Atypical facial clefts: Tessier number 3 and 4 clefts.

PURPOSE OF REVIEW: Tessier number 3 and 4 clefts result from failed fusion of facial processes during embryogenesis, and cause functional, psychosocial, and cosmetic morbidity. Given their rarity and heterogeneity, they present a unique challenge to the reconstructive surgeon, with limited literature for guidance. The purpose of this update is to summarize Tessier number 3 and 4 clefts with a focus on recent literature and expert opinion. RECENT FINDINGS: The incidence of atypical facial clefts has been estimated between 1.4 and 4.9 per 100 000 live births. Several retrospective chart reviews have been published in recent years; however, the epidemiologic data remains limited. Surgical management must be individualized and guided by classic reconstructive principles. The goal of surgery is to return the three soft tissue components (lip, nasomalar, and eyelid) to their proper anatomical location. SUMMARY: Tessier number 3 and 4 clefts are rare, demonstrate a wide spectrum of clinical presentation, and remain challenging to gain a breadth of experience for any single surgeon. They are classified based on their location along well defined anatomical axes. Component repair is performed with attention to the lip, nasomalar, and eyelid regions to restore facial symmetry and function.

Online Speech Therapy for Cleft Palate Patients in Rural Nepal: Innovations in Providing Essential Care during COVID-19 Pandemic.

BACKGROUND: Speech therapy is important for ideal functional outcome after cleft palate surgery. Nationwide lockdown due to outbreak of COVID-19 in Nepal restricted the ability of patients to travel to nearby outreach centers for regular speech therapy. The objectives were to assess the feasibility and challenges of conducting online speech therapy with postpalatoplasty children during COVID-19 pandemic; and evaluate the ways to overcome them. METHODS: Patients with cleft palate surgery done at least 3 months prior were given online speech therapy. Feasibility, advantages and challenges of online speech therapy were evaluated through interviewing the guardians and speech therapy providers. RESULTS: A total of 89 patients were included in the study. Only 11.2% had secondary palatine procedures. Almost all the children (97.8%) had face to face speech therapy prior to study period. Best use of time, use of audiovisual aid, no need to travel and rapid progress were the most commonly perceived strengths of online speech therapy. The most frequent challenges were internet connectivity, unclear voice, lack of direct interaction and unstable power supply. Recommended ways to improve online speech therapy were cited as better internet connectivity, having a fixed schedule and availing free or affordable Wifi. CONCLUSIONS: Despite the challenges, online speech therapy provided us with a way to reach out to the cleft palate children when face-to-face therapy was not possible due to COVID-19 pandemic. We see its role even during non-pandemic situations for the children who are unable to visit the speech therapy centers.

Mobile Surgical Scouts Increase Surgical Access for Patients with Cleft Lip and Palate in Nepal.

Background: In Nepal's remote regions, challenging topography prevents patients with cleft lip and palate (CLP) from seeking care. Objective: To measure the effect of a mobile surgical scout program on CLP surgical care in remote regions of Nepal. Methods: Forty-four lay people were trained as mobile surgical scouts and over 5 months traversed remote districts of Nepal on foot to detect and refer CLP patients for surgical care. Surgical patients from remote districts were compared with matched time periods in the year before intervention. Diagnostic accuracy of the surgical scouts was assessed. Findings: Mobile surgical scouts accurately diagnosed (90%) and referred (82%) patients for cleft surgery. Before the intervention, CLP surgeries from remote districts represented 3.5% of cleft surgeries performed. With mobile surgical scouting, patients from remote districts comprised 8.2% of all cleft surgeries (p = 0.007). When transportation and accompaniment was provided in addition to mobile surgical scouts, patients from remote districts represented 13.5% (p ≤ 0.001) of all cleft surgeries. Conclusion: Task-shifting the surgical screening process to trained scouts resulted in accurate diagnoses, referrals, and increased access to cleft surgery in remote districts of Nepal.

Prevalence of Dental Anomalies in the Patient with Cleft Lip and Palate Visiting a Tertiary Care Hospital.

INTRODUCTION: Dental anomaly is one of the major problems in a child born with cleft lip and palate. These anomalies have deleterious effects on the dentition leading to aesthetic problems, impairment of mastication andimproper phonation. The aim of our study was to find out the prevalence of dental anomalies in patient with cleft lip and/or palate radiographically. METHODS: A descriptive cross-sectional study was conducted from the 208 radiographs, collected by the convenience samplingtechnique with cleft lip and/or palate in Department of Burns, Plastic and Reconstructive Surgery, Nepal Cleft and Burn Centre, Kirtipur Hospital from January 2017 to July 2019.Ethical clearance for the study was obtained from Institutional Review Committee. Demographic data were collected and radiographs were evaluated for possible dental anomalies. Data obtained were entered and analysed in Statistical Package for Social Sciences version 23. RESULTS: Dental anomalies were highly prevalent among cleft lip and palate patients with at least one anomaly present in 188 (90.4%) of patients with male 120 (57.4%) presenting more anomalies than female 88 (42.6%) population. The most common anomaly was dental agenesis 161 (77.9%). The prevalence of positional anomaly, morphological anomaly and supernumerary teeth were found to be 54 (26%), 33 (15.9%) and 20 (10%) respectively. Lateral incisor showed the highest incidence of agenesis among all other missing teeth 223 (65.2%). CONCLUSIONS: The prevalence of dental anomalies among patients with cleft lip and/or palate was found to be high. Tooth agenesis was the most common anomaly observed in the study with lateral incisor having the highest incidence of agenesis.

Optimizing speech outcomes for cleft palate.

PURPOSE OF REVIEW: Cleft lip with or without palate is one of the most common pediatric birth anomalies. Patients with cleft palate often have speech difficulties from underlying anatomical defects that can persist after surgery. This significantly impacts child development. There is a lack of evidence exploring, which surgical techniques optimize speech outcomes. The purpose of this update is to report on recent literature investigating how to optimize speech outcomes for cleft palate. RECENT FINDINGS: The two-flap palatoplasty with intravelar veloplasty (IVVP) and Furlow double-opposing Z-plasty has the strongest evidence for optimizing speech. One-stage palatal repair is favored at 10-14 months of age, while delays are associated with significant speech deficits. For postoperative speech deficits, there is no significant difference between the pharyngeal flap, sphincter pharyngoplasty, and posterior pharyngeal wall augmentation. Surgical management should be guided by closure pattern and velopharyngeal gap but few studies stratify by these characteristics. SUMMARY: According to recent evidence, the two-flap palatoplasty with IVVP and Furlow palatoplasty result in the best speech. The pharyngeal flap, sphincter pharyngoplasty, and posterior pharyngeal wall augmentation are all viable techniques to correct residual velopharyngeal insufficiency. Future research should focus on incorporating standardized measures and more robust study designs.

A task-shifted speech therapy program for cleft palate patients in rural Nepal: Evaluating impact and associated healthcare barriers.

INTRODUCTION: Though access to surgical care for cleft lip/palate has expanded in low- and middle-income countries (LMICs), post-palatoplasty speech therapy is often lacking due to limited healthcare infrastructure and personnel. This mixed-methods study seeks to: 1) evaluate the impact of task-shifted speech therapy on a standardized speech score; 2) describe the experiences of families with post-operative cleft care and associated barriers; and 3) understand how to optimize cleft care by exploring the experiences of children who had nominal improvements after task-shifted speech therapy. METHODS: A convergent parallel mixed-methods study was conducted in Nepal. Standardized speech scores were compared by a blinded speech-language pathologist before and after the speech intervention. Semi-structured interviews (SSIs) and focus groups with families evaluated cleft care experiences and barriers. Qualitative and quantitative data were merged and analyzed. RESULTS: Thirty-nine post-palatoplasty children with speech deficits (ages 3-18) underwent task-shifted speech therapy, and demonstrated significant improvements in composite speech scores targeted by exercises (p<0.0001) and weakness (p=0.0002), with improvements in misarticulation (p=0.07) and glottal stop (p=0.05) that trended towards significance. Forty-seven SSIs demonstrated that the greatest barriers to follow-up were family responsibilities (62%), travel/distance (53%), and work (34%). In five focus groups, families expressed a desire to improve their child's speech and seek formal speech therapy. The speech intervention was found to be beneficial because of the compassionate staff, free lodging/food, and ability to socialize with other cleft patients and families. After merging quantitative and qualitative data, we noted that younger children between 3 and 5 years old and families who traveled greater distances for healthcare access benefited less from the speech therapy intervention. CONCLUSIONS: Task-shifted speech therapy has the potential to improve cleft lip/palate speech in LMICs. Multiple biosocial issues limit access to appropriate post-operative care.

Nepali Linguistic Validation of the Velopharyngeal Insufficiency Effects on Life Outcomes Instrument: VELO-Nepali.

OBJECTIVE: To translate and validate the velopharyngeal insufficiency (VPI) effects on life outcomes (VELO) instrument into Nepali, and test its internal consistency and validity. DESIGN: Quality-of-life instrument translation and validation. SETTING: Community served by Nepal's craniofacial referral hospital. PARTICIPANTS: Twenty-three postpalatoplasty children with VPI, 19 family guardians of VPI cases, and 29 non-VPI controls. INTERVENTIONS: The VELO instrument was translated to Nepali by 2 independent bilingual translators, reconciled, backward-translated, compared, and modified using patient cognitive interviews. All VPI children, guardians, and controls completed the VELO-Nepali. MAIN OUTCOME MEASURE(S): The VELO internal consistency was evaluated using Cronbach α coefficient. Concurrent validity and discriminant validity were assessed using 2-sample t test: assuming unequal variances. RESULTS: The VELO was translated and optimized using cognitive interviews. The VELO-Nepali demonstrated excellent internal consistency, with Cronbach α coefficients of 0.93, 0.94, and 0.90 for VPI cases, guardians of VPI cases, and non-VPI controls, respectively. The VELO-Nepali exhibited strong discriminant validity between VPI cases (x¯ = 45.4, standard deviation [SD] = 22.1) and non-VPI controls (x¯ = 84.9, SD = 12.3), (P < .001). The VELO-Nepali showed strong concurrent validity with similarities in VPI case scores (x¯ = 45.4, SD = 22.1), and guardian scores (x¯ = 52.9, s = 22.8; P = .473). CONCLUSION: The translated VELO-Nepali demonstrates strong internal consistency, discriminant validity, and concurrent validity, and can assess quality of life for Nepali VPI patients. This instrument represents the first VPI quality of life assessment validated in Nepali, and supports the feasibility of its implementation in other low- and low-middle-income countries.

Review of the First 108 Free Flaps at Public Health Concern Trust-NEPAL Hospitals: Challenges and Opportunities in Developing Countries.

BACKGROUND: Free tissue transfer is one of the most important and essential techniques in reconstructive surgery. The underlying complexity, steep learning curve, high cost, and fear of failure make it very difficult to establish as a regular service in developing countries such as Nepal. METHODS: A retrospective cohort study design was used to analyze the challenges with and opportunities for reconstructive surgery in Nepal. Medical records were reviewed for patient demographics, indications, types of free flaps, hospital stay, complications, and involvement of a microsurgery teaching workshop. RESULTS: A total of 16 microsurgical workshops were carried out by 3 international organizations over the study period (2007-2017). Altogether 108 free flaps in 103 subjects were reviewed during the study period at different hospitals of the Public Health Concern Trust-NEPAL (phect-NEPAL) and National Trauma Center. Of 103 patients, 60 were males and 43 were females with an average age of 34.5 years (range, 8-73 years). The most common indications for microsurgical reconstruction were tumor, trauma, and burns. Radial artery forearm flap, anterolateral thigh flap, and free fibular flap were the most common types of flaps. Ten different types of flaps were performed. Four cases needed more than 1 flap; one of them needed 3 flaps. Flap success rate approached 90%. Four patients died in the hospital postoperatively. CONCLUSION: Reconstructive microsurgery is challenging in Nepal and more generally in developing settings. However, persistent technical support such as training and workshops can make it feasible.

Age of individuals undergoing cleft lip and cleft palate surgeries in Nepal.

INTRODUCTION: Timely repair of cleft lip and palate maximises the benefits of surgery. Developing countries have large number of adults with unrepaired clefts. The impact of a cleft program can be determined by observing the trends of lower age at surgery. Public Health Concern Trust, Nepal has been providing a comprehensive nationwide cleft service since 1999. This study was conducted to see any change in the age at surgery. METHODS: A retrospective cross sectional study was conducted to analyse the data of all the individuals' age at primary cleft surgery from July 1999 to June 2010. Mean and median age of individuals as well as the proportion of individuals operated on at the right age in different years were calculated and compared. RESULTS: The median age for cleft lip surgery decreased from 100 to 24 months. Similarly the median age for cleft palate surgery decreased from 70 to 28 months. Proportion of surgeries carried out in the recommended age also increased. A change in the policy of the program reaching out to more remote areas and removing the age barrier for surgery resulted in older adults receiving surgery and increased median age especially for cleft palate repairs. CONCLUSIONS: A nationwide cleft program for a decade had a small impact on age at surgery. There are still many individuals who are missing the ideal age for surgery. The program needs to reach more remote areas. This information will be useful for governmental as well as non-governmental organizations working in the area of clefts.