Short-term outcome after the prenatal diagnosis of right aortic arch.

OBJECTIVES: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

Neonatal respiratory distress due to a third or fourth branchial pouch sinus: A systematic review.

OBJECTIVES: Third and fourth branchial pouch sinuses can be rare causes of respiratory distress in neonates. An overview of this distinct clinical entity is missing in literature. To aid clinicians in recognizing and adequately treating this unique entity, we conducted a systematic review to discuss patient characteristics, diagnostic considerations and treatment strategy. METHODS: MEDLINE and EMBASE were searched from inception to December 29th, 2020. Original studies concerning patients with respiratory symptoms as a result of a third or fourth branchial pouch sinus, as confirmed with rigid endoscopy, videofluoroscopy or during surgery. RESULTS: Thirty-nine studies describing 56 patients (66% male, aged 0-30 days) were analyzed. Symptoms included cervical mass (76.8%), stridor (55.4%), dyspnea (35.7%) and cyanosis (17.9%) due to a third (39.3%) or fourth (60.7%) branchial pouch sinus. Intubation was performed before treatment in 31.3%. The piriform sinus opening was identified with rigid endoscopy in 81.1%. Surgery was the treatment of choice in the majority of patients (85.7%), with a success rate of 100% and a complication rate of 10.7%. Endoscopic cauterization was successful in 40% and endoscopic cauterization followed by sclerotherapy was successful 100%, with no complications. CONCLUSION: Third or fourth branchial pouch sinuses can lead to respiratory distress in neonates. It is important to recognize this distinct clinical picture for adequate diagnosis and treatment. Rigid endoscopy is indicated to demonstrate an opening in the piriform sinus and provides the opportunity to directly perform treatment with endoscopic cauterization. If this is insufficient to relief respiratory symptoms due to a persistent cyst, sclerotherapy or surgical excision should be considered.

A modified surgical technique for aortopexy in tracheobronchomalacia.

OBJECTIVES: Tracheobronchomalacia (TBM) is characterized by collapse of trachea, bronchi or both, leading to dyspnoea, expiratory stridor, coughing or recurrent airway infections. Surgical treatment with aortopexy is warranted for severe TBM. We describe a modified aortopexy technique with aortic wall strap sutures that evenly distributes the traction force over the full width of the aortic arch. The aim of this study was to determine the outcomes of this modified anterior aortopexy technique. METHODS: Retrospective chart review of all patients undergoing aortopexy with aortic wall strap sutures for TBM between January 2010 and June 2020 in 2 tertiary hospitals in the Netherlands. RESULTS: Twenty-four patients [median age 9 months (interquartile range 2-117 months); 71% male] underwent aortopexy with the modified technique for TBM (52%), tracheomalacia (40%) or bonchomalacia (8%). Aortopexy was successful in 91.7%, defined as relief or decrease of respiratory symptoms and no need for respiratory support. Complications occurred in 8.3% and mortality was 4%. CONCLUSIONS: Aortopexy with non-absorbable strap sutures seems an effective and safe treatment for severe TBM. This study supports the hypothesis that strap sutures provide a solid and reliable traction force, but future comparative studies should confirm the benefit of strap sutures over conventional techniques.

Reliability of the reflux finding score for infants in flexible versus rigid laryngoscopy.

OBJECTIVES: The Reflux Finding Score for Infants (RFS-I) was developed to assess signs of laryngopharyngeal reflux (LPR) in infants. With flexible laryngoscopy, moderate inter- and highly variable intraobserver reliability was found. We hypothesized that the use of rigid laryngoscopy would increase reliability and therefore evaluated the reliability of the RFS-I for flexible versus rigid laryngoscopy in infants. METHODS: We established a set of videos of consecutively performed flexible and rigid laryngoscopies in infants. The RFS-I was scored twice by 4 otorhinolaryngologists, 2 otorhinolaryngology fellows, and 2 inexperienced observers. Cohen's and Fleiss' kappas (k) were calculated for categorical data and the intraclass correlation coefficient (ICC) was calculated for ordinal data. RESULTS: The study set consisted of laryngoscopic videos of 30 infants (median age 7.5 (0-19.8) months). Overall interobserver reliability of the RFS-I was moderate for both flexible (ICC = 0.60, 95% CI 0.44-0.76) and rigid (ICC = 0.42, 95% CI 0.26-0.62) laryngoscopy. There were no significant differences in reliability of overall RFS-I scores and individual RFS-I items for flexible versus rigid laryngoscopy. Intraobserver reliability of the total RFS-I score ranged from fair to excellent for both flexible (ICC = 0.33-0.93) and rigid (ICC = 0.39-0.86) laryngoscopies. Comparing RFS-I results for flexible versus rigid laryngoscopy per observer, reliability ranged from no to substantial (k = -0.16-0.63, mean k = 0.22), with an observed agreement of 0.08-0.35. CONCLUSION: Reliability of the RFS-I was moderate and did not differ between flexible and rigid laryngoscopies. The RFS-I is not suitable to detect signs or to guide treatment of LPR in infants, neither with flexible nor with rigid laryngoscopy.

Diagnostic yield of a routine magnetic resonance imaging in tinnitus and clinical relevance of the anterior inferior cerebellar artery loops.

OBJECTIVE: To assess the diagnostic yield of a routine magnetic resonance imaging (MRI) scan in patients with (unilateral) chronic tinnitus, to define the frequency of incidental findings, and to assess the clinical relevance of potentially found anterior inferior cerebellar artery (AICA) loops. STUDY DESIGN: Retrospective cohort study. SETTINGS: Tertiary Tinnitus Care Group at the University Medical Center Utrecht. PATIENTS: Three hundred twenty-one patients with chronic tinnitus. INTERVENTION: Routine diagnostic magnetic resonance imaging (MRI) and diagnostic auditory brainstem responses (ABR) when an AICA loop was found. MAIN OUTCOME MEASURE: Relationship between abnormalities on MRI and tinnitus. RESULTS: In 138 patients (45%), an abnormality on the MRI scan was described. In only 7 patients (2.2%), the abnormality probably related to the patient's tinnitus. Results were not significantly better in patients with unilateral tinnitus (abnormalities in 3.2%). Incidental findings, not related to the tinnitus, were found in 41% of the patients. In 70 patients (23%), an AICA loop was found in the internal auditory canal. No significant relationships were found between the presence of an AICA loop and the side of the tinnitus, abnormalities on the ABR or complaints specific to nerve compression syndrome. CONCLUSION: A routine MRI is of little or no value in patients with tinnitus with persistent complaints. Anterior inferior cerebellar artery loops are often encountered on an MRI scan but rarely relate to the tinnitus and should thus be considered incidental findings. It is advised to only perform an MRI when on clinical grounds a specific etiology with tinnitus as the symptom seems probable.

Socio-demographic, health, and tinnitus related variables affecting tinnitus severity.

OBJECTIVES: Tinnitus is a highly prevalent symptom with potential severe morbidity. Fortunately, only a small proportion of the population experience problems due to their tinnitus in such a degree that it adversely affects their quality of life (clinically significant tinnitus). It is not known why these individuals develop more burden from tinnitus. It seems likely that the severity of tinnitus can be influenced by different factors, such as socio-demographic or tinnitus characteristics or additional health complaints. It remains unclear from the current literature as to what are the main independent variables that have a bearing on tinnitus severity. This study addresses this problem by investigating variables previously described in the literature as well as additional variables. The aim of this study is to identify socio-demographic, health, and tinnitus variables that independently relate to tinnitus severity the most. DESIGN: This is a retrospective cohort study performed at the Tinnitus Care Group of the University Medical Center, Utrecht, in 309 consecutively seen chronic tinnitus patients. At this care group, patients are examined according to a structured diagnostic protocol, including history-taking by an otorhinolaryngologist and audiologist, physical examination, and audiometry. Based on results from previous research and theoretical considerations, a subset of data acquired through this diagnostic protocol were selected and used in this study. Univariate and multivariate correlations with tinnitus severity were investigated for 28 socio-demographic, health, and tinnitus variables. Tinnitus severity was measured with the Tinnitus Questionnaire (TQ) and the Tinnitus Handicap Inventory (THI). RESULTS: Eighteen variables related univariately with the TQ and 16 variables related univariately with the THI. Among these, 14 variables related univariately with both the TQ and the THI. Multivariate analyses showed three variables with an independent significant effect on both the TQ and the THI: percentage of tinnitus awareness during the day, self-reported depression and/or anxiety, and loudness on a Visual Analogue Scale. Three additional variables contributed independently significantly to the explained variance in either the TQ or the THI: level of education, somatic complaints, and tinnitus variability in loudness and/or pitch on a Visual Analogue Scale. These variables were among the first not to reach significance on the other questionnaire. CONCLUSIONS: Tinnitus severity is shown to be strongly related to percentage of tinnitus awareness during the day, self-reported depression and/or anxiety, subjectively experienced loudness, level of education, existence of additional somatic complaints, and subjectively experienced variability in loudness and/or pitch. Further research is needed to investigate the causal relationship between these variables and tinnitus severity. This knowledge may contribute to a better recognition, follow-up, and/or counseling of more vulnerable patients.

Bilateral low-frequency repetitive transcranial magnetic stimulation of the auditory cortex in tinnitus patients is not effective: a randomised controlled trial.

BACKGROUND: Although some therapies may be beneficial for some patients in reducing tinnitus, there is no curative therapy. Repetitive transcranial magnetic stimulation (rTMS) has been applied as a treatment for chronic tinnitus, but the effect remains controversial. MATERIAL AND METHODS: Fifty patients were treated with rTMS or placebo. Treatment consisted of 2,000 TMS pulses on each auditory cortex, at a rate of 1 Hz and an intensity of 110% of the individual motor threshold, on 5 consecutive days. rTMS and placebo effects were evaluated directly after treatment, after 1 week, and after 1, 3 and 6 months. Primary outcome was the Tinnitus Questionnaire (TQ). Secondary outcomes were the Tinnitus Handicap Inventory (THI) and a visual analogue scale. RESULTS: At none of the follow-up evaluation moments a significant difference between rTMS and placebo was observed with respect to changes in TQ or THI scores relative to pretreatment scores. Multilevel modelling (MLM) analyses did not show a global treatment effect either. Patients with a higher degree of burden showed slightly greater improvement after rTMS (only significant on the THI with MLM analyses). CONCLUSION: Bilateral low-frequency rTMS of the auditory cortex was not effective in treating tinnitus.

Prevalence of HIV and syphilis in pregnant women in Leon, Nicaragua.

The objective of this study was to determine the prevalence of HIV and syphilis and to identify risk factors among pregnant women visiting antenatal clinics in León, Nicaragua. During February to April 2004, blood samples from pregnant women were collected after written consent had been obtained. The samples were tested for antibodies against HIV and Treponema pallidum. A questionnaire was also completed. In total, 1,059 women were included. Antibodies against HIV were not detected in any of the women, whereas antibodies against T. pallidum were detected in 16 (1.5%). Risk factors for syphilis included illiteracy, lower education, and two or more pregnancies. HIV does not seem to be highly prevalent among pregnant women in León (prevalence, 0%; 95% CI, 0.0-0.3). The higher proportion of women infected with syphilis than found in earlier studies suggests that sexually transmitted diseases are circulating in the general population and highlights the increasing importance of HIV prevention.