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Background: The incidence of re-stenosis or re-atresia after reconstruction of the Outer Ear Canal (OEC) in patients with Congenital Malformation of the Middle and Outer Ear (CMMOE) is very high (up to 48%), and it has been a difficult problem for otologists not being able to solve.Aims/Objectives: To explore new strategies and methods to improve re-stenosis or re-atresia after reconstruction of the OEC in patients with CMMOE.Material and Methods: According to the characteristics of reconstructed OEC (r-OEC) re-stenosis or re-atresia summarized by us, a number of new prevention strategies and methods have been proposed and related patent products have been designed, including the improvement of covering epithelium types and skin grafting methods (7 types), simulated drum ring function to prevent the formation of negative pressure in the cavity, and strengthen postoperative support to reduce skin shrinkage and bone hyperplasia. The postoperative effects of different ages and preoperative OEC malformations are statistically analyzed.Results: The incidence of re-stenosis/re-atresia is 14.3% (5/35) in the thin sectional skin of the temporal scalp overlap splicing skin grafting, which was significantly better than 45.5% (15/33) in the whole piece mosaic splicing and barrel skin grafting from the inner thin sectional thigh skin and overlay splicing other methods, including the inner thigh thin sectional skin, chest medium thick skin and subcutaneous pedicle + chest medium thick skin (p<0.05). The patent artificial drum ring and the model stent of the OEC have obvious effects. The mean operation age of postoperative atresia, stenosis, and good groups are 9.3, 13.1, and 12.5 years old, respectively. The proportion of preoperative atresia is 91.3%, 85.7%, and 57.7%, respectively. The total incidence of re-atresia and re-stenosis of r-OEC for two groups of atresia and stenosis of OEC before surgery is 40.5% (49/121) and 13.3% (8/60), respectively.Conclusions and Significance: The best result is found in overlapping the splicing thin sectional skin of the temporal scalp, combined with artificial drum ring implantation, effective support of postoperative model stent of OEC and post-pubertal surgery selection are new and effective strategies and methods to prevent re-stenosis or re-atresia of r-OEC. Atresia or stenosis of the OEC before the operation is the influence factor of the postoperative effect.

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OBJECTIVE: This study aims to investigate and analyze the connection between PITX2 polymorphisms and the susceptibility of congenital esophageal atresia. METHODS: From January 2015 to June 2020, 46 children with congenital esophageal atresia undergoing surgery were recruited for the study and placed in an observation group, and 40 neonates born in pediatrics during the same period were also recruited for the study and placed in a control group. The alleles and distribution frequencies of the polymorphisms of PITX2 gene rs2200733 were analyzed, and the odds ratio (OR) of esophageal atresia caused by the rs2200733 polymorphism were calculated using a logistic analysis. RESULTS: In the observation group, there were 23 patients (50.00%) with the TT genotype of rs2200733, 21 patients with the TC genotype (45.65%), and 2 patients with the CC genotype (4.35%). In the control group, there were 13 patients with the TT genotype (32.50%), 17 patients with the TC genotype (42.50%), and 10 patients with the CC genotype (25.00%), and the differences in the genotypes between the two groups were statistically significant (P<0.05). The frequencies of the T-alleles and C-alleles of rs2200733 in the observation group were 72.83% and 27.17% respectively, while the frequencies of the control group were 53.75% and 46.25% respectively, and the differences in the rs2200733 allele frequencies were statistically significant (P>0.05). Taking the CC genotype as a reference, the neonates with the TC genotype (OR=2.978, 95% CI=1.084~7.952, P=0.042) or the neonates with the TT genotype (OR=4.778, 95% CI=1.208~13.492, P=0.009) had an increased risk of esophageal atresia, of which the TT genotype indicated a higher risk. CONCLUSION: The polymorphic site rs2200733 (T/C) of the PITX2 gene is connected to the incidence of congenital esophageal atresia. The T-allele is a risk factor for congenital esophageal atresia, and compared with the CC genotype, the TT genotype has an increased risk of esophageal atresia.

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Introduction and objectives: Congenital atresia of the external auditory canal (EAC) is a congenital defect present in one in every 10,000–20,000 births. It causes conductive hearing loss, with an air-bone gap of 50–60dB. Early amplification is essential in bilateral cases to ensure normal language development. The aim of this study is to present the osseointegrated hearing implant as a treatment for bilateral EAC atresia, reviewing the audiometric results and the rate of complications.Material and methodsRetrospective analysis of patients diagnosed with bilateral congenital EAC atresia under follow-up in the pediatric ENT clinic of the ENT and Head and Neck Surgery department of a Portuguese Tertiary Hospital, between 2003 and 2019. We reviewed the medical records and collected information on the assessment of the initial audiometric status. In the cases submitted for implantation with an osseointegrated hearing implant, we analyzed the details of follow-up, including immediate and long-term post-operative complications, as well as the audiometric results.ResultsWe present 8 pediatric patients, 6 girls and 2 boys, with a diagnosis of bilateral congenital EAC atresia. The audiometric assessment revealed moderate to severe bilateral conductive hearing loss with a mean speech recognition threshold (SRT) of 51dB. Six patients underwent osseointegrated hearing implantation. All 6 patients showed good audiometric results, with an average SRT of 20dB and closure of the air-bone gap.ConclusionsThe osseointegrated hearing implant was an effective treatment option in these patients, without significant morbidity or complications. Osseointegrated hearing implantation should be considered first line treatment for children with bilateral congenital EAC atresia, as it presents good functional results and a high level of patient satisfaction. (AU)

Introducción y objetivos: La atresia congénita del canal auditivo externo (CAE) es un defecto congénito presente en uno de cada 10.000-20.000 nacimientos. Origina una pérdida auditiva conductiva, con un gap aire-hueso de 50-60dB. La amplificación temprana es fundamental en casos bilaterales para garantizar el normal desarrollo del lenguaje. El objetivo de este estudio es presentar el implante auditivo osteointegrado como tratamiento para la atresia bilateral del CAE, revisando los resultados audiométricos y la tasa de complicaciones.Material y métodosAnálisis retrospectivo de pacientes diagnosticados con atresia congénita bilateral del CAE en seguimiento en la consulta de otorrinolaringología pediátrica del departamento de otorrinolaringología y cirugía de cabeza y cuello, en un centro hospitalario terciario portugués, entre 2003 y 2019. Revisamos los registros médicos y recopilamos información sobre la evaluación del estado audiométrico inicial. En los casos sometidos a la implantación con implante auditivo osteointegrado, se analizaron los detalles del seguimiento, incluyendo las complicaciones postoperatorias inmediatas y a largo plazo, así como los resultados audiométricos.ResultadosPresentamos los casos de 8 pacientes pediátricos, 6 niñas y 2 niños, con diagnóstico de atresia congénita bilateral del CAE. La evaluación audiométrica reveló una pérdida auditiva de conducción bilateral de grado moderado a grave, con un Speech Recognition Threshold (SRT) medio de 51dB. Seis pacientes han sido sometidos a implantación con implante auditivo osteointegrado. Los 6 pacientes presentaron buenos resultados audiométricos, con un SRT medio de 20dB y cierre del gap aire-hueso. (AU)

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INTRODUCTION AND OBJECTIVES: Congenital atresia of the external auditory canal (EAC) is a congenital defect present in one in every 10,000-20,000 births. It causes conductive hearing loss, with an air-bone gap of 50-60dB. Early amplification is essential in bilateral cases to ensure normal language development. The aim of this study is to present the osseointegrated hearing implant as a treatment for bilateral EAC atresia, reviewing the audiometric results and the rate of complications. MATERIAL AND METHODS: Retrospective analysis of patients diagnosed with bilateral congenital EAC atresia under follow-up in the pediatric ENT clinic of the ENT and Head and Neck Surgery department of a Portuguese Tertiary Hospital, between 2003 and 2019. We reviewed the medical records and collected information on the assessment of the initial audiometric status. In the cases submitted for implantation with an osseointegrated hearing implant, we analyzed the details of follow-up, including immediate and long-term post-operative complications, as well as the audiometric results. RESULTS: We present 8 pediatric patients, 6 girls and 2 boys, with a diagnosis of bilateral congenital EAC atresia. The audiometric assessment revealed moderate to severe bilateral conductive hearing loss with a mean speech recognition threshold (SRT) of 51dB. Six patients underwent osseointegrated hearing implantation. All 6 patients showed good audiometric results, with an average SRT of 20dB and closure of the air-bone gap. CONCLUSIONS: The osseointegrated hearing implant was an effective treatment option in these patients, without significant morbidity or complications. Osseointegrated hearing implantation should be considered first line treatment for children with bilateral congenital EAC atresia, as it presents good functional results and a high level of patient satisfaction.

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OBJECTIVE: To conduct a retro- and prospective analysis of the clinical characteristics and results of surgical treatment of patients with congenital temporal bone cholesteatoma. PATIENTS AND METHODS: On the basis of the SPb Scientific Research Institute of ENT of the Ministry of Health of Russia 23 patients were diagnosed with congenital temporal bone cholesteatoma for the period from 2011 to 2018. There were 11 men (47.8%), 12 women (52.2%), the age of patients - from 2 to 44 years. The M.J. Levenson criteria were used to verify congenital middle ear cholesteatoma. (1986). According to intrasurgery findings, we identified congenital cholesteatoma of the middle ear stages according to the ChOLE classification. Other types of congenital temporal bone cholesteatoma included the patients with congenital atresia of the external auditory opening, combined with congenital temporal bone cholesteatoma; we found a patient with exostoses obstructing the external auditory canal combined with congenital cholesteatoma of the middle ear; and a patient with congenital cholesteatoma of the temporal bone pyramid. RESULTS: An increase in the incidence of congenital temporal bone cholesteatoma was noted, which is probably due to increased awareness of specialists and the wider use of imaging research methods. A relationship was revealed between the stage of congenital cholesteatoma according to the ChOLE classification and the necessary volume of surgical intervention. Of the total number of cases of congenital temporal bone cholesteatoma, 26.1% were the patients with anomalies in the development of the outer and middle ear. CONCLUSIONS: All patients with suspected congenital temporal bone cholesteatoma require a temporal bone MSCT and middle ear MRI in DWI mode.

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This article presents the literature data and our own findings on the surgical treatment and options for solving the problem of restenosis in congenital atresia of the choanae (CAC) in children. PURPOSE: In order to solve the issues of prevention of the fibrosis in the postoperative period in patients with CAC, the prospects of using the carboxymethyl cellulose gel-sodium salt (CGSS) were assessed. PATIENTS AND METHODS: This medicine was used in patients, who were: primarily operated, after a period of wearing a protector (n=17); primarily operated without using a protector, at the final stage of the operation and in the early postoperative period (n=15); reoperated because of the restenosis, also at the final stage of the operation and in the early postoperative period (n=12). RESULTS: We present our results of application of the carboxymethyl cellulose gel-sodium salt for the prevention of scar adhesions in this condition: endoscopy of the nasal cavity and choanal region in all 44 patients with follow-up observation (from 1 year to 5 years) showed no signs of restenosis. CONCLUSION: The use of sodium carboxymethyl cellulose sodium salt in the intra- and postoperative period may be one of the ways of solving the problem of restenosis after surgery in children with CAC along with the use of endoscopic techniques for performing choanotomy.

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OBJECTIVE: To introduce an innovation that combines single port laparoscopic and Wharton-Sheares-George cervicovaginal reconstruction using a small intestinal submucosa (SIS) graft in a patient with congenital cervical atresia, absence of vagina, and incomplete uterine septum (U2aC4V4). DESIGN: Video article introducing a new surgical technique. SETTING: University hospital. PATIENT(S): A 15-year-old patient with congenital cervical atresia, absence of vagina, and incomplete uterine septum had primary amenorrhea and cyclic lower abdominal pain. The magnetic resonance imaging did not show hematometra and the endometrium was 6 mm when she had lower abdominal pain. INTERVENTION(S): A neovagina (depth, 7 cm; width, 2.5 cm) was created using the Wharton-Sheares-George neovaginoplasty. By single laparoscopy, the bladder was separated from the anterior surface of the uterus. With the mold in the neovagina created by the assistant, the apex of neovagina was opened. Then the lower uterine segment was exposed and incised. A T-shaped intrauterine device was connected to an 8-cm-long catheter scissored from a 14-F Foley catheter and was inserted into the uterus to prevent cervical or vaginal stenosis. The upper end of the graft was applied onto the lower uterine segment with delayed absorbable sutures. The lower end was sutured to the high vaginal or vestibular mucosa. MAIN OUTCOME MEASURE(S): The feasibility and effect of combination single port laparoscopic with vaginal cervicovaginal reconstruction in the congenital atresia of cervix. RESULT(S): The operation was successful. The operating time was 90 minutes. Hospitalization was 3 days. There were no intraoperative and postoperative complications. The patient had resumption of menses at three cycles postoperatively, and she had no dysmenorrhea. No cervical or vaginal stenosis occurred because of the Foley catheter. CONCLUSION(S): Single port laparoscopic combined with vaginal cervicovaginal reconstruction provided a minimally invasive, safe, and effective surgical option for the young patient with congenital atresia of cervix. It was successful and without complications or cervical or vaginal stenosis.

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This article presents the literature data and our own findings on the surgical treatment congenital atresia of the choanae (CAC) in children. AIM: To find the causes of restenosis after a choanotomy in children with CAC. PATIENTS AND METHODS: We analyzed the results of surgical treatment of 144 children with the use of an endoscopic transnasal technique (n=94) and without using endoscopy (n=67). In patients with signs of restenosis, endoscopic revision of the choanal opening, computed tomography results clarifying the anatomy of the abnormal zone, anamnestic data, and the presence of concomitant pathology were assessed. RESULTS: The primary causes of the recurrence of the CAC are the concomitant anatomical features of the structure of the choanal zone, insufficient period of protector insertion in the postoperative period, and surgical intervention without visual control. CONCLUSION: Endoscopic transnasal choanotomy with intraoperative rhino- and epipharingoscopic imaging, which is performed taking the individual characteristics of the anatomy of the anomalous zone into account, is the most optimal surgical procedure in pediatric patients. In combination with the use of a protector with a distal extended zone, high efficiency and rarity of restenosis are achieved.

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Objective: To investigate the MRI manifestations of congenital vaginal atresia, analyze its imaging features, and improve the understanding of the disease. Methods: MRI findings and clinical data of 12 patients with congenital vaginal atresia confirmed by hysteroscopy and laparoscopic surgery were retrospectively analyzed. Vaginal atresia was classified according to vaginal dysplasia in AFS female genital malformation classification system. Results: In this study, 12 cases of congenital vaginal atresia were diagnosed by combined preoperative MRI with operative diagnosis. Among them, 10 patients all had type-I congenital vaginal atresia, and their uterus and cervix were normal (1 patient had ectopic renal malformation combined with left ovarian endometriosis cyst and 1 patient with uterine empyema). The other two cases were diagnosed congenital vaginal atresia type II (1 case merged with residual uterus, 1 case with cervical dysplasia). MRI mainly manifested as dilatation and hemorrhage in the uterine cavity, cervical canal and vaginal upper segment. T1WI showed high signal, T2WI showed slightly lower and slightly higher signal. The dilated vagina was above the perineal level. Conclusion: MRI features of congenital vaginal atresia have certain characteristics. MRI cannot only accurately assess the type of vaginal dysplasia and its associated complications, but also make objective evaluation and diagnosis, so it can be used as the best effective preoperative image evaluation.

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The objective of the present study was to develop and introduce into the clinical practice the method for the combined aesthetic and functional rehabilitation of the patients presenting with congenital atresia of the external auditory canal (CAEAC) and the concomitant microtia. A total of 8 patients at the age from 6 to 21 years with unilateral CAEAC and microtia were given the surgical treatment. During the intervention, atresia was resolved using the trans-mastoid approach, tympanoplasty of autofasciae and ossiculoplasty making use of the partial titanium prosthesis and the placement of cranial osteointegratable titanium implants. At the second stage of the surgical intervention the 3D silicone prosthesis of the auricle shaped on an individual basis were used. The long-term follow-up observations have demonstrated the stable formation of the tube of the external auditory canal, with the bone-air interval amounting to 15-20 dB. The auricular prosthesis was the mirror image of the natural ear and completely concealed the congenital defect.

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OBJECTIVE: The aim of this study was to introduce a new technique which is combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of cervix and to evaluate the feasibility and the safety of it. METHODS: This is a prospective observational study of 10 patients with congenital atresia of cervix who underwent combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft for cervicovaginal reconstruction from February 2013 to August 2014 in our hospital. All of the surgical procedures were carried out by the same operation team. Patient data were collected including operating time, estimated blood loss, hospital stay post-surgery, complications, total cost, and median vaginal length at 3 month, resumption of menstruation, vaginal stenosis and stricture of the cervix postoperatively. RESULTS: The operative procedure lasted 237±46 (175-380) min. The estimated blood loss was 160±76 (50-300) ml. The hospital stay post-surgery was 12±2 (9-18) days. None of the patients had complications or required a blood transfusion. The mean total cost was $3352±1025. The average vaginal length at 3 month was 8.3±1.1 (8-10) cm. All patients had resumption of menstruation. The patients were followed for a mean of 5±2 (1-10) months. Cervical or vaginal stenosis did not occur in any of the patients. CONCLUSIONS: Our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in10 patients with congenital atresia of cervix were positive, with successful results and without complications, and cervical or vaginal stenosis.

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OBJECTIVE: To introduce a new technique that combines laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of the cervix. DESIGN: Video article introducing a new surgical technique. SETTING: University hospital. PATIENT(S): A 16-year-old patient with congenital cervical atresia, vaginal dysgenesis, and ovarian endometrial cyst. INTERVENTION(S): An original technique of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft for cervicovaginal reconstruction. MAIN OUTCOME MEASURE(S): A midline incision at the vaginal introitus was made, and a 9-cm canal was made between the bladder and the rectum using sharp and blunt dissection along the anatomic vaginal route, with the aid of laparoscopy to ensure correct orientation. A 14 × 12 cm split thickness skin graft was harvested from the right lateral thigh. By laparoscopy, the level of the lowest pole of the uterine cavity was exposed and the cervix was incised by shape dissection. The proximal segment of the harvested skin to the lower uterine segment was secured, and the distal segment was sutured with the upper margin of vulva vaginally. Surgical technique reports in anonymous patients are exempted from ethical approval by the Institutional Review Board. The patient gave consent to use the video in the article. RESULT(S): The procedure was successfully completed. Since February 2013, our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in 10 patients with congenital atresia of cervix were positive, with successful results and without complications or cervical, or vaginal stenosis. CONCLUSION(S): Our technique is feasible and safe for congenital atresia of cervix, with successful results and without complications or cervical or vaginal stenosis.

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