Surgical sequence, timing and volume, and variation in dento-facial outcome, speech and secondary surgery in children with unilateral cleft lip and palate: The Cleft Care UK Study.

OBJECTIVES: To estimate both the association of surgical variables in complete unilateral cleft lip and palate (cUCLP) in the UK with outcomes at age 5 years, and the association of secondary speech surgery, volume of surgery, and surgeon with the same outcomes. SETTING AND SAMPLE POPULATION: The Cleft Care UK study, a cross-sectional study of 268 5-year-olds, born from 2005 to 2007, with cUCLP. MATERIALS AND METHODS: Information on surgical variables was extracted from a standardized questionnaire. Dento-facial outcomes were derived from dental study casts of dental arch relationships. Three speech outcomes - intelligibility, structure and articulation - were derived using the Cleft Audit Protocol for Speech-Augmented tool. RESULTS: Surgical and outcome data were available for 211 (79%) children from all cleft centres in the UK. Later soft palate surgery was associated with a 17% increased chance of a poor intelligibility score (P = .02), and high volume surgery with a 249% increased chance of a good articulation score (P = .01). There were no between surgeon effects identified. No association between the surgical variables examined and dento-facial outcome, or secondary speech surgery by the age of 5 years were found. CONCLUSION: This study found associations between surgical variables and speech outcomes at 5 years of age, but not between surgical variables and dento-facial outcome, nor between surgical variables and secondary speech surgery. High surgical volume should be maintained, and any changes towards later surgery monitored for changes in speech outcome.

Psychometric Validation of the CLEFT-Q Patient Reported Outcome Measure: A Prospective Study to Examine Cross-Sectional Construct Validity.

OBJECTIVE: CLEFT-Q is a condition-specific patient-reported outcome measure (PROM) for patients with cleft lip and/or palate (CL/P). The aim of this study was to examine the cross-sectional construct validity of the CLEFT-Q scales. DESIGN: Construct validity was assessed through a prospective study that tested hypotheses regarding correlations of scores with other PROMs that measure related constructs. SETTING: Seven cleft centres in Canada, the USA, and UK were involved. PATIENTS/PARTICIPANTS: Patients were aged eight to 29 years with CL/P. INTERVENTIONS: Before undergoing rhinoplasty, orthognathic, cleft lip scar revision, and alveolar bone graft, participants were asked to complete the following PROMs: CLEFT-Q (9 scales), Child Oral Health Impact Profile (socio-emotional subscale) and Cleft Hearing Appearance and Speech Questionnaire (features 1 subscale). MAIN OUTCOME MEASURE(S): The correlation coefficients examining the relationship between the scales were the main outcome measures. Correlations (Spearman) were calculated and interpreted as follows: <0.3 weak, 0.30 to 0.50 moderate, ≥0.50 strong. RESULTS: Participants (n = 177) were mostly male (61%) and aged between eight and 11 years (42%). Overall, 38 of 52 (73%) hypotheses tested were supported. More specifically, 20 of 26 (77%) hypotheses about correlations between the appearance scales were supported, two of three (67%) hypotheses about correlations between the health-related quality of life scales were supported, and 16 of 23 (70%) hypotheses about correlations between the appearance and health-related quality of life scales were supported. CONCLUSIONS: Cross-sectional construct validity of the CLEFT-Q scales adds further evidence of the psychometric properties of this instrument.

The Incidence and Outcomes for Children with Cleft Palate and/or Lip and CHARGE Syndrome.

OBJECTIVES: Published literature on children with cleft palate and/or lip (CP + /-L) and CHARGE syndrome (CS) is limited. This study investigated cleft characteristics including surgery, and feeding and communication outcomes in children identified with CP + /-L and CS. DESIGN: Retrospective cross-sectional review. SETTING: Regional Referral Centre for Paediatric Cleft Surgery. PATIENTS: All children diagnosed with CP + /-L and CS (based on clinical features and/or CHD7 mutation testing) between 1989-2019. MAIN OUTCOME MEASURES: Cleft type, timing of CP + /-L repair, reasons for 'delayed' repair, feeding methods and communication modality. RESULTS: Twenty-two children with CP + /-L and CS were identified. Cleft sub-types (%) were: Eleven (50%) had bilateral cleft lip and palate (BCLP), six (27%) had unilateral cleft lip and palate (UCLP) and five (23%) had cleft palate (CP). Cleft repair was delayed compared to protocol care for non-syndromic children with CP + /-L. Median age for lip repair + /- vomerine flap was 9 months (range 4-22 months), and palate repair was 21 months (range 11-40 months). Median age for isolated CP repair was 13 months (range 7-23). Surgery for cardiac anomalies (36%) before cleft repair, and (59%) were classed as having severe systemic disease at the time of cleft surgery. Only 27% of the children in this study had both full oral feeding and verbal communication. CONCLUSIONS: Children with CP + /-L and CS had severe cleft types and complex medical problems leading to delayed cleft surgery. Feeding and speech outcomes were better in the children aged over ten years.

The Method of Surgical Lip Repair Affects Speech Outcomes in Children With Bilateral Cleft Lip and Palate.

OBJECTIVE: It was hypothesized that lip repair protocols in children with bilateral cleft lip and palate (BCLP) would affect development of bilabial consonants /m/ /b/ /p/. This study compared speech outcomes in 2 surgical groups. DESIGN: A retrospective case note investigation. SETTING: UK Cleft Centre (2000-2009). PATIENTS: Forty-nine children with complete BCLP, of whom 26 had a 1-stage and 23 a 2-stage bilateral cleft lip repair. INTERVENTIONS: One-stage cleft lip repair versus a 2-staged cleft lip repair. MAIN OUTCOME MEASURES: Bilabial consonant production at 18 months, 3 and 5 years of age. Cleft Speech Characteristics (CSCs) at age 5. RESULTS: At age 18 months, 81% of the 1-stage lip repair group and 4% of the 2-stage lip repair group produced bilabial consonants (P <·0001, Fisher test). At age 3 years, 81% of the 1-stage and 26% of the 2-stage lip repair groups produced bilabial consonants (P =·0133, Fisher test). At age 5 years, both groups had similar bilabial consonant production, but children in the 2-stage lip repair group had more frequent and severe CSCs (P = ·0037, χ2). CONCLUSION: A 1-stage lip repair for children with complete BCLP resulted in better bilabial consonant production at 18 months and 3 years of age than a 2-stage lip repair. At age 5 years both groups had bilabial consonants but children in the 2-stage lip repair group had worse CSCs. The surgical protocol for bilateral cleft lip repair affected speech outcome in children with BCLP.

Range and timing of surgery, and surgical sequences used, in primary repair of complete unilateral cleft lip and palate: The Cleft Care UK study.

OBJECTS: To describe the range of surgery used to repair the lip and palate in the UK with specific interest in the sequence/timing used in complete unilateral cleft lip and palate (cUCLP). SETTING AND SAMPLE POPULATION: The Cleft Care UK study, a cross-sectional study of 268 5-year-olds, born from 2005 to 2007, with complete unilateral cleft lip and palate. MATERIALS & METHODS: Information on surgery was extracted from medical notes by surgeons during research clinics and transcribed onto a standardized questionnaire. RESULTS: Surgical data were available for 251 (94%) children from all cleft centres in the UK (n = 18). Over a two-year period, 32 surgeons used 10 different surgical sequences in primary repair of the cleft lip and palate. The most frequently used sequence was repair of cleft lip and anterior hard palate followed by repair of posterior hard palate and soft palate (70%). Four surgical sequences were used only once. Most surgeons had a preferred sequence, but 38% (11/29) used more than one sequence during the study period. The timing of repair of the lip, the hard palate and the soft palate varied with surgical sequence, and also between surgeons, even adjusting for the different sequences used. CONCLUSION: Despite centralization of cleft services in the UK, there remains considerable variation in both the sequence and timing of surgical repair of cleft lip and palate in infancy. Further work is required to understand whether these factors are associated with differences in outcome.

Impact of Completing CLEFT-Q Scales That Ask About Appearance on Children and Young Adults: An International Study.

OBJECTIVE: To describe the impact of completing the CLEFT-Q appearance scales on patients with cleft lip and/or palate and to identify demographic and clinical characteristics and CLEFT-Q scores associated with reporting a negative impact. DESIGN: International cross-sectional survey. SETTING: Recruitment took place between October 2014 and November 2016 at 30 craniofacial clinics located in 12 countries. PATIENTS: Aged 8 to 29 years with cleft lip and/or palate. MAIN OUTCOME MEASURE(S): Participants were asked 4 questions to evaluate the impact of completing the field test version of a patient-reported outcome measure (the CLEFT-Q) that included 154 items, of which 79 (51%) asked about appearance (of the face, nose, nostrils, teeth, lips, jaws, and cleft lip scar). RESULTS: The sample included 2056 participants. Most participants liked answering the CLEFT-Q (88%) and the appearance questions (82%). After completing the appearance scales, most participants (77%) did not feel upset or unhappy about how they look, and they felt the same (67%) or better (23%) about their appearance after completing the questionnaire. Demographic and clinical variables associated with feeling unhappy/upset or worse about how they look included country of residence, female gender, more severe cleft, anticipating future cleft-specific surgeries, and reporting lower (ie, worse) scores on CLEFT-Q appearance and health-related quality-of-life scales. CONCLUSION: Most participants liked completing the CLEFT-Q, but a small minority reported a negative impact. When used in clinical practice, CLEFT-Q scale scores should be examined as soon as possible after completion in order that the clinical team might identify patients who might require additional support.

Single Suture Synostosis and Isolated Cleft Palate in Non-Apert Syndrome Patients.

INTRODUCTION: Single suture craniosynostosis (SSC) and isolated cleft palate (ICP) in non-Apert syndrome patients rarely occur together. Management includes airway optimization, timing surgery appropriately, and assessing both cranial vault aesthetics and speech outcomes. The aim of this study was to compare treatment pathways and outcomes in patients with both conditions to standard treatment for these conditions in isolation. METHODS: Patient hospital medical records were retrospectively reviewed for demographic data, timing of surgery, aesthetic outcome (using the Whitaker grading system for head shape), and speech outcome (from speech therapy records of general development and speech assessment). RESULTS: Six patients with SSC and ICP were identified over an 18-year period. Cranial surgery was performed between 4 and 16 months and cleft surgery between 6 and 34 months of age in all cases. Documentation of cleft surgery and genetic testing was not available for 1 patient. One patient with hemi-palatal absence had an obturator inserted at 34 months. Two patients were found to have Fragile X and Emanuel syndrome, respectively.No increased perioperative airway risk was highlighted in any case. Four cases were designated Whitaker grade 1, 1 case was designated grade 2, and 1 case was not graded as no cranial surgery was performed. Half of the patients had general and speech developmental delay, while the other half demonstrated no signs of developmental delay. DISCUSSION/CONCLUSION: Examination of 6 patients with SSC and ICP suggests the presence of both conditions does not adversely impact management or outcome for each condition, or increase perioperative risk.

The Cleft Multidisciplinary Collaborative: Establishing a Network to Support Cleft Lip and Palate Research in the United Kingdom.

BACKGROUND: As a growing paradigm of health research, trainee collaboratives can influence clinical practice through the generation of cost-effective multicenter audit and research projects. The aims of the present article are to outline and discuss the establishment of a multidisciplinary collaborative in the context of cleft lip and/or palate (CL/P). METHODS: The Cleft Multidisciplinary Collaborative (CMC) was formed in April 2016 under the overarching supervision of the National Institute for Health Research. Membership of the CMC is open to all members of the CL/P multidisciplinary team, who are encouraged to submit ideas for new research projects that will benefit clinical practice. RESULTS: To date, 48 clinical participants are involved in the CMC. These participants represent all 17 cleft teams from the United Kingdom and encompass a wide range of disciplines. The CMC has undertaken 2 major projects so far. The first involved collection of phenotype data to support a national cohort study. The second, still in progress, is a systematic review investigating factors associated with outcomes for velopharyngeal competence following cleft palate repair. CONCLUSIONS: The concept of a multidisciplinary collaborative in CL/P has been demonstrated through the generation of a United Kingdom-wide network of committed clinicians and researchers and the effective undertaking of 2 large research projects. As the CMC gathers momentum, it hopes to attract funding to support its activities, to promote more involvement from the allied health and nursing professions, to encourage a more ingrained research culture within the CL/P community, and to promote the wider ambition of a global collaborative.

Psychometric findings and normative values for the CLEFT-Q based on 2434 children and young adult patients with cleft lip and/or palate from 12 countries.

BACKGROUND: Patients with cleft lip and/or palate can undergo numerous procedures to improve appearance, speech, dentition and hearing. We developed a cleft-specific patient-reported outcome instrument to facilitate rigorous international measurement and benchmarking. METHODS: Data were collected from patients aged 8-29 years with cleft lip and/or palate at 30 hospitals in 12 countries between October 2014 and November 2016. Rasch measurement theory analysis was used to refine the scales and to examine reliability and validity. Normative CLEFT-Q values were computed for age, sex and cleft type. RESULTS: Analysis led to the refinement of an eating and drinking checklist and 12 scales measuring appearance (of the face, nose, nostrils, teeth, lips, jaws and cleft lip scar), health-related quality of life (psychological, social, school, speech distress) and speech function. All scales met the requirements of the Rasch model. Analysis to explore differential item functioning by age, sex and country provided evidence to support the use of a common scoring algorithm for each scale for international use. Lower (worse) scores on CLEFT-Q scales were associated with having a speech problem, being unhappy with facial appearance, and needing future cleft-related treatments, providing evidence of construct validity. Normative values for age, sex and cleft type showed poorer outcomes associated with older age, female sex and having a visible cleft. INTERPRETATION: The CLEFT-Q represents a rigorously developed instrument that can be used internationally to collect and compare evidence-based outcomes data from patients aged 8-29 years of age with cleft lip and/or palate.

Post-operative intravenous fluid administration for infant cleft surgery: An observational study.

OBJECTIVE: To investigate post-operative intravenous fluid administration and length of stay in a single site cleft centre. Previous publications have linked increased length of stay following primary cleft surgery to the administration of intravenous fluids post-operatively. MATERIALS AND METHODS: One hundred and ten primary cleft operations were conducted from May 2015 to April 2016 on non-syndromic infants. At West Midlands Cleft Centre, there are three cleft surgeons and 20 paediatric anaesthetists. This observational study compares classification of cleft type and surgical procedure with intravenous fluid administration, time taken to tolerate oral feeding, and length of stay. RESULTS: Cleft lip repair infants had the shortest length of stay in hospital, 25 h 8 min (median) and 33% had intravenous fluids. The palate repair only and lip and palate repair children had a median length of stay of 29 h 20 min and 29 h 0 min respectively, A total of 79% and 76% of these groups had intravenous fluids administered. Cleft lip repair infants fed in significantly less time than palate alone or lip and palate operations (p values 0.00 and 0.03, respectively). CONCLUSION: Cleft lip repair only infants feed well post-operatively and rarely require intravenous fluids. Infants having cleft repair involving the palate are slower to feed orally, and may require fluids due to poor oral intake. Intravenous fluids following lip repair is associated with longer hospital stay. We suggest intravenous fluids may not be needed routinely following cleft lip repair, but should always be considered following repair involving the palate.

Understanding cleft lip and palate. 4: Surgery and continuing care.

The fourth paper in this series on cleft lip and palate discusses the surgery involved in the care of children and young people with clefts of the lip and/or palate, orthodontic treatment and psychological support for this group of patients and their families.

Understanding cleft lip and palate. 3: feeding the baby.

The third paper in this series on cleft lip and palate gives an overview of feeding a baby with a cleft lip and/or palate. It includes a description of the feeding assessment that all babies receive from the Cleft Specialist Nurse, the different methods of feeding that are likely to succeed with each cleft type, and other associated care and interventions.

Understanding cleft lip and palate. 1: an overview.

Cleft lip and/or cleft palate affects about 1:700 babies born in the UK and may occur as a single anomaly or in association with other congenital abnormalities as part of a syndrome. This article, the first in a series, provides a brief overview of the different types of cleft lip and palate and describes the organisation of regional cleft services in England and Wales.

Understanding cleft lip and palate. 2: The first five years.

The second in a series on cleft lip and palate, this article outlines the diagnosis of these congenital anomalies, the problems that a child with a cleft lip and/or palate and their family may encounter in the first five years of life, and the multidisciplinary care needed. The input of individual members of the cleft team is described, including nurse specialists, speech and language therapists, paediatric dentists, ear, nose and throat surgeons, and audiologists.

CLEFT-Q: Detecting Differences in Outcomes among 2434 Patients with Varying Cleft Types.

BACKGROUND: Measuring the patient perspective is important in evaluating outcomes of cleft care. Understanding how treatment outcomes vary depending on cleft type may allow for better planning of treatments, setting of expectations, and more accurate benchmarking efforts. The CLEFT-Q is a patient-reported outcome measure for patients with cleft lip and/or palate. METHODS: The 12 CLEFT-Q scales measuring appearance (i.e., face, nose, nostrils, lips, cleft lip scar, teeth, and jaws), function (i.e., speech), and health-related quality of life (i.e., psychological, school, social, and speech-related distress) were field tested in a cross-sectional study in 30 centers in 12 countries. Patients with cleft lip and/or cleft palate aged 8 to 29 years were recruited from clinical settings. Differences in CLEFT-Q scores by cleft subtypes were evaluated using one-way analysis of variance or Kruskal-Wallis H tests, with Tukey or Dunn procedure with Bonferroni corrections post hoc analyses, respectively. Scores are presented using radar charts to visualize all outcomes simultaneously. RESULTS: The field test included 2434 patients. Scores on all CLEFT-Q scales varied significantly with cleft subtype. Patients with unilateral or bilateral cleft lip and/or palate scored lower on all appearance scales compared with patients with cleft palate or unilateral incomplete cleft lip. Scores on the speech function and speech-related distress scales decreased with each progressive group in the Veau classification. Patients with complete bilateral cleft lip and palate scored lowest on the social, school, and psychological scales. CONCLUSIONS: Patient-reported outcomes measured with the CLEFT-Q vary significantly with cleft type. Visualizing multiple outcomes simultaneously with radar charts allows for an understanding of a patient's overall status in a single graph.

Outcomes of Cleft Palate Repair in Patients with Pierre Robin Sequence: A Matched Case-Control Study.

BACKGROUND: Pierre Robin sequence results from a cascade of events that occur during embryologic development and frequently presents with cleft palate. Some studies have shown speech outcomes to be worse in patients with Pierre Robin sequence after cleft palate repair. METHODS: A cohort of Pierre Robin sequence patients who all required an airway intervention and nasogastric feeding in the neonatal period were identified and speech outcomes assessed at 5 years of age. A cleft- and sex-matched non-Pierre Robin sequence, cleft palate-only comparison group was also identified from the same institution and study period. RESULTS: A total of 24 patients with Pierre Robin sequence that required airway and nutritional support in the neonatal period were matched for age, sex, and cleft type to a group of 24 non-Pierre Robin sequence cleft patients. There was no significant difference in the incidence of oronasal fistula between the groups. Secondary surgery for velopharyngeal incompetence was significantly more (p = 0.017) in the Pierre Robin sequence group, who also had significantly greater nasality (p = 0.031) and cleft speech characteristic (p = 0.023) scores. CONCLUSIONS: The authors hypothesize that other factors may exist in Pierre Robin sequence that may lead to poor speech outcomes. The authors would suggest counseling parents of children with Pierre Robin sequence that have required a neonatal airway intervention, that speech development may be poorer than in other children with cleft palate, and that these children will have a significantly higher incidence of secondary speech surgery. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

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.

Identifying the effect of cleft type, deprivation and ethnicity on speech and dental outcomes in UK cleft patients: a multi-centred study.

BACKGROUND: Outcome measures are increasingly important in the modern National Health Service. In the care of children born with cleft lip and/or palate there are many different outcomes to consider but only a few reliable, validated outcome measures exist. The dmft (decayed, missing and filled teeth) index and cleft speech characteristics (CSCs) are used regularly by cleft teams throughout the UK to assess outcomes in children with cleft lip and/or palate. We hypothesized that these two outcome measures might be significantly influenced by the demographics of the populations studied independent of the care provided. METHODS: A retrospective review of all patients aged between five and six referred to three regional cleft centres during a twelve month period were included in the study. Speech and dental outcomes were compared with patient ethnicity, cleft type and level of deprivation as determined by the Carstairs score. RESULTS: The data of 287 patients were used. Speech was significantly affected by cleft type (p < 0.03), whereas dentition was significantly affected by ethnicity (p = 0.002) and deprivation (p = 0.012). CONCLUSIONS: This study demonstrates that the demographics of cleft populations can significantly affect the measures of outcome used to assess the quality of care provided by cleft teams. It has also demonstrated that these demographics are not evenly distributed across the country and that some cleft teams will have a more 'at risk' population than others. LEVEL OF EVIDENCE: Risk, level II.