Immediate versus delayed surgery in congenital choanal atresia: A systematic review.

OBJECTIVES: To evaluate immediate versus delayed surgical intervention on treatment outcomes in the management of congenital choanal atresia. METHODS: This study adhered to Preferred Reporting Items for Systematic Review and Meta-Analysis guidelines in reporting a systematic review of the literature. OVID Medline, EMBASE and Pubmed databases were searched using relevant key terms. Inclusion and exclusion criteria were designed to capture studies examining immediate versus delayed primary surgery for congenital choanal atresia. Timing of surgery was classified as immediate or delayed based on median age of intervention stratified by type of obstruction. Primary outcomes were primary treatment failure, respiratory function and mortality rates. RESULTS: A total of 2765 abstracts were identified and screened by 2 independent reviewers. Of the 688 articles reviewed in full text, 23 articles met the study criteria and were subjected to quality assessment. The full study assessment and quality control measures yielded 23 studies (representing 362 patients) for pooled patient-level analysis in the systematic review. Primary treatment failures occurred in 24.8% of patients that underwent immediate surgery and 42.6% of patients that underwent delayed surgery for bilateral choanal atresia (p = 0.01). There were no differences in mortality rates (5.6% vs 4.2%; p = 1.00) or qualitative measures of respiratory function. There were no difference in treatment outcomes for patients with unilateral choanal atresia (p > 0.05). CONCLUSIONS: Through an analysis of pooled individual patient data, this systematic review of the literature demonstrated that there was significantly higher rates of treatment failure in patients that underwent delayed surgery for bilateral choanal atresia. Clinical trials and large prospective cohort studies investigating outcomes following immediate and delayed surgical intervention will provide further insight into treatment strategies.

Atresia bilateral de coanas en la adolescencia / Bilateral choanal atresia in adolescence / Atresia bilateral de choana na adolescência

Introducción: la atresia de coanas es una anomalía congénita nasal con una incidencia de 1/70.000 nacidos vivos en Argentina. Se presenta con dificultad respiratoria desde el nacimiento en el caso de las bilaterales. Presentaremos 2 casos de pacientes masculinos con atresia de coanas bilateral diagnosticados tardíamente. Para su tratamiento se utilizó técnica endoscópica transeptal con colgajos doblados sin tutores y azitromicina como antiinflamatorio de mucosa respiratoria en el postquirúrgico. Material y método: Se valoraron en forma descriptiva y retrospectiva 2 pacientes adolescentes diagnosticados e intervenidos quirúrgicamente en el Servicio de Otorrinolaringología del Hospital Posadas, mediante técnica endoscópica transeptal con colgajos doblados sin tutores. Se analizaron variables como tipo de placa atrésica, edad de diagnóstico, malformaciones asociadas y resultados quirúrgicos. Como tratamiento médico postquirúrgico, se utilizó azitromicina a dosis antiinflamatorias. Resultados: La técnica endoscópica transnasal con colgajos doblados sin tutor que hemos realizado en estos dos pacientes reduce el riesgo de reestenosis, no requiere reintervenciones posteriores para retirar el stent y genera una mejor tolerancia del paciente en el postoperatorio. El uso de azitromicina postoperatoria disminuyó el proceso inflamatorio favoreciendo la cicatrización, lográndose una correcta permeabilización de las coanas y una respiración nasal normal bilateral. Conclusiones: El diagnóstico temprano en este tipo de pacientes es fundamental, debido a la característica de respiradores nasales estrictos que presentan los neonatos. No se han encontrado reportes de pacientes diagnosticados en la adolescencia, por lo cual el diagnóstico y el tratamiento, en este caso, plantearon también un desafío.

Introduction: choanal atresia is a nasal congenital anomaly with an incidence of 1 / 70,000 live births in Argentina. It presents with respiratory difficulty from birth in bilateral cases. We will present 2 cases of male patients with bilateral choanal atresia of late diagnose. We used a trans-septal endoscopic technique with folded flap without tutors and azithromycin as an anti-inflammatory of the respiratory mucosa in the postoperative time. Material and method: A descriptive and retrospective study was carried out on 2 adolescent patients diagnosed and surgically treated in the Otorhinolaryngology Service of the Posadas Hospital, using transeptal endoscopy with a folded flap technique without tutors. Variables such as atresia plaque type, age of diagnosis, associated malformations, complications and surgical results were analyzed. Azithromycin was used at anti-inflammatory doses as a post-surgical medical treatment. Results: The transnasal endoscopic technique with folded flaps that we performed in these two patients reduces the risk of restenosis, does not require subsequent reinterventions to remove the stent and generates a better patient tolerance in the postoperative period. As for the use of postoperative azithromycin, the inflammatory process decreased, favoring cicatrization, being able to achieve a correct permeabilization of the choanas and a normal bilateral nasal breathing. Conclusions: We believe that early diagnosis in this type of patients is essential, due to the characteristic of strict nasal breathers presented by the neonates. There were no reports of patients diagnosed in adolescence, so the diagnosis and treatment, in this case, also posed a challenge.

Introdução: a atresia de choana é uma anomalia congênita nasal com incidência de 1 / 70.000 nascidos vivos na Argentina. Esse transtorno/a mesma apresenta dificuldade respiratória desde o nascimento no caso seja bilateral. (Em este estudo) apresentaremos dois casos de pacientes do sexo masculino com atresia de choana bilateral diagnosticada tardiamente. Uma técnica endoscópica trans-septal com aba curvada sem tutores e azitromicina como anti-inflamatório da mucosa respiratória foi utilizada como tratamento pós-cirurgico. Material e método: Foram avaliados de forma descritiva e retrospetiva dois pacientes adolescentes diagnosticados e tratados cirúrgicamente no serviço de Otorrinolaringologia do Hospital Posadas pela técnica transeptal endoscopica sem tutores aba dobrada. São analisados valores como tipo de placa de atresia, idade do diagnóstico, malformações associadas, complicações e resultados cirúrgicos. Como tratamento médico pós-cirúrgico, a azitromicina foi utilizada em dosagens anti-inflamatórias. Resultados: A técnica endoscópica trans-nasal com abas dobradas sem tutor que foi realizada em estes dois pacientes, reduz o risco de re-estenose, não precisando de novas intervenções cirúrgicas para remover o stent e gerar uma melhor tolerância do paciente no pós-operatório. Quanto ao uso de azitromicina pós-operatória, o processo inflamatório diminuiu, favorecendo a cicatrização, conseguindo uma permeabilização correta da choana e uma respiração nasal bilateral normal. Conclusões: Acreditamos que o diagnóstico precoce neste tipo de pacientes é fundamental, devido à característica de respiradores nasais estritos apresentada pelos neonatos. Não houve relatos de pacientes diagnosticados na adolescência, pelo que o diagnóstico e o tratamento, neste caso, também representaram um desafio.

The CHARGE association: the role of tracheotomy.

OBJECTIVES: To evaluate the need for a tracheotomy and its timing during the evolution of an association of malformations, including coloboma, heart defects, choanal atresia, developmental and growth retardation, genitourinary malformation, and ear anomalies (CHARGE association). DESIGN: Retrospective study from January 1988 through December 1997. SETTING: Four academic tertiary care centers. PATIENTS AND METHODS: Forty-five patients with CHARGE association having at least 3 cardinal malformations (growth retardation excluded) and review of the malformations and respiratory manifestations encountered. All the patients underwent endoscopic exploration on several occasions. We reviewed the nature and the timing of therapeutic interventions performed on the airway. RESULTS: Two patients died (one patient of septicemia, the other of unknown causes). Abnormalities of blood gas levels and/or sleep were found in 30 patients (67%), were responsible for cardiorespiratory arrest in 9 (20%), and required admission to the intensive care unit in 21 (47%). Pharyngolaryngeal anomalies leading to dyspnea (discoordinate pharyngolaryngomalacia, glossoptosis, retrognathia, laryngeal paralysis, cleft, stenosis, and difficult intubation) were found in 26 patients (58%). Tracheobronchial anomalies (esophagotracheal fistula, esophageal atresia, and tracheomalacia) were present in 18 patients (40%). Resection of the aryepiglottic folds was attempted 3 times, but without success. Tracheotomy was necessary in 13 patients (29%) at a median age of 2.4 months (mean duration, 25 months). Among these infants, the posterior nasal choanae were patent in 10 patients at the time of tracheotomy. Gastroesophageal reflux was encountered in 36 patients (80%). Prolonged enteral feeding was necessary in 21 patients (47%), with gastrostomy in 16 (of whom 9 needed a tracheotomy). These feeding difficulties and airway problems were highly correlated. CONCLUSIONS: We encountered multiple, complicated airway abnormalities. Resection of aryepiglottic folds was inadequate. Often, a tracheotomy could not be avoided in these patients, regardless of choanal patency. Tracheotomy needs to be performed early to avoid hypoxic events. In some selected patients, ventilation using bilevel positive airway pressure may be an alternative.