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.

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.

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.

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.

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.

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.

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.

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.

The continuing multidisciplinary needs of adult patients with cleft lip and/or palate.

OBJECTIVE: Babies born with cleft lip and/or palate are followed-up throughout their growth in childhood. During adulthood, they may require further functional and/or aesthetic treatment. Adult multidisciplinary cleft clinics have been in place in the West Midlands, U.K. since June 2000. The aim of this study was to review the number and nature of problems these adult patients had and the types of treatment they required. DESIGN: All adult patients seen in 2004 were identified. Case notes were reviewed for patient epidemiology, problems at initial presentation, and interventions carried out until their last clinic visit in 2004. RESULTS: In 2004, there were 145 patients seen in the adult cleft clinic. Of those, 55 patients attended as part of their continuing care. Ninety were newly referred as adults to the cleft service. Patients ranged in age from 15 to 70 years and had, on average, three clinical problems each. One hundred and sixteen patients were listed for surgery of varying types, nine patients had nonsurgical speech-related intervention, 21 patients had restorative dental intervention, and 16 patients needed an extended clinical psychology assessment. CONCLUSION: For some patients who have had a cleft lip and/or palate, problems continue into adulthood or arise later in life. These problems are often multiple and treatment often requires the input of more than one specialist. The results of this audit support the need for coordinated multidisciplinary care for adults who have had a cleft lip and/or palate.

Management of infants with Pierre Robin sequence.

OBJECTIVE: Several methods of treating babies with Pierre Robin sequence have been described since the condition itself was first documented in 1923. The main aim of treatment has been to relieve upper airway obstruction. Treatment methods used range from positioning of the baby to invasive surgery. The aim of this article was to describe the assessment, treatment, and monitoring methods used for babies referred with Pierre Robin sequence (PRS). SETTING/PATIENTS: From December 1995 to May 2000, 22 consecutive patients were admitted to Birmingham Children's Hospital with PRS. Their airway and nutritional status were assessed and continuously monitored. INTERVENTIONS: Treatment concentrated on the relief of airway obstruction with a nasopharyngeal airway (NPA) and nutritional support of the babies until they grew out of their respiratory and feeding difficulties. MAIN OUTCOME MEASURES: Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. RESULTS: All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. CONCLUSION: Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.