The Prevalence of Apraxia Characteristics in Patients with Velocardiofacial Syndrome as Compared with Other Cleft Populations

2007 ◽  
Vol 44 (2) ◽  
pp. 175-181 ◽  
Author(s):  
Ann W. Kummer ◽  
Linda Lee ◽  
Lindsay Schaadt Stutz ◽  
Adam Maroney ◽  
Jennifer Weidenbach Brandt

Objective: The purpose of this study was to assess the oral-motor function of children with a history of cleft lip and palate, cleft palate only, velocardiofacial syndrome, and children with normal oral structures to determine if children with velocardiofacial syndrome have more apraxia characteristics than the other populations have. Design: The Apraxia Profile (Hickman, 1997) was administered to all participants in a prospective study. Setting: The investigation was conducted at Cincinnati Children's Hospital Medical Center. Participants: In this study, 10 children with cleft lip and palate, 10 with cleft palate only, 7 with velocardiofacial syndrome, and 47 with normal structures were tested. Results: This study revealed that when compared with children with normal structures, children with cleft lip and palate did not demonstrate significant apraxia characteristics, children with cleft palate only demonstrated some apraxia characteristics, and children with velocardiofacial syndrome demonstrated the most apraxia characteristics. Conclusions: There appears to be a high prevalence of apraxia characteristics in the speech of patients with velocardiofacial syndrome. This information has implications for the type of treatment recommended. Further investigation is warranted with a larger patient population and a focus on the association of abnormal brain structure with apraxia in this population.

1992 ◽  
Vol 29 (5) ◽  
pp. 470-474 ◽  
Author(s):  
Bente Felix-Schollaart ◽  
Jan B. Hoeksma ◽  
Jean Paul Van De Velde ◽  
Jerôme I. Puyenbroek ◽  
Birte Prahl-Andersen

The reproductive history was studied to evaluate if the three types of solitary, nonsyndromic clefts: cleft lip (CL), cleft lip and palate (CLP), and cleft palate (CP) are homogeneous entities. Occurrence of fetal loss, maternal health, and drug consumption of the mother during the pregnancy were compared in cases involving three types of clefts. Data was gathered from 87 children with clefts, 55 males and 32 females. Spontaneous abortions and vaginal bleeding were found to occur significantly more often in the (older) mother of a CLP child. This suggests that the factors involved in the etiology of CLP differ from the factors involved in CL and CP. Therefore, grouping of data of the three types of clefts in studies on the etiology should be avoided.


2003 ◽  
Vol 40 (2) ◽  
pp. 176-179 ◽  
Author(s):  
Orit Reish ◽  
Yehuda Finkelstein ◽  
Ronit Mesterman ◽  
Ariela Nachmani ◽  
Baruch Wolach ◽  
...  

Objective Velocardiofacial syndrome (VCFS) is the most common multiple anomaly disorder associated with palatal clefting. Cytogenetic hemizygous deletion of 22q11 region is found in 80% of patients. The frequency of 22q11 deletion in patients presenting with isolated palatal anomalies has not been fully assessed. Our objective was to determine the frequency of the deletion in patients with isolated palatal anomalies. Design Patients were referred because of velopharyngeal insufficiency because of isolated congenital palatal anomalies. Diagnosis of palatal anomalies was confirmed by videonasopharyngoscopy, multiview videofluoroscopy and cephalometry. Other clinical findings suggestive of VCFS were sought, and subjects with these characteristics were excluded from the study. Peripheral blood samples from all patients were analyzed cytogenetically utilizing fluorescent in situ hybridization for the 22q11 region. Results Thirty-eight patients aged 3 to 31 years were included in the study. Nine had cleft palate, 7 cleft lip and palate, 10 overt and 11 occult submucous cleft palate, and 1 had a deep nasopharynx. No deletion of 22q11 region was detected in any of the evaluated patients. Conclusions A routine screening for the 22q11 deletion in older children and adults presenting with an isolated palatal anomaly may not be required. Because other signs related to VCFS such as facial dysmorphism and behavioral or psychiatric disorders may evolve at an older age, young patients should be followed up and reevaluated for additional relevant symptoms that may lead to deletion evaluation. In light of the fact that the current literature is inconsistent, the relative small size of this study and the significant consequences of missed 22q11.2 deletion, more information is needed before definitive recommendations can be made.


2017 ◽  
Vol 157 (4) ◽  
pp. 676-682 ◽  
Author(s):  
Terence E. Imbery ◽  
Lindsay B. Sobin ◽  
Emily Commesso ◽  
Lindsey Koester ◽  
Sherard A. Tatum ◽  
...  

Objective Describe longitudinal audiometric and otologic outcomes in patients with cleft palates. Study Design Case series with chart review. Setting Single academic medical center. Methods Charts of 564 patients with a diagnosis of cleft palate (59% syndromic etiology, 41% nonsyndromic) from 1998 to 2014 were reviewed. Patients without at least 1 audiometric follow-up were excluded from analysis. Patient demographics, surgeries, audiometric tests, and otologic data were recorded for 352 patients. Results Forty-five percent had isolated cleft palates, 34% had unilateral cleft lip and palate, and 21% had bilateral cleft lip and palate. Patients were followed for a mean of 50.3 months with a mean of 3.2 separate audiograms performed. Patients received a mean of 2.93 pressure equalization tubes. Increased number of pressure equalization tubes was not associated with incidence of cholesteatoma, which was identified in only 4 patients. Nine patients underwent eventual tympanoplasty with an 89% closure rate. Analysis of mean air-bone gap by cleft type did not reveal significant differences ( P = .08), but conductive losses and abnormal tympanometry persisted into teenage years. Conclusions Patients with cleft palates have eustachian tube dysfunction, which, in our cohort, resulted in persistent conductive hearing loss, highlighting the importance of long-term follow-up. Cholesteatoma incidence was low and not associated with number of tubes, which at our institution were placed prophylactically. Tympanoplasty was successful in those with persistent perforations.


2006 ◽  
Vol 43 (6) ◽  
pp. 665-672 ◽  
Author(s):  
Catharina A. M. Bongaarts ◽  
Martin A. van't Hof ◽  
Birte Prahl-Andersen ◽  
Iris V. Dirks ◽  
Anne M. Kuijpers-Jagtman

Objective: Evaluation of the effect of infant orthopedics on maxillary arch dimensions in the deciduous dentition in patients with unilateral cleft lip and palate. Design: Prospective two-arm randomized controlled clinical trial with three participating cleft palate centers. Setting: Cleft palate centers of the Radboud University Nijmegen Medical Center, Academic Center of Dentistry Amsterdam, and University Medical Center Rotterdam, the Netherlands. Patients: Children with complete unilateral cleft lip and palate (n = 54) were included. Interventions: Patients were randomly divided into two groups. Half of the patients (IO+) had a presurgical orthopedic plate until surgical closure of the soft palate at the age of 52 weeks; the other half (IO−) did not undergo presurgical orthopedics. Mean outcome measures: Maxillary arch dimensions were assessed on dental casts at 4 and 6 years of age with measurements for arch width, arch depth, arch length, arch form, and the vertical position of the lesser segment. Contact and collapse were assessed also. Results: There were no clinically significant differences found between IO+ and IO− for any of the variables. Conclusions: Infant orthopedics had no observable effect on the maxillary arch dimensions or on the contact and collapse scores in the deciduous dentition at the ages of 4 and 6 years. Considering the Dutchcleft results to date, there is no need to perform infant orthopedics for unilateral cleft lip and palate patients.


2006 ◽  
Vol 43 (6) ◽  
pp. 702-709 ◽  
Author(s):  
Julie Reid ◽  
Nicky Kilpatrick ◽  
Sheena Reilly

Objective: To examine the natural history of feeding skills in babies with clefts and identify risk factors and predictors of poor feeding. Participants: Sixty-two babies with clefts were examined at 2 weeks, 3 months, and 14 months of age. Main Outcome Measures: Feeding ability, oral motor function, and feeding efficiency were assessed. Univariate analyses were used to determine whether oral motor function and sequelae varied according to feeding ability or cleft condition. Multivariable logistic regressions were used to determine risk factors for poor feeding. Results: Poor feeding skills were detected in one third of newborns. The prevalence of poor feeding reduced to 19% at 3 months of age and 15% at 14 months of age. Oral motor dysfunction and sequelae (particularly nasal regurgitation) were more commonly observed in babies with poor feeding skills irrespective of comorbidity. The main risk factor for poor feeding was a diagnosis of syndrome or Pierre Robin sequence (PRS). At 2 weeks of age, babies with syndrome or PRS were 15 times more likely to have poor feeding skills than their nonsyndromic counterparts. When syndrome or PRS was controlled for, babies with cleft palate and cleft lip and palate were equally likely to have poor feeding skills. Parental report of feeding efficiency was predictive of poor feeding in young babies. Conclusions: Poor feeding skills are relatively common in newborns with cleft palate and cleft lip and palate. Treatment for feeding problems may be needed beyond the first year of life, especially for babies born with PRS or a syndrome.


2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
P. Agbenorku ◽  
M. Yore ◽  
K. A. Danso ◽  
C. Turpin

Background. Cleft lip and cleft palate are among the most common orofacial congenital anomalies. This study is to establish Orofacial Clefts Database for Kumasi, Ghana, with a view to extend it to other cities in future to obtain a national orofacial anomaly database. Methods. A descriptive prospective survey was carried out at eleven selected health facilities in Kumasi. Results. The total number of live births recorded was 27,449. Orofacial anomalies recorded were 36, giving an incidence of 1.31/1000 live births or 1 in 763 live births. The mean maternal age of cleft lip/palate babies was 29.85 years (range 18–40 years). The male : female ratio for the orofacial anomalies babies was 1.3 : 1; the male : female ratio was 0.5 : 1 in the cleft lip group, 1.3 : 1 in the cleft lip and palate group, and 4 : 1 in the cleft palate group. The majority of clefts were unilateral (69.4%, n=25), with females (n=14) outnumbering males (n=11). A family history of cleft was recorded with five babies (13.9%). Associated congenital anomalies were recorded in seven (19.4%) cleft lips and/or palates. Conclusion. The incidence of 1 in 763 live births found in this study indicates that cleft lip/palate is a common congenital anomaly in Kumasi.


2020 ◽  
Vol 11 (SPL3) ◽  
pp. 363-367
Author(s):  
Monisha K ◽  
Senthil Murugan P ◽  
Aravind Kumar

Cleft lip and palate (CLP) is one of the most prevalent malformations occurring in the head and neck region. Cleft lip and palate is the second most birth defect in the US after club foot. The incidence of Cleft lip and cleft palate is also very common in Indian Population with the rate of 1 in 700 births approximately. In India, the main reason for the formation of Cleft Lip and cleft palate is consanguineous marriage due to less awareness among people. Cleft lip can be unilateral or bilateral and may involve or palate. Again it can be further classified as Complete or Incomplete cleft lip and /or Cleft palate. Most of the patients were deprived of treatment, mainly due to their unawareness and their lower status. Cleft patients need comprehensive, cleft care management. So the aim of this study is to find the incidence of bilateral cleft lip or palate in patients who reported toSaveetha Dental College and Hospital, Chennai. This study is done with 76 patients40 males, 36 females)who visited a Saveetha Dental College during one year between June 2019-April 2020. All available data were extracted from patients case sheets and results were obtained through SPSS analysis. In this study, we observed that 90.5 % of patients reported with unilateral cleft lip and palate, where only 9.1% of patients reported with bilateral cases. Males were having high prevalence with 52.6 % and females 47.4%. conclusion, male patients had higher cleft lip and palate compared to females. The incidence of bilateral cases seen among cleft lip and palate is fewer in males.


2018 ◽  
Vol 55 (5) ◽  
pp. 747-752 ◽  
Author(s):  
Roderic M. F. Schwirtz ◽  
Frans J. Mulder ◽  
David G. M. Mosmuller ◽  
Robin A. Tan ◽  
Thomas J. Maal ◽  
...  

Objective: To determine if cropping facial images affects nasolabial aesthetics assessments in unilateral cleft lip patients and to evaluate the effect of facial attractiveness on nasolabial evaluation. Design: Two cleft surgeons and one cleft orthodontist assessed standardized frontal photographs 4 times; nasolabial aesthetics were rated on cropped and full-face images using the Cleft Aesthetic Rating Scale, and total facial attractiveness was rated on full-face images with and without the nasolabial area blurred using a 5-point Likert scale. Setting: Cleft Palate Craniofacial Unit of a University Medical Center. Patients: Inclusion criteria: nonsyndromic unilateral cleft lip and an available frontal view photograph around 10 years of age. Exclusion criteria: a history of facial trauma and an incomplete cleft. Eighty-one photographs were available for assessment. Main Outcome Measures: Differences in mean CARS scores between cropped versus full-face photographs and attractive versus unattractive rated patients were evaluated by paired t test. Results: Nasolabial aesthetics are scored more negatively on full-face photographs compared to cropped photographs, regardless of facial attractiveness. (Mean CARS score, nose: cropped = 2.8, full-face = 3.0, P < .001; lip: cropped = 2.4, full-face = 2.7, P < .001; nose and lip: cropped = 2.6, full-face = 2.8, P < .001). Conclusion: Aesthetic outcomes of the nasolabial area are assessed significantly more positively when using cropped images compared to full-face images. For this reason, cropping images, revealing the nasolabial area only, is recommended for aesthetical assessments.


2019 ◽  
Vol 4 (1) ◽  
pp. 621-624
Author(s):  
Sanjeev Kumar Thakur ◽  
Baleshwar Yadav ◽  
Raj Kumar Bedajit ◽  
Kailash Khaki Shrestha ◽  
Shankar Man Rai

Introduction: Cleft lip and palate is one of the most common congenital anomaly, which contributes to eustachian tube dysfunction leading to impaired middle ear ventilation and middle ear pathology. It has been stated that the timely repair of cleft palate reduces the incidence of otitis media with effusion. Objective: To describe the ear findings in cleft palate patients who have undergone repair of cleft palate. Methodology: A total of 36 (20 male and 16 female) post cleft repair patients underwent Otoscopy and Tympanometry between January to December 2018. Cases with isolated cleft lip, ears with perforation of tympanic membrane and attico-antral disease were excluded. Results: The age range was from 4 years to 24 years, with a mean of 11.34 years. Maximum patients were of the age group of 10- 20 years (18, 50%) followed by that of 5-10 years range (15, 41.66%). Total number of ears examined were 72. Out of a total of 72 ears, 70 (97.2%) had abnormal otoscopic finding with dull tympanic membrane in 37 (51.39%) ears, retracted in 31 (43.05%) and bulging tympanic membrane in 2 (2.78%) cases. Type B curve was the commonest (36, 50%), followed by Type As (24, 33.3%). Type C curve was found in 11(15.3%) ears. Type A curve was found only in one ear. The duration of post-repair of cleft palate ranged from 6 months to up to 16 years with a mean of 8.52; 11 cases(30.56%) had history of repair of cleft palate of more than 10 years duration.16 cases (44.44%) had that of more than 5 years and 9 cases (25%) had less than 5 years history of duration of repair. When correlation was done statistically, the tympanometry findings and duration of repair had a very low correlation (Table 5). The time period of repair had no significant effect on tympanometric ear findings. Conclusion Abnormal otoscopic findings and tympanometric findings were common in post cleft palate repaired patients. The duration of cleft palate repair had low correlation to the tympanometric ear findings.


2009 ◽  
Vol 46 (1) ◽  
pp. 24-29 ◽  
Author(s):  
Wei Zheng ◽  
James D. Smith ◽  
Bing Shi ◽  
Yu Li ◽  
Yan Wang ◽  
...  

Objective: To present the tympanometric findings in 552 patients (115 over 10 years of age) with unrepaired cleft palate (256 had audiologic findings) and to show the natural history and outcome of these cases. Setting: The cleft lip and palate clinic for the Division of Cleft Lip and Palate Surgery at the West China College of Stomatology, Sichuan University, Chengdu, People's Republic of China. Design: Pure-tone audiometric and tympanometric evaluations were performed on 552 patients with an unrepaired cleft palate. Results were analyzed by looking at the patient's age and cleft palate type. Results: This study demonstrated an age-related decrease in the frequency of hearing impairment and abnormal tympanometry. The frequency of hearing impairment and abnormal tympanometry in patients with submucous cleft palate was significantly lower than in patients from the other four major cleft palate categories (p  =  .001, p  =  .006, respectively). Conclusions: The middle ear function and hearing levels of unrepaired cleft palate patients improved with age, but at least 30% of the patients’ ears demonstrated a hearing loss and abnormal tympanometry in each age group, including those over 19 years of age. In the crucial language-learning stage, the frequency of hearing impairment and abnormal tympanometry was as high as 60%. Considering these results, palate repair and surgical intervention, such as tube insertion, for otological problems should be considered at an early age.


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