Permanent tooth agenesis in non-syndromic Robin sequence and cleft palate: prevalence and patterns

Objectives: Partial tooth agenesis is frequently observed in Robin sequence. Tooth anomalies are increasingly considered as an extended phenotype of the cleft palate population. The study objective was to compare the prevalence and patterns of tooth agenesis in a group of patients with non-syndromic Robin sequence (ns-RS) and a group with non-syndromic cleft palate (ns-CP). Materials and methods: The panoramic radiographs of 115 ns-RS and 191 ns-CP patients were assessed for agenesis of the permanent dentition (excluding third molars) and the patterns recorded using the Tooth Agenesis Code. Results: Partial tooth agenesis was observed in 47.8% of ns-RS and 29.8% of ns-CP patients with a greater prevalence in the mandibula than in the maxilla, particularly in ns-RS. The teeth most frequently absent in both groups were the mandibular second premolars and maxillary lateral incisors. Tooth agenesis was bilateral in two-thirds of affected ns-RS patients and one-half of ns-CP patients. In ns-RS, bilateral agenesis of the mandibular second premolars was more frequently observed in female than that in male patients. Completely symmetrical patterns of hypodontia were found in around 45% of ns-RS patients with tooth agenesis compared to 35% in ns-CP. No association was found between the extent of the palatal cleft and the severity of hypodontia. Conclusion: Tooth agenesis is more prevalent in ns-RS than that in ns-CP, demonstrates a much greater predilection for the mandible in ns-RS, and bears no relation to the extent of the palatal cleft. Clinical relevance: When compared to ns-CP, additional developmental disturbances are likely involved in the etiology of tooth agenesis in ns-RS. Future research could help identify the underlying genetic traits and aid in classifying patients in those with and without expected tooth agenesis in order to facilitate orthodontic management strategies

Best practices for the diagnosis and evaluation of infants with robin sequence:a clinical consensus report

Importance: Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. Objective: To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. Evidence Review: Based on a literature review and expert opinion, a clinical consensus report was generated. Findings: Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the possibility of multilevel obstruction exists. The initial assessment of the clinical features and severity of respiratory distress is important and has practical implications. Signs of upper airway obstruction can be intermittent and are more likely to be present when the infant is asleep. Therefore, sleep studies are recommended. Feeding problems are common and may be exacerbated by the presence of a cleft palate. The clinical features and their severity can vary widely and ultimately dictate the required investigations and treatments. Conclusions and Relevance: Agreed-on recommendations for the initial evaluation of RS and clinical descriptors are provided in this consensus report. Researchers and clinicians will ideally use uniform definitions and comparable assessments. Prospective studies and the standard application of validated assessments are needed to build an evidence base guiding standards of care for infants and children with RS

Compression of the deep branch of the ulnar nerve due to volar dislocation of the fifth carpometacarpal joint

Volar dislocation of the base of the fifth metacarpal bone is an unusual event. When it occurs, damage to the deep branch of the ulnar nerve may be expected because of its close relation to the carpometacarpal joint. A case of neuropathy of the deep ulnar motor branch due to such a dislocation is reported. Mild wasting of the first dorsal interosseous muscle and a severe loss of adduction strength of the thumb was observed 7 weeks after reduction of the dislocation. Complete functional recovery occurred 4 months after the initial injury

Classification Systems of Cleft Lip, Alveolus and Palate:Results of an International Survey

Objective: This study aimed to identify commonly used classification systems by cleft providers around the world, including the perceived indications and limitations of each system. Design: A cross-sectional survey. Participants: A total of 197 registrants from three international cleft/craniofacial meetings. Interventions: Participants were sent a web-based questionnaire concerning cleft classification systems. Main outcome measures: Frequency of commonly used classification systems, their perceived indications and limitations. Results: A total of 197 respondents from 166 different centers completed the questionnaire. Healthcare professionals from all disciplines responded, with the most frequent respondents being plastic surgeons (38.1%), maxillofacial surgeons (28.4%) and orthodontists (23.9%). Eighteen different classification systems were in use. The most frequently used systems were the International Statistical Classification of Diseases and Related Health Problems (ICD-10) (35.5%), LAHSHAL (34.0%), and Veau (32.5%) classification systems. Most respondents (32.5%) indicated that anatomical and morphological characteristics are essential components of a classification system. However, respondents indicated that their current classification systems lacked sufficient description of cleft extension and severity. Conclusions: Great variety in the use of classification systems exists among craniofacial specialists internationally. The results recommend the usage of the LAHSHAL classification of OFCs, due to its comprehensiveness, relatively high implementation rate globally, convenience of usage and complementarity with the ICD-10 system. Moreover, it can overcome deficiencies inextricably linked to ICD-10, such as incapacity to describe laterality and clefts of the alveolus. More international exposure to the merits of using the LAHSHAL classification system would be highly recommended

The Effectiveness of Three-Dimensional Osteosynthesis Plates versus Conventional Plates for the Treatment of Skeletal Fractures:A Systematic Review and Meta-Analysis

Purpose: To assess the difference between preformed anatomically shaped osteosynthesis plates and patient-specific implants versus conventional flat plates for the treatment of skeletal fractures in terms of anatomical reduction, operation time, approach, patient outcomes, and complications. Material and Methods: MEDLINE (1950 to February 2023), EMBASE (1966 to February 2023), and the Cochrane Central Register of Controlled Trials (inception to February 2023) databases were searched. Eligible studies were randomised clinical trials, prospective controlled clinical trials, and prospective and retrospective cohort studies (n ≥ 10). Inclusion criteria were studies reporting the outcomes of preformed anatomically shaped osteosynthesis plates and patient-specific implants versus conventional flat plates after treating skeletal fractures. Outcome measures included anatomical reduction, stability, operation time, hospitalisation days, patients’ outcomes, and complications. Two independent reviewers assessed the abstracts and analysed the complete texts and methodologies of the included studies. Results: In total, 21 out of the 5181 primarily selected articles matched the inclusion criteria. A meta-analysis revealed a significant difference in operation time in favour of the preformed anatomical plates and patient-specific implants versus conventional plates. Significant differences in operation time were found for the orbital (95% CI: −50.70–7.49, p = 0.008), upper limb (95% CI: −17.91–6.13, p &lt; 0.0001), and lower limb extremity groups (95% CI: −20.40–15.11, p &lt; 0.00001). The mean difference in the rate of anatomical reduction in the lower limb extremity group (95% CI: 1.04–7.62, p = 0.04) was also in favour of using preformed anatomical plates and patient-specific implants versus conventional plates. Conclusions: This systematic review showed a significant mean difference in surgery time favouring the use of preformed anatomical plates and patient-specific implants for orbital, upper, and lower limb extremity fractures. Additionally, preformed anatomical plates and patient-specific implants in the lower limb group result in a significantly higher rate of anatomical reduction versus conventional flat plates.</p

Assessment of Health-Related Quality of Life in Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion

BACKGROUND: Numerous studies have proven the efficacy of mandibular distraction osteogenesis or tongue-lip adhesion in Robin sequence infants with upper airway obstruction. However, none has compared health-related quality of life outcomes. METHODS: In the present retrospective study, Robin sequence infants younger than 1 year, who underwent mandibular distraction osteogenesis or tongue-lip adhesion, were included (2006 to 2016). The infants' caregivers were asked to complete a questionnaire based on the Glasgow Children's Benefit Inventory. RESULTS: The response rate was 71 percent (22 of the 31 questionnaires; mandibular distraction osteogenesis, 12 of 15; and tongue-lip adhesion, 10 of 16) and median age at surgery was 24 days (range, 5 to 131 days). Median total Glasgow Children's Benefit Inventory scores after mandibular distraction osteogenesis and after tongue-lip adhesion were 21.9 (interquartile range, 9.4) and 26.0 (interquartile range, 37.5), respectively (p = 0.716), indicating an overall benefit from both procedures. Positive changes were observed in all subgroups emotion, physical health, learning, and vitality. In syndromic Robin sequence, both procedures demonstrated a lower positive change in health-related quality of life compared with isolated Robin sequence (p = 0.303). CONCLUSIONS: Both surgical procedures demonstrated an overall benefit in health-related quality-of-life outcomes, with no significant differences. The authors' findings contribute to the debate regarding the use of mandibular distraction osteogenesis versus tongue-lip adhesion in the surgical treatment of Robin sequence; however, studies evaluating health-related quality of life in larger Robin sequence cohorts are necessary to identify which procedure is likely to be best in each individual Robin sequence infant. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III

Will the right Robin patient rise, please? Definitions and criteria during management of Robin sequence patients in the Netherlands and Belgium

Robin Sequence (RS) is characterized by micrognathia and upper airway obstruction (UAO), with or without cleft palate, causing respiratory and feeding problems. Management options are: positioning; nasopharyngeal airway (NPA); tongue-lip adhesion (TLA); mandibular distraction (MDO); and tracheostomy. Controversy exists in literature regarding RS definition and management. Here we describe definitions, management strategies and criteria in opting for management strategies, used by Dutch and Belgian cleft teams. A specifically designed questionnaire was sent to members of all 16 Dutch and Belgian cleft teams. 14 cleft teams returned 35 questionnaires. All used micrognathia as definition criterion, 93.4% cleft palate, 51.5%glossoptosis and 45.7% UAO. Six different RS definitions were used; even within a single team >1 definition was used. All teams used different management strategies: all used positioning, 10 NPA, 6 TLA, 7 MDO, 8 tracheostomy, 5 refer patients with invasive treatment indication. Criteria in opting management modalities were: O2-saturation (89.3%), clinical presentation (86.2%), growth and feeding problems (69.0%), polysomnography (62.1%), and differed within teams. The Dutch and Belgian cleft teams use variable RS definitions, different management modalities and criteria in choosing management strategies. A single, strict definition and evidence-based management guidelines should be formulated for optimal patient car

Atypical Outcomes of Nasal and Lip Appearance After Unilateral Cleft Lip Repair: Judgment by Professionals, Patients, and Laypeople

Objective: To gain more insight into the assessment of “atypical” nasal and lip appearance outcomes compared to “typical” appearance outcomes after unilateral cleft lip and palate (UCLP) repair, when judged by professionals, patients with repaired UCLP, and laypeople. Design: An online survey containing 3 series of photographs with various degrees of “typical” and “atypical” nasal and lip appearance outcomes after UCLP repair was sent to 30 professionals, 30 patients with repaired UCLP, and 50 laypeople in 2 countries. Participants were instructed to rank the photographs from excellent to poor based on overall appearance. Mean rank positions of photographs were analyzed and differences in mean rank score between “typical” and “atypical” results were assessed using a T-test. Agreement of ranking between the 3 groups was assessed with an analysis of variance analysis. Setting: Amsterdam UMC, location VUmc, Netherlands and Boston Children’s Hospital, Boston, USA. Patients: Photographs of 6- to 18-year-old patients with repaired UCLP. Results: “Atypical” appearance outcomes were ranked significantly less favorably (small nostril: P = 0.00; low vermillion border: P = 0.02; whistling deformity: P = 0.00) compared to “typical” outcomes. Difference between professionals, patients and laypeople in rank positioning the photographs was not statistically significant (P = 0.89). Conclusions: Noses with a smaller nostril and lips containing a whistling deformity were perceived as poorer outcome compared to the “typical” results. Professionals, patients, and laypeople are in agreement when assessing these outcomes