• Maxillofacial Plastic and Reconstructive Surgery
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• 제42권
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• pp.26.1-26.6
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• 2020

Background: Orofacial clefts (OFCs) comprise a wide range of malformations, including cleft lip, cleft palate, and cleft lip with cleft palate, which can vary in terms of etiology, severity, and disease burden. Objective(s): This study aimed to evaluate the correlation between various risk factors and orofacial cleft disorder spectrum in newborns. Study design: A total of 323 cases and 400 controls were enrolled in this study and evaluated in terms of the maternal history of abortion or miscarriage, child's sex, maternal and paternal age, maternal history of systemic disease, history of medication therapy during pregnancy, birth order, consanguineous marriage, and complications during pregnancy. Results: Analysis of the results suggested that consanguineous marriage, a maternal history of abortion/miscarriage, and complications during pregnancy could potentially increase the risk of OFCs in children (P < 0.05). However, the analyses revealed that the other variables could not potentially increase the risk of OFCs (P > 0.05). Conclusion(s): Multiple cofactors may simultaneously contribute to the formation of such abnormalities; therefore, a comprehensive, multidisciplinary care program is necessary to ensure a successful pregnancy period and the birth of a healthy newborn.

• 대한소아치과학회지
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• 제51권1호
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• pp.66-79
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• 2024

This study aimed to assess the prevalence and distribution pattern of dental anomalies in the permanent teeth of patients with non-syndromic cleft lip with or without cleft palate. Additionally, it aimed to compare differences in dental anomalies between cleft and non-cleft areas, considering gender and cleft phenotype. Panoramic radiographs of 164 patients diagnosed with non-syndromic orofacial clefts were retrospectively analyzed by a single examiner to confirm dental anomalies. The dental anomalies investigated included tooth agenesis, supernumerary teeth, microdontia, rotation, ectopic eruption, and enamel hypoplasia. Cleft phenotypes were categorized into 7 types based on medical and dental records. A significantly higher prevalence of supernumerary teeth was observed in males than females within non-cleft areas (p = 0.017), with no significant differences in other dental anomalies. In non-cleft area, patients with cleft palate exhibited a high prevalence of tooth agenesis (p < 0.0001) and microdontia (p = 0.012) compared to other cleft phenotypes. Maxillary incisor rotation was closely associated with adjacent tooth agenesis in unilateral cleft lip and palate cases (p = 0.034). This study suggests that the additional subphenotype based on dental anomalies in patients with orofacial cleft may serve as applicable clinical markers.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제43권4호
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• pp.247-255
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• 2017

Objectives: The aim of this study was to compare the quality of life (QoL) of parents/caregivers of children with cleft lip and/or palate before and after surgical repair of an orofacial cleft. Materials and Methods: Families of subjects who required either primary or secondary orofacial cleft repair who satisfied the inclusion criteria were recruited. A preoperative and postoperative health-related QoL questionnaire, the 'Impact on Family Scale' (IOFS), was applied in order to detect the subjectively perceived QoL in the affected family before and after surgical intervention. The mean pre- and postoperative total scores were compared using paired t-test. Pre- and postoperative mean scores were also compared across the 5 domains of the IOFS. Results: The proportion of families whose QoL was affected before surgery was 95.7%. The domains with the greatest impact preoperatively were the financial domain and social domains. Families having children with bilateral cleft lip showed QoL effects mostly in the social domain and 'impact on sibling' domain. Postoperatively, the mean total QoL score was significantly lower than the mean preoperative QoL score, indicating significant improvement in QoL (P<0.001). The mean postoperative QoL score was also significantly lower than the mean preoperative QoL score in all domains. Only 3.2% of the families reported affectation of their QoL after surgery. The domains of mastery (61.3%) with a mean of $7.4{\pm}1.8$ and finance (45.1%) with a mean score of $7.2{\pm}1.6$ were those showing the greatest postoperative impact. The proportion of families whose QoL was affected by orofacial cleft was markedly different after treatment (95.7% preoperative and 3.2% postoperative). Conclusion: Caring for children with orofacial clefts significantly reduces the QoL of parents/caregivers in all domains. However, surgical intervention significantly improves the QoL of the parents/caregivers of these children.

• Archives of Plastic Surgery
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• 제48권6호
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• pp.630-634
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• 2021

To date, there have been no reports of patients showing a Tessier number 7 cleft with unilateral complete cleft lip and palate. Furthermore, no studies have established the sequence, plan, or timing of surgical methods for treating patients presenting the above anomalies simultaneously. We report a case of a Tessier number 7 cleft with unilateral complete cleft lip and palate. Two months after birth, lip adhesion was performed on the unilateral complete cleft lip and total excision was performed on the skin tag. At 4 months of age, Tessier number 7 cleft was corrected. At 6 months of age, surgery involving two small triangular flaps was performed on the unilateral incomplete cleft lip after performing lip adhesion. At 13 months of age, two-flap palatoplasty with a vomer flap was performed on the complete cleft palate. At 6 years of age, open rhinoplasty was performed on the unilateral cleft lip nose deformity. At 9 years of age, bone grafting was performed for the alveolar cleft. At follow-up appointments up to 13 years of age, there were no major complications. Here, we present this patient, surgical procedures and timelines, and show our results demonstrating good postoperative outcomes.

• Archives of Plastic Surgery
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• 제46권1호
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• pp.16-22
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• 2019

Background Various surgical techniques have been used to correct Tessier number 7 craniofacial cleft, which involves macrostomia, ear deformity, and hemifacial microsomia. To achieve symmetrical and satisfactory results in patients with macrostomia, the authors performed a 1-mm medial overcorrection on the cleft side and evaluated the results of this procedure. Methods A retrospective medical record review of patients diagnosed with Tessier number 7 craniofacial cleft from March 1999 to February 2017 was performed. Using clinical photographs, outpatient clinic records, and operative records, information was recorded regarding concurrent congenital anomalies, postoperative complications, and follow-up. Using Photoshop CS2, the length of both sides of the lip was compared. The ratio of these lengths was calculated to evaluate lip symmetry. Results Of the patients treated at the Department of Plastic and Reconstructive Surgery at Kyungpook National University Chilgok Hospital, 11 (male-to-female sex ratio, 7:4) were diagnosed with Tessier number 7 craniofacial cleft. Concurrent congenital anomalies included skin tag, hemifacial microsomia, and cleft palate. The mean duration of follow-up was $78.273{\pm}72.219$ months and the mean ratio of the lengths of both sides of the lip was $1.048{\pm}0.071$. Scar widening occurred as a postoperative complication in some patients. No cases of wound infection, bleeding, or wound dehiscence occurred. Conclusions For the successful correction of macrostomia, plastic surgeons should consider both functional and aesthetic problems of the lip. Adequate repair of the orbicularis oris muscle, skin closure with Z-plasty, and medial overcorrection of the neo-oral commissure led to good results in our patients.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제44권4호
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• pp.159-166
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• 2018

Objectives: The aim of the study was to compare wound healing complications following the use of either absorbable or non-absorbable sutures for skin closure in cleft lip repair. Materials and Methods: This was a randomized controlled trial conducted at the Department of Oral and Maxillofacial Surgery, Lagos University Teaching Hospital, Idi Araba, Lagos State, Nigeria. Sixty subjects who required either primary or secondary cleft lip repair and satisfied all the inclusion criteria were recruited and randomized into two groups (Vicryl group or Nylon group). The surgical wounds in all subjects were examined on 3rd, 7th, and 14th postoperative days (POD) for presence or absence of tissue reactivity, wound dehiscence, and local wound infection. Results: Hemorrhage, tissue reactivity, wound dehiscence, and local wound infection were identified as wound healing complications following cleft lip repair. The incidence of postoperative wound healing complications on POD3 was 33.3%. Tissue reactivity was more common throughout the evaluation period with the use of an absorbable (Vicryl) suture compared to a non-absorbable (Nylon) suture, although the difference was statistically significant only on POD7 (P=0.002). There were no significant differences in the incidences of wound dehiscence and infection between the two groups throughout the observation period. Conclusion: There were no statistically significant differences in the incidences of wound dehiscence and surgical site wound infection following the use of either Vicryl or Nylon for skin closure during cleft lip repair. However, more cases of tissue reactivity were recorded in the Vicryl group than in the Nylon group on POD7. Particular attention must be paid to detect the occurrence of wound healing complications, most especially tissue reactivity, whenever a Vicryl suture is used for skin closure in cleft lip repair.

• 대한구순구개열학회지
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• 제7권1호
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• pp.17-24
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• 2004

Cleft lip and palate are most common congenital defomity to affect the orofacial region. Cleft lip and palate are caused by abnormal development of primary and secondary palate. Its causative mechanism is not completely understood, but genetic and environmental factors play important roles. Lots of epidemiologic surveys have been done extensively about incidence, racial influence, sex ratio, parent age, associated syndrome and genetic factors. These researches are useful to dissolve many problems in prevention and treatment of cleft lip and palate. We performed epidemiologic survey of cleft lip and palate who visited the department of Oral and Maxillofacial Surgery, Guro hospital of Korea University from 1995 to 2004.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제32권1호
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• pp.19-26
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• 2006

Objective: In order to elucidate the retrogressive degeneration of orofacial cleft, the fissured tissues of prenatal and postnatal cleft lip and palate were examined by histological and immunohistochemical methods. Design: Totally 42 cases of prenatal (n=17) and postnatal (n=25) cleft lip and/or palate were examined in comparison with 10 cases of normal lip and oral mucosa using immunohistochemical stainings of MMP-3, MMP-9, MMP-10, cathepsin G, PCNA, E-cadherin, TGase 2, HSP-70, vWF, and VEGF. Main Outcome Measures: In the fissured tissue the sebaceous glands were strongly positive for PCNA and grew into the underlying fibromuscular tissue (24/42). Some hyperplastic sebaceous glands of prenatal cleft lip produced infundibular follicular cyst (9/17). The skin and mucosal epithelia from the postnatal cleft lip and palate (10/25) showed severe basal hyperplasia (11/25) and melanocyte infiltration (7/25). Results: The immunostaining of MMP-3 and HSP-70 were strongly positive in the hyperplastic sebaceous glands and nearby atrophying muscle bundles of the fissured tissue, while MMP-9, MMP-10, and cathepsin G were almost negative. The immunoreactions of the other antibodies used in this study were similar between in the fissured tissues and in the normal controls. Conclusions: These data suggest that the over-expression of MMP-3 is closely related to the sebaceous gland hyperplasia, epithelial dysplasia, and the muscle degeneration, and that the over-expression of MMP-3 in the fissured tissue may continuously aggravate the cleft condition in the later life.

• Asian Pacific Journal of Cancer Prevention
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• 제14권11호
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• pp.6803-6806
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• 2013

Background: Interferon regulatory factor 6 (IRF6) is a transcription factor with distinct and conserved DNA and protein binding domains. Mutations within the protein binding domain have been significantly observed in subjects with orofacial cleft relative to healthy controls. In addition, recent studies have identified loss of expression of IRF6 due to promoter hypermethylation in cutaneous squamous cell carcinomas. Since mutational events occurring within the conserved domains are likely to affect the function of a protein, we investigated whether regions within the IRF6 gene that encodes for the conserved protein binding domain carried mutations in oral squamous cell carcinoma (OSCC). Materials and Methods: Total chromosomal DNA extracted from 32 post surgical OSCC tissue samples were amplified using intronic primers flanking the exon 7 of IRF6 gene, which encodes for the major region of protein binding domain. The PCR amplicons from all the samples were subsequently resolved in a 1.2% agarose gel, purified and subjected to direct sequencing to screen for mutations. Results: Sequencing analysis resulted in the identification of a mutation within exon 7 of IRF6 that occurred in heterozygous condition in 9% (3/32) of OSCC samples. The wild type codon TTC at position 252 coding for phenylalanine was found to be mutated to TAC that coded for tyrosine (F252Y). Conclusions: The present study identified for the first time a novel mutation within the conserved protein binding domain of IRF6 gene in tissue samples of subjects with OSCC.

• Journal of Oral Medicine and Pain
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• 제30권1호
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• pp.87-106
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• 2005

삼차신경계의 일차연접부위에서 구강 및 악안면 영역의 저역치기계자극수용기에서 유래하는 감각정보의 전달 및 처리 기전을 이해하고자, 고양이 콧수염에서 유래하는 들신경섬유를 사용하여 단일축삭 내 기록법에 의해 HRP를 표식한 후, 삼차신경중간핵에서 시편을 제작하고 표식종말에 대한 연속절편을 형성하였다. 총 30개의 표식 신경종말을 대상으로 전자현미경을 이용하여 삼차신경중간핵에서의 신경섬유 종말 및 연접이전축삭종말의 미세구조적 특징, 발현빈도, 연접양식 등을 분석하여 다음과 같은 결과를 얻었다. 1. 표식종말은 직경 45$\sim$55 ㎚의 균일한 형태의 밝고 둥근 모양의 연접소포를 함유하고 있었으며, 가지돌기와는 연접이후 치밀질이 잘 발달되어 있고, 연접틈새가 크며, 여러 곳에서 넓은 연접구조를 보이는 비대칭연접을, 다형의 연접소포를 함유하는 비표식 축삭종말과는 연접이후 치밀질이 뚜렷하게 발달되지 않으며, 연접면적이 좁은 대칭연접을 형성하였다. 2. 각 표식종말은 인접한 신경구조물들과 최소 1개에서부터 최대 15개까지 신경연접을 형성하여 단위 표식종말 당 평균 4.77$\pm$3.37개의 신경연접이 관찰되었으며, 5개 이상의 신경구조물들과 연접을 형성하는 비교적 복잡한 연접양상이 다수의 표식종말(46.7%, n=14)에서 관찰되었다. 3. 표식종말이 세포체와 직접 연접하는 양상은 관찰되지 않았으며, 가지돌기와는 단위 표식종말 당 1.83$\pm$1.37개의 신경연접을 형성하였다. 가지돌기와 연접을 이루는 표식종말의 대부분(85.0%)은 원위부 가지돌기인 가지돌기체와 연접을 이루었으며(n=47, 1.57$\pm$1.38/1 bouton), 근위부 가지돌기(n=6, 0.20$\pm$0.41/1 bouton)나 가지돌기 가시(n=2, 0.07$\pm$0.25/1 bouton)와 연접을 이루는 경우는 드물었다. 4. 표식종말의 76.7%(n=23)에서 다양한 형태의 연접소포를 함유하는 축삭종말인 p-ending과 축삭사이연접을 형성하였으며 (2.93$\pm$2.36/1 bouton), p-ending이 표식종말 및 이에 연접하는 가지돌기와 동시에 연접을 형성하는 연접세동이도 60.0% (n=18)에서 관찰되었다. 이상의 결과를 종합하여 보았을 때, 고양이 콧수염에서 유래하는 들신경섬유 종말은 삼차신경중간핵에서 특징적인 연접양상을 나타내었으며, 이는 감각정보의 분별, 통각의 정동반응 등 복잡한 감각정보의 처리에 관여하는 삼차신경중간핵의 기능과 밀접한 상관관계가 있는 것으로 사료되며, 향후 신경종말의 미세구조에 대한 정량적인 분석과 연접이전 억제에 관여하는 신경전달물질의 동정 등 생리학적인 기능에 대한 더욱 광범위한 연구가 필요하리라고 사료된다.