Introduction Choanal atresia (CA) is an uncommon congenital anomaly. There are various syndromes that are associated with CA. The purpose of this study is to determine the differences in CA\'s presentation and outcome when associated with other congenital anomalies and syndromes. Method This is a retrospective review study of all children (18 years and younger) who underwent CA repair in a tertiary referral healthcare center from January 2005 to April 2022. Demographics, comorbidities, radiological testing, operative reports, and outpatient reports were collected. Success was determined as a child with bilateral patent choana that is able to breathe from both nostrils comfortably. Result Twenty-four patients met the criteria for inclusion in this study. Bilateral CA was present in 15 (62.5%) patients. Mixed CA was the most common variant. There were various congenital anomalies in association with CA patients who are yet to be classified into a syndrome. The most common congenital anomaly was cleft lip and palate. Bony and mixed types were significantly associated with non-syndromic patients (p<0.05). Twenty patients (83%) were diagnosed with CA at age of less than one year, and four patients were diagnosed after one year of age. There were 36 surgeries performed on 24 patients, of which 27 were endoscopic and nine were using Hugher dilator. The overall success rate for CA repair was 50%. The median number of revisions per patient was 0.5. Conclusion CA is a challenging anomaly to repair. There are various factors that influence the outcome of children with CA. Otolaryngologists should counsel the patient and their families regarding possible need for revision especially in those with other craniofacial anomalies.

Introduction  The risk of skull base injury during choanal atresia repair can be mitigated via thorough understanding of skull base anatomy. There is a paucity of data describing differences in skull base anatomy between patients with coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities (CHARGE) syndrome and those without. Objectives  The aim of this study was to measure nasal and skull base anatomy in patients with isolated bilateral choanal atresia (BCA), CHARGE syndrome, and other syndromic congenital anomalies. Methods  Retrospective chart review of patients with bilateral choanal atresia and computed tomography of the face between 2001 and 2019 were evaluated. Choanal width, height, mid-nasal height, and skull base slope were measured radiographically. Differences in anatomy between healthy patients, those with CHARGE syndrome, and those with other congenital anomalies were compared. Results  Twenty-one patients with BCA and relevant imaging were identified: 7 with isolated BCA, 6 with CHARGE syndrome, and 8 with other congenital anomalies. A t -test indicated insignificant difference in skull base slope, choanal height, choanal width, or mid-nasal skull base height between isolate BCA cases and patients with any congenital anomaly. When comparing CHARGE to isolated BCA cases, mid-nasal height was shorter in CHARGE patients ( p  = 0.03). There were no differences in measurements between patients with congenital anomalies excluding CHARGE ( p  > 0.05). Two patients in the congenital anomaly group were found to have bony skull base defects preoperatively. Conclusion  This study represents the largest description of skull base and nasal anatomy in patients with CHARGE syndrome and BCA. Surgeons should be aware of the lower skull base in CHARGE patients to avoid inadvertent skull base injury.

OBJECTIVE: To study the effect of dose-adjusted mitomycin-c (MMC) on the recurrence rate of choanal atresia (CA), and the complication rate associated with this concentration.
METHODS: This prospective cohort study was conducted between May 2012 and March 2020 at a tertiary referral center. It included patients of all ages who were diagnosed with CA and scheduled to undergo surgical repair. The MMC group received 4.0 mg/mL of topical MMC. Both groups were followed up for the surgical outcomes and complication rates.
RESULTS: Twenty-one patients (15 females) underwent 25 CA repair procedures. The mean age was 44.85 months (standard deviation = 72.85). MMC was used in 12 (57.1%) of 21 patients. Revision CA repair was warranted in three of the nine patients who did not receive topical MMC compared to one of the 12 patients who received topical MMC. The MMC group required 1.08 ± 0.29 surgeries (range, 1-2), whereas the non-MMC group required 1.44 ± 0.73 surgeries (range, 1-3). Functional success was achieved in 17 (81%) patients who remained symptom-free until their last follow-up visit.
CONCLUSIONS: High-concentration MMC was considered safe in the pediatric and adult populations. Although high-concentration MMC could reduce the need for revision surgery, further studies are required to determine whether the effect is significant in a larger sample population.

BACKGROUND: Multiple surgical approaches have been proposed to repair the congenital choanal atresia. However, there remains no general consensus about the optimal surgical technique. This study aimed to describe and evaluate outcomes of the endoscopic septonasal flap technique combined with bioabsorbable steroid-eluting stents for repair of congenital choanal atresia in neonates and infants.
METHODS: Clinical data of 37 neonates and infants with congenital choanal atresia who received nasal endoscopic surgery with the flap technique between January 2018 and July 2020 were analyzed retrospectively. All patients underwent the ultra‑low‑dose paranasal sinus computed tomography imaging preoperatively to confirm diagnosis and plan the surgery. In these patients, the mirrored L-shaped flap technique was performed for bilateral atresia and the cross-over L-shaped flap technique was performed for unilateral atresia. A total of 22 patients had silicone stents postoperatively and 15 patients had bioabsorbable steroid-eluting stents postoperatively. Silicone stents were removed at one month postoperatively under secondary general anesthesia, while no anesthesia was needed to remove the bioabsorbable steroid-eluting stents. Postoperative follow-up ranged from 10 months to 3 years.
RESULTS: The septonasal flap technique was performed in all patients. Compared with the silicone stents group, the average operative duration and the hospital length of stay in the bioabsorbable steroid-eluting stents group were decreased [(97.46 ± 15.37) min vs (83.49 ± 19.16) min t = 13.733, P < 0.001] [(12.8 ± 3.22) d vs (7.67 ± 3.91) d t = 15.082, P < 0.001], the average number of procedures was reduced [(2.04 ± 0.64) vs (1.00 ± 0.001), t = 82.689, P < 0.001], the differences were statistically significant. There were no reports of postoperative restenosis and complications in the bioabsorbable steroid-eluting stents group, and follow-up endoscopic examinations showed patency and stable nasal passages in all cases.
CONCLUSIONS: The endoscopic septonasal flap technique can effectively expose and expand the choanal bony structure for repair of congenital choanal atresia in neonates and infants. The combined use of this technique along with bioabsorbable steroid-eluting stents can help prevent the need for revision procedures and also against stent-related injuries.

BACKGROUND: To evaluate the clinical management of choanal atresia (CA) in tertiary centers across Canada.
METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with choanal atresia to a participating center.
RESULTS: The health charts of 215 patients (59.6% female) with choanal atresia (CA) were reviewed. Mean age of initial surgical repair was 0.8 months for bilateral CA, and 48.6 months for unilateral CA. Approaches of surgical repair consisted of endoscopic transnasal (31.7%), non-endoscopic transnasal (42.6%), and transpalatal (25.2%). Stents were used on 70.7% of patients. Forty-nine percent of patients were brought back to the OR for a planned second look; stent removal being the most common reason (86.4%). Surgical success rate of initial surgeries was 54.1%. Surgical technique was not associated with rate of restenosis [χ2 (2) = 1.6, p = .46].
CONCLUSIONS: The present study is the first national multi-institutional study exploring the surgical outcomes of CA over a 30-year period. The surgical repair of CA presents a challenge to otolaryngologists, as the rate of surgical failure is high. The optimal surgical approach, age at surgical repair, use of stents, surgical adjuncts, and need for planned second look warrant further investigation.

BACKGROUND: To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada.
METHODS: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center.
RESULTS: The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome.
CONCLUSIONS: The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted.

Background Although choanal atresia (CA) was first described 250 years ago, its description and understanding remain incomplete, as is the distinction between unilateral and bilateral CA. Among the surgical techniques introduced to manage this condition are intranasal Hegar\'s dilator (IHD) and transnasal endoscopic excision (TNEE). Objectives This study retrospectively evaluated the outcomes and effectiveness of IHD and TNEE in the treatment of patients with CA, including differences in the incidence of re-stenosis with these techniques. Methods Patients diagnosed with CA who underwent surgical interventions in the Otolaryngology Department of Al-Noor Specialist Hospital, Makkah, Saudi Arabia, between 1997 and 2017 were analyzed. Postoperative outcomes including re-stenosis rates were compared in patients who underwent IHD and TNEE. Factors associated with patient outcomes were analyzed, including ages at diagnosis and surgery, nationality, gender, type of atresia (unilateral/bilateral and bony/membranous/mixed), surgical intervention (IHD or TNEE), and re-stenosis and need for revision surgery after IHD and TNEE. Results A total of 30 patients were diagnosed with CA, including 21 (70%) girls and 9 (30%) boys. Of them, 18 (60%) patients were diagnosed at younger than one month of age, 28 (93%) were Saudi nationals, and 20 (67.70%) were aged younger than three months at the time of surgery. Of these 30 patients, 17 (56.70%, all Saudi nationals) underwent IHD, and 13 (43.30%), including 15 Saudi nationals, underwent TNEE. The 17 patients who underwent IHD included 13 (76.50%) girls and 4 (23.50%) boys, whereas the 13 patients who underwent TNEE included 8 (61.50%) girls and 5 (38.50%) boys. Fifteen patients (50%) had mixed-type CA, nine (30%) had bony-type CA, and six (20%) had membranous-type CA. Twenty-six (86.67%) patients underwent primary surgery, whereas four (13.33%) underwent revision surgery; of the latter, three (75%) had undergone primary IHD, and one had undergone primary TNEE. Only one (3.33%) patient experienced re-stenosis after revision surgery, which consisted of IHD. Twelve patients (40%) underwent stenting, with one developing re-stenosis. The relationships between surgical approach and re-stenosis after primary and secondary surgery were not statistically significant. Conclusion The outcomes in patients with CA treated with IHD and TNEE are comparable. Rates of re-stenosis and need for revision surgery do not differ significantly in patients treated with these surgical approaches.

OBJECTIVE: To compare the outcomes of endoscopic repair of bilateral congenital choanal atresia using a flap technique without stenting versus endoscopic repair using stenting without a flap.
METHODS: A prospective randomised controlled study was conducted, comprising 72 patients with bilateral congenital choanal atresia. The patients were randomised into two groups. Group A (42 patients) underwent endoscopic repair using a mirrored L-shaped flap without stenting, and group B (30 patients) underwent endoscopic repair using stenting without a flap.
RESULTS: At a mean follow-up period of 18.2 months, endoscopic assessment revealed a patent posterior choana in 81 per cent and 83.33 per cent of patients in group A and group B respectively. Choanal stenosis occurred in 21.40 per cent and 33.33 per cent of patients in group A and group B respectively. Granulation tissue was observed in 28.6 per cent and 53.3 per cent of patients in group A and group B respectively.
CONCLUSIONS: The endoscopic approach utilising a flap without stenting is safe and effective, with a high success rate.

Introduction  The main histological features of the nasal mucosa 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.

BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.