|Year : 2019 | Volume
| Issue : 1 | Page : 9-12
Choanal atresia, risk of failure
Abdullah Alhelali, Abdulsalam Alahmari, Sulaiman Alajlan, Alammar Ahmed Y
Department of ENT, King Saud University Medical City, Riyadh, Saudi Arabia
|Date of Web Publication||6-Feb-2020|
MD Abdullah Alhelali
Pediatric Otolaryngology fellow, Department ENT, King Saud University Medical City, Riyadh, P.O. Box 25052, Riyadh 12642
Source of Support: None, Conflict of Interest: None
Background: Choanal atresia (CA) is a rare congenital anomaly that is characterized by posterior nasal choanal obstruction either unilateral or bilateral. Endonasal endoscopic repair is the current modality of treatment. Restenosis is still a common concerning complication post-surgery. In this study, we studied the impact of many factors on the recurrence rate.
Objectives: This study aimed at evaluating the success rate of endonasal endoscopic repair of choanal atresia and the influence of many factors on the recurrence rate: age at intervention, laterality, use of mitomycinC , presence of syndromes and adenoid hypertrophy.
Design: Retrospective study by chart review
Settings: King Saud University Medical City, Riyadh, Saudi Arabia.
Material and Methods: We considered all cases of choanal atresia operated in our center for the period from January 2014 to May 2017. We included only the primary cases (operated for the first time) in this study. We excluded any case that was operated on before in any other center, missed to follow-up or with incomplete medical records.
Results: The sample comprised 22 cases of CA, with an equal distribution of males and females. Four of the patientsexperienced recurrence(recurrence rate 18%), and the mean follow-upperiod was 21.3 months. The recurrence rate was higher in syndromic patients (2 out of 3 versus 2out of 19 nonsyndromic). Mean age at intervention in successful cases was 17 months and in the recurrence group was 7.15 months. Unilateral cases were more common than bilateral (14 versus 8). The rate of recurrence was 10% in themitomycin group and 25% in the nonmitomycin group.
Conclusions: The recurrence rate of post-choanal atresia repair was 18% and recurrence in syndromic patientswas higher in this study.
Keywords: Choanal atresia, congenital nasal obstruction, endoscopic endonasal repair
|How to cite this article:|
Alhelali A, Alahmari A, Alajlan S, AlammarA. Choanal atresia, risk of failure. Saudi J Otorhinolaryngol Head Neck Surg 2019;21:9-12
|How to cite this URL:|
Alhelali A, Alahmari A, Alajlan S, AlammarA. Choanal atresia, risk of failure. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2023 Jun 4];21:9-12. Available from: https://www.sjohns.org/text.asp?2019/21/1/9/277844
| Introduction|| |
Choanal atresia is a well-known cause of congenital nasal obstruction and is a rare disease with an incidence of 1:5000-7000 human births. Four theories of choanal atresia have been described over the years:(1) persistence of the buccopharyngeal membrane from the foregut; (2) abnormal persistence or location of the mesoderm forming adhesions in thenasochoanal region; (3) abnormal persistence of the nasobuccal membrane of Hochstetter, and (4) misdirection of neural crest cell migration.
Most congenital choanal atresia cases(two-thirds) are unilateral, and the majority (70%) have mixed bony- membranous choanal obliteration. Approximately 50% of all patients may have associated congenital comorbidities, such as CHARGE or Crouzon syndromes.
Choanal atresia is usually diagnosed by a pediatrician early during the screening of the child after delivery. Clinical evaluation by history and physical examination is highly helpful. Insertion of 6 or 8 Fr suction catheter will provide insight into the etiology and the side of the obstruction. Flexible nasal endoscopy will give direct visualization of the point of obstruction in the nasal cavity and can be used to visualize the atretic plate. Definitive evaluation is achieved with thin-slice CT scan of the sinuses.
Bilateral CA presents with dyspnea and cyclic cyanosis, which are relieved by crying, because the child is an obligatory nasal breather in the first weeks of life. Such a situation necessitates an emergency intervention to secure the airway by oropharyngeal airway, orotracheal intubation or tracheostomy. Infants with unilateral CA rarely present with respiratory symptoms and are often diagnosed later on. In these cases,it is preferable to delay the definitive management as the chance of successful repair is higher.
Surgical repair is the definitive management of CA. Many approaches have been described, although the endoscopic trans-nasal approach is the most widely used and accepted [7, 8].The success rate of this approach is high. Various studies have reported primary repair success rates ranging from 67 to 88%.The mean success rate oftrans-nasal endoscopic repair was 85.3% out of a total of 238 cases in a meta-analysis of 20 studies. Many risk factors of restenosis have been described in the literature. These factors include nasopharyngeal reflux, gastroesophageal reflux, age <10days, bilateral CA with purely bony atretic plate, and the presence of associated malformations [6, 11-13].
In this study, we studied the recurrence rate in our center and measure the risk factors that may influence the success rate. We studied the effect of age, laterality, syndromes, and mitomycin C use.
| Materials and Methods|| |
This study was conducted at the Department of Otolaryngology at King Saud University Medical City, Riyadh, Saudi Arabia, after approval for this research was granted by our hospital’s institutional review board. We collected data from all primary choanal atresia cases that underwent surgical intervention during the period from January 2014 to May 2017. All cases were reviewed retrospectively using paper and/ or computerized records. We included only the primary cases (first-time procedures). We excluded any case that was operated on before in any other center, missed to follow-up, or had incomplete medical record files.
In all patients, we used endoscopic endo-nasal techniques. We started with draping and nasal packing with a pledget soaked in 0.05 xylometazoline hydrochloride and then injection of lidocaine with diluted adrenaline (dilution 1:200,000) at the posterior septum and atretic plate. The opening of the choana(e)was initiated at the most inferior-medial part of the atretic plate with suction or freer . After the confirmation of nasopharynx, we dilated this opening with a microdebrider. Backbiting forceps were usually used for the posterior septectomy, and then a small burr drill was used to drill any remnant of thick bone and for lateral drilling of the pterygoid plate. We used Mitomycin C randomly in alternating cases. Mitomycin C was applied locally for a total of 4 minutes by a pledget soaked in 4 mg/ml.
Postoperatively, patients were typically discharged the next day on nasal saline irrigation and local steroid drops or spray for 4 weeks and nasal decongestant for 3 days, in addition to antireflux and pain medication. The patients werefollowed upthe next week and then monthly.
All patients were documented to be free from any related nasal symptoms, and patent and wide choanae were considered to confirm successful cases. Symptoms of nasal blockage and discharge relevant to documented restenosis of the choana posteriorly were considered to reflect failed procedures.
| Results|| |
Twenty-two cases of CA were included in this study,males to femaleswere equally distributed (50% each). Fourteen (63.63%) cases had unilateral choanal atresia,and 8 (36.36%) caseshadbilateral atresia. From the sample of 22 cases, there were 19(86.36%) nonsyndromic cases and 3(13.63%) cases with syndromes (2 CHARGE and 1 Down Syndrome). We used MMC in 10 cases (45.45%) and did not apply MMC in 12 (54.54%) cases. In 5 (22.72%) patients, there was an enlarged adenoid, and the patientunderwent adenoidectomy at the same operation. The rate of recurrence in this study was 18.2% (4 cases out of 22);Graph 1.
There was no significant difference in the distribution of mean age at intervention between the recurred and nonrecurred groups (p=0.2). The mean (±SD) age of those with recurrence was 7.15(±7.45) months, and the mean (± SD) age of those without recurrence was 27.27 (± 31.4) months. There was a difference observedin the age of cases; the age range of those with recurrence was 8 days to 15 months (median=6.7months),while the age range of those without recurrence was 10 days to 8 years (median=12 months);[Table 1]. There was no difference in recurrence rate between the cases that received MMC application at the end of the procedure and those that did not (pvalue=0.5); [Table 2]. The recurrence was higher in syndromic cases; 2 out of 3 cases (66.7%) hadrestenosis. The recurrence rate of the nonsyndromic group was 10.5% (2 out of 19) (pvalue=0.07);[Table 2].
|Table 2: Effects of MMC, syndromes, adenoidectomy, and laterality on recurrence.|
Click here to view
Adenoidectomy was done in 5 patients at the same operation without any impact on the recurrence rate. Two of these patients had restenosis (40%), while the nonadenoidectomy group had a restenosis rate of (11.8%) (pvalue=0.2); [Table 2].
The restenosis rate was higher in bilateral cases (37.5%) than in unilateral cases (7.1%); however, this difference was not significant (pvalue=0.1); [Table 2].
| Discussion:|| |
Since endonasal endoscopic repair of choanal atresia was implemented for the first time by Stankiewicz, it has become the most widely performed approach. This approachadds many benefits, such as excellent direct vision and illumination and magnification, and more importantly,has improved the safety of the procedure by avoiding palatal, dental and skull base trauma as wellby reducing operative time [2, 3]. The goal of such surgery is to prevent functional complications of the nose and upper jaw and reduce the necessity for revision surgery.
The most challenging part of CA repair, especially in neonates,isthe narrow nasal cavity, and in some cases,deviation of the nasal septum, turbinate enlargement or an arched palate.
Restenosis has been reported to be greaterin congenital bilateral bony atresia cases and especially in caseswith CHARGE syndrome [11, 15]. Malformations of the rhinopharynx were reported previously in patients with CHARGE, and one study found that half of syndromic patients with CA had rhinopharyngeal vertical stenosis posterior to the atreticchoanae. Which may influence the outcome. In our study, apparently, we have higher recurrence rate in syndromic patient. Two out of three syndromic cases had recurrence, while, 2 out of 19 non-syndromic had recurrence (66.7% versus 10.5%).
Postoperative care is crucial, with frequent cleaning and follow up are important to prevent recurrence[! 11]. Extra-esophageal reflux control has been advocated in some studies [1, 10, 11], and saline irrigation and careful aspiration and steroid application have also been recommended [11, 14, 17, 18].
In our center and many other centers as well have abandoned the use of stents due to their side effects and the lack ofclear evidence of added benefits. Some studies reported higher recurrence rate with stenting [11, 15, 18-20]. Stents were also reported to induce many complications, such as granulation tissue formation, intranasal inflammation and infection, ulceration or erosion of the nares, columellar necrosis, septal injuries or perforation, dislodgement, discomfort and pain [2, 10, 21-23]. These findings and the morbidity and inconvenience of these stents for the child and the family represent strong arguments against their use. Mitomycin C is an antiproliferative and antitumor antibiotic agent that inhibits fibroblast growth and proliferation. It has been used to reduce the inflammatory process, to prevent scarring and to improve surgical outcomes. Controversial reports havebeen published, with some showing no difference in outcomes afteradding MMC [12, 25],while others showed improved outcomes without complications. Carter et al. reported decreased granulation tissue formation and fewer subsequent surgical debridements with topical mitomycin C treatment. In this study, we used higher dose of mitomycin C than previously mentioned studies and longer and longer time of application (4mg/ml applied locally for 4 minutes). The recurrence rate was higher in non-MMC group however, was not statistically significant (p=0.5)
In conclusion, the recurrence rate in this study was 18%. Syndromic children had a higher likelihood of unsuccessful procedures than did children with isolated CA anomaly. We found no significant influence of MMC on the recurrence rate. The failure of the procedure tends to be higher in bilateral than in unilateral cases, although the difference was not statistically significant.
| References|| |
Kwong K M. Current Updates on Choanal Atresia. Front Pediatr, 2015. 3: 52.
Hengerer A S, T M Brickman, A Jeyakumar. Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience. Laryngoscope.
2008; 118(5): 862-6.
Josephson G D, et al. Transnasal endoscopic repair of congenital choanal atresia: long-term results. Arch Otolaryngol Head Neck Surg.
1998; 124(5): 537-40.
Stankiewicz J A. The endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg.
1990; 103(6): 931-7.
Ramsden J D, P Campisi , V Forte. Choanal atresia and choanal stenosis. Otolaryngol Clin North Am.
2009;42(2): 339-52, x.
Newman J R, et al. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg.
2013; 139(1): 71-5.
McLeod I K, Brooks D B , Mair E A . Revision choanal atresia repair. Int J Pediatr Otorhinolaryngol.
2003. 67(5): 517-24.
Llorente J L, et al. Endoscopic treatment of choanal atresia. Acta Otorrinolaringol Esp.
2013; 64(6): 389-95.
De Freitas R P, R G Berkowitz. Bilateral choanal atresia repair in neonates--a single surgeon experience. Int J Pediatr Otorhinolaryngol.
2012; 76(6): 873-8.
Durmaz A, et al. Transnasal endoscopic repair of choanal atresia: results of 13 cases and meta-analysis. J Craniofac Surg.
2008; 19(5): 1270-4.
Teissier, N., et al. Predictive factors for success after transnasal endoscopic treatment of choanal atresia. Arch Otolaryngol Head Neck Surg.
Kim H, et al. Clinical features and surgical outcomes of congenital choanal atresia: factors influencing success from 20-year review in an institute. Am J Otolaryngol.
Corrales C E, P.J. Koltai. Choanal atresia: current concepts and controversies. Curr Opin Otolaryngol Head Neck Surg.
Brihaye P, et al. Comprehensive management of congenital choanal atresia. Int J Pediatr Otorhinolaryngol.
2017; 98: p. 9-18.
Velegrakis S, et al. Long-term outcomes of endonasal surgery for choanal atresia: 28 years experience in an academic medical centre. Eur Arch Otorhinolaryngol.
2013; 270(1): 113-6.
Coniglio J U, Manzione J V, Hengerer A S. Anatomic findings and management of choanal atresia and the CHARGE association. Ann Otol Rhinol Laryngol.
1988;97(5 Pt 1): 448-53.
Schoem S R.Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg.
2004; 131(4): 362-6.
Carter J M, Lawlor C , Guarisco J L. The efficacy of mitomycin and stenting in choanal atresia repair: a 20 year experience. Int J Pediatr Otorhinolaryngol.
2014; 78(2): 307-11.
El-Anwar M W, Nofal A A, El-Ahl M A. Endoscopic repair of bilateral choanal atresia, starting with vomer resection: Evaluation study. Am J Rhinol Allergy.
2016; 30(3): 95-9.
Zuckerman J D, Zapata S , Sobol S E. Single-stage choanal atresia repair in the neonate. Arch Otolaryngol Head Neck Surg.
Bedwell J R, Choi S S. Are stents necessary after choanal atresia repair?. Laryngoscope.
2012; 122(11): 2365-6.
Saafan M E. Endoscopic management of congenital bilateral posterior choanal atresia: value of using stents. Eur Arch Otorhinolaryngol.
2013; 270(1): 129-34.
Devambez M, Delattre A , Fayoux P. Congenital nasal pyriform aperture stenosis: diagnosis and management. Cleft Palate Craniofac J.
Holland B W , McGuirt W F Jr., Surgical management of choanal atresia: improved outcome using mitomycin. Arch Otolaryngol Head Neck Surg.
2001; 127(11): 1375-80.
[Table 1], [Table 2]