Search

Background: Traumatic ulceration of the tongue due to congenital teeth is known as Riga-Fede disease (RFD). The literature on RFD consists largely of anecdotal case reports, thereby lacking valid clinical recommendations. While managing a newborn with RFD complicated by Ludwig’s angina, this knowledge gap was recognized. Therefore, this systematic review was conducted to gain insight into the optimal management and the possible complications of RFD. Methods: A literature search was conducted using PubMed, Google Scholar, and Science Direct, supplemented with African Journals Online, Indmed, and manual searches. Papers published between 1900 and July 2023 in any language were retrieved. Neonates with natal or neonatal teeth and ulceration of the oral mucosa were included. Papers with incomplete data and review articles were excluded. Results: A total of 84 newborns with RFD from 62 published reports (including the case reported herein) were analyzed. Most of the reports were from the Indian subcontinent, and the frequency of reporting has increased since the year 2000. Tongue ulceration in all cases was due to mandibular incisors. There was no significant difference in the median duration of healing between the tooth extraction group (14 days) and the conservative management group (18 days). There was only a very weak positive correlation between the duration of symptoms and healing time, which was statistically insignificant. Conclusion: Healing of mucosal ulcers in RFD is often protracted and does not appear to be influenced by the duration of symptoms or by the modality of treatment. Extraction of the offending teeth is more common (75% of cases) than conservative management, such as grinding (ameloplasty) or resin capping of teeth. RFD is mostly a benign disorder; however, serious complications such as meningitis, Ludwig’s angina, and soft tissue abscesses can rarely occur with Staphylococcus aureus infections.

Background: Autosomal recessive cutis laxa type-1C is also known as Urban-Rifkin-Davis syndrome (URDS). It is known to affect cardiopulmonary, integumentary, gastrointestinal, musculoskeletal and genitourinary systems. However, its frequent association with congenital diaphragmatic hernia has not previously been highlighted. Case Presentation: A newborn male with cutis laxa presented with respiratory distress at birth. The cause of dyspnoea was perinatal strangulation of the stomach in hiatus hernia. After surgical repair of the hernia, his respiratory distress temporarily improved but kept recurring periodically by various mechanisms in sequence namely pulmonary hypertension, tracheomalacia, pulmonary emphysema and finally he succumbed to pneumothorax. Genetic analysis revealed his skin condition as autosomal recessive cutis laxa type-1C which is also known as URDS. Exome sequencing revealed a novel frameshift mutation c.426delC (p.Cys143Alafs*41) of the LTBP4 gene in the exon 5 of Chromosome 19. Conclusion: Out of the 28 cases of URDS reported in the world literature 57% had congenital diaphragmatic hernia (CDH) and 53% of them died during infancy. Such a high incidence of CDH is not observed in other subtypes of elastic disorders. Thus, congenital diaphragmatic defects appear to be a characteristic diagnostic feature of URDS in patients with cutis laxa.

Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest.

Background: Tinnitus is the result of aberrant neuronal activity. As a novel treatment form, neuromodulation is used to modify neuronal activity of brain areas involved in tinnitus generation. Among the different forms of electric stimulation, transcranial random noise stimulation (tRNS) has been shown to be a promising treatment option for tinnitus. In addition, recent studies indicate that the reduction in tinnitus can be more pronounced when different modalities of stimulation techniques are combined ("bimodal stimulation"). TRNS can be used in combination with acoustic stimulation (AS), a further treatment option recognized in the literature. The aim of the proposed study is to investigate whether simultaneous tRNS and AS improve levels of tinnitus loudness and distress., Methods: The intervention consists of bilateral high-definition tRNS (HD-tRNS) over the auditory cortex combined with the application of AS which is studied in a crossover design. The visits will be performed in 26 sessions. There will be 20 treatment sessions, divided into two blocks: active and sham HD-tRNS. Within the blocks, the interventions are divided into group A: HD-tRNS and AS, and group B: HD-tRNS alone. Furthermore, in addition to the assessments directly following the intervention sessions, there will be six extra sessions performed subsequently at the end of each block, after a period of some days (follow-ups 1 and 2) and a month after the last intervention (C). Primary outcome measures are analog scales for evaluation of subjective tinnitus loudness and distress, and the audiological measurement of minimum masking level (MML). Secondary outcome measures are brain activity as measured by electroencephalography and standardized questionnaires for evaluating tinnitus distress and severity., Discussion: To the best of our knowledge, this is the first study which uses HD-tRNS combined with AS for tinnitus treatment. The crossover design permits the comparison between HD-tRNS active vs. sham and with vs. without AS. Thus, it will be possible to evaluate the efficacy of the combined approach to HD-tRNS alone. In addition, the use of different objective and subjective evaluations for tinnitus enable more reliable and valid results., Trial Registration: Swiss Ethics Committee (BASEC-Nr. 2020-02027); Swiss Federal Complementary Database (kofam.ch: SNCTP000004051 ); and ClinicalTrials.gov (clinicaltrials.gov: NCT04551404 )., (© 2022. The Author(s).)