The role of extracorporeal life support and timing of repair in infants with congenital diaphragmatic hernia.

Extracorporeal life support (ECLS) serves as a rescue therapy for patients with congenital diaphragmatic hernia (CDH) and severe cardiopulmonary failure, and only half of these patients survive to discharge. This costly intervention has a significant complication risk and is reserved for patients with the most severe disease physiology refractory to maximal cardiopulmonary support. Some contraindications to ECLS do exist such as coagulopathy, lethal chromosomal or congenital anomaly, very preterm birth, or very low birth weight, but many of these limits are being evaluated through further research. Consensus guidelines from the past decade vary in recommendations for ECLS use in patients with CDH but this therapy appears to have a survival benefit in the most severe subset of patients. Improved outcomes have been observed for patients treated at high-volume centers. This review details the evolving literature surrounding management paradigms for timing of CDH repair for patients receiving preoperative ECLS. Most recent data support early repair following cannulation to avoid non-repair which is uniformly fatal in this population. Longer ECLS runs are associated with decreased survival, and patient physiology should guide ECLS weaning and eventual decannulation rather than limiting patients to arbitrary run lengths. Standardization of care across centers is a major focus to limit unnecessary costs and improve short-term and long-term outcomes for these complex patients.

Computed tomography multi-planar and 3D image assessment protocol for detailed analysis of inner ear malformations in patients undergoing cochlear implantation counseling.

<b><br>Introduction:</b> Congenital inner ear malformations resulting from embryogenesis may be visualized in radiological scans. Many attempts have been made to describe and classify the defects of the inner ear based on anatomical and radiological findings.</br> <b><br>Aim:</b> The aim was to propose and discuss computed tomography multi-planar and 3D image assessment protocols for detailed analysis of inner ear malformations in patients undergoing cochlear implantation counseling.</br> <b><br>Material and methods:</b> A retrospective analysis of 22 malformed inner ears. CT scans were analyzed using the Multi-Planar Reconstruction (MPR) option and 3D reconstruction.</br> <b><br>Results:</b> The protocol of image interpretation was developed to allow reproducibility for evaluating each set of images. The following malformations were identified: common cavity, cochlear hypoplasia type II, III, and IV, incomplete partition type II and III, and various combinations of vestibule labyrinth malformations. All anomalies have been presented and highlighted in figures with appropriate descriptions for easier identification. Figures of normal inner ears were also included for comparison. 3D reconstructions for each malformation were presented, adding clinical value to the detailed analysis.</br> <b><br>Conclusions:</b> Properly analyzing CT scans in cochlear implantation counseling is a necessary and beneficial tool for appropriate candidate selection and preparation for surgery. As proposed in this study, the unified scans evaluation scheme simplifies the identification of malformations and reduces the risk of omitting particular anomalies. Multi-planar assessment of scans provides most of the necessary details. The 3D reconstruction technique is valuable in addition to diagnostics influencing the decision-making process. It can minimize the risk of misdiagnosis. Disclosure of the inner ear defect and its precise imaging provides detailed anatomical knowledge of each ear, enabling the selection of the appropriate cochlear implant electrode and the optimal surgical technique.</br>.

Risk of congenital anomalies in children who have a sibling with cancer: A matched cohort study.

PURPOSE: We assessed the risk of congenital anomalies in children who have a sibling with cancer. METHODS: We performed a matched cohort study of children born between 2006 and 2022 in Quebec. The exposure was having a sibling with cancer. Exposed children were matched to unexposed children based on sex, number of siblings, birth order, and year. The outcome included heart defects, orofacial clefts, and other anomalies. Using conditional logistic regression, we estimated odds ratios (OR) and 95 % confidence intervals (CI) for the association between having a sibling with cancer and the likelihood of having a congenital anomaly. RESULTS: A total of 2403 children who had a sibling with cancer were matched to 240,257 unexposed children. Congenital anomalies were more frequent in children who had a sibling with cancer compared with unexposed children (10.3 % vs 8.9 %). Overall, having a sibling with cancer was only weakly associated with congenital anomalies (OR 1.18, 95 % CI 1.04-1.35). Exposed children tended to have greater odds of polydactyly/syndactyly (OR 1.89, 95 % CI 1.11-3.21) and urinary defects (OR 1.50, 95 % CI 1.09-2.08) compared with unexposed children. CONCLUSIONS: Children who have a sibling with cancer have an only weakly elevated risk of congenital anomalies.

Long-term follow-up of patients with congenital diaphragmatic hernia.

Neonates with congenital diaphragmatic hernia encounter a number of surgical and medical morbidities that persist into adulthood. As mortality improves for this population, these survivors warrant specialized follow-up for their unique disease-specific morbidities. Multidisciplinary congenital diaphragmatic hernia clinics are best positioned to address these complex long-term morbidities, provide long-term research outcomes, and help inform standardization of best practices in this cohort of patients. This review outlines long-term morbidities experienced by congenital diaphragmatic hernia survivors that can be addressed in a comprehensive follow-up clinic.

Human diprosopus: Case report of a rare congenital abnormality.

Diprosopus is a congenital anomaly in which partial or complete duplication of craniofacial structures occurs. Because it is rare, the mortality rate is high, and information concerning this anomaly is scarce. This study describes a case of human diprosopus in a 9-year-old male individual, who has severe complications associated with the central nervous, cardiovascular, respiratory, and digestive systems. Since birth, he has been monitored in a specialized hospital environment, where he has undergone several surgeries and multidisciplinary treatments. Regarding the craniofacial aspects, he had agenesis of the corpus callosum, floor of the nasal cavity, and floor of the anterior cranial fossa, in addition to the presence of bone dysplasia, ocular hypertelorism and cleft palate with nasal and oral teratoma. Regarding dental characteristics, the patient has duplication of the maxilla, mandible, tongue, and some teeth. After complementary imaging exams, several supernumerary teeth were found, with some being impacted and in complex regions, with an indication for extraction due to the risks of impaction, irruptive deviation, root resorption, and associated cystic or tumoral lesions. Because of the numerous complications, knowledge, and preparation of the entire team is necessary for the correct management of the case.

Pancreatic congenital anomalies and their features on CT and MR imaging: a pictorial review.

OBJECTIVE: To review the congenital anomalies of the pancreas with their main clinical manifestations and key imaging findings on CT and MRI. BACKGROUND AND CLINICAL SIGNIFICANCE: Anomalies of pancreatic development are frequent and generally asymptomatic, but can mimic and predispose individuals to pancreatic or peripancreatic pathologies, such as pancreatitis or malignancy. Their correct diagnosis may help avoid unnecessary further investigations and procedures, or establish adequate treatment when they manifest clinically. Differentiating pancreatic congenital anomalies from their main radiological mimics constitutes a challenge for the radiologist and requires familiarity with key imaging findings. CONCLUSION: The imaging findings of CT and MRI are essential for the correct diagnosis of congenital pancreatic anomalies.

Cervicothoracic (C6, C7 & T1) spina bifida occulta - A case report.

INTRODUCTION: Spina bifida (SB), as one of the spine abnormalities, is caused by incomplete closure of the neural tube and generally includes two types: open and closed. This study aims to introduce one of the rare cases of closed SB. CASE PRESENTATION: A 34-year-old male patient was admitted to the hospital with neck pain. Radiographic and computed tomography (CT) imaging revealed bifid spinous processes from C6 to T1 vertebrae, indicative of a rare case of closed SB in the cervicothoracic region. CLINICAL DISCUSSION: Closed SB, while often regarded as benign, can be associated with various pathologies that require follow-up. CONCLUSION: Diagnosing this abnormality is crucial for addressing potential complications that may arise from it.

Intrauterine Growth Restriction and Placenta Previa: A Clinical Case Report of an Advanced-Age Pregnancy.

Exploring the intricacies of managing high-risk pregnancies complicated by intrauterine growth restriction (IUGR), placenta previa, and a single umbilical artery requires a comprehensive understanding of their etiologies, mechanisms, and treatment recommendations. This case report delves into the clinical course of a 34-year-old smoker with a pre-pregnancy body mass index of 14.2 kg/m2, shedding light on the considerations posed by a pregnancy in which several risk factors are superimposed on one another. IUGR, affecting 10%-15% of pregnancies, elevated the risk of adverse outcomes during labor and delivery, necessitating careful antenatal monitoring. Placenta previa, with an incidence of 0.3% to 2% in pregnancies, introduced further complications impacting delivery modes and raising the risk of hemorrhage. This report aims to showcase the interconnectedness between these various obstetrical complications and risk factors, to guide maternal-fetal-medicine specialists in making informed decisions during the management of high-risk pregnancies.

Efforts to improve outcomes among neonates with complex intestinal atresia: a single-center low-income country experience.

PURPOSE: Intestinal obstruction caused by intestinal atresia is a surgical emergency in newborns. Outcomes for the jejunal ileal atresia (JIA), the most common subtype of atresia in low-income countries (LIC), are poor. We sought to assess the impact of utilizing the Bishop-Koop (BK) approach to JIA in improving outcomes. METHODS: A retrospective cohort study was performed on children with complex JIA (Type 2-4) treated at our national referral hospital from 1/2018 to 12/2022. BK was regularly used starting 1/1/2021, and outcomes between 1/2021 and 12/2022 were compared to those between 1/2018 and 12/2020. Statistical significance was set at p < 0.05. RESULTS: A total of 122 neonates presented with JIA in 1/2018-12/2022, 83 of whom were treated for complex JIA. A significant decrease (p = 0.03) was noted in patient mortality in 2021 and 2022 (n = 33, 45.5% mortality) compared to 2018-2020 (n = 35, 71.4% mortality). This translated to a risk reduction of 0.64 (95% CI 0.41-0.98) with the increased use of BK. CONCLUSION: Increased use of BK anastomoses with early enteral nutrition and decreased use of primary anastomosis improves outcomes for neonates with severe JIA in LIC settings. Implementing this surgical approach in LICs may help address the disparities in outcomes for children with JIA.

Role of practice standardization in outcome optimization for CDH.

Standardization of care seeks to improve patient outcomes and healthcare delivery by reducing unwanted variations in care as well as promoting the efficient and effective use of healthcare resources. There are many types of standardization, with clinical practice guidelines (CPGs), based on a stringent assessment of evidence and expert consensus, being the hallmark of high-quality care. This article outlines the history of CPGs, their benefits and shortcomings, with a specific focus on standardization efforts as it relates to congenital diaphragmatic hernia management.

Heimler syndrome with a complaint of blurred vision caused by compound heterozygous variants in PEX1.

INTRODUCTION: Heimler syndrome (HS) is a rare disorder that includes sensorineural hearing loss (SNHL), nail abnormalities, and enamel hypoplasia. Patients with this syndrome can also exhibit ocular manifestations. At present, only a few cases of HS have been reported, existing knowledge of this syndrome is limited, and many cases have been misdiagnosed or even missed. This is the first report of Heimler syndrome with blurred vision as the first complaint, which was diagnosed by genetic analysis in the ophthalmology department. CASE DESCRIPTION: An 8-year-old girl complained of bilateral visual blur and night blindness from birth. Ophthalmic examinations revealed bilateral retinitis pigmentosa with cystoid macular edema, visual impairment with hyperopia and astigmatism. Hearing test revealed bilateral severe sensorineural hearing loss. Dental examinations revealed enamel hypoplasia. In addition, whole-exome sequencing (WES) identified two pathogenic variants in PEX1: the previously reported missense variant c.2966T > C (p.I989 T), and the novel frameshift variant c.1671_1672del (p.G558Sfs*33). CONCLUSION: Heimler syndrome is caused by compound heterozygous PEX1 pathogenic variants, c.2966T > C (p.I989 T) and c.1671_1672del (p.G558Sfs*33), which contributed to the diversity of clinical and genetic profiles in this patient. The main clinical manifestations include bilateral retinitis pigmentosa with cystoid macular edema, sensorineural hearing loss, and enamel hypoplasia. Systemic examinations are suggested for patients suspected of having pigmentary retinal dystrophy, especially combined with hearing-related impairments. Genetic testing can help us to make a definitive diagnosis.

Prenatal Diagnosis of Hemimegalencephaly Using Radiological Methods: A Case Report.

Hemimegalencephaly is a rare congenital anomaly characterized by an increase in the size and dysplastic involvement of one cerebral hemisphere, which can be partial or complete. It may also be associated with anomalies in the cerebellum and brainstem and, in some cases, be a part of different syndromes. The result of these abnormalities leads to intractable epilepsy and developmental delay. Diagnosis is typically made through imaging studies in the postnatal period, but it can also be done before birth. We present the case of a 23-week pregnant patient in whom a prenatal diagnosis of hemimegalencephaly was made, highlighting the need for fetal magnetic resonance imaging (MRI) to confirm the diagnosis.

Clinical characteristics of postoperative necrotizing enterocolitis in patients with congenital jejunoileal atresia and its risk factors.

Objective: To review postoperative necrotizing enterocolitis (NEC) in patients with jejunoileal atresia (JIA) and to explore the potential risk factors related to the concurrence of NEC. Methods: Patients diagnosed with JIA who received surgical treatment from January 2016 to June 2021 were enrolled. Demographics, viral infection of the fetus, transfusion within 48 hours before NEC, sepsis before JIA repair, pathological and anatomical classification of JIA, combined malformation, occurrence time of NEC after the operation, treatment, and prognosis of patients were analyzed. Patients were divided into NEC group and non-NEC group, and all patients were followed up for 3-6 months to observe for complications. Results: A total of 180 patients with JIA were included, of whom 12 were diagnosed with NEC after surgery and 1 patient with NEC died during follow-up. The average age, birth weight, gestational age, proportion of premature infants, proportion of preoperative infections, and pathological classification of JIA did not significantly differ between the two groups. The probability of patients with proximal jejunal atresia (PJA) in the NEC group (58.3%) was higher than that in the non-NEC group (22.6%) (p=0.011), and patients with PJA had longer parenteral nutrition time than patients without PJA (26.64±9.21 days vs 15.11±6.58 days, p<0.001). Conclusion: PJA was more likely to be associated with concurrent NEC after surgery, which is a highly NEC-related risk factor inherent in JIA.

Post-urethroplasty complications in hypospadias repair: a systematic review and meta-analysis comparing polydioxanone and polyglactin sutures.

Background: Polyglactin (PG) and polydioxanone (PDS) sutures are extensively used based on the surgeon's preference. The development of post-reconstruction urethrocutaneous fistula (UCF) is variably attributed to the choice of suture material for urethroplasty. This meta-analysis compares complications of hypospadias repair using PG and PDS sutures. Methods: The systematic review and meta-analysis were performed as per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The authors conducted thorough searches in databases including MEDLINE, EMBASE, CENTRAL, Scopus, Google Scholar, and clinical trial registries. Outcome measures included UCF, meatal stenosis, wound infection, urethral stricture, glans dehiscence, and overall complications. Quantitative analysis was used with fixed or random-effect models to find the pooled risk ratio and I2 heterogeneity. Results: The criteria for inclusion were met by five comparative studies with the inclusion of 1244 children altogether. Pooled analysis failed to show a statistically significant difference in the incidence of meatal stenosis, urethral stricture, wound infection, and total complications using PG and PDS sutures. However, it showed a reduction in the incidence of UCF with PDS suture hypospadias repairs (risk ratio=0.66, 95% CI 0.48 to 0.92). Conclusions: PDS sutures are associated with decreased incidence of UCF than PG after hypospadias repair. The incidence of meatal stenosis, urethral stricture, wound infection, and total complications was not affected by the type of suture material used for repair. Clinical implications: This meta-analysis suggests decreased incidence of UCF when PDS sutures are used for hypospadias repair which may impact the choice of suture material for repair. PROSPERO registration number: CRD42023409710.

Pulmonary artery diameter ratio as a prognostic indicator of congenital diaphragmatic hernia.

Background: Following on from an earlier study published in 2008 about left pulmonary artery (LPA) flow measured on serial echocardiography being strongly prognostic in left-sided congenital diaphragmatic hernia (CDH) and the ratio of LPA to right pulmonary artery (RPA) diameters being a simple and reliable indicator for commencing nitric oxide (NO) therapy, the ratio of LPA:RPA diameters (PA ratio or PAR) was hypothesized to possibly reflect cardiopulmonary stresses accompanying CDH better. Methods: Subjects with isolated left-sided CDH treated between 2007 and 2020 at a single pediatric surgical center were recruited and classified according to survival. Data obtained retrospectively for subject demographics, clinical course, LPA/RPA diameters, and PAR were compared between survivors and non-survivors. The value of PAR for optimizing the prognostic value of PA diameter data in CDH were analyzed with receiver operating characteristic (ROC) curve analysis. Results: Of 65 subjects, there were 54 survivors (82.3%) and 11 non-survivors (17.7%); 7 of 11 non-survivors died before surgical repair could be performed. Mean PAR for survivors (0.851±0.152) was significantly higher than for non-survivors (0.672±0.108) (p=0.0003). Mean PAR for non-survivors was not affected by surgical repair. Characteristics of survivors were: LPA ≥2 mm (n=52 of 54; mean PAR=0.866±0.146) and RPA ≥3 mm (n=46 of 54; mean PAR=0.857±0.152). Non-survivors with similar LPA and RPA diameters to survivors had significantly lower mean PAR. ROC curve cut-off for PAR was 0.762. Subjects with high PAR (≥0.762) required high-frequency oscillatory ventilation/NO less than subjects with low PAR (<0.762) (p=0.0244 and p=0.0485, respectively) and subjects with high PAR stabilized significantly earlier than subjects with low PAR (1.71±0.68 days vs 3.20±0.87 days) (p<0.0001). Conclusions: PAR would appear to be strongly correlated with clinical outcome in CDH and be useful for planning management of cardiopulmonary instability in CDH.

Duplicación del colédoco asociado a unión biliopancreática anómala: Reporte de caso / Common bile duct duplication associated with anomalous biliopancreatic junction: A case report

Introducción. La duplicación del colédoco es una anomalía congénita poco frecuente. En la mayoría de los casos este defecto se asocia a cálculos en la vía biliar, unión pancreatobiliar anómala, pancreatitis, cáncer gástrico o colangiocarcinoma. Por esta razón, el diagnóstico y el tratamiento temprano son importantes para evitar las complicaciones descritas a futuro. Métodos. Se presenta el caso de una paciente de 30 años, con antecedente de pancreatitis aguda, con cuadro de dolor abdominal crónico, a quien se le realizaron varios estudios imagenológicos sin claro diagnóstico. Fue llevada a manejo quirúrgico en donde se documentó duplicación del colédoco tipo II con unión pancreatobiliar anómala. Resultados. Se hizo reconstrucción de las vías biliares y hepatico-yeyunostomía, con adecuada evolución postoperatoria y reporte final de patología sin evidencia de tumor. Conclusión. El diagnóstico se hace mediante ecografía endoscópica biliopancreática, colangiorresonancia o colangiopancreatografía retrógrada endoscópica. El tratamiento depende de si está asociado o no a la presencia de unión biliopancreática anómala o cáncer. Si el paciente no presenta patología neoplásica, el tratamiento quirúrgico recomendado es la resección del conducto con reconstrucción de las vías biliares.

Introduction. Double common bile duct is an extremely rare congenital anomaly. This anomaly may be associated with bile duct stones, anomalous biliopancreatic junction, pancreatitis, bile duct cancer, or gastric cancers. Thus, early diagnosis and treatment is important to avoid complications. Clinical case. We report a rare case of double common bile duct associated with an anomalous biliopancreatic junction in a 30-year-old female, with prior history of acute pancreatitis, who presented with chronic abdominal pain. She underwent several imaging studies, without clear diagnosis. She was taken to surgical management where duplication of the type II common bile duct was documented with anomalous pancreatobiliary junction. Results. Reconstruction of the bile ducts and hepatico-jejunostomy were performed, with adequate postoperative evolution and final pathology report without evidence of tumor. Conclusion. Diagnosis is usually performed by an endoscopic ultrasound, magnetic resonance cholangiopancrea-tography, or endoscopic retrograde cholangiopancreatography. Treatment depends on the presence of anomalus biliopancreatic junction or concomitant cancer. In cases without associated malignancy, resection of bile duct and biliary reconstruction is the recommended surgical treatment.

Tubularized incised plate urethroplasty and grafted tubularized incised plate urethroplasty: systematic review, meta-analysis and trial sequential analysis.

Background: Hypospadias is one of the most common genital birth defects. There are around 300 various techniques available for the repair of hypospadias. This study aims to compare the reported outcomes of Tubularized incised plate urethroplasty (TIP) and Grafted TIP (GTIP) repair in children undergoing primary hypospadias repair. Methods: This meta-analysisadhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and we framed our research question using the population, intervention, control and outcomes format. We conducted comprehensive electronic searches across various databases, employing a Boolean search strategy with predefined search terms. Only randomized controlled trials (RCTs) were included for quantitative analysis. Results: Totally, 10 RCTs met our inclusion criteria for quantitative analysis. The results indicated that urethrocutaneous fistula, glans dehiscence, and stricture rates were comparable between the two groups. The incidence of meatal stenosis was found to be significantly lower in the GTIP group with a relative risk (RR) of 0.32 (95% confidence interval (CI) 0.15 to 0.67). Conclusion: The coucomes UCF, glans dehiscence, and stricture rates were comparable between the two groups. Notably, the incidence of meatal stenosis was found to be significantly lower in the grafted TIP group. In terms of operative time, our quantitative synthesis demonstrated that the TIP group had a shorter operative time than the GTIP group with significant heterogeneity.

Neonatal outcomes and follow-up of children born to women with pregnancy-associated cancer: a prospective observational study.

BACKGROUND: During the last decade, there has been a growing number of cases of children born from pregnancy-associated cancer (PAC), however there are currently insufficient data on the follow up to be observed in this category of newborns. Objective of the study was to evaluate the neonatal outcomes of infants born to mother with PAC, the potential adverse effect of chemotherapy during pregnancy and the risk of metastasis to the fetus. METHODS: Maternal clinical data and neonatal outcomes of child born to mothers diagnosed with PAC were collected; infants were divided into those were and were not exposed to chemotherapy during fetal life and their outcomes were compered. RESULTS: A total of 37 newborn infants from 36 women with PAC were analyzed. Preterm delivery occurred in 83.8% of the cases. No significant differences in neonatal outcomes were found between infants who were and were not exposed to chemotherapy during pregnancy. The median follow-up period was 12 months. CONCLUSIONS: PAC treatment during the second or third trimester does not seem to be dangerous for the fetus, however infants born from PAC must be carefully evaluated for to rule out the consequences of chemotherapy and exclude the presence of metastasis. Long-term follow-up, especially in children exposed to chemotherapy, should be encouraged to obtain relevant data on long-term toxicity.

Hidden blood loss and bleeding characteristics in children with congenital scoliosis undergoing spinal osteotomies.

PURPOSE: Spinal osteotomies are often essential in the treatment of congenital scoliosis. Risk factors for bleeding in these patients needing extracavitatory approaches, especially hidden blood loss, are sparsely investigated. We aimed to investigate the bleeding characteristics and hidden blood loss in paediatric patients undergoing spinal osteotomies for congenital scoliosis. METHODS: A retrospective analysis identified all patients with congenital scoliosis were retrospectively identified from the prospectively collected spine register from 2010 to 2022. Operative technique, perioperative laboratory results and imaging studies were extracted. The primary outcome was total blood loss including intraoperative, drain output and hidden blood loss. RESULTS: Fifty-seven children (32 boys) with a mean age of 8.3 years underwent spinal osteotomy for congenital scoliosis. Posterolateral hemivertebrectomy was sufficient in 34 (59%) patients, while vertebral column resection (VCR) was required in 23 patients. Total bleeding averaged 792 (523) ml accounting for 42% of the estimated blood volume. Hidden blood loss accounted for 40% of total bleeding and 21% of estimated blood volume with a mean of 317 (256) ml. VCR was associated with greater intraoperative and total bleeding than hemivertebrectomies (p = 0.001 and 0.007, respectively). After adjusting for patient weight and fusion levels, hidden blood loss was larger in hemivertebrectomies (4.18 vs. 1.77 ml/kg/fused level, p = 0.049). In multivariable analysis, intraoperative blood loss was inversely correlated with preoperative erythrocyte levels. Younger age was associated with significantly greater drain, hidden and total blood loss. CONCLUSION: Hidden blood loss constitutes a significant portion (40%) of total bleeding in congenital scoliosis surgery. Younger age is a risk factor for bleeding and the hidden blood loss should be taken into consideration in their perioperative management.

[Contemporary diagnosis and management of congenital microtia and aural atresia : Part 2: Overview of therapeutic approaches]. / Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen : Teil 2: Übersicht der verschiedenen Therapieansätze.

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.