Bilateral Congenital Choanal Atresia Research Articles

Practical Tips for Surgical Management of Bilateral Choanal Atresia.

Choanal atresia refers to congenital blockage of posterior choana of nose by the presence of a bony or membranous soft tissue. It causes respiratory distress innewborn which requires emergency surgical intervention. Various surgical methods areavailable for correction of choanal atresia and endoscopic approach is the commonlyused one. However there is risk of re-stenosis after surgery. This article focuses onsurgical refinements to improve the surgical outcome.It is a retrospective study done on eight newborns with bilateral congenital choanalatresia. Data included gestational age, any ante-natal issues, breathing activity at birth, diagnostic tests for choanal atresia and Head to Foot examination findings. Initialdiagnostic work up included CT scan of paranasal sinus and Echocardiography to ruleout associated cardiac anomalies. All the newborns were treated initially in NICU withventilator support and then taken up for endoscopic correction of atresia. After surgery, newborns were successfully weaned off from ventilator.Among the 8 newborns, there were 5 males and 3 females and their gestational agewas full term. (except in one). Initial presentation included respiratory distress on day 1of life with difficulty in inserting feeding tube through the nose. Imaging showedbilateral atresia in 7 newborns and unilateral atresia in one newborn. 5 of themunderwent atresia surgery using endoscopic approach. One newborn required revisionsurgery. Remaining newborns remained symptom free during the follow up period.Currently endoscopic approach remains a safer method for correction of choanalatresia with very minimal chance of re-stenosis. Surgical refinements like adequatewidening of neo-choana and usage of mucosal flaps to cover the raw area has beenfound to improve the surgical outcome.

Evaluation of the factors affecting the necessity for revision surgery in choanal atresia

ObjectiveThis study aims to compare the effects of different surgical techniques for congenital choanal atresia (CCA), and particular emphasis is given to the analysis of the factors affecting the surgical outcome. The necessity for revision surgery and surgical outcomes were retrospectively investigated in patients undergoing revision surgery according to a recently proposed classification system. Material and methodA retrospective study was conducted on patients operated for CCA between January 2007 and December 2018 at a university hospital. Data in the medical records, including patient age when the initial surgery was performed, gender, additional anomalies and medical conditions, the duration of gestation, side and type of atresia, type of surgery and need for revision surgery were reviewed. ResultsForty-five patients treated for CCA in our university hospital were screened. The mean follow-up duration was 82.16 months. Revision surgery was required in 9 of 26 cases, which are included in the study (34.6%). Fourteen patients presented with bilateral CCA, while 12 were unilateral cases. Sixteen patients (61.5%) had comorbid medical conditions. While the removal of vomer and mucoperiosteal flap use affects successful surgical repair, no differences were observed in choanal atresia type, laterality, use of stent and the presence of additional medical conditions. ConclusionThe most important factors affecting surgical success for CCA are the removal of the vomer and closure of all bare bone tissues using a mucoperiosteal flap. Use of stents provides no additional benefit, apart from preventing synechiae formation.

The efficacy of endoscopic plasty for children with bilateral congenital choanal atresia

Objective:To evaluate the clinical effect of endoscopic repair for the treatment of children with bilateral congenital choanal atresia. Methods:A retrospective analysis was conducted in our department that six children who were diagnosed as bilateral congenital choanal atresia by nasopharyngeal endoscopy and nasal CT scanning. All cases underwent endoscopic repair and placed the silicone stent to support the enlarged nostrils for six months, and these patients were followed up to observe re-atresia rate after taking out of the supporting tube. Results:Six cases' nasal ventilation were effectively improved after operation without serious complications, 5 cases showed a satisfactory curative effect after taking out of the supporting tube, only 1 case with bony atresia found choanal re-atresia during follow-up. Conclusion:The clinical effect of using endoscopic repair for children with bilateral congenital choanal atresia showed remarkable efficacy with a low re-atresia.

Endoscopic transnasal repair of 5 cases of bilateral congenital choanal atresia starting with resection of the posterior portion of the septum and vomer

目的： 探讨鼻中隔后端犁骨切除后鼻孔成形术治疗先天性后鼻孔闭锁的疗效。 方法： 回顾性分析深圳市儿童医院2017年3月至2019年6月收治的5例先天性后鼻孔闭锁患儿的资料，其中男2例，女3例；年龄33 d至2岁4个月；2例双侧先天性后鼻孔闭锁（CHARGE综合征），1例单侧后鼻孔闭锁和单侧后鼻孔狭窄（Crouzon综合征）；2例单侧先天性后鼻孔闭锁。所有病例在3.0 mm 0度耳内镜经鼻腔切除鼻中隔后端骨质、去除犁骨、闭锁板骨质并打磨患侧翼内板，连通双侧后鼻孔。术后不放置长期扩张支架。术后2周、1、3、6、12个月常规随访，最长随访超过2年。观察手术疗效。 结果： 5例患儿均恢复良好的鼻腔通气功能，随访期间未见后鼻孔造孔出现再次狭窄和闭锁。无其他异常并发症出现。 结论： 耳内镜下鼻中隔后端、犁骨、闭锁板切除术治疗后鼻孔闭锁可行，且术后可以不放置长期扩张支架，手术疗效确定，造孔无再次狭窄或闭锁。.

Congenital bilateral choanal atresia- Endonasal endoscopic surgery- Report of two cases

Congenital choanal atresia (CCA) is the development failure of the nasal cavity to communicate with nasopharynx. It is an uncommon congenital anomaly of nose with an incidence of approximately 1 in 5000-7000 live births. Choanal atresia is caused by failure of resorption of the nasobuccal membrane during embryonic development. Choanal atresia has a significant association with CHARGE syndrome. Surgical intervention is recommended in the first weeks of life in bilateral cases because this is a life threatening condition. Two cases of congenital bilateral choanla atresia (CCA) was admitted in the department of Otolaryngology & Head-Neck Surgery at Bangabandhu Sheikh Mujib Medical University (BSMMU) with the complaints of intermittent attacks of cyanosis, dyspnea, respiratory distress and history of inability to take feeds. On examination both babies had respiratory distress, mucoid discharge from both nasal cavities. A soft rubber catheter was passed into both nares revealed the diagnosis of bilateral CCA which was confirmed by flexible nasoendoscopy. Bilateral CCA was managed with a nasopharyngeal airway. Surgery is the definitive treatment with two main approaches namely transnasal and transpalatal. We discuss successful management of two neonates with bilateral CCA by endonasal endoscopic approach.
 Bangladesh J Otorhinolaryngol; April 2018; 24(1): 85-89

Bilateral Congenital Choanal Atresia in a Female Neonate: Importance of Bedside Diagnosis

Bilateral Congenital Choanal Atresia in a Female Neonate: Importance of Bedside Diagnosis

Supposed otolaryngology emergency which presents late: Bilateral congenital choanal atresia

Supposed otolaryngology emergency which presents late: Bilateral congenital choanal atresia

Bilateral congenital choanal atresia in a 16-year old girl at Muhimbili National Hospital, Tanzania

Background: Bilateral choanal atresia refers to a congenital anomaly whereby a child is born with bilateral imperforate posterior nares. In most cases the diagnosis is established soon after birth. Establishment of the diagnosis requires a high index of suspicion by clinicians. The objective was thus to report an unusual case which went undiagnosed for many years and review the few available literatures.Case presentation: A 16-year old girl presented to the otorhinolaryngology clinic of Muhimbili National Hospital in Tanzania with a long standing history of bilateral nasal obstruction. The condition was reported to be present since birth and this was accompanied by mouth breathing and persistent bilateral nasal discharge. The patient also reported a history suggestive of respiratory distress after birth which necessitated oxygen therapy and difficulty in breastfeeding where she was kept on expressed breast milk during the first few weeks of life. Nasal endoscopy was done clinic and the diagnosis of bilateral congenital choanal atresia was made. Computerized tomography scan of the nose and paranasal sinuses revealed bilateral choanal atresia of mixed type. Surgical perforation of the atretic choanae without stenting was done endoscopically under general anaesthesia.Conclusion: Bilateral congenital choanal atresia is an otorhinolaryngological emergency and should be established promptly both in resource limited settings and in areas where advanced tools are in situ to prevent its morbidity and mortality if it goes unestablished.

Completely Endoscopic Approach Using a Skeeter Drill to Treat Bilateral Congenital Choanal Atresia in a 33 Week Born Pre-term Baby

Choanal atresia (CA) is a relatively rare condition manifesting with respiratory distress. Endoscopic approaches have superseded transnasal and transpalatal approaches. We present a case of a premature baby of 1.10kg, who developed respiratory distress and was diagnosed with bilateral CA. A nasal airway was created endoscopically using a skeeter drill.

Comprehensive management of congenital choanal atresia

ObjectivesTo present results of a one-stage minimally invasive surgical procedure for congenital choanal atresia (CCA). Seven outcome measures were applied. Materials and methodsRetrospective study conducted between 1999 and 2015. The same endonasal endoscopic approach with multiflaps and no stenting was used on 36 children. The flaps were attached with fibrine glue. There were 50% unilateral and 50% bilateral cases, 70% primary and 30% secondary surgery. The mean age at primary surgery for bilateral atresia was 10 days and for unilateral atresia 4 years. Associated loco-regional disorders were: hypoplasia of the inferior turbinate, rhinopharyngeal stenosis and rhinopharyngeal web. ResultsThe average follow-up time was 6 years, ranging from 1 to 14 years. There was a functionally patent choanae in 94% of children, and 6% showed severe restenosis with a diameter less than 4 mm, which needed one revision surgery each. Charge patients were not associated with worse outcome. There was no external nasal valve stenosis and no permanent Eustachian tube dysfunction. Synechiae occurred in 3 patients with hyperplastic inferior turbinate. No patients showed any disharmonious nasal growth. In neonates with isolated bilateral CCA, breast-suction could be started within 1 day (range 1–2 days), and pain-killers were needed on average for 1.5 days (range 1–4 days). The hospital stay for unilateral isolated CCA was on average 1.5 days (range 1–2 days) and for bilateral isolated CCA, 8 days (range 3–20 days). Postoperative procedures under a short general anesthesia were necessary in 12 cases, 10 of them were infants under 6 months of age. ConclusionSurgery could be performed safely in the newborn in the early stage of life, even for unilateral atresia. Tendency for restenosis can be minimized by: 1. the construction of an as large as possible uni-neochoanae by removing the posterior part of the vomer and by drilling away the medial pterygoid; 2. in case of rhinopharyngeal stenosis, part of the endochondral clivus bone should be resected; 3. all raw surfaces should be covered by multiple mucosal flaps secured with fibrin glue; 4. no stenting; 5. appropriate postoperative care.

Bilateral Complete Congenital Choanal Atresia in an 18-month-old Female Child with Rare Clinical Presentation: Transnasal Endoscopic Repair with Stent

ABSTRACT Congenital choanal atresia (CCA) is the developmental failure of the nasal cavity to communicate with the nasopharynx. Surgical repair is recommended in the first weeks of life in bilateral congenital choanal atresia (BCCA), because this is a life-threatening situation in newborns as they are obligate nose breathers. This article reports on an 18-month-old female child, with delayed presentation of bilateral choanal atresia, treated successfully endoscopically and stent placement postoperatively. Case report The patient is an 18-month-old female child, born full term, by cesarean section (for cephalopelvic disproportion) with birth weight 3,750 gm to a 30-year-old primigravida. There were no immediate prenatal or postnatal medical or surgical problems in the mother. The child cried immediately after birth, there was no postnatal respiratory distress, cyanosis, or any major hospital admission. On examination, microcephaly (head circumference 46 cm below 2 standard deviation of the mean), hypertelorism, ani-Mongolian slanted eyes, left side cleft lip with high arched palate with malocclusion were observed. She presented late with c/o nasal discharge and mild breathing difficulty on breastfeeding. The catheter test revealed the diagnosis of BCCA. Noncontrast computed tomography (CT) scan nose was done, which showed nonposterior choana blocked with bony plates on both the sides (Fig. 1). The paranasal sinuses, orbit, and anterior nasal cavities were within normal limits. Transnasal endoscopic repair was done under general anesthesia. Endoscopy revealed a bony atresia (Fig. 2), which was drilled out keeping the direction medially and inferiorly. Feeding tubes were fashioned as stents and placed for a period of 6 weeks. There were no postoperative complications. The patient was referred to pediatric surgery for other problems. Endoscopy confirmed bilateral patency at 12 weeks postoperatively. How to cite this article Mohindra S, Gupta AK. Bilateral Complete Congenital Choanal Atresia in an 18-month-old Female Child with Rare Clinical Presentation: Transnasal Endoscopic Repair with Stent. Clin Rhinol An Int J 2016;9(3):153-154.

Endoscopic management of congenital choanal atresia: Second look is mandatory

Congenital choanal atresia (CA) is a rare abnormality characterized by unilateral or bilateral obstruction of the posterior end of the nasal cavity. With an incidence of 1:1000 to 1:8000 births, it is more in female than in males and unilateral more common than bilateral. Surgical procedures aim to provide adequate functional choanal patency and a low rate of rest enosis, with shorter surgery and hospitalization times, to minimize morbidity and mortality, this study done to evaluate the effectiveness and safety of endoscopic nasal surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia and to emphasis in the important of second look procedure at the time of removal of stent to assess the wideness of neochoana, and to remove any granulation tissue ,or polyps, it involved 13 patients, 10 with unilateral CA and 3 bilateral CA, all involved in surgery and second look.Bangladesh J Otorhinolaryngol; October 2015; 21(2): 76-79

Endoscopic repair of bilateral choanal atresia, starting with vomer resection: Evaluation study.

Choanal atresia is the most common inborn nasal anomaly, and its definitive treatment is surgical to achieve proper bilateral nasal patency. Four main surgical approaches were used for repair: transantral, transpalatal, transseptal, and transnasal. The transnasal endoscopic approach is the most widely accepted nowadays. In this study, we aimed to evaluate the long-term results of a transnasal endoscopic repair of congenital choanal atresia started with resection of the posterior portion of the vomer and was completed without the use of stents in a large series of patients. Twenty-five patients (age range, 3-15 days) with bilateral congenital choanal atresia had surgery by using stentless endoscopic transnasal repair by starting with resection of the vomer bone. Postoperative control included office fiberoptic nasal endoscopy. During the follow-up period of 15-66 months (mean [standard deviation], 35.76 ± 16.8 months), 18 patients (72%) had a wide choana with adequate nasal breathing; Six patients (24%) had narrowing of the choana (<50%), still with adequate and satisfactory airway without feeding difficulties, and one patient (4%) developed restenosis (>50%) after 7 months, which necessitated repeated surgery. One patient (the fourth case) developed a palatal defect, which healed conservatively. No other complications were detected. The described technique proved to be effective and easier, with good long-term satisfactory results in a large series of patients. This technique allows early use of both nasal passages for simultaneous endoscope and instrument insertion to excise both atretic plates without the use of stents or flaps.

Endoscopic bilateral congenital choanal atresia repair of 112 cases, evolving concept and technical experience

ObjectiveThis study aims to present 18 years’ experience with endoscopic treatment of bilateral congenital choanal atresia as regard to management concept, surgical technique, results, pitfalls, and complications. Patients and methodsA retrospective study including 112 cases of bilateral congenital choanal atresia, treated at Mansoura University Hospital endoscopically in the period from January 1998 to March 2015. As far as we know, this is the largest study group on transnasal endoscopic choanal atresia repair in literature. ResultsOne hundred and twelve infants (87 females, 25 males) were included in the study. Age at operation ranged between 1 day and 28 days (8.75 days in average), and body weight average was 2.76kg. All patients were diagnosed at birth except 3 infants. In this study, 85 cases were mixed atresia, 25 cases were bony atresia, and only two cases were membranous. In all cases, obliterated choana bone and vomer bone was removed, lateral wall drilling was used in 33 cases. Follow up ranged between 6 months and 18 years (95.6 months in average). The most common complication was restenosis, occurred in 42% (47 cases). Second-look procedure was done in 68 cases. The need for second-look evaluation with stent group was 74.5% (62 out of 83 infants), whereas in non-stent group was 20.6% (6 out of 29 infants). ConclusionEndoscopic repair of bilateral choanal atresia is a safe, effective technique with minimal complication. Usage of 30 degree sinuscope permits better visualization and higher accessibility for the surgical instruments. Surgically formed wide single neochoana with removal of all intervening tissue surroundings, and good follow up permit higher success rate without stenting. Advanced learning curve permits tailoring the perfect surgery with minimal tissue injury and better outcome. Post-operative choanal dilatation using esophageal dilators under endoscopic examination decrease the need for stenting and second-look evaluation.

Congenital bilateral adult choanal atresia undiagnosed until the second decade: How we did it.

Background:Bilateral congenital choanal atresia that presents in adulthood is rare. There are only eight reported cases in the literature.Method:We present a ninth case of adult bilateral choanal atresia diagnosed at the age of 20 years. Can a bilateral choanal atresia present so late in life when such a condition is considered incompatible with life?Results and conclusion:Clinical details, diagnosis, and surgical steps with a clear depiction of photographs and comparison with all other previously reported cases, which can help novel otolaryngologists in their clinical practice are discussed.

To stent or not to stent? A meta-analysis of endonasal congenital bilateral choanal atresia repair.

The use of nasal stents as a postoperative adjunct following repair of choanal atresia remains controversial. The study objective was to systematically review the literature regarding the efficacy and safety of stenting following transnasal endoscopic repair of bilateral choanal atresia. Systematic review with meta-analysis. A comprehensive search in PubMed, EMBASE, CINAHL, and the Cochrane Library was conducted. Inclusion criteria included articles written in the English language with five or more subjects and clear intervention data and outcomes. Two independent reviewers screened studies for eligibility, appraised the level of evidence, extracted data, and resolved discrepancies by consensus. Successful surgery was defined as the absence of restenosis. Of 154 identified studies, 15 met inclusion criteria. Levels of evidence varied from level 3 to 4. Mean age at surgery ranged from 5 days to 25 months. Thirteen studies (n = 167 patients) included patients who were stented; the weighted pooled proportion of successful surgery was 65% (95% confidence interval [CI], 49-76%). Mean duration of stenting ranged from 48 hours to 16 weeks. Six studies (n = 42) evaluated patients who were not stented; the weighted pooled proportion of successful surgery was 64% (95% CI, 42-84%). Complications associated with stenting included alar injury, vestibular stenosis, columellar tear, and stent dislodgement or blockage. Success rates for bilateral choanal atresia repair were similar with and without the use of nasal stents. The use of nasal stents may be associated with more complications. There is insufficient data to determine if mitomycin C is a useful therapeutic adjunct.

Adulthood Presentation of Bilateral Complete Congenital Choanal Atresia with other Congenital Anomalies: A Case Report with Review of Literature

ABSTRACT Bilateral choanal atresia is an otorhinolaryngology emergency. It is a potentially life-threatening disorder because the affected newborn is an obligate nose breather. We report clinical and radiological evaluation of a patient with congenital bilateral choanal atresia, who presented at the 20-year of age with other congenital anomalies. Patient was diagnosed as congenital bilateral choanal atresia, right accessory nasal deformity, high arched palate with congenital phthisis bulbi of his left eye. He was posted for transnasal endoscopic surgery under general anesthesia. Unique feature of this case is left congenital phthisis bulbi along with bilateral congenital choanal atresia probably the first case report having both anomalies in a single patient. While performing surgery, we should carefully study the anatomy of the basisphenoid, as in our case we did not attempt to correct atresia on right side. How to cite this article Agarwal VK, Bist SS, Mishra SK, Bharti B. Adulthood Presentation of Bilateral Complete Congenital Choanal Atresia with other Congenital Anomalies: A Case Report with Review of Literature. Clin Rhinol An Int J 2015;8(1):24-26.

Patients With Congenital Choanal Atresia Treated by Transnasal Endoscopic Surgery

Congenital choanal atresia (CCA) is defined as a congenital failure in the development of communication between the nasal cavity and nasopharynx in newborns. It is the most common congenital anomaly of nose. Most of the patients presented with unilateral CCA. Neonates with bilateral CCA have severe respiratory distress after birth. Airway control with orotracheal intubation should be done immediately to save the life of neonate with bilateral CCA. After airway control, surgery should be performed as soon as possible. The aim of surgery is to create a patent nasal passage and to prevent restenosis with minimal morbidity. Transnasal endoscopic surgery of CCA has become the most popular treatment method. The retrospective review of 33 patients with CCA was presented in this study. The patients who were diagnosed at the neonate period and operated on in 6 months after birth were grouped as I (neonate). The patients who were diagnosed 6 months after birth were grouped as II (child-young adult). All patients were treated by transnasal endoscopic surgery. Stent were put in all patients to prevent restenosis. Although the restenosis rate was higher in group I (53.8%) than in group II (23.1%), it did not cause a statistically significant difference. Overall success rate of our surgical approach was 61.5%. Despite the surgical developments and many treatment modalities, restenosis is still the most challenging problem in CCA.

Congenital Bilateral Choanal Atresia with Duodenal Atresia and Lagophthalmos

ABSTRACTBilateral choanal atresia is an uncommon congenital malfor mation and its association with duodenal atresia and lagophthalmos is very rare. It is a lifethreatening condition where immediate intervention is always needed. A systemic examination is required to find its association with other anomalies. Though different approaches for its treatment are described endoscopic transnasal approach is the most acceptable less traumatic approach till date. We are having a case report of a premature baby with choanal and duodenal atresia with lagophthalmos and was managed successfully with endoscopic transnasal approach.How to cite this articleGupta S, Pradhan S. Congenital Bilateral Choanal Atresia with Duodenal Atresia and Lagophthalmos. Int J Otorhinolaryngol Clin 2014;6(3):137139.

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. DOI: http://dx.doi.org/10.3329/bjms.v13i1.14454 Bangladesh Journal of Medical Science Vol. 13 No. 01 January2014: 91-94