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.

Bilateral choanal atresia – An unusual case of severe respiratory distress in a newborn without nasal flaring

Respiratory distress in a newborn can be due to various causes and some need active intervention. Choanal atresia (CA) is a rare congenital anomaly with its incidence estimated to be 1 case/5,000–8,000 births. It is characterized by narrowing or blockage of the nasal passages. It is important to make an early clinical diagnosis with emphasis on timely management as it can be life-threatening. The pediatrician may be not able to pass a feeding tube through the neonate’s nostril even on repeated attempts. Detailed evaluation should be performed for the CHARGE association. High-resolution computed tomography can aid the diagnosis and transnasal endoscopic surgery is the preferred treatment modality. Here is a case report of a term neonate born with severe respiratory distress who was diagnosed to have bilateral CA on evaluation and managed with nasal endoscopic surgery.

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.

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.

Endoscopic Transnasal Repair of Bilateral Choanal Atresia in Neonates: Our Experience

Introduction: Congenital choanal atresia is the developmentalfailure of the nasal cavity to communicate with nasopharynx.The newborn baby presents with intermittent attacks ofcyanosis and respiratory distress soon after birth. Inabilityto pass nasal catheters in both the nares reveals the diagnosisof bilateral Choanal Atresia. Study aimed to present ourexperience with a endoscopic approach for transnasal repairof choanal atresia.Material and Methods: Seven patients with mean age 7 dayswith bilateral choanal atresia,underwent endoscopic repairusing a mucoperichondrial flap from the nasal septum. Thebony stenosis was opened with a surgical curette or drill, andthe raw surface was covered by the flap.Results: A total of 7 choanae were operated. With meanfollow-up 27 months. Out of seven patients operated by us,five patient survived with a patent choana.We had two deaths,one patient expired during the procedure due to bleeding andone patient expired three hour after the procedure due to CCF.Conclusion: Endoscopic repair of choanal atresia is a safe andrapid procedure.

Impact of Successful Choanal Atresia Repair on the Nasal Mucosa: A Preliminary Study

Abstract Introduction Themain histological features of the nasalmucosa in choanal atresia are distorted cilia, marked increase of mucous submucosal glands associated with marked reduction of goblet cell density, and lymphocytic cellular infiltration. Objective To study the nasal mucosal changes in cases of choanal atresia after successful repair compared with pre-repair mucosal histological features. Methods Tissue samples were taken from the inferior turbinate of 3 patients (1 bilateral and 2 unilateral) who were successfully operated. Then, the biopsies were subjected to histopathological, histochemical and immunohistochemical studies. After that, the results were compared with pre-repair findings in the choanal atresia side and in the normal side. Results Four biopsies (4 repaired choanal atresia sides) of the mucosa of the inferior turbinate revealed that 1 patient (who had a bilateral choanal atresia repaired), after achieving a patent choana for 8 months, had not completely recovered a normal nasal mucosa. The other 2 patients, after 18 and 23 months of achieving a patent choana, showed normal nasal cavities. Conclusion The main histological features of the nasal mucosa in choanal atresia could be reversed by surgery, making the patients regain their choanal patency, with their mucosae changing back to normal gradually with time.

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 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.

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.

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...

Isolated Congenital Bilateral Choanal Atresia and Nasopharyngeal Atresia- a case report.

A term neonate developed respiratory distress, paradoxical cyanosis (relieved by crying) soon after birth. Inability to insert No.5 French infant feeding tube through the nose into the pharynx led to the diagnosis of Bilateral Choanal Atresia , which was confirmed by HRCT Scan of the nose. Insertion of an oropharyngeal tube reduced the respiratory distress. Extensive investigations did not reveal any other congenital anomaly. The baby was treated with Transnasal Surgery.

Analysis of respiratory problems in CHARGE syndrome: a single center study

PURPOSE: CHARGE syndrome consists of multiple malformation including coloboma, heart defect, choanal atresia, growth or developmental retardation, genital anomalies, and ear anomalies. The aim of this study was to evaluate the respiratory problems in children with CHARGE syndrome. METHODS: Out of 9 patients with CHARGE syndrome, medical records from 8 patients showing respiratory distress or respiratory failure were retrospectively reviewed. We investigated the causes of respiratory problems by physical examination, endoscopy, echocardiogram, computed tomography, rigid bronchoscopy, swallowing test, and 24-hour impedence monitoring. RESULTS: Five patients required endotracheal intubation soon after birth due to bilateral choanal atresia (n=2) and congenital heart diseases (n=3). Three patients were intubated within a month because of surgery for complex heart diseases (n=2) or recurrent apnea (n=1). Tracheostomy was performed in 3 patients who showed primary or secondary subglottic stenosis. Among 8 patients who had aspiration or respiratory distress after feeding, cricopharyngeal incoordination and gastroesophageal reflux disease were found in 7 and 2 children, respectively. One patient died of aspiration during oral feeding. CONCLUSION: Patients with CHARGE syndrome manifest respiratory distress or failure due to various causes including congenital anomaly in the airway, cardiac anomaly, neurologic or gastrointestinal problems. Therefore, pediatricians should be alert to the respiratory symptoms and signs in CHARGE syndrome and take active intervention from the birth to improve their long-term prognosis.

Tratamiento quirúrgico de la atresia de coanas: caso clínico / Surgical repair of choanal atresia

La atresia de coanas es la alteración congénita más frecuente del desarrollo nasal. Puede ser unilateral o bilateral, y según eso, la clínica puede aparecer desde el momento del nacimiento, o bien permanecerá asintomática hasta la edad adulta. En el neonato, si es bilateral, se manifiesta por una disnea inspiratoria y una cianosis cíclica al lactar, que se alivian con el llanto; y en el adulto, la clínica es de insuficiencia respiratoria nasal unilateral, con rinorrea. El tratamiento es quirúrgico, con varias vías posibles de abordaje, siendo la vía endoscópica la más utilizada hoy en día. La complicación más frecuente tras la cirugía es la reestenosis. Presentamos el caso de una paciente con atresia unilateral, que fue intervenida con éxito en nuestro hospital, y describimos la técnica.

Choanal atresia is the most frecuent congenital anomaly of nasal development. It can be unilateral or bilateral, and according to it, it will give symptoms from the moment of the birth, or will remain asymptomatic up to the adult age. In the newborn it demonstrates for an acute respiratory distress and a cyclic cyanosis by feeding, that relieve with crying; and in the adult, the clinic is of unilateral nasal obstruction, with rhinorhea. The treatment is surgical, with several possible surgical approaches. Transnasal endoscopic repair is the most used nowadays, because it's a safe and successful technique, and reestenosis is the most frequent complication. We present the case of a patient with unilateral atresia, that was operated in our hospital, and we describe the technique used.

Progress in diagnosis and treatment of neonatal upper airway obstruction / 国际儿科学杂志

The most common etiology of neonatal upper airway obstruction is congenital abnormalities,including choanal atresia,Pierre Robin sequence,occupational lesions,laryngomalacia,and so on.Some patients are after birth with dyspnea,severe cases with suffocation,and even death.Due to lack of specific clinical manifestations in early neonatal periode,it is usually misdiagnosed as other respiratory diseases.Therefore,early identification of the etiology,as soon as possible to relieve the obstruction,is particularly critical in the prevention of suffocation for the upper airway obstruction in the neonatal period.

Management of congenital choanal atresia (CCA) after multiple failures: A Case Report

Nasal obstruction in neonates is a potentially fatal condition because neonates are obligatory nasal breathers. Bilateral choanal atresia is therefore a neonatal emergency. Several approaches for corrections of choanal atresia are available including the helium laser: YAG. A 5-year-old Chinese girl born with bilateral choanal atresia, had birth asphyxia that required intubation. She underwent multiple surgeries for correction of choanal atresia at other hospitals but failed to improve. She was referred to Universiti Kebangsaan Malaysia Medical Center (UKMMC) after presenting with intermittent respiratory distress and cyanosis following an upper respiratory tract infection. A repeat computed tomography (CT) scan done preoperatively showed complete bony stenosis over the left choana and finding was confirmed by examination under general anesthesia. She underwent endoscopic transnasal removal of left bony atretic plate. There was no intra or postoperative complications. During follow up 10 years later, the airway on both sides remains patent.