Case report on bilateral cleft palate with protuded premaxilla

Cleft palate (ICD 10-Q 35.9) with Protruding of premaxilla is common feature in patient with bilateral cleft lip and palate it is due to the under trained growth at anterior nasal septal and vomero-premaxillary suture without lateral continuities. Hippocrates (400BC) AND Galen(150AD) mansion cleft lip, but not cleft palate in their writing, Cleft palate –Fanco.(1556), Repair of cleft lip –as early as 255-206 BC in CHINA. The first successful closure of a soft palate defect was reported in 1764 by LEMONNIERa French dentist.

Cleft Lip Palate in a Patient with 5q14.3 Deletion Syndrome: A Possible Unreported Feature?

5q14.3 deletion syndrome (MIM#613443) is an uncommon but well-known syndrome characterized by intellectual disability, epilepsy, hypotonia, brain malformations, and facial dysmorphism. Most patients with this syndrome have lost one copy of the <i>MEF2C</i> gene (MIM*600662), whose haploinsufficiency is considered to be responsible for the distinctive phenotype. To date, nearly 40 cases have been reported; the deletion size and clinical spectrum are variable, and at least 6 cases without <i>MEF2C</i> involvement have been documented. We herein report the clinical and cytogenomic findings of an 11-year-old girl who has a 5q14.3q21.1 de novo deletion that does not involve <i>MEF2C</i> but shares the clinical features described in other reported patients. Moreover, she additionally presents with bilateral cleft-lip palate (CLP), which has not been previously reported as a feature of the syndrome. The most frequent syndromic forms of CLP were ruled out in our patient mainly by clinical examination, and Sanger sequencing was performed to discard the presence of a <i>TBX22</i> gene (MIM*300307) defect. Our report suggests CLP as a possible unreported feature and redefines the critical phenotypic regions of 5q14.3 deletion syndrome.

Pre-surgical Alveolar Molding: An Adjunct to Surgical Repair in Cleft Lip and Palate Rehabilitation

Bilateral cleft lip and palate (CLP) with a severely protruded premaxillary segment can present as a challenge during surgical lip repairing. Pre-surgical alveolar molding, as described in this case report, is one such technique which aids in the approximation of the cleft segments thereby improving the esthetic result of the surgical repair. The present case highlights the importance of a simple molding appliance in re-positioning the protruded premaxillary segments of a neonate with bilateral CLP thereby serving as an adjunct to the surgical repair.

Evaluation of Lip Morphology and Nasolabial Angle in Non-Syndromic Cleft Lip and/Palate and Non-Cleft Individuals

Objective: To investigate the variation between the non-syndromic cleft lip and/or palate (NSCLP) and non-cleft (NC) subjects in relation to the lip morphology (LM) and nasolabial angle (NLA). Materials and Methods: Lateral cephalogram (Late. Ceph.) of 123 individuals (92 NSCLP [29 = bilateral cleft lip and palate (BCLP), 41 = unilateral cleft lip and palate (UCLP), 9 = unilateral cleft lip and alveolus (UCLA), 13 = unilateral cleft lip (UCL)], and 31 NC) who did not undergo any orthodontic treatment were investigated. By WebCeph, an artificial intelligence- (A.I.) driven software, 2 (two) parameters of LM, namely upper lip to E line (LM-1) and lower lip to E line (LM-2), and NLA analysis was carried out for each individual. Multiple tests were carried out for statistical analysis. Results: The mean ± SD observed for LM-1, LM-2, and NLA for NC individuals were 1.56 ± 2.98, 0.49 ± 3.51, and 97.20 ± 16.10, respectively. On the other hand, the mean ± SD of LM-1, LM-2, and NLA for NSCLP individuals were 4.55 ± 4.23, 1.68 ± 2.82, and 82.02 ± 14.66, respectively. No significant variation was observed with respect to gender and side. NSCLP (different types) and NC individuals showed significant disparities in LM-1 and NLA. Conclusion: It can be concluded that parameters of lip morphology such as LM-1, LM-2, and NLA vary among NSCLP and NC individuals.

Van der woude syndrome with associated scaphocephaly: a rare case report

The purpose of this case is to provide a descriptive report of a rare combination of Van der Woude syndrome, recurrent in three generations of the same family, in a female patient with bilateral cleft lip and palate associated with a sagittal craniosynostosis (scaphocephaly).

Oral Rehabilitation of Bilateral Cleft Lip and Palate Patient with Simultaneous LeFort I Osteotomy and Zygomatic Implants: A Case Report

The aim of this study is to evaluate Le Fort I Osteotomy and zygomatic implantation without any graft placement for management of a cleft lip and palate patient. This case report describes oral rehabilitation of a 33-year-old patient with bilateral cleft lip-palate and oronasal fistula and atrophic pre-maxilla. As treatment, the patient received simultaneous Le Fort I osteotomy, palatoplasty and two zygomatic implant insertions. The prosthetic superstructure included zygomatic implant-supported removable hybrid prosthesis on bar locator and metal-ceramic fixed bridges in the posterior region. As conclusion, this protocol can be promising for management of patients with cleft lip-palate and malocclusion.

Treatment Outcome in Bilateral Cleft lip and Palate Patients Evaluated With the Huddart-Bodenham Scoring System and the Bilateral Cleft lip and Palate Yardstick: A Systematic Review

Objectives To assess treatment outcome (transversal and sagittal dental arch relationships) and its determinants in complete bilateral cleft lip and palate (BCLP) evaluated with the modified Huddart-Bodenham scoring system and the BCLP Yardstick. Materials and methods Multiple electronic databases were searched without time limitation. Randomized clinical trials, cohort and case control studies using BCLP Yardstick and/or modified Huddart-Bodenham system to judge treatment outcome of patients with BCLP were included. The Risk of Bias in Nonrandomized Studies of Interventions tool and Grading of Recommendations, Assessment, Development, and Evaluation was used. Results Of the 528 studies identified by the electronic search, only eight retrospective studies met the inclusion criteria and were included. A total of 12 cleft centers were represented. All treatment protocols differed and background information was underreported. The results for the BCLP yardstick showed that all except the centers in New Zealand had a mean score lower than 3, indicating good treatment results. However, these studies had a moderate to high risk of bias. The modified Huddart-Bodenham scores were negative in all studies. No further meta-analysis was done due to heterogeneity and high risk of bias. The quality of evidence was graded as very low. Conclusion Results for the dental arch relationship of studies in complete BCLP and possible determinants were not synthesized due to very low quality of evidence. Clinical research for patients with BCLP should focus on sound methodological designs to enable evidence-based decision making to improve treatment for patients with BCLP and thereby hopefully their quality of life.