Anatomical Study of Domain Rescue of Palatal Length in Patients With a Wide Cleft Palate: Buccal Flap Reconstruction in Primary Palatoplasty.

BACKGROUND: This study characterizes the potential loss of velar length in patients with a wide cleft and rescue of this loss of domain by local flap reconstruction, providing anatomic evidence in support of primary lengthening of the soft palate during palatoplasty. METHODS: A retrospective review was conducted of all patients with a cleft palate at least 10mm in width, who underwent primary palatoplasty with a buccal flap prior to 18 months of age over a 2-year period. All patients underwent primary palatoplasty with horizontal transection of the nasal mucosa, which was performed after nasal mucosa repair, but prior to muscular reconstruction. The resulting palatal lengthening was measured and the mucosal defect was reconstructed with a buccal flap. RESULTS: Of the 22 patients included, 3 (13.6%) had a history of Pierre Robin sequence, and 5 (22.7%) had an associated syndrome. No patients had a Veau I cleft, 7 (31.8%) had a Veau II, 12 (54.5%) had a Veau III, and 3 had (13.6%) a Veau IV cleft. All patients had a right buccal flap during primary palatoplasty. The mean cleft width at the posterior nasal spine was 10.6 ± 2.82mm, and mean lengthening of the velum after horizontal transection of the nasal mucosa closure was 10.5 ± 2.23mm. There were 2 (9.1%) fistulas, 1 (4.5%) wound dehiscence, 1 (4.5%) 30-day readmission, and no bleeding complications. CONCLUSIONS: Patients with a wide cleft palate have a potential loss of 1cm velar length. The buccal flap can rescue the loss of domain in palatal length, and potentially improve palatal excursion.

Cleft Summit 2022: The Impact of a Unified Voice.

OBJECTIVE: The inaugural Cleft Summit aimed to unite experts and foster interdisciplinary collaboration, seeking a collective understanding of velopharyngeal insufficiency (VPI) management. DESIGN: An interactive debate and conversation between a multidisciplinary cleft care team on VPI management. SETTING: A two-hour discussion within a four-day comprehensive cleft care workshop (CCCW). PARTICIPANTS: Thirty-two global leaders from various cleft disciplines. INTERVENTIONS: Cleft Summit that allows for meaningful interdisciplinary collaboration and knowledge exchange. MAIN OUTCOME MEASURES: Ability to reach consensus on a unified statement for VPI management. RESULTS: Participants agreed that a patient with significant VPI and a dynamic velum should first receive a surgery that lengthens the velum to optimize patient outcome. A global, multicenter prospective study should be done to test this hypothesis. CONCLUSION: The 1st Cleft Summit successfully distilled global expertise into actionable best-practice guidelines through iterative discussions, fostering interdisciplinary collaboration and paving the way for a transformative multi-center prospective study on VPI care.

Palatal surface development from 6 years of age to early adulthood: data modelling using 3D geometric morphometrics.

OBJECTIVES: The study followed the modelling of postnatal growth of a healthy palate of the Central European (Czech) population sample based on transverse data on sex and age from 6 to 19 years. MATERIALS AND METHODS: Digitised 3D models of 212 healthy palatal surfaces were evaluated using 3D geometric morphometrics and superimpositions. The individuals were grouped based on age (preschool, younger and older school age, younger and older adolescents, young adults) and sex (♂ n = 101, ♀ n = 111). RESULTS: Female palatal development was non-linear and was interrupted between the 10-12 years and then proceeded intensively until the age of 15 when it ceased. In contrast, male-modelled growth was consistent throughout the follow-up and continued linearly until at least 19 years of age. The palate did not widen further with increasing age, and primarily palatal vaulting and heightening were found. The characteristics and distribution of areas with extensive modelled growth changes were comparable in females and males, as confirmed by the location of principal components (PC1 and PC2) within modal space and growth trajectories. The extent of sexual dimorphism increased from 15 years of age due to pubertal spurt combined with earlier completion of palatal development in females. CONCLUSIONS: The study showed modelled healthy palatal development from 6 years of age to early adulthood, which might be utilised as reference standards for the Central European population sample. CLINICAL RELEVANCE: The comparison of normal reference subjects with patients with cranio-maxillo-facial dysmorphologies represents the first step in diagnosing and establishing effective therapy.

Formula Otorrhea in Pediatric Patients With Cleft Palate.

The case series details 2 unusual cases of male newborns with cleft lip and palate (CLP) that later developed formula otorrhea. Both patients underwent bilateral myringotomies with the insertion of pressure equalizing (PE) tubes for chronic otitis media with effusion (OME). Chronic otorrhea associated with feeding occurred post-PE tube insertion and the otorrhea was later confirmed to be due to reflux of formula. Patients were treated with antibiotic ear drops, routine ear cleaning, anti-reflux medication, and reflux precautions. After definite cleft palate repair, formula otorrhea completely resolved. When patients with CLP develop chronic OME or otorrhea following PE tube placement, reflux of formula into the middle ear should be considered and treated accordingly.

Total Aplasia of Paranasal Sinus Associated With a Syndromic Condition.

Total aplasia of paranasal sinus (TAPS) is extremely rare, although the fact that partial aplasia is very common. TAPS seems to be limited to only 5 case reports in the literature until now. We present the case of a 29-year-old patient who has a syndromic face appearance but whose TAPS was detected incidentally. The maxillary, sphenoid, ethmoid, and frontal sinuses were totally aplastic. Furthermore, clinodactyly and high-arched palate were observed. The patient's appearance was consistent with a syndromic face because of some findings on inspection such as hypertelorism, shortening of the palpebral fissure, protruded and wide nasal base, high arched palate and zygomatic hypoplasia. The patient's profile was more suitable for Teacher-Collins syndrome than other syndromes, however, a certain diagnosis was not made genetically. To the best of our knowledge, this is the first reported association between TAPS and a syndromic condition.

Comparative Study of Maxillary Growth in Patients With Unilateral Cleft Treated With and Without Pre-Surgical Orthopedics.

Compare the maxillary growth of patients with Unilateral Cleft treated with pre-surgical Functional Maxillary Orthopedic (FMO) and that of who underwent a surgical procedure with no previous use of appliances.Prospective study, the patients were divided into 2 groups. G1, was composed of 12 patients who received no pre-surgical intervention and cheilorhinoplasty at 6 months of age and G2, included 12 patients treated using Pre-Surgical Funtional Maxilary Orthopedic; they underwent no surgery during the study. Measurements were taken at three times: before the first month of life, at 6 months and between 9-12 months of age. Cleft distance (anterior, medium and posterior), maxillary width (anterior, medium and posterior) and minor and major segment width were analyzed.Alveolar Cleft decrease was 79.82% in G1 and 52% in G2. Posterior Cleft decrease was 24.1% in G1 and 41.77% in G2. Greater Segment Width increase was 24.53% for the patients in G1, and 37.47% for the patients in G2. As for Inter Canine Width, a decrease of 5.16% in G1 and an increase of 9.19% in G2 were found. Medium Arch Width only increased in a statistically significant manner in G2 9.02%.Surgery allowed for the closure of the alveolar cleft. FMO made it possible to close the anterior and the posterior clefts through the growth of the maxillary segments, increased the transverse growth of the maxilla and could prevent maxillary collapse. Each team must individually evaluate whether to indicate or not the use of preoperative orthopedics.

Racial Disparities in the Timing of Alveolar Bone Grafting.

Standard bone grafting between ages 6 and 12 has become the preferred treatment of choice for alveolar clefts. Given the importance of surgical timing in complete cleft palate repairs, it is important to identify any populations at-risk for delayed alveolar bone grafting. The purpose of this study is to identify whether a racial disparity is present nationally in the timing of alveolar bone grafting.Retrospective analysis Setting: Hospitals participating in the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP).Inclusion criteria involved patients who underwent alveolar bone grafting identified by current procedure terminology (CPT) code 42210 between years 2012 and 2019. Patients were stratified by age at time of operation based on the following parameters: early bone grafting (before 6 years of age), standard bone grafting (between 6 and 12 years of age), and late bone grafting (after 12 years of age).Racial and ethnic differences in the age of patients at the time of alveolar bone grafting.Overall, 20.28% of the cohort received alveolar bone graft after 12 years of age. African American (29.33%) and Hispanic (24.42%) patients received late alveolar bone grafting more frequently than other racial and ethnic groups (P < .001).Racial and ethnic disparities are present in the frequency at which patients receive late alveolar bone grafting for complete cleft palates. Given the suboptimal surgical results of late compared to standard alveolar bone grafting it is important to further investigate the driving factors of these disparities.

A Rare Case of Cleft Palate Associated With Tongue Hamartoma: A Case Report and Systematic Review.

INTRODUCTION: Palate development involves a genetic regulation through a complex molecular mechanism that may be disrupted by environmental factors, resulting in impaired fusion and cleft palate formation. An encounter with a case of cleft palate due to dorsal tongue hamartoma prompted us to perform this systematic review. OBJECTIVE: To review the clinical profile and management approach for a case with cleft palate and tongue hamartoma. DESIGN: A systematic literature search was conducted using keywords related to cleft palate and tongue hamartoma in PubMed, Scopus, MEDLINE, and Scielo databases through December 2021, with no time or language restrictions. PATIENTS, PARTICIPANTS: Studies reporting patients with cleft palate and tongue hamartoma were included. MAIN OUTCOME MEASURE(S): Information related to clinical profile, diagnostic tests, histopathology, management, and outcomes were extracted.Fourteen relevant publications were identified with 16 cases reported so far. Among them, thirteen patients were females (81.25%), and 3 were males (18.75%). The age of presentation varied from birth to 19 years. Oral-facial-digital syndrome (type II) was the most commonly associated syndrome.Congenital tongue hamartoma with cleft palate is a rare presentation, which can present as an isolated entity or part of a syndrome. Genetic evaluation is warranted, particularly for multiple hamartomatous lesions. The preferred treatment is immediate excision of hamartoma while following a standard timeline for palatoplasty.

A Comprehensive Assessment of Cleft Lip and Palate Websites and Patient Education Information.

OBJECTIVE: The objective of this study is to evaluate online educational resources on cleft lip and palate teams. DESIGN: A Cross-Sectional Study. SETTING: An International, Multi-Institutional Study. PARTICIPANTS: All American Cleft Palate and Craniofacial Association-approved teams with websites. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Websites were assessed for patient education and support content. Affiliation between presence of materials and U.S. Newsweek Top 100 Hospitals was also assessed. RESULTS: 187 teams were included. Presence of educational videos were available in 29.4% of websites, educational infographics in 18.2%, written materials in 66.8%, perioperative instructions in 19.3%, diagnosis information in 34.8% and treatment information in 63.1%. Information on team members were available on 77.0% of websites, resources in languages other than English in 38.5%, and support group resources in 25.7%. Cleft lip and palate diagnosis information was significantly associated with geographic region, with the largest impact from the West region (P = .03). There was a significant difference between teams affiliated with U.S. Newsweek Top 100 hospitals: Top 100 hospitals had a higher presence of educational infographics, perioperative instructions, definitions, diagnosis, and treatment (P < .01). There was no significant difference between presence of educational videos (P = .37). CONCLUSION: While many websites had basic educational materials, very few included detailed information on peri-operative planning, as well as additional forms of information including videos, infographics, and non-English languages. Providing comprehensive patient education materials online is an important supplement for patients with cleft lip and palate and should be prioritized by cleft teams.

Labial Vestibular Flap Closure of the Cleft Palate Is Advantageous for Maxillary Development.

Objective: Using labial vestibular flap was performed to close the primary alveolar and hard palate cleft at the second stage of early 2-stage closure surgery for unilateral cleft lip and palate for minimizing the damage to the maxillary periosteum. We analyzed maxillary development to clarify the influence of cleft palate surgery. Design: Retrospective longitudinal study in 5 years after primary palatal closure. Setting: Institutional study Patients: Study subjects included 214 patients with nonsyndromic complete unilateral cleft lip and palate who were consecutively treated in our clinic. Main Outcome: We used a 3D dental model scanner to assess maxillary development in patients aged 3 months to 5 years after using either the conventional pushback method (PB) (51 cases) or 2-stage closure (Local palatal flap closure: LF [67 cases] and Labial vestibular flap closure: VF [96 cases]). Results: Comparing the measurement results, the major axis of maxilla, width, intercanine distance, and intermolar distance was significantly larger in the LF group compared to the PB group. After the age of 3, the cleft side of VF group had grown significantly to compare with LF group in width. It was also confirmed that the inserted labial mucosal flap itself grew. Enlargement of the labial mucosal flap was observed at all sites except the canine. Conclusion: Good maxillary growth occurred in the following order: VF groups > LF group > PB group. Poor growth was correlated with the extent of periosteal damage during surgery and the degree of postoperative bone surface exposure.

Presurgical Orthopedic Treatment of a 3-Year-Old Child with Unoperated Complete Bilateral Cleft Lip and Palate and Protruding Premaxilla.

Unoperated patients with bilateral complete cleft lip and palate (BCCLP), who do not receive orthopedic treatment in infancy and present with significant sagittal excess of the premaxilla, are among the most difficult patients to treat. Delay in primary reconstruction reduces the elastic characteristics of the tissues. The resulting rigidity of bony structures accompanied by the wide alveolar and palatal clefts associated with sagittal excess of the premaxilla pose a great challenge for both surgeon and orthodontist. There is no unique protocol for treatment of these conditions. This paper describes the use of orthopedic treatment in an unoperated 3-year-old male child with BCCLP and a protrusive premaxilla whose appearance and function were both severely affected. A modified Meazzini technique, as described in operated BCCLP patients with a downward-displaced premaxilla, was used. The device itself consists of the 2 independent components mutually connected by a 17 × 25 steel arch. The first is an active fan-type expander. The second consists of an acrylic cap covering the premaxilla and 3 brackets used for the steel arch application. They are connected by a rectangular steel 17 × 25 archwire. Reduction of the premaxillary sagittal excess of is carried out with an elastic chain. The use of this orthopedic appliance is a reliable and valuable presurgical treatment in the therapy of children with BCCLP and protrusive premaxilla whose orthopedic and surgical treatment have been delayed.

A Unique Case Report of Meningeal Hamartoma Within Alveolar Cleft.

This case report demonstrates a rare finding of a pediatric patient with Morning Glory anomaly and Moyamoya Disease with a palatal meningeal hamartoma discovered as a mass within a previously repaired incomplete cleft of the alveolus. Oral meningeal hamartomas are exceedingly rare with only two palatal cases described and none within a cleft palate or alveolus. These findings prompt a review of oral hamartomas with meningeal subclassification. Further discussion describes the relationship of the proposed origins of meningeal hamartomas within the setting of cleft palate development.

Effect of Veau Class on Levator Veli Palatini Muscle Composition.

OBJECTIVE: To examine levator veli palatini muscle composition in patients with nonsyndromic cleft palate and investigate the impact of Veau class. DESIGN: Prospective cohort study. SETTING: Tertiary care academic hospital. PATIENTS/PARTICIPANTS: Thirteen patients with nonsyndromic cleft palate were recruited. INTERVENTIONS: During primary palatoplasty, a sample of levator veli palatini muscle was excised and prepared for histological analysis. MAIN OUTCOME MEASURES: Fat and collagen content were determined utilizing Oil Red and Sirius red stains, respectively, while muscle fiber cross-sectional areas were calculated from H&E-stained samples, with analysis using histomorphometric methods. Immunofluorescent staining of myosin heavy chain isoforms was performed. RESULTS: Patients underwent repair at 10.8 months of age (interquartile range [IQR] 10.2-12.9). Fat content of the levator veli palatini muscle was low in both groups, ranging from 0% to 5.2%. Collagen content ranged from 8.5% to 39.8%; neither fat nor collagen content showed an association with Veau classes. Mean muscle fiber cross-sectional area decreased with increasing Veau class, from 808 µm2 (range 692-995 µm2) in Veau II to 651 µm2 (range 232-750 µm2) in Veau III (P = .02). There was also a nonsignificant decrease in proportion of type I muscle fibers with increasing Veau class (44.3% [range 31.4%-84.4%] in Veau II vs 35.3% [range 17.4%-61.3%] in Veau III). CONCLUSIONS: Muscle fiber area in levator veli palatini muscles decreases in Veau III clefts in comparison to Veau II. The impact of these differences in velopharyngeal dysfunction requires further analysis of a larger cohort.

Histologic Aspect of the Curved Vomerine Mucosa in Cleft Lip and Palate.

BACKGROUND: Common surgical techniques aim to turn the entire vomerine mucosa with vomer flaps either to the oral side or to the nasal side. The latter approach is widely performed due to the similarity in color to the nasal mucosa. However, we lack a histologic description of the curved vomerine mucosa in cleft lip and palate malformations. METHODS: We histologically examined an excess of curved vomerine mucosa in 8 patients using hematoxylin-eosin, periodic acid-Schiff, Elastin van Gieson, and Alcian blue stains. Tissue samples were obtained during surgery at 8 months of age. RESULTS: Our histological analysis of the mucoperiosteum overlying the curved vomer revealed characteristics consistent with those of an oral mucosa or a squamous metaplasia of the nasal mucosa, as exhibited by a stratified squamous epithelium containing numerous seromucous glands. Some areas showed a palisaded arrangement of the basal cells compatible with metaplasia of respiratory epithelium, but no goblet cells or respiratory cilia were identified. Abundant fibrosis and rich vascularity were present. CONCLUSION: The vomer mucosa showed no specific signs of nasal mucosa. These findings should be considered in presurgical cleft orthopedics and palatal surgery for further refinement. Shifting the vomer mucosa according to a fixed physiologic belief should not overrule other important aspects of cleft repair such as primary healing and establishing optimal form and function of palatal roof and nasal floor.

Intraoral Digital Impression for Speech Aid/Obturator in Children: Report of 2 Cases.

Cleft patients may develop an abnormal opening (fistula) between the oral and the nasal cavities. Surgical repair minimizes the adverse effect on speech and feeding. However, an obturator prosthesis is a nonsurgical approach to help close the communication. The purpose of the case report presented is to show the clinical use of an intraoral digital impression in the fabrication of obturator/speech aid appliances in children with cleft lip and palate deformity. Minimal adjustments were needed, and patients and caregivers responded positively. Prostheses demonstrated good stability and retention at delivery. The use of digital technology seems to have several benefits as an alternative method for capturing impressions, especially in young children with cleft lip and palate deformity.

Cleft Lip and/or Palate in Infants Prenatally Exposed to Opioids.

OBJECTIVE: To determine the prevalence and odds ratios for cleft lip and/or palate (CL/P) among infants prenatally exposed to opioids with or without neonatal opioid withdrawal syndrome (NOWS). DESIGN: This study represents an exploratory, retrospective cohort study design of newborn medical health records from 2011 to 2016. SETTING: Records were drawn from a regional health system located in South Central Appalachia. POPULATION AND STUDY SAMPLE: The original population yielded 3 cohorts of infants: (1) infants with opioid exposure (OE) but not requiring pharmacological intervention (OE; N = 168); (2) infants with NOWS requiring pharmacological intervention (N = 294); and (3) infants with no opioid exposure (NOE; N = 16 090), the primary comparison group. MAIN OUTCOME: Infants in the NOWS and OE groups showed significantly increased prevalence and odds ratios for CL/P when compared to those in the NOE group. RESULTS: Prevalence rates per 1000 live births for infants with OE (35.71) and infants with NOWS (6.80) were significantly higher than those for infants with NOE (1.37). Comparison of infants with OE to the NOE group revealed significantly increased odds for CL/P, isolated cleft palate (CP), cleft lip (CL), and cleft lip and palate (CLP) (27.05, 41.81, 19.26, 19.37, respectively; all Ps < .008). The odds ratios for infants with NOWS compared to the NOE group were significantly higher for CL/P and CP (5.00 and 10.98, respectively; Ps < .03) but not for CL and CLP. CONCLUSION: The results provide additional evidence that prenatal OE should be considered among the critical environmental risk factors that can contribute to CL/P.

The transcriptional regulator MEIS2 sets up the ground state for palatal osteogenesis in mice.

Haploinsufficiency of Meis homeobox 2 (MEIS2), encoding a transcriptional regulator, is associated with human cleft palate, and Meis2 inactivation leads to abnormal palate development in mice, implicating MEIS2 functions in palate development. However, its functional mechanisms remain unknown. Here we observed widespread MEIS2 expression in the developing palate in mice. Wnt1Cre -mediated Meis2 inactivation in cranial neural crest cells led to a secondary palate cleft. Importantly, about half of the Wnt1Cre ;Meis2f/f mice exhibited a submucous cleft, providing a model for studying palatal bone formation and patterning. Consistent with complete absence of palatal bones, the results from integrative analyses of MEIS2 by ChIP sequencing, RNA-Seq, and an assay for transposase-accessible chromatin sequencing identified key osteogenic genes regulated directly by MEIS2, indicating that it plays a fundamental role in palatal osteogenesis. De novo motif analysis uncovered that the MEIS2-bound regions are highly enriched in binding motifs for several key osteogenic transcription factors, particularly short stature homeobox 2 (SHOX2). Comparative ChIP sequencing analyses revealed genome-wide co-occupancy of MEIS2 and SHOX2 in addition to their colocalization in the developing palate and physical interaction, suggesting that SHOX2 and MEIS2 functionally interact. However, although SHOX2 was required for proper palatal bone formation and was a direct downstream target of MEIS2, Shox2 overexpression failed to rescue the palatal bone defects in a Meis2-mutant background. These results, together with the fact that Meis2 expression is associated with high osteogenic potential and required for chromatin accessibility of osteogenic genes, support a vital function of MEIS2 in setting up a ground state for palatal osteogenesis.

Hypogonadism: Is It Always Hypogonadotropic in an Adolescent With a Cleft Palate? A Surprising Case of Klinefelter Syndrome.

Pituitary hormone deficiencies may occur in children with midline defects; in these cases, hypogonadism is usually hypogonadotropic. Herein, we report a boy at the age of 13.8 years with mild mental retardation, previously operated for complete cleft palate (isolated) and presented with hypoglycemia due to isolated secondary adrenal insufficiency, who further had a decrease in testicular size with increased follicle-stimulating hormone level (hypergonadotropic hypogonadism) and diagnosed with Klinefelter syndrome. Klinefelter syndrome in childhood is rarely diagnosed and cases are observed in a wide spectrum. Although some regional duplications of the X chromosome also show midline defects such as spina bifida-neural tube defects, mental retardation, hypopituitarism (mostly growth hormone deficiency); coexistence of Klinefelter syndrome and isolated secondary adrenal deficiency/midline defect in our case may also be coincidental. However, to our knowledge, this is the first case in literature with this association in a patient with a 47, XXY karyotype.

Spontaneous Palatal Cleft Closure.

This case report presents a palatal cleft that healed spontaneously, with complete formation of mucosa and bone. Even though the nasal structures could initially be observed through the cleft palate, a thin membrane sealed any communication between the oral and nasal cavities. The origin of this tenuous membrane cannot be fully understood with current discernment of palate formation, but it probably served as a basis for the formation of the other tissues. No previous record of nonintervened spontaneous closure of a cleft palate has been reported.

Spontaneous Growth of the Palatal Plates in the Cleft Lip and Palate.

OBJECTIVE: This is a prospective study examining palatal casts from patients with unilateral cleft lip and palate (UCLP) in the first month of life, immediately before cheiloplasty, and immediately before palatoplasty. None of the patients receives presurgical orthopedics (nasoalveolar molding). DESIGN: In this prospective study, upper arch plaster models were taken 3 times during the treatment: in the first month of life (T1), before the cheiloplasty (T2), and before the palatoplasty (T3). Anatomic landmarks were defined and linear anthropometric measurements were obtained afterward. Dimensional analysis was performed using 3D software. Two-way analysis of variance followed by Tukey test was performed for statistical analysis. SETTING: Tertiary, institutional. PARTICIPANTS: Twelve patients with UCLP of either sex with less than 1 month of life and without any other syndrome. INTERVENTION: No intervention was performed. MAIN OUTCOME MEASURE: Reduction of the cleft without using orthopedics apparatus. RESULTS: There was a statistically significant reduction in the cleft gap comparing T1 to T3. There was also a significant reduction in the intercanine width comparing T2 and T3, and T1 and T3. There was significant increase in the posterior arch width comparing T2 and T3, and T1 and T3. The palatal plate's width increased in all times analyzed. CONCLUSION: The palatal cleft narrows spontaneously as well in both midpoint and posterior point during the first 6 months of patient's treatment. This event was enhanced by cheiloplasty. This surgery might have a greater influence on the anterior arch width than in the posterior arch region.