Choanal atresia: Diagnosis and management

Objective: Choanal atresia (CA) is a rare congenital malformation caused by the obliteration of the posterior choanae by an atretic plate. The aim of our study is to describe the diagnosis and management modalities of CA and to determine the factors associated with recurrence. Materials and methods: This is a retrospective study based on the medical records of patients with CA managed in our department in the period between 2002 and 2021. We studied the clinical features and management modalities of each patient. For patients who developed a recurrence, we determined the factors associated with recurrence based on a bivariate analysis. Results: We studied the medical records of 26 patients with either a bilateral (n=8) or a unilateral (n=16) form of CA. The median age at surgery was two days for bilateral forms and 5 years and 4 months for unilateral forms. At computed tomography scan, CA was mixed (n=20), bony (n=4) or membranous (n=2). All patients underwent intranasal endoscopic surgical treatment using cold instruments alone in membranous forms and combined to the drilling of the atretic plate in bony and mixed forms. The surgical management included the resection of the posterior part of the vomer bone and the placement of nasal stents in 10 and 16 patients respectively. We recorded 6 cases of recurrence requiring a surgical re-intervention. The presence of associated cranio-facial malformations was the only factor associated with recurrence (p=0,001). Conclusion: Choanal atresia diagnosis was based on nasal endoscopy and CT scan. Surgical treatment using transnasal endoscopic approach was an effective and safe technique. Associated local malformations was a factor associated with re-stenosis

Atresia de coana unilateral: reporte de un caso pediátrico / Unilateral coana atresia. A pediatric case report

La atresia de coanas se caracteriza por la obliteración de la abertura nasal posterior. Es la anomalía congénita más frecuente de las fosas nasales. Tiene una incidencia de 1 cada 5000 a 7000 neonatos, con predominio en el sexo femenino. Puede presentarse en forma aislada o asociada a otros síndromes como el CHARGE (coloboma [C], malformaciones cardíacas [H], atresia de coanas [A], retraso psicomotor y/o en el crecimiento [R], hipoplasia de genitales [G], malformaciones auriculares y/o sordera [E, por su sigla en inglés]). Las manifestaciones clínicas son la obstrucción nasal, cianosis y dificultad respiratoria desde el nacimiento cuando es bilateral. Las atresias unilaterales se caracterizan por insuficiencia ventilatoria nasal y rinorrea unilateral, y pueden pasar inadvertidas. El diagnóstico se realiza mediante endoscopia y estudios por imágenes. El tratamiento es quirúrgico; existen diferentes técnicas y vías de abordaje. Se presenta el caso de un paciente masculino de 7 años con atresia unilateral de coana derecha con resolución microendoscópica, colocación de tutor externo, con buena resolución.

Choanal atresia is characterized by obliteration of the posterior nasal opening. It is the most common congenital anomaly of the nasal passages. It has an incidence of 1 in 5000 to 7000 newborns; predominantly female. It can occur in isolation or in association with other syndromes such as CHARGE (coloboma [C], cardiac malformations [H], choanal atresia [A], psychomotor and/or growth retardation [R], genital hypoplasia [G], atrial malformations and/or deafness [E]. Clinicallypresents nasal obstruction, cyanosis and respiratory distress from birth when bilateral, unilateral atresias are characterized by nasal ventilatory insufficiency and unilateral rhinorrhea, which may go unnoticed. Diagnosis is made by endoscopy and imaging tests. Treatment is surgical, with different techniques and approaches.A 7-year-old male patient is presented with unilateral atresia of the right choana with microendoscopic resolution, placement of an external tutor, with good resolution.

Anomalías congénitas nasales: Estudio retrospectivo y revisión de la literatura / Congenital Anomalies of the Nose: retrospective study and literature review

INTRODUCTION. Congenital malformations occur in 1 in 5,000 to 40,000 live births. They present as a morphological abnormality of the nasal pyramid or as a picture of moderate to severe nasal obstruction. OBJECTIVE. Evaluate the etiology, associated comorbidities, management and mortality of congenital malformations of the nose in neonates and infants. MATERIALS AND METHODS. Cross-sectional retrospective descriptive study, 105 medical records were reviewed, of which 26 corresponded to patients with congenital nasal malformations, at the Carlos Andrade Marín Specialty Hospital in Quito - Ecuador, between January 2009 and May 2022; the tabulation and analysis of data was carried out in the Excel program. The patients were classified according to Losee et al. in 4 types: hypoplasia, hyperplasia, clefts and tumors RESULTS. Nasal anomalies occurred in males in 73.07%, all presented nasal obstruction, the diagnosis was made by flexible nasofibroscopy; in 42.3% of the cases, the evaluation was complemented with computed tomography. The most frequent congenital pathology was stenosis - choanal atresia with 53.8%, followed by craniofacial clefts with 15.39%. In 42.3% of the cases there was an association with genetic syndromes, neurological, ocular and intestinal pathology. 69.23% of the patients received clinical treatment with nasal lavages, nasal corticosteroids and positive pressure by cannula, while 30.77% were resolved surgically, being: 2 unilateral choanal atresia, 1 middle fossa stenosis, 4 cleft lip and palate and 1 encephalocele. Mortality was 7.69% CONCLUSION. Hypoplasias and clefts are the predominant pathologies, flexible nasal nasofibroscopy is the diagnostic test of choice. The clinical treatment was successful in the initial management in 69.23% of cases. Surgery was performed for the repair of facial clefts; being the hypoplasias or masses managed by endoscopic approach.

INTRODUCCIÓN. Las malformaciones congénitas se presentan en 1 de cada 5.000 a 40.000 nacidos vivos. Se presentan como una anomalía morfológica de la pirámide nasal o como un cuadro de obstrucción nasal moderada a severo. OBJETIVO. Evaluar la etiología, comorbilidades asociadas, manejo y mortalidad de las malformaciones congénitas de nariz en neonatos y lactantes. MATERIALES Y MÉTODOS. Estudio descriptivo retrospectivo transversal, se revisaron 105 historias clínicas de las cuales 26 correspondieron a pacientes con malformaciones congénitas nasales, en el Hospital de Especialidades Carlos Andrade Marín de Quito - Ecuador, entre enero de 2009 a mayo de 2022; la tabulación y análisis de datos se realizó en el programa Excel. Los pacientes fueron clasificados según Losee et al. en 4 tipos: hipoplasia, hiperplasia, hendiduras y tumores. RESULTADOS. Las anomalías nasales se presentaron en el sexo masculino en el 73.07%, el motivo de consulta fue la obstrucción nasal, el diagnóstico se realizó mediante nasofibroscopía flexible; en el 42,3% de los casos se complementó la evaluación con tomografía computarizada. La patología congénita más frecuente fue la estenosis - atresia de coana con un 53,8%, seguida de hendiduras craneofaciales con un 15.39%. En el 42.3% de los casos existió asociación con síndromes genéticos, patología neurológica, ocular e intestinal. El 69,23% de los pacientes recibió tratamiento clínico con lavados nasales, corticoides por vía nasal y presión positiva por cánula, mientras que el 30,77% se resolvió quirúrgicamente, siendo: 2 atresia unilateral de coana, 1 estenosis de fosa media, 4 hendiduras labio palatinas y 1 encefalocele. La mortalidad fue del 7,69%. CONCLUSIÓN: Las hipoplasias y las hendiduras son las patologías que predominaron, la nasofibroscopia flexible nasal es el examen diagnóstico de elección. El tratamiento clínico fue exitoso en el manejo inicial en el 69,23% de casos. La cirugía se realizó para la reparación de hendiduras faciales; siendo las hipoplasias o masas manejadas por abordaje endoscópico.

Atresia de coana bilateral en adulto con malformación craneofacial asociada: un diagnóstico poco habitual / Bilateral choanal atresia in adult with associated craneofacial malformation: an unusual diagnosis

La atresia de coana es una rara malformación congénita improbable de encontrar de forma bilateral en un adolescente o adulto. Hasta la fecha, no se ha descrito ningún caso de atresia bilateral en un adulto con una malformación asociada de cabeza y cuello que haya requerido tratamiento conjunto. El tratamiento de elección de la atresia de coana bilateral continúa siendo la cirugía endoscópica, con controversia en el uso intraoperatorio de mitomicina o la colocación de stents para evitar estenosis. Lo que no está claro es el orden de tratamiento y la simultaneidad del procedimiento si se asocian otras posibles patologías que tengan una indicación quirúrgica. Presentamos un caso clínico que cumple con todos estos requisitos.

Choanal atresia is a rare congenital malformation that is unlikely to be found bilaterally and is seldom diagnosed in adulthood. To date, no clinical case of bilateral atresia has been described with a head and neck malformation that requires surgical treatment in an adult. The preferred treatment is still endoscopic sinonasal surgery with discrepancies of the use or not of intraoperative topical mitomycin or the placement of stents to avoid restenosis. What is not clear is the order of treatment and simultaneity of the procedure with other possible associated pathologies that have a surgical indication. We present a clinical case that meets all these requirements.

Nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants / 中华耳鼻咽喉头颈外科杂志

Objective: To describe and evaluate the surgical effect and prognosis of nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants. Methods: The clinical data of 38 newborns and infants with congenital choanal atresia who underwent nasal endoscopic surgery in Beijing Children's Hospital between January 2016 and May 2018 were retrospectively analysed, including 13 males and 25 females. The age ranged from 5 days to 3 years old at the time of operation (15 cases were newborns). The clinical data, imaging data, treatment effect and prognosis were collected. According to the different surgical methods, the patients were divided into the conventional operation group and the modified mucosal flap technique group. The designs of the modified mucosal flap technique were designed according to the type of congenital choanal atresia. The cross-over L-shaped flaps were performed in patients with unilateral atresia, and the mirrored L-shaped flaps were performed in patients with bilateral atresia. All the patients were followed up for 2-3 years, and the follow-up parameters included the times of operations, length of hospital stay, restenosis rate and incidence of complications. Study data was analyzed using SAS version 9.4 statistical software. Results: Sixteen cases underwent conventional operation while 22 patients underwent modified mucosal flap technique under nasal endoscope. The lightest weight (2 200 g) and the youngest age (5 days) of the patients came from the modified mucosal flap technique group. Compared with the conventional operation group under nasal endoscope, the modified mucosal flap technique group had fewer times of operations (1.14±0.47 vs 2.69±1.20, t=5.552, P<0.001), shorter hospital stay ((7.70±3.22) d vs (14.37±19.16) d, t=2.960, P=0.005), lower rate of postoperative restenosis (9.1% vs 43.8%, χ²=6.156, P=0.013), and lower rate of the incidence of complications (13.6% vs 43.8%, χ²=5.955, P=0.015), the differences were statistically significant. Conclusion: The nasal endoscopic modified mucosal flap technique is feasible for repairing congenital choanal atresia in newborns and infants, which can significantly reduce the incidence of postoperative restenosis and complications.

Atresia congénita bilateral de coanas en una adolescente: reporte de un caso / Congenital bilateral choanal atresia in an adolescelent: a case report

La atresia congénita de coanas se caracteriza por la presencia de placas que obliteran la comunicación entre la cavidad nasal y la nasofaringe desde el nacimiento. Se considera como incompatible con la vida cuando la condición es bilateral. El siguiente es el caso de una paciente femenina, de 17 años, sin síndromes asociados, con obstrucción y descarga nasal anterior bilateral, asociado a respiración oral desde el periodo neonatal. El diagnóstico de atresia bilateral de coanas fue confirmado por medio de endoscopía nasal y tomografía computarizada (TC). La imagen confirmó la presencia de placas atrésicas de composición mixta. La paciente recibió tratamiento quirúrgicamente por vía transnasal con resección de las placas y modelado de neocoana.

Congenital choanal atresia is characterized by the presence of plates obliterating the communication between the nasal cavity and the nasopharynx from birth. If bilateral, this condition is incompatible with life. This following is the case of a 17-year-old female patient, without associated syndromes, with bilateral nasal obstruction and anterior discharge, associated to oral breathing, starting in her neonatal period. The diagnosis of bilateral choanal atresia was confirmed by nasal endoscopy and computed tomography (CT) scan, due to the presence of atretic plates of mixed composition. The patient was surgically treated with plate resection and modeling of a neochoana by means of a transnasal surgical approach.

A novel CHD7 mutation in an adolescent presenting with growth and pubertal delay

Mutations in the CHD7 gene, encoding for the chromodomain helicase DNA-binding protein 7, are found in approximately 60% of individuals with CHARGE syndrome (coloboma, heart defects, choanal atresia, retarded growth and development, genital hypoplasia, ear abnormalities and/or hearing loss). Herein, we present a clinical case of a 14-year-old male presenting for evaluation of poor growth and pubertal delay highlighting the diagnostic challenges of CHARGE syndrome. The patient was born full term and underwent surgery at 5 days of life for bilateral choanal atresia. Developmental milestones were normally achieved. At age 14 his height and weight were

Estenosis de la apertura piriforme y síndrome de incisivo central único: casos clínicos / Piriform aperture stenosis and solitary median maxillary central incisor: clinical cases

El síndrome incisivo central único es un trastorno que involucra anomalías de la línea media. Se puede presentar con dificultad respiratoria poniendo en peligro la vida del recién nacido, debido a malformaciones nasales. Estas malformaciones incluyen atresia de coanas y la estenosis de la apertura del orificio piriforme. Debe pensarse en esta última en todo recién nacido con estridor y dificultad respiratoria de grado variable, asociado a la dificultad de pasar una sonda a través de la región anterior de las fosas nasales. El diagnóstico se confirma por tomografía computarizada del macizo craneofacial y el examen nasofibroscópico. La conducta terapéutica dependerá de la gravedad de los síntomas, pero en general si es que no hay compromiso respiratorio severo, se prefiere una conducta expectante, y esperar el crecimiento craneofacial del niño, para aumentar permeabilidad nasal. Presentamos dos casos clínicos.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include choanal atresia and pyriform aperture stenosis. The last to be thought in any newborn with stridor and respiratory distress associated with the difficulty of passing a tube through the anterior region of the nostrils. The diagnosis is confirmed by craniofacial CT scan and nasolaryngoscope evaluation. The therapeutic approach depends on the severity of symptoms.

Surgical treatment of choanal atresia with transnasal endoscopic approach with stentless single side-hinged flap technique: 5 year retrospective analysis / Tratamento cirúrgico de atresia de coana com abordagem endoscópica transnasalcom técnica de retalho único e articulação lateral sem colocação de stent: análise retrospectiva de 5 anos

Abstract Introduction: Choanal atresia is a rare congenital malformation of the nasal cavity characterized by the complete obliteration of the posterior choanae. In 67% of cases choanal atresia is unilateral, affecting mainly (71%) the right nasal cavity. In contrast to the unilateral form, bilateral choanal atresia is a life-threatening condition often associated with respiratory distress with feeding and intermittent cyanosis exacerbated by crying. Surgical treatment remains the only therapeutic option. Objective: To report our experience in the use of a transnasal endoscopic approach with stentless single side-hinged flap technique for the surgical management of choanal atresia. Methods: A 5 year retrospective analysis of surgical outcomes of 18 patients treated for choanal atresia with a transnasal technique employing a single side-hinged flap without stent placement. All subjects were assessed preoperatively with a nasal endoscopy and a Maxillofacial computed tomography scan. Results: Ten males and eight females with a mean age at the time of surgery of 20.05 ± 11.32 years, underwent surgery for choanal atresia. Fifteen subjects (83.33%) had a bony while 3 (26.77%) a mixed bony-membranous atretic plate. Two and sixteen cases suffered from bilateral and unilateral choanal atresia respectively. No intra- and/or early postoperative complications were observed. Between 2 and 3 months after surgery two cases (11.11%) of partial restenosis were found. Only one of these presented a relapse of the nasal obstruction and was subsequently successfully repaired with a second endoscopic procedure. Conclusion: The surgical technique described follows the basic requirements of corrective surgery and allows good visualization, evaluation and treatment of the atretic plate and the posterior third of the septum, in order to create the new choanal opening. We believe that the use of a stent is not necessary, as recommended in case of other surgical techniques involving the use of more mucosal flaps.

Resumo Introdução: A atresia de coanas é uma malformação congênita rara da cavidade nasal caracterizada pela obliteração completa da coana posterior. Em 67% dos casos a atresia coanal é unilateral, acomete principalmente (71%) a cavidade nasal direita. Diferentemente da forma unilateral, a atresia coanal bilateral é uma condição com risco de vida, frequentemente associada a insuficiência respiratória à alimentação e cianose intermitente exacerbada pelo choro. O tratamento cirúrgico ainda é a única opção terapêutica. Objetivo: Relatar a nossa experiência no uso de uma abordagem endoscópica transnasal com a técnica de retalho articulado de um lado só sem colocação de stent para o tratamento cirúrgico da atresia coanal. Método: Análise retrospectiva de 5 anos dos desfechos cirúrgicos de 18 pacientes tratados para atresia coanal com uma técnica transnasal com um único retalho de articulação lateral, sem colocação de stent. Todos os indivíduos foram avaliados no pré-operatório com endoscopia nasal e um exame de tomografia computadorizada maxilofacial. Resultados: Dez homens e oito mulheres com idade média de 20,05 ± 11,32 anos à cirurgia foram submetidos a cirurgia para atresia de coanas. Quinze (83,33%) apresentavam placa atrésica óssea e três (26,77%) placa atrésica ósseo-membranosa mista. Dois e 16 casos tinham atresia coanal bilateral e unilateral, respectivamente. Não foram observadas complicações intra e/ou pós-operatórias precoces. Entre 2 e 3 meses após a cirurgia dois casos (11,11%) de restenose parcial foram identificados. Apenas um deles apresentou uma recidiva da obstrução nasal e, portanto, foi reparado com sucesso com um segundo procedimento endoscópico. Conclusão: A técnica cirúrgica descrita segue os requisitos básicos de cirurgia corretiva e possibilita boa visualização, avaliação e tratamento da placa atrésica e do terço posterior do septo, a fim de criar a nova abertura coanal. Pensamos que o uso de um stent não é necessário, tal como recomendado no caso de outras técnicas cirúrgicas que envolvem o uso de mais retalhos de mucosas.

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.

Atresia de coana unilateral: o uso de molde na cirurgia da reestenose / Unilateral choanal atresia: the use of cast in surgery for restenosis

Introdução: A atresia de coana (AC) é a anomalia nasal congênita mais comum. Estima-se a incidência de 1 caso para cada 8000-10000 nascidos vivos. Relato do caso: Paciente feminina, 9 anos, com obstrução nasal em fossa nasal direita (FND) desde o nascimento. Apresentava roncos e apneia em vigência de infecção de via aérea superior. À rinoscopia anterior direita, secreção hialina abundante. À videonasoendoscopia flexível não foi possível visualizar cavum pela FND, observando-se estase de secreção em região posterior; à esquerda cavum livre, sem lesão expansiva. Tomografia de nariz e seios paranasais evidenciou atresia de coana mista à direita. Foi realizada cirurgia para correção da malformação. Após 30 dias, observou-se reestenose do cavum. Na reintervenção, a coana foi ampliada com remoção do osso palatino e do septo posterior, realizada dilatação com balão e posicionado molde de silicone. Realizada aplicação de Mitomicina. A remoção do molde foi feita em bloco cirúrgico após 42 dias, com permeabilidade coanal preservada. Nasofibroendoscopia após 6 meses mostrou fossas nasais pérvias. Discussão: Ainda há controvérsias na literatura quanto à efetividade das diferentes técnicas cirúrgicas e ao uso de stents e de agentes antineoplásicos no pós-operatório. Neste relato, a dilatação com balão pode ter contribuído para o sucesso da cirurgia, porém o molde foi fundamental para evitar a reestenose. Considerações finais: O uso do molde mostrou um excelente resultado nesta reintervenção de correção de atresia de coana. A baixa reação inflamatória e o sucesso do pós-operatório mostraram que esta é uma opção a ser utilizada para evitar a reestenose (AU)

Introduction: Choanal atresia (CA) is the most common congenital nasal anomaly. Its incidence is estimated as 1 case for every 8,000-10,000 live births. Case report: Female patient, 9 years old, with obstruction in the right nasal cavity (RNC) since birth. She presented snoring and apnea in the presence of upper airway infection. At right anterior rhinoscopy, abundant hyaline secretion. Flexible videonasoendoscopy failed to visualize cavum through RNC, with secretion stasis in posterior region; left cavum free, without expansive lesion. Tomography of the nose and paranasal sinuses revealed mixed choanal atresia on the right. Surgery was performed to correct the malformation. Thirty days later, restenosis of the cavum was observed. At reoperation the choana was enlarged with removal of the palatine bone and posterior septum, balloon dilatation was performed and a silicone cast was positioned. Mitomycin application was performed. Removal of the cast was done in surgical ward after 42 days, with preserved choanal permeability. Nasofibroscopy at 6 months postoperatively showed permeable nasal cavities. Discussion: There is still controversy in the literature regarding the effectiveness of different surgical techniques and the use of stents and anti-neoplastic agents in the postoperative period. In this report, balloon dilatation may have contributed to the success of the surgery, but the cast was crucial to avoid restenosis. Final considerations: The use of the cast had an excellent outcome in this re-intervention of choanal atresia correction. The low inflammatory reaction and postoperative success have shown that this is an effective option to be used to avoid restenosis (AU)

False Computed Tomography Findings in Bilateral Choanal Atresia

Abstract Introduction Choanal atresia (CA) is a challenging surgical problem defined as a failure in the development of communication between the nasal cavity and nasopharynx. Objective The objective of this study is to describe computed tomography (CT) findings in cases with bilateral choanal atresia. Methods The study involved performing axial and coronal non-contrast CT scanning with 2-3 mm sections on14 neonates that had bilateral CA. We used fiberoptic nasal endoscopy to confirm the diagnosis. We evaluated coronal CT to study the skull base area in such neonates. Results This study included 14 neonates with bilateral CA; with mean age of 7 3.5 days. Mixed atretic plates were found in 12 (85.7%) cases while two (14.3%) had pure bony atresia. Isolated CA was detected in 9 cases (64.3%) and 5 (35.7%) cases had associated anomalies. Coronal CT showed soft tissue density in the nasal cavity that appeared to extend through an apparent defect in the nasal roof (cribriform plate), falsely diagnosed by radiologists as associated encephalocele. At the time of surgical repair, all patients showed thick tenacious mucous secretions in both nasal cavities and revealed no encephalocele. Nasal roof remained intact in all cases. Conclusion The thick secretion of bilateral CA could give a false encephalocele appearance on the CT. It is highly recommended to perform proper suction of the nasal cavity of suspected CA cases just before CT scanning.

Advances in treatment of congenital posterior choanal atresia / 临床耳鼻咽喉头颈外科杂志

Congenital posterior choanal atresia is a rare congenital deformity. Due to the influence of nose and face and intellectual development, its preferred surgical treatment. But surgical treatment methods and technology are in dispute. The using of high resolution display systems combine with micro tools can effectively reduce postoperative scarring and adhesion formation which is the key point to prevent restenosis. There is also the focus of controversy whether should place an expansive tube after operation. Currently nasal endoscopic approach has become the first choice for otolaryngologist treating congenital posterior choanal atresia.

A Case of Bilateral Choanal Atresia without Stenting / 대한이비인후과학회지

Bilateral choanal atresia is a rare disorder characterized by bilateral obstruction of the posterior end of the nasal cavity. It can be present in isolation or associated with multiple disorders such as coloboma, heart defect, choanal atresia, retarded growth, genital hypoplasia, ear abnormalities (CHARGE) syndrome. Because congenital bilateral choanal atresia presents as respiratory distress at birth, immediate diagnosis and adequate treatment is required. Traditionally, using stents was a part of the postoperative treatment to provide a low rate of restenosis but recently it is controversial. Currently nasal endoscopic approach is mainly used with or without stenting. We report a case of CHARGE syndrome with bilateral choanal atresia treated by transnasal endoscopic approach without stenting.

Congenital Complete Arhinia: Report of a Rare Case.

Congenital absence of the nose (arhinia) is extremely rare. Arhinia leads to severe airway impairment and poor feeding in the neonate. Th e degree of nasal absence varies from case to case. Here, we present a case of congenital complete arhinia associated with other craniofacial anomalies. Arhinia leads to severe respiratory distress and feeding problems in newborn warranting emergency management, but our case diff ers as the child was adapted to mouth breathing and naso-gastric tube feeding, giving us the opportunity to delay the defi nitive management for a few weeks yet avoiding the risk of operating in a small child to create nasal airway or tracheostomy on an emergency basis.

Atresia de coanas bilateral em recém nascido: relato de caso / Bilateral choanal atresia in RN: case report

Os autores relatam o caso de um Recém Nascido Pré Termo de 36 semanas, parto cesária, nascido no dia 09/09/2011, em Juiz de Fora - Minas Gerais, peso de nascimento: 1.480 gramas, estatura 43 centímetros, P.C.: 30,5 centímetros, sexo: masculino, Apgar 1° minuto: 2 e do 5° minuto: 8, evoluindo com desconforto respiratório e necessitando de ambu e máscara em sala de parto, em seguida foi levado para o Centro de Terapia Intensiva, sendo entubado e permanecendo em ventilação mecânica. Foi pedido parecer para otorrinolaringologia e realizou-se a Tomografia Computadorizada, onde apresentou sinais compatíveis com Atresia de Coanas Bilateral.

Atresia de coanas, revisión y una mirada desde la evidencia / Choanal atresia, review and a look from the evidence

La atresia de coanas es la anomalía congénita nasal más común. Cuando es bilateral, se presenta con dificultad respiratoria desde el nacimiento. La atresia unilateral se manifiesta con insuficiencia ventilatoria y rinorrea unilateral, pudiendo pasar inadvertida. El diagnóstico se sospecha ante la ausencia de paso de aire en las fosas nasales y la imposibilidad de hacer progresar una sonda nasogástrica. Se confirma mediante examen endoscópico y tomografía computarizada. El tratamiento definitivo es quirúrgico, existiendo diferentes técnicas y vías de abordaje. Se presenta una revisión de la literatura, con especial énfasis en el tratamiento pos-quirúrgico con stents y mitomicina-C a través de una mirada desde la evidencia.

Choanal atresia is the most common congenital nasal anomaly. When bilateral, it presents with respiratory distress at birth. Unilateral atresia is manifested by respiratory failure and unilateral rhinorrhea, and may go along unnoticed. Diagnosis is suspected in the absence of airflow in the nasal cavity and for the inability to advance a nasogastric tube. Diagnosis is confirmed by endoscopic examination and computed tomography. The definitive treatment is surgical, and there are different techniques and surgical approaches. A review of the literatureis presented, with special emphasis onthepost-surgical treatmentwithstents and Mitomycin-Cview from the evidence.

Retrospective Study of a Series of Choanal Atresia Patients

Introduction: Although it has been more than 250 years since the first description of choanal atresia (CA), there are still doubts about this abnormality. The differences between unilateral and bilateral forms are seldom discussed. Objectives: Aggregate data from patients diagnosed with CA, grouping patients with unilateral and bilateral forms. Methods: Retrospective study. Results  Eighteen patients were included: 12 (66.6%) presented bilateral atresia, of which 77.8% were mixed bony-membranous type and 22.2% were pure bony type. From the 12 patients with bilateral atresia, 10 presented related malformations, 3 of whom had CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genitourinary problems, ear abnormalities). From the remaining 6 patients with unilateral atresia, only 2 showed malformations, 1 renal and 1 cardiac. All patients with unilateral atresia needed only 1 surgical procedure, and patients with the bilateral form needed a median of 2.85 interventions (p = 0.003). The median age of surgical procedure in the unilateral group was 6 years, ranging from 6 months to 18 years, and in the bilateral group was 25 days, ranging from 6 days to 6 years (p = 0.003). The median interval between diagnosis and surgery was 9 months in the unilateral group, ranging from 1 month to 18 years, and in the bilateral group was 1 day, ranging from 1 day to 2 months (p = 0.001). Discussion and Conclusions:  Success rates with the endoscopic approach vary from 62 to 100%. Nonetheless, most of these reports present results without considering the number of compromised sides. In our opinion, unilateral and bilateral cases involve distinct patients (taking into account the related malformations), have diverging clinical presentations, and show discrepant restenosis rates and therefore could be considered in different groups of analysis...

Choanal atresia; diagnosis and simple endoscopic repair at remote primary care centers

Choanal atresia [CA] is a rare, congenital malformation resulting as a failure in communication between the posterior nasal cavity and the nasopharynx. The clinical course is often asymptomatic in unilateral CA leading to higher rates of misdiagnosis, in comparison to bilateral CA, which manifests itself as a surgical emergency at birth. Most cases present as isolated malformations, but it may also be associated with other congenital anomalies in 20-50% of cases. Currently, the most important diagnostic tool for CA is computerized tomography [CT] and confirmatory diagnosis is usually achieved with the help of nasoendoscopic examination. Although, different surgical approaches have been used in the past, transnasal endoscopic repair is currently preferred over others. Herein, we describe our experience of three cases and share our simple stentless endoscopic technique, to facilitate physicians working in low facility units for a timely diagnosis and prompt treatment

Transnasal endoscopic choanal plasty for repairing congenital choanal atresia / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To observe the effect of transnasal endoscopic choanal plasty for repairing congenital choanal atresia.</p><p><b>METHOD</b>A retrospective analysis of 15 patients with choanal atresia in Beijing Children's Hospital from January 2006 to September 2012 was made. All the 15 patients accepted electronic nasopharyngoscope examination and CT scanning of the paranasl sinus. The age was between 4 years old and 6 years old. Seven of them were male and 8 were female. Six cases were unilateral atresia, 9 were bilateral atresia. Ten cases (16 sides) were membranous atresia, 2 cases (4 sides) were mixed type atresia and 3 cases (4 sides) were bony atresia. All the patients accepted transnasal endoscopic choanal plasty. A tube was placed in order to support the enlarged nostrils. The nasal tube should be retained for 3 months and kept patency by routine care. Three months after operation, the tube could be removed. The patients were told to do physiological saline nasal irrigation and receive regular review by transnasal endoscopy.</p><p><b>RESULT</b>The patients were followed up for 1 year postoperatively by electronic nasopharyngoscope examination. There was no failure case in all the 15 patients. The total effective rate was 100%.</p><p><b>CONCLUSION</b>Transnasal endoscopic choanal plasty had better efficacy and safety in the treatment of congenital choanal atresia.</p>