Multidisciplinary Care for Moebius Syndrome and Related Disorders: Building a Management Protocol.

Moebius syndrome is a collection of orofacial anomalies with highly variable features affecting many different systems but characterised by bilateral facial palsy and absent eye abduction. We largely regard Moebius syndrome as a diagnosis of exclusion. Lack of awareness and knowledge means that children often fall between services, leading to treatment delays and difficulty interfacing with social care and schools, with long-term impact on physical health and psychosocial development. We developed a multidisciplinary team comprising core clinicians (lead physician, geneticist, speech and language therapist, psychologist and specialist nurse) and an expanded group to encompass the other affected systems. The interactions between our specialties lead to the development of a treatment protocol, which we present. The protocol harnesses the aspects of care of children with a range of other rare diseases at a specialised paediatric centre and synthesises them into a holistic approach for MBS and related conditions. Management is sequenced on an "ABC-style" basis, with airway, feeding, vision and speech taking priority in the early years. We define management priorities as airway stabilisation with swallow assessment, ocular surface protection and maintenance of nutritional support. Management principles for issues such as speech, reflux, drooling and sleep issues are outlined. In later years, psychological support has a prominent role geared towards monitoring and interventions for low mood, self-esteem and bullying.

How Does Hypodontia Compare in Nonsyndromic Pierre Robin Sequence Versus Isolated Cleft Palate and Isolated Cleft Lip?

OBJECTIVE: To assess the prevalence and patterns of hypodontia in nonsyndromic Pierre Robin sequence (PRS) and compare it with hypodontia in nonsyndromic isolated cleft palates and isolated cleft lips. DESIGN: Retrospective cohort study. SETTING: Alder Hey Children's Hospital, United Kingdom. PATIENTS: Patients with nonsyndromic PRS (group 1), isolated cleft palate (group 2), and isolated cleft lip (group 3). MAIN OUTCOME MEASURES: Hypodontia in the permanent dentition assessed from orthopantomographs. RESULTS: A total of 154 patients were included. Group 1 had the highest incidence of hypodontia with 47% having at least one tooth congenitally absent. Groups 2 and 3 had reduced rates of hypodontia with 27% and 19% of the groups missing teeth, respectively; 93% of cases of hypodontia in group 1 involved the absence of at least one second premolar. Of these patients, there was found to be bilateral agenesis of second premolars in 50% of cases. CONCLUSIONS: Patients with PRS and cleft palates are more likely to have hypodontia than those with isolated cleft palates or unilateral cleft lips. Patients with PRS have more severe hypodontia than those with isolated cleft palates or unilateral cleft lips. Bilateral agenesis of lower second premolars is a commonly seen pattern among patients with PRS. In this large UK study, a similar prevalence and pattern of hypodontia to other nonsyndromic PRS populations worldwide has been demonstrated.

Identifying associations between dental arch relationship scores, relative deprivation and other cleft audit outcomes. Part 1.

OBJECTIVES: To assess whether dental arch relationship scores are associated with speech, audiological and psychology outcomes in patients with complete unilateral cleft lip and palate (UCLP) and bilateral cleft lip and palate (BCLP). SETTING AND SAMPLE POPULATION: One hundred 5-year-old children with non-syndromic complete UCLP and forty-two 5-year-olds with non-syndromic complete BCLP from the United Kingdom. MATERIAL AND METHODS: Data were collected from five-year audit clinics for dental arch relationships, speech, hearing and psychology outcomes. Associations were assessed between ordinal prognostic covariates and the dental arch relationship outcome using a Cochrane-Armitage test for trend and comparisons with binary prognostic covariates using chi-square/Fisher's exact test. This was extended to assess associations between five-year audit outcomes and a measure of deprivation. RESULTS: There was no evidence of associations between dental arch relationships and outcomes for audiology and speech. Individual domain analysis of the psychology outcomes within the UCLP cohort reported a poor outcome for Pro-social score to be associated with a poor 5-year-old index score (P = .018). For the BCLP sample, a good outcome for the BCLP Deciduous Dentition Yardstick was associated with a good conduct score (P = .01). CONCLUSION: There was no association with the 5-year-old index/BCLP Deciduous Dentition Yardstick score and outcomes reported for audiology and speech. For the UCLP and BCLP cohorts, a poor dental arch relationship outcome may be associated with issues with some aspects of psychological well-being, associated with peer interactions and conduct. The deprivation index of samples had no association with the outcomes assessed.

Identifying associations between dental arch relationship scores, relative deprivation and other cleft audit outcomes. Part 2.

Identify associations between dental arch relationship scores, oral health status and deprivation index in patients with complete unilateral cleft lip and palate (UCLP) and bilateral cleft lip and palate (BCLP). Ninety-two 5-year-old children with non-syndromic complete UCLP and thirty-nine 5-year-old children with non-syndromic complete BCLP from the United Kingdom. Data were collected from the 5-year audit outcomes submitted to the Cleft Registry and Audit Network (CRANE). The index of multiple deprivation (IMD) and Welsh index of deprivation were used to assess a relative measure of deprivation. Comparisons of 5-year-old index/BCLP Deciduous Dentition Yardstick outcome against IMD and dmft are performed using multivariable linear regression models. Both UCLP and BCLP had a high percentage of children with dmft >0 (47% and 49%, respectively). The mean dmft for the UCLP cohort was 2.8 and 2.6 for the BCLP cohort. In the UCLP group, a poorer 5-year-old index was associated with an increased dmft score (P = .023) and higher level of deprivation (P = .010). In the BCLP group, there was no significant associations between BCLP Deciduous Dentition Yardstick, dmft and IMD. A poorer dental arch relationship outcome may be associated with higher level of area deprivation and oral health status, in children with UCLP. Those with a poor outcome for the 5-year-old Index are more likely to have increased caries experience.

Implementation of photographic triage in a paediatric dental, orthodontic, and maxillofacial department during COVID-19.

BACKGROUND: During the COVID-19 pandemic, limitations were placed on face-to-face encounters in dentistry and oral and maxillofacial surgery (OMFS) in order to promote physical distancing and reduce viral propagation. To facilitate continued assessment of dental, orthodontic, and maxillofacial emergencies, a photographic triage system was initiated at Alder Hey Children's Hospital (AHCH). We will discuss the benefits this system offers at a patient, clinician, departmental, and NHS service level. AIM: To share our experience of photographic triage during the first 3 months of COVID-19 lockdown, lessons learned, and recommendations. DESIGN: Prospective data collection over 3 months. RESULTS: 220 photographic referrals were received, and swelling (30%) and dental trauma (27%) were the most common presenting complaints. 57% of referrals were not seen, 23% were seen semi-urgently, and 20% booked for outpatient review. Of those seen, 7 children were seen elsewhere and 44 were seen face-to-face at AHCH, with 8 being admitted. CONCLUSION: Photographic triage reduced physical encounters and proved useful in training junior staff, assessment of new patient referrals, and first on-call from home. Implementation should be considered throughout dental, orthodontic, and OMFS departments nationwide. In the event of a COVID-19 resurgence or emergence of a new pandemic, photographic triage could facilitate physical distancing and service provision.

Bilateral cleft lip and nose repair.

Over the last three decades bilateral cleft lip and nose repair has been characterised by a trend towards more detailed reconstruction of the nasolabial muscles together with simultaneous correction of the deformity. An understanding of the true nature of the nasal deformity in bilateral cleft lip and palate (BCLP) has gradually emerged, and has inspired new approaches to their repair. This article discusses recent trends and controversies in primary cheilorhinoplasty for BCLP.

A longitudinal three-center study of dental arch relationship in patients with bilateral cleft lip and palate.

OBJECTIVE: To compare and evaluate longitudinally the dental arch relationships from 4.5 to 13.5 years of age with the Bauru-BCLP Yardstick in a large sample of patients with bilateral cleft lip and palate (BCLP). DESIGN: Retrospective longitudinal intercenter outcome study. PATIENTS: Dental casts of 204 consecutive patients with complete BCLP were evaluated at 6, 9, and 12 years of age. All models were identified only by random identification numbers. SETTING: Three cleft palate centers with different treatment protocols. MAIN OUTCOME MEASURES: Dental arch relationships were categorized with the Bauru-BCLP yardstick. Increments for each interval (from 6 to 9 years, 6 to 12 years, and 9 to 12 years) were analyzed by logistic and linear regression models. RESULTS: There were no significant differences in outcome measures between the centers at age 12 or at age 9. At age 6, center B showed significantly better results (p=.027), but this difference diminished as the yardstick score for this group increased over time (linear regression analysis), the difference with the reference category (center C, boys) for the intervals 6 to 12 and 9 to 12 years being 10.4% (p=.041) and 12.9% (p=.009), respectively. CONCLUSIONS: Despite different treatment protocols, dental arch relationships in the three centers were comparable in final scores at age 9 and 12 years. Delaying hard palate closure and employing infant orthopedics did not appear to be advantageous in the long run. Premaxillary osteotomy employed in center B appeared to be associated with less favorable development of the dental arch relationship between 9 and 12 years.