Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_31_21
S. Rout, Ritesh Panda, Mainak Mallik
Background: Suture removal in infants and children operated for cleft lip is one of the cumbersome task for the surgeons because of missing compliance. For this reason, tissue adhesives have gained popularity among the surgeons as well as the patients over the past two decades for skin wound closure. Although there are several published reports of case series using tissue adhesive for skin closure during cleft lip repair, very few have established its advantage over conventional suture technique. Objective: The objective of this study was to compare the esthetic outcome of skin closure in cleft lip repair with suture technique and tissue adhesive, with respect to the quality of scar. Methodology: A retrospective observational study was conducted by analyzing the photographic records of patients who underwent surgical repair of cleft lip between January 2015 and December 2017. The patients were divided into two groups, one for whom skin closure performed with 5-0 monofilament polyamide suture and the other with tissue adhesive. Esthetic outcome in terms of the quality of scar was assessed using a 5 point Likert's scale by 10 independent observers who remained blind of the surgeon performed the surgery, to reduce the assessor-related bias to minimum. The statistical analysis was performed using the SPSS version 27.0 and the difference between mean scores of both the groups was calculated. Results: A total of 70 patients were included in this study with 35 in each group. The mean follow-up period of adhesive and the suture group was 13.97 and 17.85 months, respectively. Overall mean of the total scores of the entire patient population was 3.92 (+/‒0.38). For the tissue adhesive group, the mean score was 3.88 (+/‒0.367) and for the suture group, the mean score was 3.96 (+/‒0.401). The difference between mean scores of individual parameters of both the groups as well as the difference between the overall mean scores of both the groups was not found to be statistically significant. Conclusion: The use of tissue adhesive for the closure of skin during cleft lip repair is as good as sutures, with respect to esthetic outcome of scar. Hence, any of these two techniques can be used for this purpose depending on the surgeon's preference.
{"title":"Esthetic outcome of cleft lip repair with the use of tissue adhesive as opposed to suture for skin closure – A retrospective comparative study","authors":"S. Rout, Ritesh Panda, Mainak Mallik","doi":"10.4103/jclpca.jclpca_31_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_31_21","url":null,"abstract":"Background: Suture removal in infants and children operated for cleft lip is one of the cumbersome task for the surgeons because of missing compliance. For this reason, tissue adhesives have gained popularity among the surgeons as well as the patients over the past two decades for skin wound closure. Although there are several published reports of case series using tissue adhesive for skin closure during cleft lip repair, very few have established its advantage over conventional suture technique. Objective: The objective of this study was to compare the esthetic outcome of skin closure in cleft lip repair with suture technique and tissue adhesive, with respect to the quality of scar. Methodology: A retrospective observational study was conducted by analyzing the photographic records of patients who underwent surgical repair of cleft lip between January 2015 and December 2017. The patients were divided into two groups, one for whom skin closure performed with 5-0 monofilament polyamide suture and the other with tissue adhesive. Esthetic outcome in terms of the quality of scar was assessed using a 5 point Likert's scale by 10 independent observers who remained blind of the surgeon performed the surgery, to reduce the assessor-related bias to minimum. The statistical analysis was performed using the SPSS version 27.0 and the difference between mean scores of both the groups was calculated. Results: A total of 70 patients were included in this study with 35 in each group. The mean follow-up period of adhesive and the suture group was 13.97 and 17.85 months, respectively. Overall mean of the total scores of the entire patient population was 3.92 (+/‒0.38). For the tissue adhesive group, the mean score was 3.88 (+/‒0.367) and for the suture group, the mean score was 3.96 (+/‒0.401). The difference between mean scores of individual parameters of both the groups as well as the difference between the overall mean scores of both the groups was not found to be statistically significant. Conclusion: The use of tissue adhesive for the closure of skin during cleft lip repair is as good as sutures, with respect to esthetic outcome of scar. Hence, any of these two techniques can be used for this purpose depending on the surgeon's preference.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"41 - 48"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45284617","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_37_20
V. Prasad, Priyawati Moungkhom, Ashutosh Kumar Singh, B. Mishra, D. Upadhyay
Objective: The objective of this study is to evaluate cleft patients, both unilateral and bilateral clefts and compare them with Skeletal base Class I patients for symmetry using orthopantomogram. Materials and Methods: Three groups of patients within age range of 15–25 years: Group I – Normal participants (mean age = 18.33 ± 2.84); Group II – Unilateral cleft lip and cleft palate participants (mean age = 18.74 ± 3.36); and Group III – Bilateral cleft lip and cleft palate participants (mean age = 17.64 ± 3.10) were assessed and compared to each other. Orthopantomogram was analyzed for vertical symmetry. Habets asymmetry index and the Kjellgren's asymmetry index were applied to assess the relative intensity of asymmetry. Results: Significant difference was seen in condylar height (P = 0.029), ramal height (P = 0.046), mandibular body length (P = 0.014), gonial angle (P = 0.035) and condylion–gonian distance (P = 0.044). Kjellberg's symmetry index/condylar ratio was maximally affected by the groups when compared with the Habets asymmetry index. Conclusion: Mandibular asymmetry has been expressed in unilateral cleft lip and palate (CLP) group. Normal group and bilateral CLP group indicated a symmetrical posterior vertical height of the mandible.
{"title":"Assessment of mandibular symmetry in cleft lip and cleft palate patients","authors":"V. Prasad, Priyawati Moungkhom, Ashutosh Kumar Singh, B. Mishra, D. Upadhyay","doi":"10.4103/jclpca.jclpca_37_20","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_37_20","url":null,"abstract":"Objective: The objective of this study is to evaluate cleft patients, both unilateral and bilateral clefts and compare them with Skeletal base Class I patients for symmetry using orthopantomogram. Materials and Methods: Three groups of patients within age range of 15–25 years: Group I – Normal participants (mean age = 18.33 ± 2.84); Group II – Unilateral cleft lip and cleft palate participants (mean age = 18.74 ± 3.36); and Group III – Bilateral cleft lip and cleft palate participants (mean age = 17.64 ± 3.10) were assessed and compared to each other. Orthopantomogram was analyzed for vertical symmetry. Habets asymmetry index and the Kjellgren's asymmetry index were applied to assess the relative intensity of asymmetry. Results: Significant difference was seen in condylar height (P = 0.029), ramal height (P = 0.046), mandibular body length (P = 0.014), gonial angle (P = 0.035) and condylion–gonian distance (P = 0.044). Kjellberg's symmetry index/condylar ratio was maximally affected by the groups when compared with the Habets asymmetry index. Conclusion: Mandibular asymmetry has been expressed in unilateral cleft lip and palate (CLP) group. Normal group and bilateral CLP group indicated a symmetrical posterior vertical height of the mandible.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"49 - 54"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47852780","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_35_21
Helen Livesey, U. Iroegbu, M. Balasubramanian
Orofacial clefts are common birth defects and Van der Woude syndrome (VWS) is the most common form of orofacial cleft syndrome, accounting for approximately 2% of patients with a cleft.[1],[2] The cardinal features of VWS are lower lip pits associated with cleft lip and/or palate.[2],[3],[4] Lip pits are reported to occur in over 80% of individuals with VWS.[2] Most reported cases of VWS have been linked to chromosome 1q32-q41.[5],[6],[7] The interferon regulatory factor 6 (IRF6) gene, which is located at 1q32-p41 region, has been implicated in several studies.[2],[8] There are over 300 IRF6 variants that have been identified in patients with VWS, with approximately 50% of these being missense variants.[9] This paper describes a 7.5-year-old male patient that is heterozygous for a missense variant in C.101A >C p.(Lys34Thr) which is likely to be pathogenic.[9] This patient has the cardinal features for VWS but also has duodenal atresia. Neither the truncating variant identified in this patient nor other variants associated with VWS have been previously linked to duodenal atresia.
{"title":"Van der Woude syndrome: Presentation of child with duodenal atresia with an interferon regulatory factor 6 variant","authors":"Helen Livesey, U. Iroegbu, M. Balasubramanian","doi":"10.4103/jclpca.jclpca_35_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_35_21","url":null,"abstract":"Orofacial clefts are common birth defects and Van der Woude syndrome (VWS) is the most common form of orofacial cleft syndrome, accounting for approximately 2% of patients with a cleft.[1],[2] The cardinal features of VWS are lower lip pits associated with cleft lip and/or palate.[2],[3],[4] Lip pits are reported to occur in over 80% of individuals with VWS.[2] Most reported cases of VWS have been linked to chromosome 1q32-q41.[5],[6],[7] The interferon regulatory factor 6 (IRF6) gene, which is located at 1q32-p41 region, has been implicated in several studies.[2],[8] There are over 300 IRF6 variants that have been identified in patients with VWS, with approximately 50% of these being missense variants.[9] This paper describes a 7.5-year-old male patient that is heterozygous for a missense variant in C.101A >C p.(Lys34Thr) which is likely to be pathogenic.[9] This patient has the cardinal features for VWS but also has duodenal atresia. Neither the truncating variant identified in this patient nor other variants associated with VWS have been previously linked to duodenal atresia.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"85 - 87"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49486566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_47_20
M. Sahu, Monal Karkar, Vaibhav Subramaniam, G. Reddy
Nasal deformities can vary in the form of severity. Several different factors such as congenital, burn and traumatic nasal fractures or soft-tissue injury/loss, neoplastic: defects resulting from surgery for the treatment of cancers causing compromise the structural architecture and functional framework of the nose. This case report describes the prosthetic rehabilitation of two female patients, one who was reported with congenital arrhinia and another one who had accidental electrical burns of the face. The surgeon's team as well as the maxillofacial prosthetics team discussed the treatment options with the parents and decided to rehabilitate with a nasal prosthesis. This clinical report is imparting an accessible and economic method for prosthetic rehabilitation of a patient.
{"title":"Nasal deformity correction with nasal prosthesis","authors":"M. Sahu, Monal Karkar, Vaibhav Subramaniam, G. Reddy","doi":"10.4103/jclpca.jclpca_47_20","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_47_20","url":null,"abstract":"Nasal deformities can vary in the form of severity. Several different factors such as congenital, burn and traumatic nasal fractures or soft-tissue injury/loss, neoplastic: defects resulting from surgery for the treatment of cancers causing compromise the structural architecture and functional framework of the nose. This case report describes the prosthetic rehabilitation of two female patients, one who was reported with congenital arrhinia and another one who had accidental electrical burns of the face. The surgeon's team as well as the maxillofacial prosthetics team discussed the treatment options with the parents and decided to rehabilitate with a nasal prosthesis. This clinical report is imparting an accessible and economic method for prosthetic rehabilitation of a patient.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"88 - 91"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45579598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_29_21
M. Jaisani, A. Dongol, P. Acharya, A. Yadav, A. Sagtani, L. Pradhan, P. Jaisani, S. Laverick
Introduction: There are only a few centers providing care to orofacial cleft patients across Nepal. However, no adequate data have been reported regarding the epidemiology, pattern, and association of cleft in children from eastern Nepal. The aim of the study was to study the epidemiological profile of cleft lip palate (CLP) patients reporting to the department of oral and maxillofacial surgery (OMFS) in Dharan, Eastern Nepal. Materials and Methods: Data retrospectively retrieved from the cleft registry in the department of OMFS were tabulated and entered into Microsoft Excel 2000. Data of all the diagnosed nonsyndromic orofacial cleft patients attending BP Koirala Institute of Health Sciences for treatment from August 2011 to August 2017 were used for the analysis. Incompletely filled records and syndromic cases were excluded. Results: Data of 220 orofacial clefts were analyzed which included 119 males and 101 females. Among these children with cleft, only 7 patients had direct family members and 18 patients had distant family members having the same condition. Among patients with CL (n = 151), left-sided CL (n = 85) was a more common presentation. Amongst patients with a cleft of the hard plate (n = 153), bilateral (n = 87) presentation was more common. A similar pattern was observed for cleft alveolus (n = 117) with the left side (n = 60) being common. In clefts affecting the soft palate (n = 192), complete cleft (n = 189) was a more common occurrence than incomplete. The majority of our patients had a CLP (n = 133) with isolated CL being the least commonly seen (n = 18). Conclusion: The majority of our patients had a CLP with isolated CL being the least common. This observation is in concordance with reports from other Caucasian and Asian populations both from developed and developing countries.
{"title":"Oro-facial clefts at a tertiary level hospital of eastern Nepal: A retrospective insight","authors":"M. Jaisani, A. Dongol, P. Acharya, A. Yadav, A. Sagtani, L. Pradhan, P. Jaisani, S. Laverick","doi":"10.4103/jclpca.jclpca_29_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_29_21","url":null,"abstract":"Introduction: There are only a few centers providing care to orofacial cleft patients across Nepal. However, no adequate data have been reported regarding the epidemiology, pattern, and association of cleft in children from eastern Nepal. The aim of the study was to study the epidemiological profile of cleft lip palate (CLP) patients reporting to the department of oral and maxillofacial surgery (OMFS) in Dharan, Eastern Nepal. Materials and Methods: Data retrospectively retrieved from the cleft registry in the department of OMFS were tabulated and entered into Microsoft Excel 2000. Data of all the diagnosed nonsyndromic orofacial cleft patients attending BP Koirala Institute of Health Sciences for treatment from August 2011 to August 2017 were used for the analysis. Incompletely filled records and syndromic cases were excluded. Results: Data of 220 orofacial clefts were analyzed which included 119 males and 101 females. Among these children with cleft, only 7 patients had direct family members and 18 patients had distant family members having the same condition. Among patients with CL (n = 151), left-sided CL (n = 85) was a more common presentation. Amongst patients with a cleft of the hard plate (n = 153), bilateral (n = 87) presentation was more common. A similar pattern was observed for cleft alveolus (n = 117) with the left side (n = 60) being common. In clefts affecting the soft palate (n = 192), complete cleft (n = 189) was a more common occurrence than incomplete. The majority of our patients had a CLP (n = 133) with isolated CL being the least commonly seen (n = 18). Conclusion: The majority of our patients had a CLP with isolated CL being the least common. This observation is in concordance with reports from other Caucasian and Asian populations both from developed and developing countries.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"30 - 35"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46946399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_28_21
V. Deraje, A. Tandon
We report a case of a patient with premature fusion of bilateral squamous sutures and perform a literature review of similar cases. We have noted in our case and in other reports that bilateral squamous synostosis does not lead to significant clinical effects such as deformed shape of the skull or raised intracranial pressure.
{"title":"The significance or insignificance of prematurely fused bilateral squamosal sutures","authors":"V. Deraje, A. Tandon","doi":"10.4103/jclpca.jclpca_28_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_28_21","url":null,"abstract":"We report a case of a patient with premature fusion of bilateral squamous sutures and perform a literature review of similar cases. We have noted in our case and in other reports that bilateral squamous synostosis does not lead to significant clinical effects such as deformed shape of the skull or raised intracranial pressure.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"82 - 84"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49184218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_25_21
N. Gupta, K. Nagar, P. Dixit, Tanmay Tiwari, Vinod K Srivastava, P. Singh
Over the last few decades, there is a paramount shift in the implementation of pediatric anesthesia. Although for most of the time, difficult airway scenarios are well-planned and executed, occasionally an unanticipated difficult situation may still arise. This is more common in patients with orofacial anomalies such as cleft lip and palate which constitute one of the most common craniofacial anomalies. Various approaches to difficult airway management in an infant undergoing cleft repair have been described in the literature. However, no approach is considered ideal. It depends on age, associated anomalies, at hand resources, and the skill of the anesthesiologist. The unique anatomical and physiological features of pediatric airways impose additional challenges. The evolution of advanced airway modalities such as video laryngoscopes (VLs) and fiber-optic bronchoscopes had a major impact on the practice of anesthesia and the management of difficult airway scenarios in children and neonates. This article focuses on airway assessment and various current techniques and equipment used in airway management of cleft anomalies. Pediatric airway devices such as supraglottic airways, VLs, and fiber-optic bronchoscopes are briefly discussed with the benefits and limitations of each device. Recent studies describe specific problems as well as advantages with the usage of these devices.
{"title":"Airway consideration in cleft patients-challenges and approaches","authors":"N. Gupta, K. Nagar, P. Dixit, Tanmay Tiwari, Vinod K Srivastava, P. Singh","doi":"10.4103/jclpca.jclpca_25_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_25_21","url":null,"abstract":"Over the last few decades, there is a paramount shift in the implementation of pediatric anesthesia. Although for most of the time, difficult airway scenarios are well-planned and executed, occasionally an unanticipated difficult situation may still arise. This is more common in patients with orofacial anomalies such as cleft lip and palate which constitute one of the most common craniofacial anomalies. Various approaches to difficult airway management in an infant undergoing cleft repair have been described in the literature. However, no approach is considered ideal. It depends on age, associated anomalies, at hand resources, and the skill of the anesthesiologist. The unique anatomical and physiological features of pediatric airways impose additional challenges. The evolution of advanced airway modalities such as video laryngoscopes (VLs) and fiber-optic bronchoscopes had a major impact on the practice of anesthesia and the management of difficult airway scenarios in children and neonates. This article focuses on airway assessment and various current techniques and equipment used in airway management of cleft anomalies. Pediatric airway devices such as supraglottic airways, VLs, and fiber-optic bronchoscopes are briefly discussed with the benefits and limitations of each device. Recent studies describe specific problems as well as advantages with the usage of these devices.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"55 - 59"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43052099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_9_21
R. Mahajan, Sheikh Ali, Sana Jameel, Samik Sharma
Van der Woude syndrome is a rare congenital condition with autosomal dominant traits. This autosomal dominant syndrome had been reported to have a penetrance of 80% with variable expression.[1] This genetic disorder is characterized by the combination of lip pits, cleft lip with or without cleft palate, and cleft palate only. Even within the families, there is a wide variability of affected gene expressions. It is very important to establish the correct diagnosis so that genetic counseling can be recommended in such cases. Here, we report cases of Van der Woude syndrome in the same family having the same genetic inheritance with variable expression.
Van der Woude综合征是一种罕见的先天性疾病，具有常染色体显性特征。据报道，这种常染色体显性综合征的外显率为80%，表达可变这种遗传疾病的特征是唇窝，唇裂伴或不伴腭裂，以及只有腭裂。即使在家族内部，受影响的基因表达也存在很大的差异。建立正确的诊断是非常重要的，这样在这种情况下可以推荐遗传咨询。在这里，我们报告了同一家族中具有相同基因遗传但表达不同的范德沃德综合征病例。
{"title":"Variable expression of Van der Woude syndrome in the same family","authors":"R. Mahajan, Sheikh Ali, Sana Jameel, Samik Sharma","doi":"10.4103/jclpca.jclpca_9_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_9_21","url":null,"abstract":"Van der Woude syndrome is a rare congenital condition with autosomal dominant traits. This autosomal dominant syndrome had been reported to have a penetrance of 80% with variable expression.[1] This genetic disorder is characterized by the combination of lip pits, cleft lip with or without cleft palate, and cleft palate only. Even within the families, there is a wide variability of affected gene expressions. It is very important to establish the correct diagnosis so that genetic counseling can be recommended in such cases. Here, we report cases of Van der Woude syndrome in the same family having the same genetic inheritance with variable expression.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"74 - 77"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46528022","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.4103/jclpca.jclpca_24_21
Navneet Singh, Neha Bhutiani, T. Tripathi, P. Rai
Restoration of missing oral tissues in patients with cleft lip and palate is the biggest challenge in their rehabilitation. In this report, we described the postdistraction rehabilitation and 1-year follow-up of the regenerated bone by implant-supported prosthesis and orthodontic tooth movement. A novel distraction appliance regenerated natural bone which is compatible for both implant placement and orthodontic tooth movement, thereby restoring adequate function and esthetics.
{"title":"Rehabilitation using dental implants following closure of large alveolar cleft defect by HYDIS-TB appliance: One-year follow-up","authors":"Navneet Singh, Neha Bhutiani, T. Tripathi, P. Rai","doi":"10.4103/jclpca.jclpca_24_21","DOIUrl":"https://doi.org/10.4103/jclpca.jclpca_24_21","url":null,"abstract":"Restoration of missing oral tissues in patients with cleft lip and palate is the biggest challenge in their rehabilitation. In this report, we described the postdistraction rehabilitation and 1-year follow-up of the regenerated bone by implant-supported prosthesis and orthodontic tooth movement. A novel distraction appliance regenerated natural bone which is compatible for both implant placement and orthodontic tooth movement, thereby restoring adequate function and esthetics.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"9 1","pages":"78 - 81"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49157644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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