Correction of congenital choanal atresia in children and adults

Congenital choanal atresia occurs once in 7,000 to 8,000 births. The atresia is bony in most cases, more common in females, and more often right-sided in patients with unilateral atresia. The incidence of associated congenital anomalies is relatively high. During the 25-year period 1951 to 1976, 36 patients with choanal atresia were treated at the Mayo Clinic; 19 were females and 17 were males. The ages at the time of surgical treatment ranged from 1 day to 58 years. Twenty-four patients had unilateral obstruction, and 12 had bilateral obstruction. Six patients (17%) had other associated congenital anomalies. Intranasal perforation and transpalatal correction were most commonly employed in children who were less than 8 years old, and transseptal correction was most often carried out in older patients. The nature of the stenting material and the age of the patient have important bearing on the outcome of the operation. Congenital choanal atresia is a bony or membranous closure of the posterior choana. It may be complete or incomplete and unilateral or bilateral. We wish to report on a series of children and adults who were treated for congenital choanal atresia at the Mayo Clinic during a 25-year period and to describe a transseptal method of correction that may be performed concurrently with septal reconstruction and external nasal surgery in older children and adults.     

Management and outcome of choanal atresia correction

OBJECTIVES: Outcome analyses of factors that may either maximize success or predict a better outcome following choanal atresia correction. METHODS: A retrospective review of children undergoing choanal atresia correction at Great Ormond Street Hospital for Children, London between January 1990 and April 1998. Children with unilateral or bilateral choanal atresia were studied. In all cases, correction was by a transnasal approach under endoscopic control. A 120 degrees Hopkins rod telescope was used to visualize the atretic plate from the nasopharynx. Straight urethral sounds were used to perforate the plate followed by use of an air drill to remove the bony component. Portex endotracheal tubes were subsequently inserted as nasal stents. RESULTS: Sixty-five children (19 M, 46 F: age range 1 day to 17 years) presented with choanal atresia and the outcomes for 46 were included in the study. Twenty-six patients (40%) had other major anomalies. In children with unilateral atresia neither duration of stenting nor presence of facial anomalies had an impact on outcome. Of those children with bilateral choanal atresia and associated facial anomalies (n=9), 56% were asymptomatic following correction. In children with isolated bilateral choanal atresia (n=19), 74% were asymptomatic following correction; 29% (n2.3 kg (n3. 5 mm (n=6) had an 83% chance of a good outcome. Those patients stented with a tube 相似文献   

Choanal atresia: a retrospective study of 39 cases]

The purpose of this study was to investigate the clinical characteristics and the surgical management in patients with choanal atresia. We performed a retrospective study at the Saint-Luc University hospital, Brussels, between January 1988 and June 2000. Surgical corrections were performed using different approach (transnasal endoscopic, transpalatal) and different instrumentations (urethral sounds, laser nd-YAG, laser CO2, microdebrider). Portex endotracheal tubes were inserted as nasal stents in the vast majority of the patients with bilateral choanal atresia. Thirty-nine children with choanal atresia (22 unilateral and 17 bilateral) (9: M, 30: F) were studied. 38 of them were surgically managed. Based on clinical inspection and On CT-Scan, choanal atresia was defined as membranous for 4 patients, osseous for 6 and mixte for 29. Associated congenital anomalies were found in 22.7% of unilateral and in 70.5% of bilateral choanal atresia (Total: 43.5%). Of those children with bilateral choanal atresia, 75% were asymptomatic after four surgical procedures. In children with unilateral choanal atresia, 45% were asymptomatic after one surgical correction and 100% after three surgical corrections. Four patients were managed using an endoscopic endonasal approach with the microdebrider and showed no evidence of recurrence. Outcome analyses of factors that may influence the results of surgery are difficult to establish since many different surgical options were taken during this period. However, it seems that bilateral choanal atresia is associated with more surgical corrections before achieving a normal nasal breathing than unilateral choanal atresia. After this review, our current strategy regarding the choanal atresia will be to begin with the endoscopic endonasal approach using the microdebrider (stents if bilateral) and to propose the transpalatal approach in case of recurrence.     

Unilateral choanal atresia: initial presentation and endoscopic repair

The purpose of this study was to investigate the severity of presenting symptomatology in patients with unilateral choanal atresia and to assess the surgical results of patients undergoing transnasal endoscopic repair. A retrospective review of all patients with choanal atresia presenting to the author between 1990 and 1997 was performed, identifying 13 patients with unilateral choanal atresia. These patients were analyzed with respect to presenting symptomatology, preoperative computed tomography scans, appearance, type of surgical repair, and surgical results for patients undergoing endoscopic transnasal repair. Eleven patients underwent successful endoscopic transnasal repair, one patient underwent conversion to transpalatal repair after an unsuccessful attempt at transnasal endoscopic repair and one patient underwent a planned transpalatal repair. Eleven patients who underwent repair by transnasal endoscopic technique have all done well with follow-up ranging from 3 months to 7 years. Although the symptomatology of unilateral choanal atresia is not as dramatic as bilateral choanal atresia, significant airway symptomatology may be present, especially in children presenting at a very young age. It appears that transnasal repair of unilateral choanal atresia is an excellent treatment modality with excellent postoperative surgical results.     

Topical mitomycin as an adjunct to choanal atresia repair

OBJECTIVE: To evaluate the use of topical mitomycin in choanal atresia repair to reduce the development of granulation tissue and cicatrix. DESIGN AND SETTING: Retrospective case series in 2 tertiary care centers. PATIENTS: Twenty patients with either unilateral or bilateral congenital choanal atresia underwent repair using the transnasal endoscopic approach, the transpalatal approach, or both. INTERVENTIONS: The surgeons favor the use of the endoscopic transnasal drillout technique for all unilateral cases of choanal atresia and for selected bilateral cases. We describe our experience and treatment paradigm for these 20 patients (15 with unilateral atresia, 5 with bilateral atresia). Topical application of mitomycin was used, and in some cases postoperative stenting, for a period of 1 to 2 weeks. In 8 cases, a second application of mitomycin was used. Follow-up ranged from 3 months to 2 years (mean, 9 months). OUTCOME MEASURE: The patency of the choanae without respiratory distress or nasal drainage, as assessed by endoscopic evaluation, determined a successful repair. RESULTS: Of the 20 patients, 17 retained patent airways. Three patients experienced improvement from a total atresia to a narrowed, stenotic choana. CONCLUSIONS: The use of mitomycin as an adjunct to the surgical repair of choanal atresia may offer improved patency with a decreased need for stenting, dilatations, and revision surgery. Newer endoscopic techniques with powered instrumentation further enhance the safety and efficacy in the repair of choanal atresia.     

Choanal atresia in premature dizygotic twins--a transnasal approach with Holmium:YAG-laser

Twins born prematurely presented with choanal atresia and were successfully treated using a transnasal, endoscopically and microscopically controlled Ho:YAG-Laser assisted technique. One twin, who had bilateral choanal atresia was operated immediately, the other one, with unilateral choanal atresia, received surgery 8 months after birth. The rare feature of twins both suffering from choanal atresia and a technique for definitive treatment of this disease in premature neonates are presented and discussed. This report of dizygotic twins with nonsyndromal choanal atresia suggests the possibility of an autosomal recessive inheritance with various penetration or an undefined teratogenic etiologic factor.     

Choanal atresia and associated anomalies: the CHARGE association

Over the past few years there has been increasing awareness of the association of choanal atresia with other congenital defects. Thirty-one cases of choanal atresia were reviewed and other congenital anomalies were documented in 19 patients. These other anomalies were identified in 75% of the 20 patients with bilateral choanal atresia but in only 36% of the 11 patients with unilateral choanal atresia. Associated defects largely occurred in a predictable pattern described by the CHARGE mnemonic: C-coloboma, H-heart disease, A-atresia choanae, R-retarded growth, G-genital hypoplasia, E-ear defects. The implications of the CHARGE association in the management of patients with choanal atresia are discussed.     

Choanal atresia and associated anomalies

The authors have studied 130 cases of choanal malformation. They found 53 bilateral atresias, 51 unilateral atresias and 26 cases of choanal stenosis. Fifty-seven of these cases were associated with other anomalies and 38 had at least two features of the CHARGE association (C, colobomas; H, heart defects; A, atresia choanae; R, retarded growth; G, genito-urinary defects; E, ear defects). The pathogenesis of choanal atresia in regard to the abnormal migration of neuroectodermal cells is discussed.     

Surgical treatment of choanal atresia

The indications for surgery of congenital choanal atresia and the different surgical approaches are discussed in detail. The transnasal approaches should be done only in emergency situations or as first-aid treatment; the transmaxillary approach is not important today. The transpalatinal approach in the head-down position under microsurgical conditions has proved to be much better. Thirty-six cases with congenital choanal atresia were observed in 15 years. Thirty-four patients were operated on in infancy or childhood and controlled later on. Four children showed bilateral choanal atresia; the other patients had unilateral complete atresia. In 8 patients a transnasal approach was done, in 6 cases there was a recurrence in the form of a complete stenosis and after transpalatinal re-operations there was a relapse in only one case. One patient was operated on the transmaxillary approach. In 25 cases the transpalatinal approach was the method of operation; relapse was seen in 5 cases only and could be revised by transpalatinal approach, one case excepted. Own experience and the present results show that the transpalatinal approach is suitable for adults as well as newborn babies and infants. On account of the better view of the operating area and optimal reconstructive conditions, the transpalatinal approach appears to be safer and long-term results are good.     

Choanal atresia and its associated anomalies. Further support for the CHARGE Association

The finding of choanal atresia warrants careful and prompt consideration of other severe congenital anomalies in the newborn. In a chart review of 41 patients with the diagnosis of choanal atresia, 17 patients (41%) had choanal atresia alone while 24 (59%) had at least one other anomaly. Twenty-four (59%) of all patients had bilateral choanal atresia, and 75% of the patients with at least one other anomaly had bilateral choanal atresia. The following features were found to be associated with bilateral choanal atresia: significant structural heart disease (21%), postnatal growth retardation (36%), psychomotor retardation (19%), sensorineural deafness (21%), velopharyngeal discoordination (15%), micrognathia (12%), blocked tear duct (10%), high-arched palate (15%), and facial asymmetry (7%). Twenty-one other anomalies were described. Coloboma was encountered in 10% of our patients and only 3 patients had all of the features originally described in the CHARGE Association. The non-random association of multiple anomalies suggested by the eponym, CHARGE is discussed, and further data is offered for evaluating the child who presents with choanal atresia.     

An update on choanal atresia surgery at Great Ormond Street Hospital for Children: preliminary results with Mitomycin C and the KTP laser

We present the results of transnasal choanal atresia correction in 46 children, as an update to the published Great Ormond Street Hospital series. Females outnumbered males 2-1, and half the cases in our series were bilateral. Eight of the children with bilateral atresia had the CHARGE association. The median number of procedures required was three over a period of up to 3 years. Eighty-two percent of children with unilateral atresia and 78% of those with bilateral atresia were asymptomatic at the time of their last follow up. Four deaths occurred, all but one in children with CHARGE association. We were not able to demonstrate any benefit from the use of Mitomycin C, the KTP laser, betamethasone nasal drops or (in unilateral cases) stents.     

Endoscopic transnasal repair of choanal atresia

OBJECTIVES: To present our experience with a new endoscopic technique for transnasal repair of choanal atresia. METHODS: Seventeen patients aged 2 months to 13 years with choanal atresia, bilateral in 6 and unilateral in 11, underwent endoscopic repair using a mucoperichondrial flap developed from the nasal septum. The bony stenosis was opened with a surgical curette or drill, and the raw surface was covered by the flap. All patients in these case series with bilateral atresia had been treated with dilatation at birth and had restenosis. RESULTS: A total of 23 choanae were operated. Follow-up ranged from 10 to 60 months. There was one case of complete restenosis and one of partial restenosis, for a success rate of 91%. CONCLUSIONS: Endoscopic repair of choanal atresia is a safe and rapid procedure even in very young children, with no complications and a high rate of success.     

Transnasal treatment of congenital choanal atresia with the KTP laser

This retrospective analysis presents a minimally invasive method for a transnasal approach to treat bilateral and unilateral choanal atresia and stenosis in infants and children. We describe an advanced surgical technique that applies the use of a KTP laser and give recommendations for preoperative diagnosis and postoperative assessment. We report 13 cases of bilateral and unilateral choanal atresia or stenosis treated over a 3 1/2-year period. We used a transnasal approach and endoscopic control. For bilateral choanal atresia, the operation was performed within the first few days of birth. For unilateral choanal atresia or stenosis, surgery was performed several weeks after birth. In all cases, an intranasal stent was inserted. Our findings demonstrate that this transnasal approach provides significant benefits. A primary advantage is the diminished risk of intraoperative or postoperative complications. Additional benefits include lower rates of re-obstruction and a decreased incidence of subsequent disease, including chronic secretory otitis media.     

Transnasal repair of unilateral choanal atresia

OBJECTIVES: To evaluate the outcomes of the surgical correction of unilateral choanal atresia using a transnasal approach. PATIENTS AND METHOD: Over a 36-months period (from 1999 to 2001), seven children underwent endoscopic endonasal repair of an unilateral choanal atresia using the microdebrider (powered instrumentation). At the end of the procedure, topical application of Mitomycin-C was performed. No postoperative nasal stenting was inserted. Clinical characteristics of these patients, CT scan examination, complications of the procedure and outcomes were analysed and compared to historical cases treated in the same institution from 1990 to 1998. RESULTS: Seven patients (2 M/5F) (age 6 to 46 months) presented with primary unilateral choanal atresia and were operated during the period from 1999 to 2001. All the patients were symptomatic before surgical correction. No patients showed other facial anomaly. Of the 7 patients procedures, 6 (85.7%) remained patent (follow-up range 12 to 36 months). Mean surgical repair per patient before obtaining patent choana was 1.14. One patient required surgical transnasal revision nine months after the initial procedure with a patent choanal after this second procedure (follow-up 9 months). Minor turbinoseptal synechiae diagnosed 6 months after the surgical correction occurred in one patient and was the only postoperative complication. When compared to historical cases of unilateral choanal atresia (19 patients from 1990 to 1998) repaired without endoscopic control and without Mitomycin-C, it was shown that this current method provided better results; mean surgical repair per patient; 1.14 vs 1.89 and 85.7% of patent choanae at twelve months vs 47.3%. CONCLUSION: An endoscopic endonasal approach without postoperative stenting, using the microdebrider seems to us the treatment of choice for unilateral choanal atresia. The exact role of the topical application of Mitomycin-C needs to be further investigated.     

Endoscopic choanoplasty without stenting for congenital choanal atresia repair

Objective

To present our experience with an endoscopic technique for congenital choanal atresia transnasal repair without the use of intranasal stents.

Methods

Twenty-one patients with congenital choanal atresia (11 bilateral aged 2-27 days and 10 unilateral aged 2-16 years) were operated upon between December 2005 and January 2009, using an endoscopic choanoplasty technique in which a laterally based mucoperiosteal flap was elevated off the posterior nasal septum and atretic plate. Endoscopic resection of bony atresia including abnormally thickened vomer and pterygoid processes was done until a widely patent ‘neo-uni-choana’ was created. Two surgeons worked simultaneously using the “four-hand” technique. No stents were used. All patients received prophylactic systemic antibiotics and local steroid-antibiotic intranasal drops for 1 week postoperatively. Parents were taught to irrigate and clean both nasal cavities using frequent saline nasal drops and a portable home suction device.

Results

Preoperative computed tomography showed that among bilateral choanal atresia (BCA) patients, 9 (81.8%) were bony-membranous and 2 (18.2%) were pure bony atresia. All unilateral choanal atresia (UCA) cases had pure bony atresia. Two neonates with BCA had associated congenital cardiac anomalies. None had the CHARGE association. Operative time ranged from 30 to 75 min (mean 51.4 ± 13.4 min.) for BCA and 35 to 90 min (mean 57.5 ± 18 min.) for UCA repairs. Mean estimated intraoperative blood loss was 35.5 ± 15.1 cc. in BCA and 204 ± 105.6 cc. in UCA surgery. No intraoperative complications such as skull base damage or CSF leak were encountered. Patients were followed-up for periods ranging from 6 to 18 months in BCA and 12 to 24 months in UCA cases. Three BCA cases showed postoperative restenosis by granulation tissue that was successfully managed by endoscopic excision with good nasal breathing thereafter. Complications included mid-nasal synechiae, not interfering with nasal breathing, in three BCA cases and a small hard palate perforation in one UCA patient that healed conservatively, without further surgery needed.

Conclusions

Transnasal endoscopic choanoplasty repair for both unilateral and bilateral choanal atresia without intranasal stenting was found to be an expedient and safe procedure that affords minimal blood loss, minimal complications with a high rate of success.     

Choanal atresia: clinical features, surgical approach, and long-term follow-up

Twenty-one patients with choanal atresia were treated from 1969 to 1985. Follow-up of 1 1/2 to 15 years postoperatively has been obtained in 17 patients. Thirteen patients had bilateral atresia and eight had unilateral atresia. All 13 patients with bilateral atresia were operated on within the first month of life. Eighteen patients were treated transnasally and two transpalatally. Ten of 15 patients treated by transnasal puncture and dilation followed by stenting of the choanae are patent after a single procedure. Five other patients, all of whom had other associated anomalies, required secondary dilations or transpalatal repair. Our results support the effectiveness of transnasal puncture and dilatation followed by stenting of the choanae as the initial surgical procedure of choice. It may be used safely in the neonate, and is often definitive.     

Congenital choanal atresia and nasal stenosis

The most common form of nasal obstruction in neonates is soft tissue edema, but congenital bony nasal anomalies are recognized as an important cause of newborn airway obstruction. We reviewed 20 cases of congenital bony nasal abnormalties such as choanal atresia and nasal stenosis referred to National Children's Hospital between 1996 and 2001. Of the 20, 8 involved choanal atresia (6 bilateral and 2 unilateral) and 12 nasal stenosis (5 nasal cavity stenosis, 2 pyriform aperture stenosis and 5 unknown). Six cases of bilateral choanal atresia and 5 of 12 cases of the nasal stenosis presented severe airway distress. Most cases in respiratory distress required immediate surgical enlargement of nasal cavity and stenting with an endotracheal tube for 4 to 8 weeks. Of 12 with nasal stenosis, 5 with mild nasal obstruction were treated with nasal drops and 3 with mild airway distress required no treatment. Five of 7 (71%) with choanal atresia and 1 of 4 with nasal stenosis (25%) required restenosis of the nasal cavity after stent removal. Restenosis occurred more often in choanal atresia than in nasal atresia. Two of 5 with nasal restenosis required stenting for 1 to 2 months again and 1 of those was followed by stenting for more 4 months. Longer stenting did not prevent choanal from restenosis, however, no standard stenting protocol exists for neonates, highlighting the need for further study.     

Acquired posterior choanal stenosis and atresia: management of this unusual complication after radiotherapy for nasopharyngeal carcinoma

PURPOSE: To report on acquired posterior choanal stenosis and atresia after radiotherapy for nasopharyngeal carcinoma. MATERIALS AND METHODS: Four patients with acquired bilateral choanal atresia and 2 with severe unilateral choanal stenosis in the posterior choanae were identified after treatment of nasopharyngeal carcinoma with radiotherapy. The mean age was 42 years (range, 29 to 48 years). Two patients had stage II, and 4 had stage III disease, according to Ho's classification. They all received a 66 Gy dose of external irradiation delivered to the nasopharynx, and a mean dose of 62.6 Gy to the neck. Five patients had an additional 20 Gy delivered to the parapharyngeal region, and 1 patient had intracavitatory brachytherapy of 18 Gy delivered to the nasopharynx. The mean onset of symptoms was 10.5 months (range, 2 to 40 months) postirradiation. All patients were treated by transnasal endoscopic resection. Merocel epsitaxis packing (Medtronic Xomed, Jacksonville, FL) was used to stent the nasal airway for 2 weeks postoperatively. RESULTS: The mean follow-up was 16.2 months (range, 14 to 18 months) after surgery. Four patients (67%) were symptom-free. Two patients (33%) had unilateral restenosis in the postnasal space that required revision surgery and further nasal stenting for 2 weeks, and both were subsequently free from further restenosis. No adverse postoperative complication occurred. CONCLUSION: Acquired posterior choanal stenosis and atresia is an unusual long-term complication after radiotherapy that can be successfully treated with transnasal endoscopic resection. A 2-week Merocel nasal stent is recommended to prevent restenosis in the posterior choanae.     

Unilateral choanal atresia: Presentation in adults

Unilateral choanal atresia usually presents later in life than the bilateral one and may present in adults. We discuss three cases of unilateral choanal atresia, which have presented in adult age group and with complaints not clearly pointing to the diagnosis. The role of CT scan in diagnosis and nasal endoscope in the treatment has been discussed.     

Choanal atresia: treatment trends in 47 patients over 33 years

From 1977 through 1985, 11 patients underwent surgical correction of choanal atresia. This group brings the total number of patients corrected at our institution since 1951 to 47. This review compares treatment and outcome in the most recent group with the previous series of 36 patients. Eight patients were female and three were male; their ages at the time of surgical treatment ranged from 1 day to 19 years. The atresia was bilateral in five patients and unilateral in six. Six patients had other physical or mental developmental problems. Mean follow-up was 5.6 years (range, 2 to 10 years). All five patients who had transnasal puncture as primary treatment required reoperation; this is a higher incidence of failure than in the previous group. None of the three patients who had transpalatal procedures needed further operation. In two of the eight patients who had transseptal repair, restenosis developed.