• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Proceedings of The Korean Cleft Lip And Palate Association
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• 1998.06a
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• pp.22-22
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• 1998

• Archives of Plastic Surgery
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• v.36 no.3
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• pp.333-335
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• 2009

Congenital palatal fistulas are rare, and few cases have been reported. Most reported cases present with a submucous cleft palate. In terms of etiology, whether the fistula is congenital or acquired has been debated. Moreover, there is not a generally accepted surgical procedure for repair of palatal fistulas. We present a case of a congenital palatal fistula with a submucous cleft palate that was successfully treated with a Furlow double - opposing Z - plasty. We discuss palatal fistulas with a review of the literature.

• Archives of Craniofacial Surgery
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• v.23 no.2
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• pp.53-58
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• 2022

Alveolar cleft belongs to the spectrum of cleft lip and/or palate, affecting 75% of cleft lip/palate patients. The goals of alveolar cleft treatment are stabilizing the maxillary arch, separating the nasal and oral cavities, and providing bony support for both erupting teeth and the nasal base via the piriform aperture. Secondary alveolar bone grafting is a well-established treatment option for alveolar cleft. Secondary alveolar bone grafting is performed during the period of mixed dentition using autologous bone from various donor sites. There are several issues relevant to maximizing the success of secondary alveolar bone grafting, including the surgical timing, graft material, and surgical technique. In this study, we reviewed issues related to surgical timing, graft materials, and evaluation methods in secondary alveolar bone grafting.

• Archives of Craniofacial Surgery
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• v.25 no.2
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• pp.51-61
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• 2024

Background: The influence of smoking on nonsyndromic clefts has been a topic of research for many years. However, few studies have investigated the effect of smoking on causing clefts in different gene pools. Methods: A meta-analysis was conducted of case-control studies related to smoking. Keywords such as "clefts," "cleft lip," "cleft palate," "orofacial cleft," and "smoking" were used to search the MEDLINE, Embase, and Cochrane databases. Results: In total, 51 articles were reviewed. The RevMan software was utilized for the analysis, and the Mantel-Haenszel method was employed to pool the odds ratios (ORs) and 95% confidence intervals. Although the overall OR, a measure of the association between exposure and outcome, was higher for smokers than for non-smokers, this association was significantly stronger in individuals from Asia and South America (1.73), and lowest in Europe (1.31). Among active and passive smokers in Asia, the OR was approximately 0.93, indicating an equivalent impact from both types of smoking. Conclusion: This analysis indirectly suggests that restriction measures targeting both active and passive smoking are crucial in Asia.

• Genomics & Informatics
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• v.5 no.2
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• pp.56-60
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• 2007

Nonsyndromic cleft lip with or without cleft palate (CL/P) is a common craniofacial birth defect that is the result of a mixture of genetic and environmental factors. While studies have identified a number of different candidate genes and loci for the etiology of CL/P, the results have not been consistent among different ethnic groups. To study the genetic association of the candidate genes in Korean patients affected by CL/P, we genotyped 97 nonsyndromic CL/P patients and 100 control individuals using single nucleotide polymorphic markers at the MTHFR, TGFA, and IRF6 genes. We report that the T3827C marker at TGFA showed significant association with nonsyndromic CL/P, but all the other markers tested were not significantly associated with nonsyndromic CL/P in Korean patients.

• The Journal of the Korean dental association
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• v.55 no.12
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• pp.870-882
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• 2017

Maxillary growth is hindered by the restricting pressure from the scar tissue formed after lip closure and palate closure surgeries of the cleft. Therefore, the anteroposterior skeletal relationship of both jaws exacerbates as patient grows. Conventional facemask treatment is valuable for dentoalveolar compensatory treatment and for very mild maxillary hypoplasia. To achieve further maxillary protraction, bone-anchored facemask or bone-anchored maxillary protraction can be attempted. For moderate maxillary hypoplasia, surgical orthodontic treatment after growth completion can be an efficient treatment reducing uncontrollable problems. For moderate to severe maxillary hypoplasia, distraction osteogenesis (DO) can be used alone or with later surgical orthodontic treatment. To compensate the severe relapse after DO, overcorrection and bone plate placement after DO are recommended. In case of hypernasality, maxillary anterior segmental distraction osteogenesis can be chosen to prevent exacerbation of the hypernasality.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.43 no.4
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• pp.267-271
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• 2017

Although it is a rare developmental malformation, van der Woude syndrome is the most common form of syndromic orofacial clefting, accounting for approximately 2% of all cleft cases. The lower lip pits with or without a cleft lip or palate is characteristic of the syndrome. Findings, such as hypodontia, limb deformities, popliteal webs, ankylogossia, ankyloblepheron, and genitourinary and cardiovascular abnormalities, are rarely associated with the syndrome. This paper reports a rare case of van der Woude syndrome in a 10-year-old male patient with a single median lower lip pit and a repaired bilateral cleft lip and cleft palate that were associated with microstomia, hypodontia, and clubbing of the left foot with syndactyly of the second to fifth lesser toes of the same foot.

• The Journal of the Korean dental association
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• v.57 no.2
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• pp.93-99
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• 2019

Ectodermal dysplasia is a genetic disorder in which various clinical manifestations involve two or more of the differentiated tissues of the ectoderm. Facial deformity, which is frequently associated with ectodermal dysplasia, appears in the form of cleft lip or cleft palate, especially in the middle facial area.Cleft and tooth defects result in decreased alveolar bone development.This leads to severe skeletal incongruity. Facial features include frontal protrusion, malar bone hypoplasia, flat nose, mandibular prominence and long lower facial height. This clinical report presents treatment including orthognathic surgery of a patient with Hypohidrotic Ectodermal dysplasia with cleft palate.

• Korean Journal of Cleft Lip And Palate
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• v.12 no.1
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• pp.7-20
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• 2009

Orthodontic treatment planning of cleft lip and palate requires consideration of the characteristic features, growth pattern and functional disorders related to cleft lip and palate patients. Tissue deficiencies and constriction of the scar tissue in surgically treated cleft lip and palate results in disturbance of maxillary growth and deficiency of midfacial region with anterior and posterior crossbite. These patients often present congenital missing of teeth, supernumerary teeth, malformed teeth, or ectopic position of teeth, which should be treated by orthodontic treatment by expanding upper arch followed by fixed appliance. Proper use of retainer and continuous follow-up is needed to prevent relapse after orthodontic treatment has finished. Also we have to pay attention to correct speech disorder which is caused by the velopharyngeal insufficiency.