Bilateral Atresia Research Articles

Osseous choanal atresia in a canine patient

AbstractA 2‐year‐old, male, intact dog presented with a history of stertorous breathing since infancy, unresponsive to antibiotic therapy. On rostral rigid endoscopy, there were no remarkable findings. However, computed tomography revealed bilateral atresia of the nasal choanae, with focal hyperossification and severe narrowing, with accompanying hypoventilation of the nasopharynx. Retrograde flexible nasopharyngoscopy confirmed the severe partial choanal atresia. Surgical intervention was declined by the owner due to reasonably acceptable quality of life; therefore, only environmental modifications and symptomatic care have been implemented so far.

Congenital atresia of posterior nares: Diagnosis and treatment at Hilla, Iraq

Abstract Background: Atresia of posterior nares (choana) is a congenital condition in which the posterior nares fail to develop either bilaterally or unilaterally. Objectives: The aim of this article is to show different methods of diagnosis and surgical procedures used for management in our department. Materials and Methods: In this study, 21 patients were included. It is a retrospective study form. The confirmation of the diagnosis is by failure of 6–8-F tube catheter to pass; CT scan of posterior choana shows the type and thickening of atretic plate (cartilage or bone), and endoscopic examination confirms it. Results: Female patients were 15, whereas male patients were 6. Age range was from 1 day to 25 years. All of the patients were within the pediatric age group except one adult patient. Unilateral cases were found in 14 patients, while 7 patients had bilateral atresia. Bilateral atresia was presented within early day life (1–3 days). They were five females and two males. The unilateral cases diagnose at a later life period (4–25 years). There were 4 males and 10 females. Proved by CT scan, mixed bony and membranous atresia were in 18 patients, bony atresia was in 3 patients, and there was no pure membranous atresia. Thirteen of the unilateral cases involved right side, while only one case had left-side atresia (7.6%). Three cases of bilateral atresia had other congenital anomalies (14%). Ten patients were treated by nonendoscopic (transnasal) surgical approach (five of them had bilateral and unilateral atresia, respectively). Endoscopic trans-septal approach was done in two patients, both had bilateral atresia. Nine patients were treated by transnasal endoscopic method. Stent was used in 13 patients postoperatively for 4–6 weeks. Two patients required revision surgery for restenosis. Conclusion: Endoscopic transnasal approach is the proper way in the surgical treatment of atresia because it is more accurate, under surgeon vision, less invasive with less morbidity in spite of longer time to perform.

Cartilage Conduction Hearing Aids in a Patient with Bilateral Atresia of the External Auditory Canal with Chromosome 18q Deletion Syndrome: A Case Report

Cartilage Conduction Hearing Aids in a Patient with Bilateral Atresia of the External Auditory Canal with Chromosome 18q Deletion Syndrome: A Case Report

Low-level complex mosaic with multiple cell lines affecting the 18q21.31q21.32 region in a patient with de novo 18q terminal deletion

We describe a 5-year-old girl who was diagnosed at birth with 18q de novo homogeneous deletion at G-banding karyotype. Her clinical condition, characterized by hypotonia, psychomotor retardation, short stature, deafness secondary to bilateral atresia of the external auditory canals, was in agreement with the 18q deletion syndrome though presence of coloboma of a single eye only suggested a mosaic condition as an unusual sign. By combining multiple technologies including array-CGH, FISH, and WGS, we found that the terminal deletion 18q21.32q23 (21 Mb) was in segmental mosaicism of the proximal region 18q21.31q21.32 (2.7 Mb), which showed a variable number of copies: one, two, or three, in 7, 41 and 55% of the cells respectively. Breakpoint junction analysis demonstrated the presence of an inv-dup del (18q) with a disomic segment of 4.7 kb between the inverted and non-inverted copies of the duplicated region 18q21.31q21.32. From these results, we propose that all three types of abnormal chr18 (the inv-dup del and the two 18q terminal deletions of different sizes) arisen from breaks in a dicentric mirror chromosome 18q, either in more than one embryo cell or from subsequent breaking-fusion-bridge cycles. The duplication region was with identical polymorphisms as in all non-recurrent inv-dup del rearrangements though, in contrast with most of them, the 18q abnormality was of maternal origin. Taking into account that distal 18q deletions are not rarely associated with inv-dup del(18q) cell lines, and that the non-disjunction of chromosome 18 takes place especially at maternal meiosis II rather than meiosis I, multiple rescue events starting from trisomic zygotes could be considered alternative to the postmitotic ones. From the clinical point of view, our case, as well as those of del(18q) in mosaic with the dic(18q), shows that the final phenotype is the sum of the different cell lines that acted on embryonic development with signs typical of both the 18q deletion syndrome and trisomy 18. Asymmetrical malformations, such as coloboma of the iris only in the right eye, confirm the underlying mosaicism regardless of whether it is still detectable in the blood.

Treatment of choanal atresia in a cohort of 29 patients: Determinants for success or failure

BackgroundChoanal Atresia is a congenital condition that presents as a blockage from the nasal cavity to the nasopharynx. According to the German statistical Institute (Statistisches Bundesamt), the incidence in Germany in 2018 reached 2.74 in 10,000 live births.It can present unilaterally or bilaterally. As newborns are obligate nasal breathers, management of bilateral atresia has to be performed early after birth. As for unilateral atresia, the optimal age for treatment was determined to be between 6 and 12 months in a recently published consensus. ObjectiveThe main purpose of this study is to characterize the patients treated for choanal atresia in the Department of Otorhinolaryngology and Head and Neck Surgery at a tertiary hospital in the south of Germany and, based in the demographic characteristics as well as intra- and post-operative treatment, to identify factors for success or failure of the surgery. A secondary goal was to describe the complications in the use of stents and analyse its influence in the results. Material and methodsThe cohort-based observational study included 29 patients, with a minimum follow up of one year, who underwent surgical endoscopic correction of both unilateral and bilateral choanal atresia from 2003 to 2020. Analysis of the demographics, intraoperative, and postoperative treatment, and their results, was performed.Multinomial logistic regression was applied for categorical values. Comparisons were performed using Fischer/chi-square test where applicable. A significance level of 0.05 was reached. ResultsThe population was comprised of 34.5% male and 65.5% female patients. The age varied from 2 days to 20 years old, with a mean of 4.98 years and Standard Deviation (SD) 6.88. The weight of the patients varied between 1.4 kg and 85.0 kg, with a mean of 19.36 and SD 22.58. Unilateral choanal atresia was present in 58.6%, and bilateral in 41.4%. Out of this population, 48.3% presented with associated malformations.The number of procedures per patient performed in general anesthesia varied from 1 to 9, with a mean of 2.52 and SD of 2.23: statistically higher for patients presenting with bilateral atresia with a p value of 0.001*. 20 patients had a tube (Vygon, France) placed at first surgery. In 5 patients, a drug-eluting stent (Propel mini (R); Intersect ENT, USA) was applied intraoperatively, without complications.In unilateral choanal atresia, surgical and functional success was attained in 88.2% of the cases. In bilateral cases, it reached 75%.We observed a strong linear relationship between the weight of the patient and the size of the choana perioperatively: R quadrat 0.596, which may be a determinant factor in the wound healing.Weight, age, and concomitant pathology achieved statistical significance in the logistic regression model (p respectively 0.001*, 0.001* and 0.010*), which shows its influence in the result of the surgery. In particular, weight inferior to 3 kg (p 0.001*, chi-square test) at the time of the first surgery is a determinant demographic factor for need of revision in order to achieve patency.There were no major complications associated with the use of a stent, besides its dislodgement. We could not infer an implication of its use on the success of the surgery in the present cohort. Limitationsfor this study were the small size of the cohort and non-standardized records for some variables. ConclusionsYounger patients presenting with low weight and concomitant pathology have worse results after surgical endoscopic treatment of choanal atresia, revealing these characteristics to be determinant for success. The size of the neochoana is related to the weight of the patient, which is a limiting factor.In particular, patients under 3 kg had to undergo more procedures in order to achieve patency.The implication of the use of locally applied medication needs to be further studied.

A Case of Familial External Auditory Canal Atresia

Introduction: Congenital аural аtresia (CAA) can occur as an isolated congenital malformation or as part of a monogenic and chromosomal syndromes. CAA can be associated with several syndromes. An 18q deletion is frequently seen in CAA. The subject of the study are three individuals from one family (mother, son and daughter). Methods:After taking a detailed history, an examination of ENT organs, tone audiometry testing, auditory brain stem responses, brain CT, external auditory canal and middle ear were performed. Results: The conducted tone audiometry testing and auditory brain stem response found bilateral sound conduction hearing loss. Conclusion: Bilateral atresia is treated surgically. Surgical treatment is difficult and not always successful, it is preferable to perform it at age 4-5.

Two cases of combined anatomical variations: maxillofacial trunk, vertebral, posterior communicating and anterior cerebral atresia, linguofacial and labiomental trunks.

Commonly, arterial anatomic variants are reported as single entities. However, different such variants can occur in a single patient. During a retrospective study of computed tomography angiograms of 52 adult patients, 2 cases were found with unilateral maxillofacial trunks. In each case different other anatomic variants were documented. The maxillofacial trunk in the first case was associated with bilateral posterior kinks of the internal carotid artery which passed beyond the transverse processes of the atlas vertebra and indented and displaced the internal jugular veins. Common carotid origins of the superior thyroid arteries were found, as well as a high origin of the contralateral facial artery. In the second case a plethora of variants were associated with a unilateral maxillofacial trunk: 1) direct occipital-vertebral arterial anastomosis; 2) ipsilateral atresia of the distal vertebral artery and of the A1 segment of the anterior cerebral artery; 3) bilateral atresia of posterior communicating arteries; 4) linguofacial and labiomental trunks; 5) terminal trifurcation of the external carotid artery. The arterial anatomical variants of the head and neck should be carefully documented prior to specific surgical and interventional procedures, as well as for understanding the compensatory anatomical pathways of circulatory insufficiencies.

350 Cross Over Septal Flap Technique for Choanal Atresia Surgery: Two Centre Series with Outcomes

Abstract Introduction Choanal atresia (CA) is a congenital obstruction of the posterior nasal aperture due to nasal cavity canalisation failure. Endoscopic endonasal surgery has led to successful CA repair and fewer complications compared to open surgery. We describe our surgical technique that uses septal mucosal flaps without need for stenting or subsequent intubation. Method A multicentre retrospective review of patients who underwent surgery using the cross over septal technique. Patient demographics and outcomes were recorded. Flap design: bilateral vertical septal mucosa incisions are performed on either side of the posterior third of the septum to form two mucoperiosteal flaps. The posterior vomer and atretic plates are removed. One flap is pedicled superiorly and rotated over the bare sphenoid rostral bone. The contralateral flap is pedicled inferiorly and rotated to cover exposed bone of nasal cavity floor. Lateral nasal wall mucosal integrity is maintained. This technique is used both for unilateral and bilateral atresia. Results Twelve patients from 2013 to 2020 were included. Age range was 0.07-50 years, male to female ratio of 1:5. Ten patients had unilateral CA, two had bilateral. Nine had bony CA, the remainder mixed. 5 patients (mean age 2.8 years) underwent second-look endoscopy under sedation an average 36 days following primary surgery. Conclusions The cross over technique for CA has low morbidity and 100% success in our multicentre series. Use of mucoperiosteal flaps to cover the exposed bone, removal of vomer and minimal instrumentation to the lateral nasal wall are the best ways to avoid postoperative stenosis.

Congenital aural atresia surgery: anterior mastoid and transmastoid approaches

BackgroundAural atresia (CAA) is a congenital abnormality with hypoplasia or aplasia of the external auditory canal. Surgical procedures of CAA is not only difficult but has still not been embraced by surgeons. Many surgeons are doubtful to perform a repair due to poor hearing results and the risk of new canal stenosis. This article describes the writers’ expertise with surgical management of aural atresia. A retrospective study of 16 cases of CAA was undertaken in the Hearing and Speech Institute between 2015 and 2018. The study consisted of 16 patients, 8 patients for each group, 10 males and 6 females between 4 and 18 years of age with a median age of 6 years. Ten patients had bilateral atresia, and 6 had unilateral atresia. Postoperatively, patients were tested for hearing recovery and follow-up lasting up to 2 years to record any complications. Purpose of the study was to compare hearing effects and risks of anterior and transmastoid approaches to external and middle ear restoration in patients with CAA.ResultsAll patients were satisfied with the surgery by improving the hearing up to 35 dB or less after 12 months. Four patients (25%) developed soft tissue meatal stenosis, with 2 patients in each group. There were no cases of bony canal stenosis. TM perforation was seen in two patients (12.51%), one patient in each group. Lateralization of the graft was seen in one patient in the transmastoid group, and no ossicular chain refixation. Five cases had postoperative otorrhea, one in the anterior mastoid group and four in the transmastoid group. There were no other complications.ConclusionsAlthough the findings of the hearing are close. The previous approach, due to fewer postoperative complications, is now our favorite technique. Proper alignment and soft-tissue strategies are keys to the effective correction of the congenitally atretic ear canal.

Nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants

Objective: To describe and evaluate the surgical effect and prognosis of nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants. Methods: The clinical data of 38 newborns and infants with congenital choanal atresia who underwent nasal endoscopic surgery in Beijing Children's Hospital between January 2016 and May 2018 were retrospectively analysed, including 13 males and 25 females. The age ranged from 5 days to 3 years old at the time of operation (15 cases were newborns). The clinical data, imaging data, treatment effect and prognosis were collected. According to the different surgical methods, the patients were divided into the conventional operation group and the modified mucosal flap technique group. The designs of the modified mucosal flap technique were designed according to the type of congenital choanal atresia. The cross-over L-shaped flaps were performed in patients with unilateral atresia, and the mirrored L-shaped flaps were performed in patients with bilateral atresia. All the patients were followed up for 2-3 years, and the follow-up parameters included the times of operations, length of hospital stay, restenosis rate and incidence of complications. Study data was analyzed using SAS version 9.4 statistical software. Results: Sixteen cases underwent conventional operation while 22 patients underwent modified mucosal flap technique under nasal endoscope. The lightest weight (2 200 g) and the youngest age (5 days) of the patients came from the modified mucosal flap technique group. Compared with the conventional operation group under nasal endoscope, the modified mucosal flap technique group had fewer times of operations (1.14±0.47 vs 2.69±1.20, t=5.552, P<0.001), shorter hospital stay ((7.70±3.22) d vs (14.37±19.16) d, t=2.960, P=0.005), lower rate of postoperative restenosis (9.1% vs 43.8%, χ²=6.156, P=0.013), and lower rate of the incidence of complications (13.6% vs 43.8%, χ²=5.955, P=0.015), the differences were statistically significant. Conclusion: The nasal endoscopic modified mucosal flap technique is feasible for repairing congenital choanal atresia in newborns and infants, which can significantly reduce the incidence of postoperative restenosis and complications.

Features and Strategies in the Management of Choanal Atresia: A 6-Year Retrospective Analysis.

Choanal atresia although rare, is the most common inborn nasal deformity and an important cause of newborn airway obstruction. This study aims to describe a single-center experience in the management of choanal atresia and emphasize the ambiguous issues regarding its surgical repair. The authors retrospectively analyzed the treatment strategy of 18 patients with choanal atresia and their outcomes during the follow-up period. Bilateral choanal atresia was diagnosed in 9 patients, 6 of those had mixed bony-membranous type (67% versus 33% who had pure bony type). Almost half of the 18 patients had a mixed bony-membranous type of atresia (56%). Interestingly, 89% of patients with bilateral atresia underwent transnasal endoscopic repair with stenting, compared to 44% of those with unilateral atresia (P = 0.04). A trend to preference of stent procedure in patients with bony type was also observed, in comparison with mixed bony-membranous type (89% versus 50%, P = 0.09). No significant difference in the need for revision treatment was noticed among the two treatment groups. Both in our data and literature there is no clear supremacy of stenting. Considering the high incidence of re-stenosis, all patients should be under close follow up for a long-term period. Inevitably, further investigation is necessary to establish an ideal surgical procedure.

Combination of the endoscopic septonasal flap technique and bioabsorbable steroid-eluting stents for repair of congenital choanal atresia in neonates and infants: a retrospective study

BackgroundMultiple 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.MethodsClinical 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.ResultsThe 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 ± 0.00), 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.ConclusionsThe 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.

Endonasal choanal atresia repair; evaluating the surgical results of 58 cases.

Choanal atresia is the most common congenital nasal anomaly, with an incidence of 1:5000-1:8000 live births. Atresia can be seen as membraneous, bony or mixed type. When it is bilateral, it is accepted lifethreatening, therefore bilateral atresia necessitates immediate intervention. Diagnosis is confirmed by endoscopic examination and computed tomography. The absolute treatment is surgical, and different approaches have been proposed. Herein, we describe our 15-year experience in the treatment of 58 patients of congenital choanal atresia with transnasal endoscopic approach, and we compare the efficacy of placement of an intranasal stent and applying mitomycin while endoscopic microsurgical repair. The study included 41 female patients (71%) and 17 male patients (29%) with congenital CA. The mean age was 3 years ranging from 10 days to 16 years. The atretic plate was bilateral in 24 patients (41%) and unilateral in 34 (59%). The most common atresia type was the mixed type with 29 patients (50%). A total of 17 patients (29%) required postoperative revision(s). Postoperative revisions were more frequent among patients with bilateral CA (50%), and with mixed CA (31%). Stenting was used additionally by surgical correction for 10 patients. After stenting, fibrosis and restenosis was seen in 7 patients (79%). Mitomycin C was applied peroperatively in 8 patients. Restenosis after mitomycin application was seen in 4 patients (50%). By our experience, endoscopic microsurgical repair of atresia proved to be an effective and safe procedure, results compared with adjuvant treatment modalities like stent or mitomycin C use, was not better. Restenosis was the major problem seen after surgical correction.

Application of retrosigmoid sinus approach in Bonebridge implantation

Background Bonebridge is an suitable option for conductive hearing loss, however, the traditional approach cannot accomplished a satisfying implantation for patients with congenital malformation or radical mastoidectomy. Objectives To evaluate the clinical application of retrosigmoid sinus approach in Bonebridge implantation and postoperative evaluation. Materials and methods 11 patients who underwent retrosigmoid sinus approach Bonebridge implantation from March 2016 to September 2019 were retrospectively analyzed, including 6 males and 5 females, aged 12–54 years old (30.6 in average). Among them, 4 cases had undergone bilateral radical mastoidectomy, 6 cases had bilateral congenital aural atresia or stenosis, and 1 case had unilateral congenital aural atresia. Results All patients underwent Bonebridge implantation through retrosigmoid sinus approach according to the preoperative image reconstruction and plan. There was no surgical injury of sigmoid sinus or cerebrospinal fluid leakage during the operation. The aided threshold obtained an increase of 32.32 dB HL; the speech recognition rates of bisyllabic words, monosyllabic words and sentence were 79.6%, 67.8% and 75.0%, respectively. After 11–53 months of follow-up, the hearing effect was stable and no long-term complications occurred. Conclusion The retrosigmoid sinus approach is an effective surgical approach for patients with congenital ear deformities or radical cavity after mastoidectomy.

Image-guided surgical navigation for bone-conduction hearing device implant placement

IntroductionFor pediatric patients, bone-conduction hearing devices (BCHD) have demonstrated excellent outcomes. Unique to this population, BCHD implant surgeries can be technically challenging in children due to thinner, developing bone and syndromes with atypical anatomy. Image-guided surgical navigation (IGSN) clarifies underlying skull structure, potentially improving outcomes. IGSN is commonly used in otorhinolaryngologic surgeries, but current use in BCHD placement surgeries remains unprecedented. We report favorable results of IGSN in BCHD implantation for three children with complex otologic anatomy: two syndromic patients with variable temporal bone thickness, and one with prior mastoidectomies. The three patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. Methods and materialsWe report using IGSN to assist in BAHA or BONEBRIDGE™ implant surgery for three medically complicated patients. For stereotactic imaging, the patients each received pre-operative high-resolution CT scans using the paranasal sinus fusion protocol without contrast.The first patient was a 6-year-old male with CHARGE-associated abnormal temporal bone anatomy, atretic left auditory nerve, and bilateral chronic tympanic membrane perforation and otorrhea resulting in bilateral mixed conductive and sensorineural hearing loss. The patient thus was unable to consistently tolerate hearing aids.The second patient was an 18-year-old male with Rosai-Dorfman disease, history of bilateral chronic mastoiditis and middle ear infections, bilateral mastoidectomies, and bilateral malleus and incus removal resulting in mixed conductive and sensorineural hearing loss.The third patient was an 11-year-old male with Treacher Collins Syndrome, bilateral microtia, and bilateral atresia of the external auditory canals resulting in bilateral conductive hearing loss. ResultsThe patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. ConclusionIntraoperative IGSN can be a beneficial adjunct to BCHD implant placement surgeries for pediatric patients with abnormal temporal bone anatomy. IGSN can help identify the optimal surgical implantation sites, thereby reducing the risk for major morbidities associated with BCHD implantations. Furthermore, our findings expand application of IGSN use to placement of both BAHA and BONEBRIDGE™.

Bilateral pulmonary vein atresia revealed with CT

2-days baby was born in week 37 of gestation. She presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were poor responsive to medical management. Mechanic...

Bilateral anophthalmia and intrahepatic biliary atresia, two unusual components of Fraser syndrome: a case report

BackgroundFraser syndrome or “cryptophthalmos syndrome” is a rare autosomal recessive disease. It is characterized by a group of congenital malformations such as: crytophthalmos, syndactyly, abnormal genitalia, and malformations of the nose, ears, and larynx. Although cryptophthalmos is considered as a main feature of Fraser syndrome, its absence does not exclude the diagnosis. Clinical diagnosis can be made by Thomas Criteria. Here we present the first documented case of Fraser Syndrome in Aleppo, Syria that is characterized by bilateral anophthalmia and intrahepatic biliary atresia.Case presentationDuring pregnancy, several ultrasound scans revealed hyperechoic lungs, ascites, and unremarkable right kidney at the 19th-week visit; bilateral syndactyly on both hands and feet at the 32nd-week visit. On the 39th week of gestation, the stillborn was delivered by cesarean section due to cephalopelvic disproportion. Gross examination showed bilateral anophthalmia, bilateral syndactyly on hands and feet, low set ears, and ambiguous genitalia. Microscopic examination of the lung, spleen, liver, ovary, and kidneys revealed abnormalities in these organs.ConclusionThe diagnosis of Fraser syndrome can be made prenatally and postnatally; prenatally by ultrasound at 18 weeks of gestation and postnatally by clinical examination using Thomas criteria. Moreover, intrahepatic biliary atresia was not described previously with Fraser syndrome; this recommends a more detailed pathologic study for Fraser syndrome cases.

Transnasal endoscopic surgery of choanal atresia after radiotherapy for nasopharyngeal carcinoma

Objective: To discuss the effect of endoscopic dilatation and plasty for choanal atresia after radiotherapy for nasopharyngeal carcinoma. Methods: Nineteen patients with choanal atresia who were admitted to the Department of Otorhinolaryngology Head and Neck Surgery of the First Affiliated Hospital of Nanchang University from Jan. 2011 to Dec. 2018 were reviewed, with 12 males and 7 females aging from 33 to 59 years old. All of the patients had a history of radiotherapy for nasopharyngeal carcinoma and were confirmed by electronic nasopharyngoscope and nasopharyngeal imaging. Among 19 patients, there were 3 cases of unilateral occlusion and 16 cases with bilateral atresia, and all of them were membranous atresia. All patients received the transnasal endoscopic surgery of resecting partial vomer bone while trying to keep normal mucosa tissues and using the septonasal mucoperiosteal flap to repair under general anesthesia. One week after operation, the patients were told to do physiological saline nasal irrigation and received regular clean and observation under endoscope. Descriptive statistical method was used to analyze the outcome. Results: The patients were followed up for 1 year postoperatively by electronic nasopharyngoscopic examination. There was no failure case in all the 19 patients including 16 patients with a wide choana and 3 patients had narrowing of the choana (<50%), with adequate and satisfactory airway. Conclusions: Transnasal endoscopic surgery was an effective treatment for choanal atresia after radiotherapy for nasopharyngeal carcinoma. Application of septonasal mucoperiosteal flap for repairing nasal and nasopharyngeal mucosa would avoid recurrence.

Use of Steroid-Eluting Stents after Endoscopic Repair of Choanal Atresia: A Case Series with Review.

To describe a single institution's experience with the use of steroid-eluting stents after endoscopic transnasal repair of choanal atresia. A case series with review of children who underwent choanal atresia repair at a tertiary children's hospital from June 2017 to January 2018 was performed. Those who had a mometasone drug-eluting stent (Propel® Mini, Intersect ENT Inc., Palo Alto, CA) placed after primary or secondary choanal atresia repair at our institution were included. The primary outcome measure was need for revision surgery due to stenosis. Postoperative regimens, duration of stenting, and need for return to the operating room (OR) were also assessed. Five patients with a median age of 22 months at the time of repair met inclusion criteria. Two (40%) had bilateral atresia and 3 (60%) had confirmed CHARGE syndrome. A total of 6 mometasone drug-eluting stents were used in the 5 cases. Three patients were reassessed at least once in the OR; however, the majority (57.1%) of postoperative evaluations were able to be performed in the office or bedside setting. The first and last evaluations occurred a mean of 14 and 124 days after surgery, respectively. There were no instances of restenosis, repeat surgical interventions, or stent-related complications noted. Placement of a mometasone drug-eluting stent is a promising method to improve postoperative results and management of choanal atresia repair by limiting the need for repeat anesthetics and OR procedures, as well as the complications of traditional stents.

Bilateral atresia of the external acoustic meatus: a case report

Bilateral atresia of the external acoustic meatus is a rare condition. It is a subtype of congenital aural atresia, which comprises a spectrum of developmental ear abnormalities. In this case report, we present the computed tomography (CT) findings of a patient with this congenital anomaly and emphasize the importance of the findings with regard to treatment options. CT revealed bilateral atresia of the external acoustic meatus in coronal and axial sections of an 8-year-old boy was admitted to hospital due to hearing loss. No congenital anomaly was detected in the middle and inner ear structures. Early detection of congenital aural atresia is critical. Speech retardation and accompanying anomalies associated with hearing loss may be seen in these patients. CT is essential for the diagnosis and classification of congenital bilateral aural atresia, identification of concomitant ear anomalies, and determination of the appropriate surgical treatment method.