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Prognostic Factors and Management of Patients with Choanal Atresia
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Prognostic Factors and Management of Patients with Choanal Atresia Eric Moreddu, MD, Marie-Eva Rossi, MD, Richard Nicollas, MD, PhD, and Jean-Michel Triglia, MD, PhD Objective To analyze prognostic factors in the management of patients with choanal atresia. Study design This is a review of 114 patients operated on for choanal atresia in a tertiary care center between November 1986 and November 2016, including clinical characteristics, surgical management, and postoperative course with final nasal patency. The data were collected in a database that was updated over time. Results Among the 114 patients, 78 were female, 77 presented with unilateral choanal atresia, and 37 presented with bilateral unilateral choanal atresia, corresponding to 151 nasal fossae. Forty-seven patients had associated abnormalities (41.2%), including CHARGE, diagnosed in 20 children (17.5%). At the end of follow-up, 91 patients (79.8%) had normal choanal patency. The identified risk factors of surgical failure were age <6 months (P = .004), weight <5 kg (P = .007), and bilateral choanal atresia (P < .001). The type of atresia, presence of associated abnormalities, surgical approach, stenting, and use of mitomycin were not significantly linked with the surgical results. Conclusions This series highlights the importance of the timing of surgery, which is guided by the clinical ability of the infant to tolerate the procedure. Endoscopic approaches are widely performed, but a transpalatal approach, necessary in some cases of bilateral choanal atresia, does not alter the results. Unilateral choanal atresia surgery should be delayed after age 6 months and/or weight >5 kg when possible. (J Pediatr 2018;■■:■■-■■). hoanal atresia is a rare congenital anomaly, occurring in 1 in 5000-7000 live births, with a female predominance.1 Bilateral choanal atresia accounts for 30% of patients, and unilateral choanal atresia accounts for approximately 70%. Bilateral choanal atresia often necessitates early surgery because newborns are obligatory nasal breathers owing to the position of the epiglottis, tongue, and soft palate, especially during feeding.2 Unilateral choanal atresia may be asymptomatic and discovered coincidentally during neonatal examination by the inability to traverse the nasal fossa with a catheter, or even later with unilateral nasal obstruction and discharge. The association with other congenital abnormalities, such as CHARGE syndrome (ie, coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, and ear abnormalities and deafness), may modify the management of choanal atresia and necessitate further appropriate examinations.3 The ideal surgical procedure aims to restore the normal nasal passage by removing the atretic layer and should be safe without damage to adjacent structures and lead to short hospitalization and convalescence. Transnasal endoscopic repair has become the most widely accepted technique, with several described variations.4-8 In some cases, a transpalatal approach remains necessary, especially in neonates with severe craniofacial abnormalities or very small nasal fossae.9,10 Mitomycin (a frequently used adjuvant therapy) and stenting of the nasal cavity are performed inconsistently after surgery.7,11-17 However, given the differences between patients and the wide variability of surgical techniques used, the factors that influence the result of the surgical management of choanal atresia remain unclear and controversial.14,15,18 The objectives of this study were to analyze the predictive factors of success in the surgical management of choanal atresia, and to propose a management algorithm based on this analysis.
C
Methods We reviewed all patients who underwent surgical treatment for choanal atresia in the pediatric otolaryngology department of a tertiary pediatric care center (La Timone Children’s Hospital, Marseille) between November 1986 and November 2016. Clinical characteristics of the patients were collected: sex, age, weight, unilaterality or bilaterality of choanal atresia, type of choanal atresia (bony, membranous, or mixed), and coexisting abnormalities, including CHARGE association.
CHARGE CT ICU MRI
Coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, and ear abnormalities and deafness Computed tomography Intensive care unit Magnetic resonance imaging
From the Department of Pediatric Otolaryngology Head and Neck Surgery, La Timone Children’s Hospital (AixMarseille University), Marseille, France The authors declare no conflicts of interest. 0022-3476/$ - see front matter. © 2018 Elsevier Inc. All rights reserved. https://doi.org10.1016/j.jpeds.2018.08.074
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THE JOURNAL OF PEDIATRICS • www.jpeds.com The following surgical variables were collected: the approach at first intervention in our department (transpalatal or endoscopic transnasal), the method of transnasal repair when performed (drilling, microdebrider, laser, balloon dilation, and associations among these techniques), the use of stenting and application of mitomycin, and the number of interventions for each technique. Postoperative data collected included duration of hospitalization, intensive care unit (ICU) admission when required, number of interventions, and final results in terms of nasal patency and follow-up. Normal patency was defined as a choanal arch with <50% restenosis using a flexible endoscope and allowing normal airflow, partial restenosis when anatomic stenosis was >50% and/or associated with symptoms (discharge or breathing difficulties) and complete stenosis when no passage was visible under fibroscopy or endoscopy. Only procedures related to the development of postoperative stenosis were considered second-stage surgeries; planned secondary procedures to remove stents were not considered second-stage surgeries. Surgical criteria for success were the final degree of patency, need for second-stage surgery, and number of surgical procedures to obtain adequate choanal arches. These data were included in a FileMaker Pro database and updated at each operation and follow-up examination over time. Statistical analyses were performed with SPSS 20 (IBM Corp, Armonk, New York), and R 3.5.1 (R Foundation for Statistical Computing, Vienna, Austria) software. Multivariate analysis was performed for the significance of prognostic factors of choanal atresia. A significance threshold of P < .05 was adopted for all statistical analyses. The Pearson c2 test was used for noncontinuous variables, and the Student t test was used for continuous variables. Logistic regression was used for multivariate analysis when confounding factors were identified. k-means clustering was used to determine age and weight cutoffs.
Results A total of 114 patients were included in the database. After management, 91 patients (79.8%) had normal patency of the choanae, 14 had partial stenosis (12.7%), 2 had complete restenosis of a unilateral choanal atresia (1.8%), 3 died of a comorbidity (2.7%), and 4 were lost of follow-up (3.6%) after immediate postoperative follow-up (Table I; available at www.jpeds.com). The mean number of interventions was 2.28, and the median number was 2. The mean duration of followup was 3.87 years (46 months), and the median duration was 2.91 years (35 months). Sex The 114 patients included 78 females (68.4%), for a female:male ratio of approximately 2.2:1. There were no significant differences between males and females (Table II). Age and Weight The mean age at first procedure was 2.25 years, with a median of 0.70 year (approximately 8 months). Patients who under-
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Table II. Patient demographic data Total Variable Sex Female Male Type of atresia Bony Mixed Membranous CHARGE Yes No Associated abnormalities Yes No
Unilateral
Bilateral
P value
N
%
n
%
n
%
78 36
68.4 31.6
52 25
67.5 32.5
26 11
70.3 29.7
.77
100 48 3
66.2 31.8 2.0
52 24 1
67.5 31.2 1.3
24 12 1
64.9 32.4 2.7
.80
20 94
17.5 82.5
10 67
13.0 87.0
10 27
27.0 73.0
.06
47 67
41.2 58.8
24 53
31.2 68.8
23 14
62.2 37.8
.002
Significant P values are in bold type.
went surgery before age 6 months had a mean hospital length of stay (23.5 days vs 11 days; P < .001) and underwent more procedures (2.55 vs 1.81; P = .004) (Table III). The mean weight at the first procedure was 9.04 kg, with a median of 6.25 kg. Patients weighing <5 kg at the first procedure had longer median hospital length of stay (33 days vs 13.51 days; P < .001), including ICU stay (7.00 days vs 1.38 days; P = .047) and underwent more procedures (3.00 vs 1.93; P = .007). They also obtained choanal patency less frequently than patients weighing >5 kg (63.8% vs 83.3%; P = .047). Unilaterality or Bilaterality Seventy-seven patients presented with unilateral choanal atresia (67.5%), including 44 with right-sided predominance, and 37 patients presented with bilateral choanal atresia (32.5%), corresponding to 151 operated nasal fossae. Patients with bilateral choanal atresia underwent surgery at a younger age (mean, 18 days vs 3.06 years, P < .001; median = 9 days) and lower weight (mean, 2.90 kg vs 12.50 kg; P < .001; median = 2.70). Patients with bilateral choanal atresia had more associated anomalies than patients with unilateral atresia (65.6% vs 31.8%; P = .001). They also had a longer hospital stay (mean, 30.38 days vs 12.12 days; P < .001) and underwent more surgical procedures (mean, 3.19 vs 1.84, P < .001). As shown in Table I, a good choanal airway was reported in 85.7% of children with
Table III. Patient age and weight Measures Weight, kg Mean Median Minimum Maximum Age, y Mean Median Minimum Maximum
Total
Unilateral
Bilateral
9.04 6.25 1.10 65.00
12.50 8.65 3.10 65.00
2.90 2.70 1.10 6.73
2.25 0.70 0.01 17.56
3.06 1.28 0.01 17.56
0.05 0.02 0.01 0.57
P value <.001
(18 d) (9 d) (2 d) (7 mo)
<.001
Significant P values are in bold type.
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Table IV. Surgical prognostic factors influencing the number of procedures for choanal atresia Factors Age at surgery, mo ≥6 <6 Weight at surgery, kg ≥5 <5 Bilateralism No Yes CHARGE No Yes Associated abnormalities No Yes Stenting (unilateral atresia) No Yes Mitomycin No Yes Transpalatal approach No Yes
Average number of procedures
P value
1.81 2.55
.004
1.93 3.00
.007
1.84 3.19
<.001
2.16 2.85
.02
2.15 2.47
.24
1.82 1.86
.28
2.01 3.31
<.001
1.84 2.89
.044
Significant P values are in bold type.
unilateral choanal atresia and in 67.6% of those with bilateral choanal atresia (P = .03). Type of Atresia Atresia was bony in 100 nasal fossae (66.2%) and mixed in 48 nasal fossae (31.8%) (Table II). Two patients had membranous atresia, 1 unilateral and the other bilateral, corresponding to 3 nasal fossae (2.0%). No difference in type of choanal atresia was identified between the patients with unilateral and those with bilateral choanal atresia. The type of atresia was not significantly associated with the outcome after a single procedure or at the end of the management, even if a trend toward better results was observed for membranous atresia. A good result was noted after a single procedure in 34.1% cases of bony atresia, 41% cases of mixed atresia, and 100% cases of membranous atresia (P = .06). Associated Abnormalities The total rate of coexisting congenital abnormalities (including CHARGE) was 41.2% (47 of 114) (Tables II and IV). Bilateral choanal atresia was significantly more frequently associated with additional abnormalities (P = .002). The patients with associated abnormalities did not undergo more operations (2.47 vs 2.16; P = .24) but the patients with partial stenosis at the end of management were more likely to have associated abnormalities (73.70% vs 26.30%) compared with those with normal patency (38.50% vs 61.50%; P =.019). The following diseases involving facial or midline abnormalities were found: Down syndrome (4 patients), isolated coloboma (2 patients), laryngomalacia (2 patients), nasal fistula (2 patients), Crouzon syndrome (2 patients), 22q11
microdeletion (2 patients), Opitz syndrome (1 patient), palatal cleft (1 patient), anal anomaly (1 patient), and esophagotracheal cleft (1 patient). A syndromic association (including CHARGE) was diagnosed in 29 of the 114 children (25.4%), and 18 children (15.8%) had associated abnormalities not formally integrated in a known syndromic association. Heart disease was diagnosed in 11 children, all of 2 with a syndromic association (8 with CHARGE, 1 with Down syndrome). CHARGE association was diagnosed in 20 children (17.5%). Patients with CHARGE association had a non-statistically significant increased prevalence of bilateral choanal atresia (50% vs 27.7%; P = .065). CHARGE syndrome did not significantly modify the final outcome of the surgical management, but was associated with an increased number of operations (2.85 vs 2.16; P = .02). All 3 patients who died during follow-up presented with CHARGE syndrome. Surgical Approach In patients with unilateral choanal atresia, surgery was performed via a transpalatal approach in 28 of 77 cases (36.4%) and an endoscopic transnasal approach in the other 49 cases (63.6%). For the 49 transnasal approaches, a microdebrider was used in 38 cases, a laser was used in 8 cases, and drilling was performed in 3 cases. Patients with bilateral choanal atresia underwent surgery via a transpalatal approach in 27 of 37 cases (73.0%) and via an endoscopic transnasal approach in the other 10 cases (27.0%). Surgery was performed using a microdebrider in 6 cases, a laser in 2 cases, and drilling in 2 cases. Combining unilateral and bilateral choanal atresia, surgery was performed in 82 nasal fossae using a transpalatal approach and in 69 using a transnasal endoscopic approach. In the latter group, a microdebrider was used in 50 nasal fossae, a laser was used in 12 nasal fossae, and drilling was performed in 7 nasal fossae. The approach used at the first procedure appeared to determine the surgical results; 72.0% of transpalatal procedures required a second-stage surgery, compared with 50.7% of endonasal procedures (P = .014) (Table V; available at www.jpeds.com). However, when adjusted for age and weight, this association became nonsignificant (P = .120). Patients operated on by a transpalatal approach underwent more procedures (2.89 vs 1.84; P = .044) (Table IV). In the same subgroup (unilateral or bilateral), no significant differences could be highlighted in terms of results of surgery between the approaches and the technique performed. Adjuvant Therapies (Stenting and Mitomycin) Stenting was performed in 85.4% (129 of 151) of the nasal fossae and in 74.5% (85 of 114) of the patients. Stenting was performed in 97.3% (36 of 37) of cases of bilateral choanal atresia and in 63.6% (49 of 77) of cases of unilateral choanal atresia. The mean duration of stenting was 27.7 days (median, 27 days), with no significant difference between unilateral and bilateral choanal atresia cases. The number of procedures was the same with or without stenting for cases of unilateral choanal atresia (1.86 vs 1.82; P = .28) (Table IV).
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Bilateral
Unilateral
choanal atresia
choanal atresia
Symptomatic
Asymptomatic
Preoperative assessment: Craniofacial CT-scan Transthoracic ultrasounds Renal function
WAIT
Nasal endoscopy under general anesthesia
Complete preoperative and associated abnormalities assessment
Passage of endoscope + instrument: possible
Passage of endoscope + instrument: NOT possible
>6 months >5 kg
Endoscopic transnasal approach
Transpalatal approach
Endoscopic transnasal approach
Figure. Management algorithm for choanal atresia.
Mitomycin application was significantly associated with a greater number of procedures (3.31 vs 2.01; P < .001) but not with final results in terms of patency (Table IV).
Discussion Our present series has allowed the elucidation of significant prognostic factors of choanal atresia surgery.9 The main prognostic factors of choanal atresia repair were age, weight at the first procedure, and bilateralism. From these observations, we can recommend algorithms for the management of unilateral and bilateral choanal atresia (Figure). In studying choanal atresia, bilateralism, age, weight, and the type of approach are so enmeshed that the results of sta-
tistical analysis must to be interpreted carefully. Our study highlights the importance of age and weight in the surgical management of choanal atresia, with a greater number of procedures needed to obtain good choanal patency before age 6 months and/or weight >5 kg. Consequently, for patients with unilateral choanal atresia, waiting until age >6 months or weight >5 kg (when possible) tends to reduce the number of procedures necessary to obtain good nasal patency. This is explained by the absolute size of the patient: the larger the patient, the wider the choanal arches can be opened and the vomer resected, with less consequent risk of scarring and restenosis. This observation is concordant with previous studies available in the literature.8 The type of choanal atresia was determined using the classification system of Brown et al, who reported 29% cases of
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pure bony atresia and 71% cases of mixed bony and membranous atresia in their series.19 In our series, the atresia was identified as pure bony in 66.2% of cases, mixed in 31.8%, and membranous in 2.0%, similar to the previous observations. Membranous atresias are rare and embryologically unclear. In our series, the diagnosis of membranous atresia on computed tomography scan was confirmed during surgery, with absence of the bony plate and no need for bony resection in the 2 cases. The timing of surgery for bilateral choanal atresia must be guided by clinical tolerance of the nasal obstruction and the presence of associated abnormalities. Globally, children with bilateral choanal atresia undergo surgery during the first week. Patients with bilateral choanal atresia more often underwent transpalatal procedures because of small nasal fossae, precluding the simultaneous use of an endoscope and instruments. This approach was more likely to fail after the first procedure, but the type of initial approach was nonsignificantly associated with the success rate when adjusted for age and weight in multivariate analysis. This is explained by the fact that the patients undergo surgeries until satisfactory nasal breathing is achieved. Consequently, the choice of approach relies on the possibility of endoscopic procedure; most published studies recommend this approach when possible.6-8,18,20-23 We also recommend the use of endoscopic techniques whenever possible. These techniques allow the restoration of nasal patency using the natural airway, without detachment of the palatal fibromucosa, with consequent lower risks of lesions of the palatal pedicle and of wound healing disorders. Furthermore, optical magnification of the operated area allows precise excision of the atretic plate. Endoscopic approaches lead to short operative times, minimal bleeding, early feeding after surgery, and short hospital stay. Over the 30 last years, the development of endoscopic techniques has reduced the indications for transpalatal approaches. The instruments used in this series followed the technical advances during this period. Drilling and mucosal debridement using a microdebrider have become gold standard techniques in endoscopic approaches. Laser, widely used in the 1990s, is now mainly used for revision procedures, when patency is insufficient due to the presence of granulation tissue or fibrous healing.5,15,24,25 Balloon dilation, developed more recently, is particularly helpful in restenosis and can be preceded by laser incisions of the fibrous ring for better efficacy.21,26,27 In our protocol, stenting was systematically performed for bilateral choanal atresia, as is recommended in neonates who undergo surgery at a young age and low weight.21 A change in approach in our series of unilateral choanal atresia cases occurred in 2008. Before 2008, 86.0% (49 of 57) of unilateral choanal atresias were stented, whereas none have been stented since 2008. Our results indicate that in cases of unilateral choanal atresia, not stenting is as safe as stenting, because this change did not change the final results in terms of patency or number of procedures (1.79 before 2008 vs 2.00 after 2008; P = .95). These results differ from those of studies that have reported poorer results when stenting is performed in endoscopic
transnasal approaches, but lead to the same conclusion with abandonment of stenting.7,16,28 Mean stent duration (27.7 days) was negatively influenced by the long-term analysis of this series. A modification of the duration of stenting was applied with the development of endoscopic repair and a study by Van den Abbeele et al recommending the avoidance of prolonged nasal stenting; the duration of stenting dropped from 36.14 days before 2002 to 10.83 days after 2002 (P < .001).7 In our current management, stenting is planned to last for 1-2 weeks. Mitomycin was used in our management as adjuvant therapy in refractory atresias and was associated with poorer results. Studies about the efficacy of mitomycin are controversial, with no prospective study demonstrating its efficacy available in the literature.11-13 In the present study, we could not evaluate the efficacy of mitomycin, and thus make no recommendations. The long duration of stay reported in our series is explained by the fact that it includes the entire perioperative period, even preoperative and postoperative ICU stays for neonates with bilateral choanal atresia. In addition, it includes every stay for initial repair and revision surgery. For initial and planned repair, which excludes cases of respiratory distress, patients are hospitalized the day before surgery and discharged according to the postoperative course. For endoscopic revision surgery, outpatient surgery has become a standard of care over the last decade in our hospital. In our series, 2 patients presented with complete restenosis of unilateral choanal atresia; 1 is scheduled for revision surgery, and the other decided not to undergo revision surgery. Over the period covered by this study, some modalities for diagnosis have also changed. Diagnostic imaging (CT scan, magnetic resonance imaging [MRI], and ultrasound) and cytogenetic testing have developed and become more accessible. All the patients of this series underwent CT scan before surgery, without major improvement in recent years in terms of diagnosis of choanal atresia (or of the absence of lateral semicircular canals). Other abnormalities may be more accurately diagnosed today, such as the absence of olfactory bulbs on recent MRI. However, the abnormalities explored in this series (eg, CHARGE, facial abnormalities, Down syndrome) are clinically diagnosed and were already well known in 1986. Other patient characteristics of our series are similar to those described in the literature.15,25 Females were predominant, and unilateral choanal atresia was more frequent than bilateral, with right-sided predominance. Associated abnormalities were frequent: approximately 60% in bilateral cases and 30% in unilateral cases. CHARGE association was the most common (approximately 20% of all patients). Recent studies described “3C” syndrome (choanal atresia, coloboma, and lateral semicircular canal hypoplasia or absence).8 Complementary investigations are mandatory before surgery. A craniofacial CT scan is required for anatomic analysis before surgery. Other investigations are useful to anticipate any increased risk of general anesthesia: transthoracic ultrasound for cardiac assessment and blood sample with renal function, given the possibility of heart and/or renal failure in syndromic associations like CHARGE. Other examinations, including cytogenetic
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THE JOURNAL OF PEDIATRICS • www.jpeds.com testing, ocular examination, audiologic testing, genitourinary ultrasound and cerebral MRI, may be performed, but surgery should not be delayed due to respiratory distress. In our series, the presence of comorbidities and CHARGE did not increase the number of procedures and only slightly altered the final results in terms of permeability. Confounding factors, such as lower weight and prematurity, may skew the results in this population. CHARGE association is severe and associated with extrarhinologic complications that were the cause of death of 3 patients in our series. Other studies have reported conflicting results regarding the effect of associated abnormalities and the course of choanal atresia, with some finding no effect on outcomes15 but others associating CHARGE with an increased risk of failure with endoscopic repair.3 The objective evaluation of nasal airflow and nasal fossae patency in children is limited by the feasibility of examination at this age. The measurement of nasal airflow is particularly difficult because it requires the child’s cooperation. For example, peak nasal inspiration flow and rhinomanometry are hardly feasible before age 5 years. Acoustic rhinometry does not seem to be useful in posterior nasal pathology.29 Objective assessment of the choanal opening requires either imaging with radiography exposure or the use of measuring instruments under general anesthesia. These techniques are not recommended in the postoperative management of choanal atresia. Thus, the majority of studies of choanal atresia have evaluated airflow and patency based on subjective findings, such as the quality of nasal breathing (based on noise, indrawing, feeding, or discomfort), presence of rhinorrhea, or evaluation of the choanal area by endoscopy or nasofibroscopy. Objective evaluation of airflow or the choanal area after choanal atresia repair in children is a challenge for future work. ■ Submitted for publication Jun 6, 2018; last revision received Aug 13, 2018; accepted Aug 29, 2018 Reprint requests: Eric Moreddu, MD, Department of Pediatric Otolaryngology Head and Neck Surgery, La Timone Children’s Hospital (Aix-Marseille University), 264 Rue Saint Pierre, Marseille 13385 CEDEX 5, France. E-mail: [email protected]
References 1. Harris J, Robert E, Källén B. Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics 1997;99:363-7. 2. Negus VE. The function of the epiglottis. J Anat 1927;62(Pt 1):1-8. 3. Schraff SA, Vijayasekaran S, Meinzen-Derr J, Myer CM. Management of choanal atresia in CHARGE association patients: a retrospective review. Int J Pediatr Otorhinolaryngol 2006;70:1291-7. 4. Khafagy YW. Endoscopic repair of bilateral congenital choanal atresia. Laryngoscope 2002;112:316-9. 5. Pototschnig C, Völklein C, Appenroth E, Thumfart WF. Transnasal treatment of congenital choanal atresia with the KTP laser. Ann Otol Rhinol Laryngol 2001;110:335-9. 6. Uzomefuna V, Glynn F, Al-Omari B, Hone S, Russell J. Transnasal endoscopic repair of choanal atresia in a tertiary care centre: a review of outcomes. Int J Pediatr Otorhinolaryngol 2012;76:613-7.
Volume ■■ 7. Van Den Abbeele T, Francois M, Narcy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg 2002;128:936-40. 8. Guls¸en S, Baysal E, Celenk F, Aytaç I, Durucu C, Kanlikama M, et al. Treatment of congenital choanal atresia via transnasal endoscopic method. J Craniofac Surg 2017;28:338-42. 9. Triglia JM, Nicollas R, Roman S, Paris J. Choanal atresia: therapeutic management and results in a series of 58 children. Rev Laryngol Otol Rhinol (Bord) 2003;124:139-43 (in French). 10. Newman JR, Harmon P, Shirley WP, Hill JS, Woolley AL, Wiatrak BJ. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg 2013;139:71-5. 11. Prasad M, Ward RF, April MM, Bent JP, Froehlich P. Topical mitomycin as an adjunct to choanal atresia repair. Arch Otolaryngol Head Neck Surg 2002;128:398-400. 12. Holland BW, McGuirt WF Jr. Surgical management of choanal atresia: improved outcome using mitomycin. Arch Otolaryngol Head Neck Surg 2001;127:1375-80. 13. Carter JM, Lawlor C, Guarisco JL. The efficacy of mitomycin and stenting in choanal atresia repair: a 20 year experience. Int J Pediatr Otorhinolaryngol 2014;78:307-11. 14. Romeh HE, Albirmawy OA. A 13-year experience and predictors for success in transnasal endoscopic repair of congenital choanal obliteration. Int J Pediatr Otorhinolaryngol 2010;74:737-42. 15. Teissier N, Kaguelidou F, Couloigner V, François M, Van Den Abbeele T. Predictive factors for success after transnasal endoscopic treatment of choanal atresia. Arch Otolaryngol Head Neck Surg 2008;134:57-61. 16. Schoem SR. Transnasal endoscopic repair of choanal atresia: why stent? Otolaryngol Head Neck Surg 2004;131:362-6. 17. Rombaux P, de Toeuf C, Hamoir M, Eloy P, Bertrand B, Veykemans F. Transnasal repair of unilateral choanal atresia. Rhinology 2003;41:31-6. 18. Velegrakis S, Mantsopoulos K, Iro H, Zenk J. Long-term outcomes of endonasal surgery for choanal atresia: 28 years experience in an academic medical centre. Eur Arch Otorhinolaryngol 2013;270:113-6. 19. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope 1996;106(1 Pt 1):97-101. 20. Deutsch E, Kaufman M, Eilon A. Transnasal endoscopic management of choanal atresia. Int J Pediatr Otorhinolaryngol 1997;40:19-26. 21. Riepl R, Scheithauer M, Hoffmann TK, Rotter N. Transnasal endoscopic treatment of bilateral choanal atresia in newborns using balloon dilatation: own results and review of literature. Int J Pediatr Otorhinolaryngol 2014;78:459-64. 22. Uri N, Greenberg E. Endoscopic repair of choanal atresia: practical operative technique. Am J Otolaryngol 2001;22:321-3. 23. Stankiewicz JA. The endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg 1990;103:931-7. 24. Tzifa KT, Skinner DW. Endoscopic repair of unilateral choanal atresia with the KTP laser: a one-stage procedure. J Laryngol Otol 2001;115:286-8. 25. Friedman NR, Mitchell RB, Bailey CM, Albert DM, Leighton SE. Management and outcome of choanal atresia correction. Int J Pediatr Otorhinolaryngol 2000;52:45-51. 26. Bedwell J, Shah RK, Bauman N, Zalzal GH, Preciado DA. Balloon dilation for management of choanal atresia and stenosis. Int J Pediatr Otorhinolaryngol 2011;75:1515-8. 27. Goettmann D, Strohm M, Strecker EP. Treatment of a recurrent choanal atresia by balloon dilatation. Cardiovasc Intervent Radiol 2000;23:4801. 28. Gosepath J, Santamaria VE, Lippert BM, Mann WJ. Forty-one cases of congenital choanal atresia over 26 years—retrospective analysis of outcome and technique. Rhinology 2007;45:158-63. 29. Leclerc JE, Leclerc JT, Bernier K. Choanal atresia: long-term follow-up with objective evaluation of nasal airway and olfaction. Otolaryngol Head Neck Surg 2008;138:43-9.
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Table I. Final surgical outcomes of choanal atresia surgery in function of laterality Total
Unilateral
Bilateral
Outcomes
n
%
n
%
n
%
No stenosis Partial stenosis Stenosis Missing data Dead
91 14 2 4 3
82.73 12.73 1.82 3.64 2.73
66 8 2 1 0
85.71 10.39 2.60 1.30 0
25 6 0 3 3
67.57 16.22 0 8.11 8.11
Table V. Surgical technique and number of nasal fossae requiring revision surgery Techniques Transpalatal approach, n Requiring revision surgery, n Endoscopic approach, n Requiring revision surgery, n
Total
Unilateral
Bilateral
82 59 69 35
28 17 49 24
54 42 20 9
The differences between the groups were significant (P = .03 for all).
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Prognostic Factors and Management of Patients with Choanal Atresia Eric Moreddu, MD, Marie-Eva Rossi, MD, Richard Nicollas, MD, PhD, and Jean-Michel Triglia, MD, PhD Objective To analyze prognostic factors in the management of patients with choanal atresia. Study design This is a review of 114 patients operated on for choanal atresia in a tertiary care center between November 1986 and November 2016, including clinical characteristics, surgical management, and postoperative course with final nasal patency. The data were collected in a database that was updated over time. Results Among the 114 patients, 78 were female, 77 presented with unilateral choanal atresia, and 37 presented with bilateral unilateral choanal atresia, corresponding to 151 nasal fossae. Forty-seven patients had associated abnormalities (41.2%), including CHARGE, diagnosed in 20 children (17.5%). At the end of follow-up, 91 patients (79.8%) had normal choanal patency. The identified risk factors of surgical failure were age <6 months (P = .004), weight <5 kg (P = .007), and bilateral choanal atresia (P < .001). The type of atresia, presence of associated abnormalities, surgical approach, stenting, and use of mitomycin were not significantly linked with the surgical results. Conclusions This series highlights the importance of the timing of surgery, which is guided by the clinical ability of the infant to tolerate the procedure. Endoscopic approaches are widely performed, but a transpalatal approach, necessary in some cases of bilateral choanal atresia, does not alter the results. Unilateral choanal atresia surgery should be delayed after age 6 months and/or weight >5 kg when possible. (J Pediatr 2018;■■:■■-■■). hoanal atresia is a rare congenital anomaly, occurring in 1 in 5000-7000 live births, with a female predominance.1 Bilateral choanal atresia accounts for 30% of patients, and unilateral choanal atresia accounts for approximately 70%. Bilateral choanal atresia often necessitates early surgery because newborns are obligatory nasal breathers owing to the position of the epiglottis, tongue, and soft palate, especially during feeding.2 Unilateral choanal atresia may be asymptomatic and discovered coincidentally during neonatal examination by the inability to traverse the nasal fossa with a catheter, or even later with unilateral nasal obstruction and discharge. The association with other congenital abnormalities, such as CHARGE syndrome (ie, coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, and ear abnormalities and deafness), may modify the management of choanal atresia and necessitate further appropriate examinations.3 The ideal surgical procedure aims to restore the normal nasal passage by removing the atretic layer and should be safe without damage to adjacent structures and lead to short hospitalization and convalescence. Transnasal endoscopic repair has become the most widely accepted technique, with several described variations.4-8 In some cases, a transpalatal approach remains necessary, especially in neonates with severe craniofacial abnormalities or very small nasal fossae.9,10 Mitomycin (a frequently used adjuvant therapy) and stenting of the nasal cavity are performed inconsistently after surgery.7,11-17 However, given the differences between patients and the wide variability of surgical techniques used, the factors that influence the result of the surgical management of choanal atresia remain unclear and controversial.14,15,18 The objectives of this study were to analyze the predictive factors of success in the surgical management of choanal atresia, and to propose a management algorithm based on this analysis.
C
Methods We reviewed all patients who underwent surgical treatment for choanal atresia in the pediatric otolaryngology department of a tertiary pediatric care center (La Timone Children’s Hospital, Marseille) between November 1986 and November 2016. Clinical characteristics of the patients were collected: sex, age, weight, unilaterality or bilaterality of choanal atresia, type of choanal atresia (bony, membranous, or mixed), and coexisting abnormalities, including CHARGE association.
CHARGE CT ICU MRI
Coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, and ear abnormalities and deafness Computed tomography Intensive care unit Magnetic resonance imaging
From the Department of Pediatric Otolaryngology Head and Neck Surgery, La Timone Children’s Hospital (AixMarseille University), Marseille, France The authors declare no conflicts of interest. 0022-3476/$ - see front matter. © 2018 Elsevier Inc. All rights reserved. https://doi.org10.1016/j.jpeds.2018.08.074
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THE JOURNAL OF PEDIATRICS • www.jpeds.com The following surgical variables were collected: the approach at first intervention in our department (transpalatal or endoscopic transnasal), the method of transnasal repair when performed (drilling, microdebrider, laser, balloon dilation, and associations among these techniques), the use of stenting and application of mitomycin, and the number of interventions for each technique. Postoperative data collected included duration of hospitalization, intensive care unit (ICU) admission when required, number of interventions, and final results in terms of nasal patency and follow-up. Normal patency was defined as a choanal arch with <50% restenosis using a flexible endoscope and allowing normal airflow, partial restenosis when anatomic stenosis was >50% and/or associated with symptoms (discharge or breathing difficulties) and complete stenosis when no passage was visible under fibroscopy or endoscopy. Only procedures related to the development of postoperative stenosis were considered second-stage surgeries; planned secondary procedures to remove stents were not considered second-stage surgeries. Surgical criteria for success were the final degree of patency, need for second-stage surgery, and number of surgical procedures to obtain adequate choanal arches. These data were included in a FileMaker Pro database and updated at each operation and follow-up examination over time. Statistical analyses were performed with SPSS 20 (IBM Corp, Armonk, New York), and R 3.5.1 (R Foundation for Statistical Computing, Vienna, Austria) software. Multivariate analysis was performed for the significance of prognostic factors of choanal atresia. A significance threshold of P < .05 was adopted for all statistical analyses. The Pearson c2 test was used for noncontinuous variables, and the Student t test was used for continuous variables. Logistic regression was used for multivariate analysis when confounding factors were identified. k-means clustering was used to determine age and weight cutoffs.
Results A total of 114 patients were included in the database. After management, 91 patients (79.8%) had normal patency of the choanae, 14 had partial stenosis (12.7%), 2 had complete restenosis of a unilateral choanal atresia (1.8%), 3 died of a comorbidity (2.7%), and 4 were lost of follow-up (3.6%) after immediate postoperative follow-up (Table I; available at www.jpeds.com). The mean number of interventions was 2.28, and the median number was 2. The mean duration of followup was 3.87 years (46 months), and the median duration was 2.91 years (35 months). Sex The 114 patients included 78 females (68.4%), for a female:male ratio of approximately 2.2:1. There were no significant differences between males and females (Table II). Age and Weight The mean age at first procedure was 2.25 years, with a median of 0.70 year (approximately 8 months). Patients who under-
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Table II. Patient demographic data Total Variable Sex Female Male Type of atresia Bony Mixed Membranous CHARGE Yes No Associated abnormalities Yes No
Unilateral
Bilateral
P value
N
%
n
%
n
%
78 36
68.4 31.6
52 25
67.5 32.5
26 11
70.3 29.7
.77
100 48 3
66.2 31.8 2.0
52 24 1
67.5 31.2 1.3
24 12 1
64.9 32.4 2.7
.80
20 94
17.5 82.5
10 67
13.0 87.0
10 27
27.0 73.0
.06
47 67
41.2 58.8
24 53
31.2 68.8
23 14
62.2 37.8
.002
Significant P values are in bold type.
went surgery before age 6 months had a mean hospital length of stay (23.5 days vs 11 days; P < .001) and underwent more procedures (2.55 vs 1.81; P = .004) (Table III). The mean weight at the first procedure was 9.04 kg, with a median of 6.25 kg. Patients weighing <5 kg at the first procedure had longer median hospital length of stay (33 days vs 13.51 days; P < .001), including ICU stay (7.00 days vs 1.38 days; P = .047) and underwent more procedures (3.00 vs 1.93; P = .007). They also obtained choanal patency less frequently than patients weighing >5 kg (63.8% vs 83.3%; P = .047). Unilaterality or Bilaterality Seventy-seven patients presented with unilateral choanal atresia (67.5%), including 44 with right-sided predominance, and 37 patients presented with bilateral choanal atresia (32.5%), corresponding to 151 operated nasal fossae. Patients with bilateral choanal atresia underwent surgery at a younger age (mean, 18 days vs 3.06 years, P < .001; median = 9 days) and lower weight (mean, 2.90 kg vs 12.50 kg; P < .001; median = 2.70). Patients with bilateral choanal atresia had more associated anomalies than patients with unilateral atresia (65.6% vs 31.8%; P = .001). They also had a longer hospital stay (mean, 30.38 days vs 12.12 days; P < .001) and underwent more surgical procedures (mean, 3.19 vs 1.84, P < .001). As shown in Table I, a good choanal airway was reported in 85.7% of children with
Table III. Patient age and weight Measures Weight, kg Mean Median Minimum Maximum Age, y Mean Median Minimum Maximum
Total
Unilateral
Bilateral
9.04 6.25 1.10 65.00
12.50 8.65 3.10 65.00
2.90 2.70 1.10 6.73
2.25 0.70 0.01 17.56
3.06 1.28 0.01 17.56
0.05 0.02 0.01 0.57
P value <.001
(18 d) (9 d) (2 d) (7 mo)
<.001
Significant P values are in bold type.
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Table IV. Surgical prognostic factors influencing the number of procedures for choanal atresia Factors Age at surgery, mo ≥6 <6 Weight at surgery, kg ≥5 <5 Bilateralism No Yes CHARGE No Yes Associated abnormalities No Yes Stenting (unilateral atresia) No Yes Mitomycin No Yes Transpalatal approach No Yes
Average number of procedures
P value
1.81 2.55
.004
1.93 3.00
.007
1.84 3.19
<.001
2.16 2.85
.02
2.15 2.47
.24
1.82 1.86
.28
2.01 3.31
<.001
1.84 2.89
.044
Significant P values are in bold type.
unilateral choanal atresia and in 67.6% of those with bilateral choanal atresia (P = .03). Type of Atresia Atresia was bony in 100 nasal fossae (66.2%) and mixed in 48 nasal fossae (31.8%) (Table II). Two patients had membranous atresia, 1 unilateral and the other bilateral, corresponding to 3 nasal fossae (2.0%). No difference in type of choanal atresia was identified between the patients with unilateral and those with bilateral choanal atresia. The type of atresia was not significantly associated with the outcome after a single procedure or at the end of the management, even if a trend toward better results was observed for membranous atresia. A good result was noted after a single procedure in 34.1% cases of bony atresia, 41% cases of mixed atresia, and 100% cases of membranous atresia (P = .06). Associated Abnormalities The total rate of coexisting congenital abnormalities (including CHARGE) was 41.2% (47 of 114) (Tables II and IV). Bilateral choanal atresia was significantly more frequently associated with additional abnormalities (P = .002). The patients with associated abnormalities did not undergo more operations (2.47 vs 2.16; P = .24) but the patients with partial stenosis at the end of management were more likely to have associated abnormalities (73.70% vs 26.30%) compared with those with normal patency (38.50% vs 61.50%; P =.019). The following diseases involving facial or midline abnormalities were found: Down syndrome (4 patients), isolated coloboma (2 patients), laryngomalacia (2 patients), nasal fistula (2 patients), Crouzon syndrome (2 patients), 22q11
microdeletion (2 patients), Opitz syndrome (1 patient), palatal cleft (1 patient), anal anomaly (1 patient), and esophagotracheal cleft (1 patient). A syndromic association (including CHARGE) was diagnosed in 29 of the 114 children (25.4%), and 18 children (15.8%) had associated abnormalities not formally integrated in a known syndromic association. Heart disease was diagnosed in 11 children, all of 2 with a syndromic association (8 with CHARGE, 1 with Down syndrome). CHARGE association was diagnosed in 20 children (17.5%). Patients with CHARGE association had a non-statistically significant increased prevalence of bilateral choanal atresia (50% vs 27.7%; P = .065). CHARGE syndrome did not significantly modify the final outcome of the surgical management, but was associated with an increased number of operations (2.85 vs 2.16; P = .02). All 3 patients who died during follow-up presented with CHARGE syndrome. Surgical Approach In patients with unilateral choanal atresia, surgery was performed via a transpalatal approach in 28 of 77 cases (36.4%) and an endoscopic transnasal approach in the other 49 cases (63.6%). For the 49 transnasal approaches, a microdebrider was used in 38 cases, a laser was used in 8 cases, and drilling was performed in 3 cases. Patients with bilateral choanal atresia underwent surgery via a transpalatal approach in 27 of 37 cases (73.0%) and via an endoscopic transnasal approach in the other 10 cases (27.0%). Surgery was performed using a microdebrider in 6 cases, a laser in 2 cases, and drilling in 2 cases. Combining unilateral and bilateral choanal atresia, surgery was performed in 82 nasal fossae using a transpalatal approach and in 69 using a transnasal endoscopic approach. In the latter group, a microdebrider was used in 50 nasal fossae, a laser was used in 12 nasal fossae, and drilling was performed in 7 nasal fossae. The approach used at the first procedure appeared to determine the surgical results; 72.0% of transpalatal procedures required a second-stage surgery, compared with 50.7% of endonasal procedures (P = .014) (Table V; available at www.jpeds.com). However, when adjusted for age and weight, this association became nonsignificant (P = .120). Patients operated on by a transpalatal approach underwent more procedures (2.89 vs 1.84; P = .044) (Table IV). In the same subgroup (unilateral or bilateral), no significant differences could be highlighted in terms of results of surgery between the approaches and the technique performed. Adjuvant Therapies (Stenting and Mitomycin) Stenting was performed in 85.4% (129 of 151) of the nasal fossae and in 74.5% (85 of 114) of the patients. Stenting was performed in 97.3% (36 of 37) of cases of bilateral choanal atresia and in 63.6% (49 of 77) of cases of unilateral choanal atresia. The mean duration of stenting was 27.7 days (median, 27 days), with no significant difference between unilateral and bilateral choanal atresia cases. The number of procedures was the same with or without stenting for cases of unilateral choanal atresia (1.86 vs 1.82; P = .28) (Table IV).
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Bilateral
Unilateral
choanal atresia
choanal atresia
Symptomatic
Asymptomatic
Preoperative assessment: Craniofacial CT-scan Transthoracic ultrasounds Renal function
WAIT
Nasal endoscopy under general anesthesia
Complete preoperative and associated abnormalities assessment
Passage of endoscope + instrument: possible
Passage of endoscope + instrument: NOT possible
>6 months >5 kg
Endoscopic transnasal approach
Transpalatal approach
Endoscopic transnasal approach
Figure. Management algorithm for choanal atresia.
Mitomycin application was significantly associated with a greater number of procedures (3.31 vs 2.01; P < .001) but not with final results in terms of patency (Table IV).
Discussion Our present series has allowed the elucidation of significant prognostic factors of choanal atresia surgery.9 The main prognostic factors of choanal atresia repair were age, weight at the first procedure, and bilateralism. From these observations, we can recommend algorithms for the management of unilateral and bilateral choanal atresia (Figure). In studying choanal atresia, bilateralism, age, weight, and the type of approach are so enmeshed that the results of sta-
tistical analysis must to be interpreted carefully. Our study highlights the importance of age and weight in the surgical management of choanal atresia, with a greater number of procedures needed to obtain good choanal patency before age 6 months and/or weight >5 kg. Consequently, for patients with unilateral choanal atresia, waiting until age >6 months or weight >5 kg (when possible) tends to reduce the number of procedures necessary to obtain good nasal patency. This is explained by the absolute size of the patient: the larger the patient, the wider the choanal arches can be opened and the vomer resected, with less consequent risk of scarring and restenosis. This observation is concordant with previous studies available in the literature.8 The type of choanal atresia was determined using the classification system of Brown et al, who reported 29% cases of
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pure bony atresia and 71% cases of mixed bony and membranous atresia in their series.19 In our series, the atresia was identified as pure bony in 66.2% of cases, mixed in 31.8%, and membranous in 2.0%, similar to the previous observations. Membranous atresias are rare and embryologically unclear. In our series, the diagnosis of membranous atresia on computed tomography scan was confirmed during surgery, with absence of the bony plate and no need for bony resection in the 2 cases. The timing of surgery for bilateral choanal atresia must be guided by clinical tolerance of the nasal obstruction and the presence of associated abnormalities. Globally, children with bilateral choanal atresia undergo surgery during the first week. Patients with bilateral choanal atresia more often underwent transpalatal procedures because of small nasal fossae, precluding the simultaneous use of an endoscope and instruments. This approach was more likely to fail after the first procedure, but the type of initial approach was nonsignificantly associated with the success rate when adjusted for age and weight in multivariate analysis. This is explained by the fact that the patients undergo surgeries until satisfactory nasal breathing is achieved. Consequently, the choice of approach relies on the possibility of endoscopic procedure; most published studies recommend this approach when possible.6-8,18,20-23 We also recommend the use of endoscopic techniques whenever possible. These techniques allow the restoration of nasal patency using the natural airway, without detachment of the palatal fibromucosa, with consequent lower risks of lesions of the palatal pedicle and of wound healing disorders. Furthermore, optical magnification of the operated area allows precise excision of the atretic plate. Endoscopic approaches lead to short operative times, minimal bleeding, early feeding after surgery, and short hospital stay. Over the 30 last years, the development of endoscopic techniques has reduced the indications for transpalatal approaches. The instruments used in this series followed the technical advances during this period. Drilling and mucosal debridement using a microdebrider have become gold standard techniques in endoscopic approaches. Laser, widely used in the 1990s, is now mainly used for revision procedures, when patency is insufficient due to the presence of granulation tissue or fibrous healing.5,15,24,25 Balloon dilation, developed more recently, is particularly helpful in restenosis and can be preceded by laser incisions of the fibrous ring for better efficacy.21,26,27 In our protocol, stenting was systematically performed for bilateral choanal atresia, as is recommended in neonates who undergo surgery at a young age and low weight.21 A change in approach in our series of unilateral choanal atresia cases occurred in 2008. Before 2008, 86.0% (49 of 57) of unilateral choanal atresias were stented, whereas none have been stented since 2008. Our results indicate that in cases of unilateral choanal atresia, not stenting is as safe as stenting, because this change did not change the final results in terms of patency or number of procedures (1.79 before 2008 vs 2.00 after 2008; P = .95). These results differ from those of studies that have reported poorer results when stenting is performed in endoscopic
transnasal approaches, but lead to the same conclusion with abandonment of stenting.7,16,28 Mean stent duration (27.7 days) was negatively influenced by the long-term analysis of this series. A modification of the duration of stenting was applied with the development of endoscopic repair and a study by Van den Abbeele et al recommending the avoidance of prolonged nasal stenting; the duration of stenting dropped from 36.14 days before 2002 to 10.83 days after 2002 (P < .001).7 In our current management, stenting is planned to last for 1-2 weeks. Mitomycin was used in our management as adjuvant therapy in refractory atresias and was associated with poorer results. Studies about the efficacy of mitomycin are controversial, with no prospective study demonstrating its efficacy available in the literature.11-13 In the present study, we could not evaluate the efficacy of mitomycin, and thus make no recommendations. The long duration of stay reported in our series is explained by the fact that it includes the entire perioperative period, even preoperative and postoperative ICU stays for neonates with bilateral choanal atresia. In addition, it includes every stay for initial repair and revision surgery. For initial and planned repair, which excludes cases of respiratory distress, patients are hospitalized the day before surgery and discharged according to the postoperative course. For endoscopic revision surgery, outpatient surgery has become a standard of care over the last decade in our hospital. In our series, 2 patients presented with complete restenosis of unilateral choanal atresia; 1 is scheduled for revision surgery, and the other decided not to undergo revision surgery. Over the period covered by this study, some modalities for diagnosis have also changed. Diagnostic imaging (CT scan, magnetic resonance imaging [MRI], and ultrasound) and cytogenetic testing have developed and become more accessible. All the patients of this series underwent CT scan before surgery, without major improvement in recent years in terms of diagnosis of choanal atresia (or of the absence of lateral semicircular canals). Other abnormalities may be more accurately diagnosed today, such as the absence of olfactory bulbs on recent MRI. However, the abnormalities explored in this series (eg, CHARGE, facial abnormalities, Down syndrome) are clinically diagnosed and were already well known in 1986. Other patient characteristics of our series are similar to those described in the literature.15,25 Females were predominant, and unilateral choanal atresia was more frequent than bilateral, with right-sided predominance. Associated abnormalities were frequent: approximately 60% in bilateral cases and 30% in unilateral cases. CHARGE association was the most common (approximately 20% of all patients). Recent studies described “3C” syndrome (choanal atresia, coloboma, and lateral semicircular canal hypoplasia or absence).8 Complementary investigations are mandatory before surgery. A craniofacial CT scan is required for anatomic analysis before surgery. Other investigations are useful to anticipate any increased risk of general anesthesia: transthoracic ultrasound for cardiac assessment and blood sample with renal function, given the possibility of heart and/or renal failure in syndromic associations like CHARGE. Other examinations, including cytogenetic
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THE JOURNAL OF PEDIATRICS • www.jpeds.com testing, ocular examination, audiologic testing, genitourinary ultrasound and cerebral MRI, may be performed, but surgery should not be delayed due to respiratory distress. In our series, the presence of comorbidities and CHARGE did not increase the number of procedures and only slightly altered the final results in terms of permeability. Confounding factors, such as lower weight and prematurity, may skew the results in this population. CHARGE association is severe and associated with extrarhinologic complications that were the cause of death of 3 patients in our series. Other studies have reported conflicting results regarding the effect of associated abnormalities and the course of choanal atresia, with some finding no effect on outcomes15 but others associating CHARGE with an increased risk of failure with endoscopic repair.3 The objective evaluation of nasal airflow and nasal fossae patency in children is limited by the feasibility of examination at this age. The measurement of nasal airflow is particularly difficult because it requires the child’s cooperation. For example, peak nasal inspiration flow and rhinomanometry are hardly feasible before age 5 years. Acoustic rhinometry does not seem to be useful in posterior nasal pathology.29 Objective assessment of the choanal opening requires either imaging with radiography exposure or the use of measuring instruments under general anesthesia. These techniques are not recommended in the postoperative management of choanal atresia. Thus, the majority of studies of choanal atresia have evaluated airflow and patency based on subjective findings, such as the quality of nasal breathing (based on noise, indrawing, feeding, or discomfort), presence of rhinorrhea, or evaluation of the choanal area by endoscopy or nasofibroscopy. Objective evaluation of airflow or the choanal area after choanal atresia repair in children is a challenge for future work. ■ Submitted for publication Jun 6, 2018; last revision received Aug 13, 2018; accepted Aug 29, 2018 Reprint requests: Eric Moreddu, MD, Department of Pediatric Otolaryngology Head and Neck Surgery, La Timone Children’s Hospital (Aix-Marseille University), 264 Rue Saint Pierre, Marseille 13385 CEDEX 5, France. E-mail: [email protected]
References 1. Harris J, Robert E, Källén B. Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics 1997;99:363-7. 2. Negus VE. The function of the epiglottis. J Anat 1927;62(Pt 1):1-8. 3. Schraff SA, Vijayasekaran S, Meinzen-Derr J, Myer CM. Management of choanal atresia in CHARGE association patients: a retrospective review. Int J Pediatr Otorhinolaryngol 2006;70:1291-7. 4. Khafagy YW. Endoscopic repair of bilateral congenital choanal atresia. Laryngoscope 2002;112:316-9. 5. Pototschnig C, Völklein C, Appenroth E, Thumfart WF. Transnasal treatment of congenital choanal atresia with the KTP laser. Ann Otol Rhinol Laryngol 2001;110:335-9. 6. Uzomefuna V, Glynn F, Al-Omari B, Hone S, Russell J. Transnasal endoscopic repair of choanal atresia in a tertiary care centre: a review of outcomes. Int J Pediatr Otorhinolaryngol 2012;76:613-7.
Volume ■■ 7. Van Den Abbeele T, Francois M, Narcy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg 2002;128:936-40. 8. Guls¸en S, Baysal E, Celenk F, Aytaç I, Durucu C, Kanlikama M, et al. Treatment of congenital choanal atresia via transnasal endoscopic method. J Craniofac Surg 2017;28:338-42. 9. Triglia JM, Nicollas R, Roman S, Paris J. Choanal atresia: therapeutic management and results in a series of 58 children. Rev Laryngol Otol Rhinol (Bord) 2003;124:139-43 (in French). 10. Newman JR, Harmon P, Shirley WP, Hill JS, Woolley AL, Wiatrak BJ. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg 2013;139:71-5. 11. Prasad M, Ward RF, April MM, Bent JP, Froehlich P. Topical mitomycin as an adjunct to choanal atresia repair. Arch Otolaryngol Head Neck Surg 2002;128:398-400. 12. Holland BW, McGuirt WF Jr. Surgical management of choanal atresia: improved outcome using mitomycin. Arch Otolaryngol Head Neck Surg 2001;127:1375-80. 13. Carter JM, Lawlor C, Guarisco JL. The efficacy of mitomycin and stenting in choanal atresia repair: a 20 year experience. Int J Pediatr Otorhinolaryngol 2014;78:307-11. 14. Romeh HE, Albirmawy OA. A 13-year experience and predictors for success in transnasal endoscopic repair of congenital choanal obliteration. Int J Pediatr Otorhinolaryngol 2010;74:737-42. 15. Teissier N, Kaguelidou F, Couloigner V, François M, Van Den Abbeele T. Predictive factors for success after transnasal endoscopic treatment of choanal atresia. Arch Otolaryngol Head Neck Surg 2008;134:57-61. 16. Schoem SR. Transnasal endoscopic repair of choanal atresia: why stent? Otolaryngol Head Neck Surg 2004;131:362-6. 17. Rombaux P, de Toeuf C, Hamoir M, Eloy P, Bertrand B, Veykemans F. Transnasal repair of unilateral choanal atresia. Rhinology 2003;41:31-6. 18. Velegrakis S, Mantsopoulos K, Iro H, Zenk J. Long-term outcomes of endonasal surgery for choanal atresia: 28 years experience in an academic medical centre. Eur Arch Otorhinolaryngol 2013;270:113-6. 19. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope 1996;106(1 Pt 1):97-101. 20. Deutsch E, Kaufman M, Eilon A. Transnasal endoscopic management of choanal atresia. Int J Pediatr Otorhinolaryngol 1997;40:19-26. 21. Riepl R, Scheithauer M, Hoffmann TK, Rotter N. Transnasal endoscopic treatment of bilateral choanal atresia in newborns using balloon dilatation: own results and review of literature. Int J Pediatr Otorhinolaryngol 2014;78:459-64. 22. Uri N, Greenberg E. Endoscopic repair of choanal atresia: practical operative technique. Am J Otolaryngol 2001;22:321-3. 23. Stankiewicz JA. The endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg 1990;103:931-7. 24. Tzifa KT, Skinner DW. Endoscopic repair of unilateral choanal atresia with the KTP laser: a one-stage procedure. J Laryngol Otol 2001;115:286-8. 25. Friedman NR, Mitchell RB, Bailey CM, Albert DM, Leighton SE. Management and outcome of choanal atresia correction. Int J Pediatr Otorhinolaryngol 2000;52:45-51. 26. Bedwell J, Shah RK, Bauman N, Zalzal GH, Preciado DA. Balloon dilation for management of choanal atresia and stenosis. Int J Pediatr Otorhinolaryngol 2011;75:1515-8. 27. Goettmann D, Strohm M, Strecker EP. Treatment of a recurrent choanal atresia by balloon dilatation. Cardiovasc Intervent Radiol 2000;23:4801. 28. Gosepath J, Santamaria VE, Lippert BM, Mann WJ. Forty-one cases of congenital choanal atresia over 26 years—retrospective analysis of outcome and technique. Rhinology 2007;45:158-63. 29. Leclerc JE, Leclerc JT, Bernier K. Choanal atresia: long-term follow-up with objective evaluation of nasal airway and olfaction. Otolaryngol Head Neck Surg 2008;138:43-9.
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Table I. Final surgical outcomes of choanal atresia surgery in function of laterality Total
Unilateral
Bilateral
Outcomes
n
%
n
%
n
%
No stenosis Partial stenosis Stenosis Missing data Dead
91 14 2 4 3
82.73 12.73 1.82 3.64 2.73
66 8 2 1 0
85.71 10.39 2.60 1.30 0
25 6 0 3 3
67.57 16.22 0 8.11 8.11
Table V. Surgical technique and number of nasal fossae requiring revision surgery Techniques Transpalatal approach, n Requiring revision surgery, n Endoscopic approach, n Requiring revision surgery, n
Total
Unilateral
Bilateral
82 59 69 35
28 17 49 24
54 42 20 9
The differences between the groups were significant (P = .03 for all).
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