Prenatal ultrasonic diagnosis of submucosal cleft palate / 中华超声影像学杂志

Objective:To summarize the ultrasound manifestations of submucosal cleft palate, and explore the diagnostic value of prenatal ultrasound for submucosal cleft palate.Methods:A total of 21 146 pregnant women who underwent fetal ultrasound examination in the second and third trimesters in the Affiliated Hospital of Jining Medical University from January 2013 to May 2018 were collected. They were all singleton pregnancy. The ultrasound image which was the horizontal plate of the palatine bone at the posterior border of the fetal hard palate was routinely obtained. The presence of bone loss at the posterior border of the hard palate was defined as a positive case. Then the palate targeted ultrasound examinations of the positive cases were performed to observe the continuity of the soft palate. The ultrasound images of positive cases were compared with the results of induction or delivery, and their postpartum diagnosis and treatment were tracked.Results:A total of 44 simple cleft palate were detected in 21 146 fetuses, including 23 dominant cleft palate and 21 submucosal cleft palate. Two cases of 21 submucosal cleft palate were induced because of other deformities, the other 19 cases were born. The follow-up of the 19 submucosal cleft palate cases showed that 15 cases visited to stomatology department before 3 years of age, and 2 cases of newborns with dominant cleft palate were misdiagnosed as submucosal cleft palate by prenatal ultrasound, and the other13 of them were clinically diagnosed as submucosal cleft palate. The ultrasound of the submucosal cleft palate showed there was no inverted "V" -shaped bone in the posterior edge of the hard palate which was connected by a membranous connection, and the soft palate was complete, but the center of soft palate was thinner or even present membranous hyperechoic in ultrasound.Conclusions:Submucosal cleft palate has characteristic ultrasound features, and prenatal ultrasound make a diagnosis and provide some basis for obtaining early diagnosis and treatment after birth.

Surgical management in a severe OSA patient diagnosed with Stickler syndrome.

Stickler syndrome is a genetic disorder of connective tissue. One of the major symptoms associated with this disorder is an oro-facial malformation, which may cause a submucous cleft or a complete cleft of the hard palate. A 32-year-old man diagnosed with Stickler syndrome and a submucosal cleft palate (SMCP) visited our hospital with a chief complaint of excessive daytime sleepiness. The patient was diagnosed with severe obstructive sleep apnea (OSA), and administration of a polysomnography test revealed an apnea-hypopnea index (AHI) of 30.9 events/hour (h). Auto-titrating continuous positive airway pressure was initiated to control the OSA symptoms and subsequently the patient showed some improvement. However, due to continuous velopharyngeal insufficiency symptoms, intravelar veloplasty was performed. Three months after surgery, the AHI had decreased to 12.4 events/h. Recent studies have described a greater risk for OSA in individuals with cleft palate, than in the general population. The present case demonstrates surgical success in a patient with OSA and SMCP, suggesting that palatal surgery may be considered an optional surgical treatment for OSA patients with SMCP.

Incidence of Symptomatic Submucous Cleft Palate in the Netherlands: A Retrospective Cohort Study Over a Period of 22 Years.

OBJECTIVE: To analyze the incidence of submucous cleft palate (SMCP) in a large national database and raise awareness among referring providers: pediatricians, speech pathologists, and dentists to minimize delay in diagnosis. DESIGN: Retrospective cohort study. SETTING: Tertiary setting. PATIENTS: Patients were extracted from the "Dutch Association for Cleft and Craniofacial Anomalies" database. A total of 6916 patients were included from 1997 until 2018 and divided into 2 groups (ie, SMCP versus cleft palate [CP]). Patients born before 1997 and adopted patients were excluded. INTERVENTIONS: Clefts were classified as either hard of soft palatal involvement based on anatomical landmarks at first consultation. MAIN OUTCOME MEASURES: Primary outcomes were the patient characteristics in both groups (ie, gender, birth weight, gestational age, and additional anomalies). Secondary outcome was the time of diagnosis among subgroups. RESULTS: In total, 532 patients were diagnosed with SMCP (7.7%). Birth weight, gestational age, and additional anomalies did not differ between subgroups, but there were more males in the SMCP group (P < .001). The median age of diagnosis of the SMCP group was significantly higher than of the CP group (987 vs 27 days; P < .001). Over the course of 22 years, the time of diagnosis for SMCP did not decrease. CONCLUSION: Submucous cleft palate represents <10% of the Dutch cleft population and 19.4% of all CP. Time of diagnosis for SMCP is significantly longer when compared with time of diagnosis of CP, and this has not changed over the study period of 22 years.

Incidence of bifid uvula and its relationship to submucous cleft palate and a family history of oral cleft in the Brazilian population / Incidence of bifid uvula and its relationship to submucous cleft palate and a family history of oral cleft in the Brazilian population

Abstract Introduction: Bifid uvula is a frequently observed anomaly in the general population and can be regarded as a marker for submucous cleft palate. Objective: In this study aimed to determine the frequency of bifid uvula and submucous cleft palate and their relationship with oral clefts in a Brazilian population. Methods: We conducted a transversal, descriptive and quantitative study of 1206 children between August 2014 and December 2015. A clinical examination of the children was conducted by means of inspection of the oral cavity with the aid of a tongue depressor and directed light. After the clinical examination in children, parents answered a questionnaire with questions about basic demographic information and their family history of oral clefts in their first-degree relatives. After application of the questionnaires, the information collected was archived in a database and analyzed by the statistical program SPSS® version 19.0, by applying Chi-Square tests. Values with p < 0.05 were considered statistically significant. Results: Of the 1206 children included in this study, 608 (50.40%) were female and 598 (49.60%) were male (p = 0.773). The average age of children was 3.75 years (standard deviation ± 3.78 years). Of the 1206 children studied, 6 (0.5%) presented with bifid uvula. Submucosal cleft palate was not found in any child. When the family histories of children were examined for the presence of nonsyndromic cleft lip and/or cleft palate, no first degree relatives presented with the congenital anomaly. Conclusion: This study revealed that the incidence of bifid uvula and submucous cleft palate in this population was quite similar to previously reported incidence rates. Our study suggests an intensification of new reviews, with broader and diverse populations, seeking to associate the occurrence of bifid uvula, submucous cleft palate and oral clefts.

Resumo: Introdução: A úvula bífida é uma anomalia frequentemente observada na população em geral e pode ser considerada como um marcador de fissura palatina submucosa. Objetivo: Determinar a frequência de úvula bífida e fissura palatina submucosa e sua relação com fissura orais em uma população brasileira. Método: Realizamos um estudo transversal, descritivo e quantitativo de 1.206 crianças entre agosto de 2014 e dezembro de 2015. O exame clínico das crianças foi realizado por meio da inspeção da cavidade oral com auxílio de um abaixador de língua e luz direcionada. Após o exame clínico nas crianças, os pais responderam a um questionário com perguntas sobre informações demográficas básicas e antecedentes de fendas orais em familiares de primeiro grau. As informações coletadas foram arquivadas em um banco de dados e analisadas pelo programa estatístico SPSS® versão 19.0, aplicando testes de Qui-Quadrado. Os valores com p < 0,05 foram considerados estatisticamente significativos. Resultados: Das 1.206 crianças incluídas neste estudo, 608 (50,40%) eram do gênero feminino e 598 (49,60%) do masculino (p = 0,773). A idade média das crianças foi de 3,75 anos (desvio-padrão ± 3,78 anos). Das 1.206 crianças estudadas, seis (0,5%) apresentavam úvula bífida. A fissura palatina submucosa não foi encontrada em nenhuma criança. Quando as histórias familiares de crianças foram examinadas quanto à presença de fissura de lábio e/ou palato não sindrômica, nenhum parente de primeiro grau apresentava esta anomalia congênita. Conclusão: Este estudo revelou que a incidência de úvula bífida e fissura palatina submucosa nesta população é bastante semelhante às taxas de incidência previamente relatadas. Nosso estudo sugere uma intensificação de novas revisões, com populações mais amplas e diversas, buscando associar a ocorrência de úvula bífida, fissura palatina submucosa e fissura orais.

Incidence of bifid uvula and its relationship to submucous cleft palate and a family history of oral cleft in the Brazilian population.

INTRODUCTION: Bifid uvula is a frequently observed anomaly in the general population and can be regarded as a marker for submucous cleft palate. OBJECTIVE: In this study aimed to determine the frequency of bifid uvula and submucous cleft palate and their relationship with oral clefts in a Brazilian population. METHODS: We conducted a transversal, descriptive and quantitative study of 1206 children between August 2014 and December 2015. A clinical examination of the children was conducted by means of inspection of the oral cavity with the aid of a tongue depressor and directed light. After the clinical examination in children, parents answered a questionnaire with questions about basic demographic information and their family history of oral clefts in their first-degree relatives. After application of the questionnaires, the information collected was archived in a database and analyzed by the statistical program SPSS® version 19.0, by applying Chi-Square tests. Values with p<0.05 were considered statistically significant. RESULTS: Of the 1206 children included in this study, 608 (50.40%) were female and 598 (49.60%) were male (p=0.773). The average age of children was 3.75 years (standard deviation±3.78 years). Of the 1206 children studied, 6 (0.5%) presented with bifid uvula. Submucosal cleft palate was not found in any child. When the family histories of children were examined for the presence of nonsyndromic cleft lip and/or cleft palate, no first degree relatives presented with the congenital anomaly. CONCLUSION: This study revealed that the incidence of bifid uvula and submucous cleft palate in this population was quite similar to previously reported incidence rates. Our study suggests an intensification of new reviews, with broader and diverse populations, seeking to associate the occurrence of bifid uvula, submucous cleft palate and oral clefts.

Molecular basis of cleft palates in mice.

Cleft palate, including complete or incomplete cleft palates, soft palate clefts, and submucosal cleft palates, is the most frequent congenital craniofacial anomaly in humans. Multifactorial conditions, including genetic and environmental factors, induce the formation of cleft palates. The process of palatogenesis is temporospatially regulated by transcription factors, growth factors, extracellular matrix proteins, and membranous molecules; a single ablation of these molecules can result in a cleft palate in vivo. Studies on knockout mice were reviewed in order to identify genetic errors that lead to cleft palates. In this review, we systematically describe these mutant mice and discuss the molecular mechanisms of palatogenesis.

Powered instrumentation for transnasal endoscopic partial adenoidectomy in children with submucosal cleft palate.

OBJECTIVES: Partial adenoidectomy is the selective removal of the obstructing part of adenoid tissue, thus relieves obstruction symptoms and preserves the velopharyngeal valve action. Patients with palatal dysfunction are candidates for the technique. This study describes the use of microdebrider, transnasally (guided by the nasal endoscope) to perform partial adenoidectomy in patients with submucosal cleft palate, who presented with adenoidal hypertrophy and also it discusses its effects on nasal obstruction and speech. SUBJECTS AND METHODS: This prospective study was carried out on twenty-three submucosal cleft palate patients who were referred to the ORL-HN department; Zagazig University Hospitals complaining of respiratory obstruction and sleep disturbances due to adenoids hypertrophy. After preoperative nasoendoscopic and speech evaluation, transnasal endoscopic, power-assisted partial adenoidectomy was done for all patients. All patients were followed up at regular visits including nasoendoscopy and speech evaluation. RESULTS: The procedure insured fast, safe, reliable, under vision and well controlled steps. Intraoperatively no major complications were recorded. During follow up, nasal obstruction and respiratory obstruction symptoms were improved. Speech outcome results were reported. CONCLUSION: The study demonstrates the feasibility of using the microdebrider for performing transnasal partial adenoidectomy (under endoscopic guidance). The procedure is precise, rapid, safe and well-tolerated with the advantage of direct visualization of a traditionally difficult-to-expose area. The study reported improvement of respiratory obstruction symptoms with good speech results.

Spectral characteristics of resonance disorders in submucosal type cleft palate patients