caused by the recurrent pathogenic variant in ERF, underline Chitayat syndrome as a cause of isolated skeletal malformations and therefore contribute to the improvement of diagnostic strategies in individuals with hand hyperphalangism.
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Dedos/anomalías , Predisposición Genética a la Enfermedad , Hallux Valgus/genética , Síndrome de Pierre Robin/genética , Proteínas Represoras/genética , Adolescente , Adulto , Niño , Preescolar , Facies , Femenino , Dedos/diagnóstico por imagen , Dedos/patología , Hallux Valgus/diagnóstico por imagen , Hallux Valgus/patología , Humanos , Síndrome de Pierre Robin/diagnóstico por imagen , Síndrome de Pierre Robin/patología , Secuenciación del Exoma , Adulto JovenRESUMEN
To evaluate the feasibility and efficacy of external suspension with absorbable poly-l-lactic acid material shaping microplates for infants with severe bronchomalacia and congenital heart disease. From November 2017 to January 2019, 11 continual patients with severe bronchomalacia and congenital heart disease underwent bronchial membrane external suspension together with cardiovascular surgery. An absorbable plate made with poly-l-lactic acid material was used as the shaping fixation material in all patients. Data included the details of the operation, and clinical results were collected. The mean age was 1.2 ± 1.0 years, and the mean weight was 7.7 ± 2.9 kg. The patients with cardiac malformations were operated on under low-temperature cardiopulmonary bypass (CPB) through median sternotomy. There were no in-hospital deaths. The CPB time, mechanical ventilation time, and length of intensive care unit stay were 123.9 ± 36.9 min, 20.7 ± 19.4 h, and 71.6 ± 54.9 h, respectively. Two patients underwent surgery through a left posterolateral incision without CPB. One was a double aortic arch repair, and the other was only bronchial membrane external suspension with prior IAA repair. No patients needed ECMO support. The mean follow-up time was 12.1 ± 5.6 months, and no patients were lost to follow-up. No cases of late death were noted, and no patients needed reoperation. According to the CT scans, no patients had bronchial restenosis. External bronchial membrane suspension with an absorbable poly-l-lactic acid material shaping plate, which had better histocompatibility, for infants with severe bronchomalacia and congenital heart disease was a safe and feasible procedure.
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Broncomalacia/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/cirugía , Broncomalacia/complicaciones , Preescolar , Femenino , Cardiopatías Congénitas/complicaciones , Humanos , Lactante , Recién Nacido , Masculino , Poliésteres/uso terapéutico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Background and objectives: Persistent wheezing (PW) is defined as prolonged or recurrent episodes of wheezing despite regular treatment. Flexible bronchoscopy (FB) is recommended to determine the etiology of PW in children. This study aimed to determine the etiology of PW based on FB findings in a national pediatric center. Materials and Methods: Children presenting with PW that underwent flexible bronchoscopy from April 2016 to August 2019 at the Mother and Child Health Institute of Serbia were included in this observational study. After endoscopic evaluation, bronchoalveolar lavage fluid (BALF) samples were taken and further analyzed. Quantitative microbiology, cytological analysis and oil-red staining of specimens were performed to determine cellular constituents and presence of lipid laden macrophages (LLM). Upper gastrointestinal series were performed to exclude gastroesophageal reflux disease, swallowing dysfunction and vascular ring. Results: Pathological findings were revealed in 151 of 172 study participants, with bacterial lower airway infection (BLAI) (48.3%) and primary bronchomalacia (20.4%) as the most common. Younger participants were hospitalized for significantly longer periods (ρ = -0.366, p < 0.001). Study participants with BLAI and associated mucus plugging were notably younger (p < 0.001). Presence of LLM in BALF was not associated with findings of upper gastrointestinal series. All patients with confirmed BLAI were treated with oral antibiotics. Although FB is considered to be invasive, there were no complications associated with the procedure. Conclusions: Flexible bronchoscopy has an exceptional diagnostic value in evaluation of PW. In younger patients with BLAI, presence of mucus plugs may complicate the clinical course, so significant benefits can be achieved with therapeutic lavage during bronchoscopy.
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Broncoscopios/normas , Broncoscopía/instrumentación , Broncoscopía/métodos , Ruidos Respiratorios/diagnóstico , Ruidos Respiratorios/fisiopatología , Adolescente , Lavado Broncoalveolar/instrumentación , Lavado Broncoalveolar/métodos , Broncoscopía/normas , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , SerbiaRESUMEN
BACKGROUND: In 1993, Chitayat et al., reported a newborn with hyperphalangism, facial anomalies, and bronchomalacia. We identified three additional families with similar findings. Features include bilateral accessory phalanx resulting in shortened index fingers; hallux valgus; distinctive face; respiratory compromise. OBJECTIVES: To identify the genetic aetiology of Chitayat syndrome and identify a unifying cause for this specific form of hyperphalangism. METHODS: Through ongoing collaboration, we had collected patients with strikingly-similar phenotype. Trio-based exome sequencing was first performed in Patient 2 through Deciphering Developmental Disorders study. Proband-only exome sequencing had previously been independently performed in Patient 4. Following identification of a candidate gene variant in Patient 2, the same variant was subsequently confirmed from exome data in Patient 4. Sanger sequencing was used to validate this variant in Patients 1, 3; confirm paternal inheritance in Patient 5. RESULTS: A recurrent, novel variant NM_006494.2:c.266A>G p.(Tyr89Cys) in ERF was identified in five affected individuals: de novo (patient 1, 2 and 3) and inherited from an affected father (patient 4 and 5). p.Tyr89Cys is an aromatic polar neutral to polar neutral amino acid substitution, at a highly conserved position and lies within the functionally important ETS-domain of the protein. The recurrent ERF c.266A>C p.(Tyr89Cys) variant causes Chitayat syndrome. DISCUSSION: ERF variants have previously been associated with complex craniosynostosis. In contrast, none of the patients with the c.266A>G p.(Tyr89Cys) variant have craniosynostosis. CONCLUSIONS: We report the molecular aetiology of Chitayat syndrome and discuss potential mechanisms for this distinctive phenotype associated with the p.Tyr89Cys substitution in ERF.
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Anomalías Múltiples/genética , Síndrome de Dandy-Walker/genética , Discapacidades del Desarrollo/genética , Huesos Faciales/anomalías , Proteínas Represoras/genética , Anomalías Múltiples/fisiopatología , Broncomalacia/genética , Broncomalacia/fisiopatología , Síndrome de Dandy-Walker/fisiopatología , Discapacidades del Desarrollo/fisiopatología , Exoma/genética , Cara/fisiopatología , Huesos Faciales/fisiopatología , Femenino , Hallux Valgus/genética , Hallux Valgus/fisiopatología , Secuenciación de Nucleótidos de Alto Rendimiento , Humanos , Recién Nacido , Masculino , FenotipoRESUMEN
BACKGROUND: Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM. METHODS: Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images. RESULTS: Oblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM. CONCLUSIONS: Patients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.
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Broncomalacia/cirugía , Broncoscopía , Evaluación del Resultado de la Atención al Paciente , Índice de Severidad de la Enfermedad , Traqueomalacia/cirugía , Broncomalacia/diagnóstico , Preescolar , Femenino , Humanos , Lactante , Tiempo de Internación , Masculino , Estudios Retrospectivos , Traqueomalacia/diagnósticoRESUMEN
The aim of this study was to: (1) find out whether laryngomalacia (LM) types are related to clinical course; (2) which patients with LM are at higher risk of other airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)]; and (3) evaluate the prevalence of LM in our region. Patients with established LM diagnosis and complete clinical and endoscopy records were enrolled. They were classified into different LM types according to classification based on the side of supraglottic obstruction. One hundred ten children were included. The most common LM appearance was type I-58 children, followed by combine types (I + II and I + III)-38. The other airway malacia were found in 47 patients: TM in 31, BM in 10, and TM with BM in 6. Other comorbidities (cardiac, neurological, and genetic disorders) were identified in 30 children. Patients with combine types of LM differ from those with single type of LM in terms of prematurity (13 vs 31 %, p = 0.04) and higher weight on the examination day (p = 0.006). Patients with other airway malacia differ from children with isolated LM in terms of prematurity (40 vs 13 %, p = 0.008), comorbidities (38 vs 19 %, p = 0.024), and lower weight on the examination day (p = 0.014). The prevalence of clinically relevant LM was one in 2600-3100 newborns. Clinical course of LM cannot be anticipated on the basis of solely endoscopic evaluation of the larynx. Comorbidities and prematurity increase the risk of other airway malacia. The prevalence of LM is relatively high in the middle-south part of Poland.
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Obstrucción de las Vías Aéreas , Broncomalacia/epidemiología , Laringomalacia , Traqueomalacia/epidemiología , Obstrucción de las Vías Aéreas/patología , Obstrucción de las Vías Aéreas/fisiopatología , Broncoscopía/métodos , Comorbilidad , Femenino , Humanos , Lactante , Recién Nacido , Laringomalacia/clasificación , Laringomalacia/epidemiología , Laringomalacia/fisiopatología , Masculino , Polonia/epidemiología , Prevalencia , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Supraglotitis/patologíaRESUMEN
This study was undertaken to assess the frequency of airway malacia in infants and young children with achondroplasia, a population well known to be at risk for a variety of respiratory problems. We also wished to evaluate what, if any, contribution airway malacia makes to the complex respiratory issues that may be present in those with achondroplasia. Retrospective chart review of all infants and young children with achondroplasia who were assessed through the Midwest Regional Bone Dysplasia Clinics from 1985 through 2012 (n = 236) was completed. Records of comprehensive clinical examinations, polysomnographic assessments, and airway visualization were reviewed and abstracted using a data collection form. Analyses were completed comparing the group with and those without evidence for airway malacia. Thirteen of 236 patients (5.5%) were found to have airway malacia. Most of those affected had lower airway involvement (9/13). The presence of airway malacia was correlated with an increased occurrence of obstructive sleep apnea as well as need for oxygen supplementation, airway surgeries and tracheostomy placement. Although estimates of the frequency of airway malacia in the general population are limited, its frequency in children with achondroplasia appears to be much higher than any published general population estimate. The presence of airway malacia appears to confound other breathing abnormalities in this population and results in the need for more invasive airway treatments.
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Acondroplasia/complicaciones , Apnea Obstructiva del Sueño/etiología , Acondroplasia/diagnóstico , Acondroplasia/genética , Humanos , Lactante , Masculino , Morbilidad , Estudios Retrospectivos , Apnea Obstructiva del Sueño/diagnóstico , Apnea Obstructiva del Sueño/epidemiologíaRESUMEN
OBJECTIVES: The objective was to report and analyse the characteristics and results of open aortopexy and thoracoscopic aortopexy for the treatment of airway malacia in a paediatric population. METHODS: We report a retrospective consecutive case series of paediatric patients undergoing aortopexy for the treatment of airway malacia at a quaternary referral centre between December 2006 and January 2021. Outcome measures included days to extubation, continued need for non-invasive ventilation, further intervention in the form of tracheostomy and death. RESULTS: 169 patients underwent aortopexy: 147 had open procedures (135 via median/limited median sternotomy and 12 thoracotomy) and 22 thoracoscopic. Mean follow up was 8.46 yrs (range 1-20 yrs). Most common site of airway malacia was the trachea (n = 106, 62.7 %), and 48 (28.4 %) had additional involvement at the bronchi with tracheobronchomalacia (TBM). 15 (8.9 %) had bronchomalacia (BM) only. Incidence of bronchial disease was lower in the thoracoscopic than open group (13.6 % vs 40.82 %; p < 0.0001). Mean time to extubation was 1.45 days, 2.59 days, 5.23 days in tracheomalacia, TBM and BM groups, respectively (p = 0.0047). Mean time to extubation was 1.35 days, 2 days, 3.67 days, and 5 days in patients with external vascular compression, TOF/OA, primary airway malacia, and laryngeal reconstruction, respectively (p = 0.0002). There were 21 deaths across the cohort, and all were in the open group. 71.4 % (n = 15) had bronchial involvement of their airway malacia. CONCLUSIONS: Open and thoracoscopic aortopexy are effective treatments for airway malacia in children. We have identified that involvement of the bronchi is a risk factor for adverse outcomes, and the optimum treatment for this patient cohort is still debatable. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective Study.
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Traqueobroncomalacia , Traqueomalacia , Humanos , Niño , Lactante , Estudios Retrospectivos , Aorta/cirugía , Traqueobroncomalacia/cirugía , Traqueomalacia/cirugía , Esternotomía/efectos adversos , Esternotomía/métodosRESUMEN
OBJECTIVES: The management of idiopathic subglottic stenosis (iSGS) poses a clinical challenge due to high recurrence rates following both endoscopic and open approaches, often leading to tracheostomy. The activation of abnormal T-cells and cytokine pathways has been linked to iSGS pathogenesis. Autologous adipose tissue centrifugation yields lipoaspirate, offering optimal anti-inflammatory effects and biocompatibility widely utilized in various medical settings. This report presents the first 3 cases employing endoscopic dilation (ED) in combination with local lipoaspirate injection to address recurrent iSGS. METHODS: A prospective observational study was conducted, involving multidisciplinary evaluation by the Tracheal Team at the University of Modena. Patients meeting specific criteria were directed to undergo ED + lipoaspirate injection. RESULTS: Three patients fulfilled the inclusion criteria. The mean number of prior endoscopic procedures performed was 8. Endoscopic examination revealed 90% stenosis in patient A, 60% stenosis in patient B, and 60% stenosis in patient C. All patients presented inflammatory tissue or incipient granulations at the stenotic site, with an average time of 6 months between previous procedures. After 15 months, none of the patients required further procedures, and endoscopic examination revealed a significant reduction or disappearance of inflammatory tissue with a stable airway lumen. CONCLUSIONS: The observed results are encouraging in terms of reducing local inflammation and halting stenosis progression, especially in cases of short-term relapsing iSGS.
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Tejido Adiposo , Laringoestenosis , Recurrencia , Humanos , Laringoestenosis/cirugía , Laringoestenosis/etiología , Masculino , Tejido Adiposo/trasplante , Estudios Prospectivos , Femenino , Persona de Mediana Edad , Dilatación/métodos , Adulto , Laringoscopía/métodos , Resultado del TratamientoRESUMEN
BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.
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Broncoscopía , Traqueobroncomalacia , Humanos , Traqueobroncomalacia/cirugía , Traqueobroncomalacia/complicaciones , Masculino , Femenino , Lactante , Preescolar , Resultado del Tratamiento , Broncoscopía/métodos , Niño , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Estudios RetrospectivosRESUMEN
OBJECTIVE: In children with tracheobronchomalacia, surgical management should be reserved for the most severe cases and be specific to the type and location of tracheobronchomalacia. The goal of this study is to describe the presentation and outcomes of children with severe tracheobronchomalacia undergoing surgery. METHODS: Retrospective case series of 20 children operated for severe tracheobronchomalacia at a tertiary hospital from 2003 to 2023. Data were collected on symptoms age at diagnosis, associated comorbidities, previous surgery, age at surgery, operative approach, time of follow-up, and outcome. Surgical success was defined as symptom improvement. RESULTS: The most frequent symptoms of severe tracheobronchomalacia were stridor (50 %), cyanosis (50 %), and recurrent respiratory infections (45 %). All patients had one or more underlying conditions, most commonly esophageal atresia (40 %) and prematurity (35 %). Bronchoscopy were performed in all patients. Based on etiology, patients underwent the following procedures: anterior aortopexy (n = 15/75 %), posterior tracheopexy (n = 4/20 %), and/or posterior descending aortopexy (n = 4/20 %). Three patients underwent anterior aortopexy and posterior tracheopexy procedures. After a median follow-up of 12 months, 16 patients (80 %) had improvement in respiratory symptoms. Decannulation was achieved in three (37.5 %) out of eight patients with previous tracheotomy. The presence of dying spells at diagnosis was associated with surgical failure. CONCLUSIONS: Isolated or combined surgical procedures improved respiratory symptoms in 80 % of children with severe tracheobronchomalacia. The choice of procedure should be individualized and guided by etiology: anterior aortopexy for anterior compression, posterior tracheopexy for membranous intrusion, and posterior descending aortopexy for left bronchus obstruction.
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Traqueobroncomalacia , Humanos , Traqueobroncomalacia/cirugía , Traqueobroncomalacia/complicaciones , Estudios Retrospectivos , Femenino , Masculino , Lactante , Resultado del Tratamiento , Recién Nacido , Preescolar , Broncoscopía , Índice de Severidad de la Enfermedad , Niño , Estudios de SeguimientoRESUMEN
BACKGROUND: Glucocorticoids are frequently required for management of cough because of inflammatory airway disease (IAD) and airway collapse (AWC). OBJECTIVES/HYPOTHESIS: To determine the efficacy and feasibility of inhaled administration of corticosteroids in controlling cough in dogs with noninfectious airway disease. ANIMALS: Thirty-six client-owned dogs. METHODS: Dogs were prospectively recruited for this placebo-controlled cross-over study. Inflammatory airway disease was diagnosed through bronchoalveolar lavage cytology. Airway collapse was diagnosed through bronchoscopy, or if dogs were unsuitable anesthetic candidates, by crackles on auscultation, radiographic changes in airway diameter, or fluoroscopy. Dogs were randomly assigned to receive placebo or fluticasone propionate for the first 2 weeks of the trial then crossed over to fluticasone. A quality of life (QOL) survey (best score 0, worst score 85) was completed at 0 and 6 weeks. A visual-analog cough survey was submitted at 0, 2, 4, and 6 weeks to assess cough, feasibility, and adverse effects of treatment. RESULTS: For 32 dogs, QOL score at study end (mean 11.3 ± 9.7) was significantly lower (P < .0001) compared to entry (mean 28.1 ± 14.1), with a median change of 69% in QOL score, indicating improved quality of life. Cough frequency, duration, and severity were significantly (P < .0001) decreased at study end. Feasibility of aerosolized delivery improved with continued use (P = .05) with only 1 dog unable to accept inhaled medication. CONCLUSION AND CLINICAL IMPORTANCE: This study supports the utility of fluticasone propionate by inhalation in management of cough in dogs with IAD and AWC.
Asunto(s)
Asma , Enfermedades de los Perros , Perros , Animales , Tos/tratamiento farmacológico , Tos/veterinaria , Calidad de Vida , Estudios Cruzados , Fluticasona/uso terapéutico , Glucocorticoides/uso terapéutico , Asma/tratamiento farmacológico , Asma/veterinaria , Androstadienos/uso terapéutico , Método Doble Ciego , Enfermedades de los Perros/tratamiento farmacológicoRESUMEN
BACKGROUND: Principal and lobar bronchial collapse is increasingly recognized as an isolated entity. OBJECTIVE: Retrospectively describe the procedure and outcomes of dogs undergoing bronchial stenting at a single referral hospital. ANIMALS: Nine client-owned dogs with variable degrees of collapse of the left principal bronchus (LPB), lobar bronchus 1 (LB1), and lobar bronchus 2 (LB2), and with clinically relevant signs of respiratory dysfunction. METHODS: Data were collected from patient records. All dogs underwent stenting of the LPB and LB2. Anatomic and functional impairment grades were assigned to each case before and 4 weeks after stenting. Data regarding response to stenting and complications were evaluated. RESULTS: Bronchial stenting was considered successful in all cases, with all dogs experiencing improved quality of life (QOL), and decreased functional impairment grade at 4 weeks post-stenting. Follow-up of >6 months was available for 6 dogs and of these, 5 were alive at 12 months, 3 were alive at 18 months, and 1 was alive at 24 months. Stent-related complications occurred in 4 dogs, and were resolvable in 3. Two dogs developed pneumothorax, 1 developed recurrent pneumonia, and 1 developed new-onset coughing. All dogs had mild and manageable coughing post-stenting. CONCLUSIONS AND CLINICAL IMPORTANCE: Stenting of the LBP and LB2 might be an effective option for dogs with advanced collapse of these bronchi and associated signs. Although all included dogs had resolution or improvement of clinical signs considered life-threatening or as affecting QOL, ongoing coughing is expected. Patient selection appears important with regard to achieving successful outcomes.
Asunto(s)
Enfermedades de los Perros , Calidad de Vida , Humanos , Perros , Animales , Estudios Retrospectivos , Bronquios/cirugía , Tráquea , Stents/veterinaria , Enfermedades de los Perros/cirugía , Enfermedades de los Perros/diagnósticoRESUMEN
OBJECTIVE: Standard methods to evaluate tracheal pathology in children, including bronchoscopy, may require general anesthesia. Conventional dynamic proximal airway imaging in noncooperative children requires endotracheal intubation and/or medically induced apnea, which may affect airway mechanics and diagnostic performance. We describe a technique for unsedated dynamic volumetric computed tomography angiography (DV-CTA) of the proximal airway and surrounding vasculature in children and evaluate its performance compared to the reference-standard of rigid bronchoscopy. METHODS: Children who had undergone DV-CTA and bronchoscopy in one-year were retrospectively identified. Imaging studies were reviewed by an expert reader blinded to the bronchoscopy findings of primary or secondary tracheomalacia. Airway narrowing, if present, was characterized as static and/or dynamic, with tracheomalacia defined as >50% collapse of the tracheal cross-sectional area in exhalation. Pearson correlation was used for comparison. RESULTS: Over a 19-month period, we identified 32 children (median age 8 months, range 3-14 months) who had undergone DV-CTA and bronchoscopy within a 90-day period of each other. All studies were unsedated and free-breathing. The primary reasons for evaluation included noisy breathing, stridor, and screening for tracheomalacia. There was excellent agreement between DV-CTA and bronchoscopy for diagnosis of tracheomalacia (κ = 0.81, p < 0.001), which improved if children (n = 25) had the studies within 30 days of each other (κ = 0.91, p < 0.001). CTA provided incremental information on severity, and cause of secondary tracheomalacia. CONCLUSION: For most children, DV-CTA requires no sedation or respiratory manipulation and correlates strongly with bronchoscopy for the diagnosis of tracheomalacia. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:410-416, 2023.