• Archives of Plastic Surgery
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• v.40 no.2
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• pp.97-103
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• 2013

Background Velopharyngeal insufficiency (VPI) may persist after primary repair of the cleft palate, and surgical correction is necessary in many cases. The purpose of this study is to evaluate the effect of double opposing Z-plasty (DOZ) in cleft palate patients suffering from VPI after primary two-flap palatoplasty. Methods Between March 1999 and August 2005, we identified 82 patients who underwent two-flap palatoplasty for cleft palate repair. After excluding the patients with congenital syndrome and mental retardation, 13 patients were included in the final study group. The average age of the patients who underwent DOZ at was 5 years and 1 month. Resonance, nasal emission, and articulation were evaluated by a speech pathologist. The velopharyngeal gaps were measured before and after surgery. Results Six patients attained normal speech capabilities after DOZ. The hypernasality grade was significantly improved after surgery in all of the patients (P=0.0015). Whereas nasal emission disappeared in 8 patients (61.5%), it was diminished but still persisted in the remaining 5 patients. Articulation was improved in all of the cases. In two cases, the velopharyngeal gap was measured using a ruler. The gap decreased from 11.5 to 7 mm in one case, and from 12.5 to 8 mm in the second case. Conclusions The use of DOZ as a surgical option to correct VPI has many advantages compared with other procedures. These include short surgery time, few troublesome complications, and no harmful effects on the dynamic physiological functioning of the pharynx. This study shows that DOZ can be another option for surgical treatment of patients with VPI after two-flap palatoplasty.

• Korean Journal of Cleft Lip And Palate
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• v.2 no.1_2
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• pp.5-14
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• 1999

The aim of treatment of cleft lip and palate is to correct the cleft and associated problems surgically and thus hide the anomaly so that patients can lead normal lives. This correction involves surgically producing a face that does not attract attention, a vocal apparatus that permits intelligible speech, and a dentition that allows optimal function and esthetics. In neonatal periods, gross distortion of tissues surrounding the cleft requires considerable effort and time due to post operative functional defect and scarring and induces milk feeding problem, malocclusion of deciduous or permanent dentition, congenital missing teeth, skeletal dysplasia. The occurrence of a cleft deformity is a source of considerable shock to the parents of an afflicted baby, and the most appropriate approach is very important things. Thus we tried to analysis of dental arch, shape and size of deformity in cleft patients. The results were obtained as follows. 1. When the cast measurements of UCLP subjects at first visit it was found that the mean length was 9.29mm at the alveolar cleft width, also that was 11.7mm at the anterior width and 14mm at the posterior cleft width. 2. Comparison of UCLP group at first visit and just lip surgery, it was found that the older group showed a insignificant reduction in the width of the cleft in the alveolar, canine, and tuberosity regions. 3. The maxillary casts of the UCLP group at 6 months differ Significantly from those of the at 3 months in both length and width. but there was no statistical difference except anterior ridge length of nonclefted site. 4. Comparison at 6 months and 18 months, there was a greater change in length of the alveolar cleft width, intercanine width, and anterior cleft width. Maxillary arch became wider at both the canine region and intertuberosity region. also posterior anteroposterior length was increased but anterior AP length was decreased from 8.1mm to 7.7mm. There was meaningful increase at intertuberosity length; however, a significant reduction in width t-t'

• Archives of Craniofacial Surgery
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• v.13 no.2
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• pp.135-138
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• 2012

Purpose: Reconstruction of the cleft earlobe is challenging. Several procedures are available to reconstruct congenital earlobe deformities. However, for large defective type, surgical procedures and designs are complex and tend to leave a visible scar. We present a simple method of reconstruction for defective type congenital cleft earlobe using a one stage technique with infraauricular transposition flap. This allows for easy and accurate size estimation and good aesthetic outcomes. Methods: A 4-year-old male patient has congenital cleft earlobe and antihelical deformity. Otoplasty for antihelical deformity correction and one stage infra-auricular transposition flap for earlobe reconstruction were performed. The flap was designed from the inferoanterior margin of the earlobe. The size of the flap was determined based on the normal side, and the width and length of the flap was 1 cm and 3 cm in size, respectively. An incision was made at the midline of the defective lobule. Further, the elevated flap was inserted. The elevated flap and the incision margins of the lobule were sutured together. Then, the donor site was closed primarily. Results: The volume and shape of the reconstructed earlobe were natural. There was no flap necrosis. The donor site had no morbidities and scar was not easily notable. Conclusion: Infra-auricular transposition flap can be designed easily and offer sufficient volume of earlobe. Furthermore, the scar is inconspicuous. In conclusion, infra-auricular transposition flap can be a good option for reconstructing a large defect type cleft earlobe.

• Journal of Genetic Medicine
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• v.9 no.2
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• pp.89-92
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• 2012

Campomelic dysplasia (CMD) is a rare, often lethal, genetic disorder characterized by multiple congenital anomalies and abnormal development of the reproductive organs in males. Mutations in the SOX9 gene are known to cause CMD. We present a Korean CMD girl with a normal 46,XX karyotype and a female reproductive organ phenotype. She was born at 2.35 kg at 38 weeks of gestation and showed characteristic phenotypes, including cleft palate, micrognathia, hypertelorism, flat nasal bridge, congenital bowing of limbs, hypoplastic scapulae, deformed pelvis, and 11 pairs of ribs. She also had an atrioseptal defect of the heart and marked laryngotracheomalacia requiring tracheostomy and tracheopexy. SOX9 mutation analysis revealed the presence of a novel nonsense mutation, $p.Gln369^*$, and the patient was genetically confirmed to have CMD. Although she showed marked failure to thrive and neurodevelopmental delay, she is now 40 months of age and is the only surviving patient with CMD in Korea.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.43 no.3
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• pp.171-177
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• 2017

Amniotic constriction band is a rare clinical entity with varied manifestations that range from a combination of congenital malformations to isolated malformations that are unique to each patient. The etiology of this entity remains unknown. Herein, we highlight two cases of amniotic constriction band that presented to our unit with unique clinical characteristics. To the best of our knowledge, an isolated circumferential band of scarring on the face with ocular involvement, as demonstrated by the first case, and a combination of bilateral complete cleft lip and palate with bilateral microphthalmia, auto-amputation of the right thumb, and a constriction band on the left thumb, as demonstrated by the second case, are extremely rare presentations of amniotic constriction band that were not previously reported in the literature and therefore necessitate a special mention. We discuss potential etiologies for these cases and review the existing literature on this entity.

• Korean Journal of Veterinary Research
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• v.62 no.4
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• pp.32.1-32.4
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• 2022

A Hanwoo cow with a delayed gestation and abdominal distension was delivered following PGF_2α injection. There was excessive amniotic fluid, and a male calf was delivered but died immediately. The calf had no eyes and nose, and a cleft palate on the upper jaw. Gross appearance and computed tomography image showed that upper teeth were spread out on both sides due to cleft palate in the upper jaw, and lower jaw and teeth were positioned upward. There were no other brain parts except cerebellum. These findings show a rare case of hydramnios related to fetal congenital deformity in a Hanwoo cow.

• The korean journal of orthodontics
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• v.29 no.4 s.75
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• pp.467-481
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• 1999

Cleft lip and/or palate is one of the most common congenital craniofacial anomalies. According to previous epidemiologic studies, incidence of cleft lip and/or palate has been increasing nowadays. However, there is no report about epidemiologic study of cleft lip and/or palate patients who visited dept. of orthodontics in Korea. So the purpose of this study was to provide the epidemiological characteristics and important basic clinical data for the diagnosis and the treatment of the cleft lip and/or palate patients. With the orthodontic and cleft charts, diagnostic models and X-ray films from 250 patients with cleft lip and/or palate who visited Dept. of Orthodontics, Seoul National University Dental Hospital during the last 11 years, the authors investigated patient's visiting yew, types of cleft, patient's gender, and Angle's classification of malocclusion, and surgery timing. The results were as follows ; 1. The number of cleft patients who visited Dept. of Orthodontics, SNUDH increased during 1988-1990 and then it declined until 1992. From 1993 to 1996, it showed a stationary trend. After 1997 it showed an overwhelmingly increasing trend. 2. In the cleft type, the ratio of cleft lip cleft lip and alveolus cleft palate : cleft lip and palate was 7.6:19.2:9.6:63.6. In cleft position, unilateral clefts were more than bilateral ones (cleft lip 79:21, cleft lip and alveolus 77:23, cleft lip and palate 75.5:24.5). In cleft side, left clefts were mote than right clefts (cleft lip 53.3:46.7 cleft lip and alveolus 59.5:40.5, cleft lip and palate 59.2:40.8). 3. In gender ratio, males were more than females in cleft lip (57.9:42.1), cleft lip and alveolus (68.8:31.2) and cleft lip and palate (76.1:23.9). But in cleft Palate females were more than males as 41.7: 58.3. 4. In the age groups, 7-12 year group was the most abundant as $52\%$, and then 0-6 year group ($20.4\%$), 13-18 year group ($17.2\%$), more than 18 yew group ($10.4\%$) were followed as descending order. 5. Most of the cleft lip repair surgeries were operated in 0-3 month ($60.3\%$) and 4-6 month ($17.9\%$). 6. The cleft palate repair surgeries were done in 1-2 year ($31.7\%$), 0-1 year ($25.6\%$), 2-3 year ($12.1\%$), more than 5 year ($11.6\%$) as descending order. 7. The lip scar revision surgeries were done before admission at elementary school in $60\%$. (4-6 you ($27.5\%$), 6-8 year ($19.6\%$), more than 10 year ($19.6\%$), 2-4 year ($13.7\%$) as descending order) 8. The rhinoplasties were done before admission at elementary school in $51.7\%$. (0-2 year ($7.1\%$), 2-4 year ($14.3\%$), 4-6 year ($21.4\%$), 6-8 year ($14.3\%$)). 9. The pharyngeal flap were done at 6 Y (72.5 months) after birth on average and there was even distribution of surgery timing. 10. In relationship between Angle's classification of malocclusion and cleft types, Class I was most abundant and Class III, Class II were followed as descending order in cleft lip group. But Class III was most abundant and Class I, Class II were followed as descending order in cleft lip and alveolus group, cleft palate group, and cleft lip and Palate group. The percentage of frequency in Class III malocclusion was overwhelmingly higher in cleft lip and palate group than any other groups. 11. Because the frequency of class III malocclusion was most prevalent in all age groups, anterior crossbite was the most common chief complaint of cleft patients.

• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.3
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• pp.209-215
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• 2013

Cleft lip and palate, the most common craniofacial anomalies, are severe congenital defects that have an incidence of 0.28 to 3.74 per 1000 live births. Although there has been great improvement in the field of cleft surgery, surgical approach cannot be the single solution to resolve the various problems encountered in patients with cleft lip and palate. The concept of presurgical infant orthopedics (PSIO) for gradual closure of the cleft gap and simplified surgical performance was first introduced by McNeil in 1950. Recently, there are many attempts not only to approximate the alveolar segments but also to reshape the nasal cartilage. Three infants with unilateral cleft lip and palate were referred from the department of Plastic Surgery for presurgical nasoalveolar molding (PNAM). Maxillary appliances using resin with orthodontic wire were fabricated. Then these appliance was applied until patients underwent lip surgery. In all cases, the patients could wear the appliance all day since they were able to eat even with the appliance on, This resulted in significant improvements in the nasal symmetry were found. Our appliance, namely K-NAM, extends the wearing time within the limited period and as a result it is expected to maximize the treatment effects. Used properly, this appliance would play a major role in enhancing nasal symmetry with satisfactory results.

• Archives of Craniofacial Surgery
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• v.25 no.1
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• pp.11-16
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• 2024

Background: The pathogenesis of orofacial cleft (OFC) is multifactorial, involving both genetic and non-genetic factors, the latter of which play a key role in the development of these anomalies. This paper addresses the incidence of OFC in Indonesia, with a focus on identifying and examining the distribution of contributory factors, including parental medical history, pregnancy history, and environmental influences. Methods: The study was conducted through the collection of primary data. An interdisciplinary research team from Indonesia administered a standardized questionnaire to parents who had children with OFC and who had provided informed consent. The case group comprised 133 children born with cleft lip and/or palate, and the control was 133 noncleft children born full-term. The risk factors associated with OFC anomalies were analyzed using the chi-square test and logistic regression. All statistical analyses were performed using SPSS version 25. A p-value of 0.05 or less was considered to indicate statistical significance. Results: The study comprised 138 children, of whom 82 were boys (59.4%) and 56 were girls (40.6%). Among them, 45 patients (32.6%) presented with both cleft lip and cleft palate, 25 individuals (18.1%) had a cleft palate only, and 28 patients (20.3%) had a cleft lip only. OFC was found to be significantly associated with a maternal family history of congenital birth defects (p<0.05), complications during the first trimester (p<0.05), consumption of local fish (p<0.05), caffeine intake (p<0.05), prolonged medication use (p<0.05), immunization history (p<0.05), passive smoking (p<0.05), and X-ray exposure during pregnancy (p<0.05). Conclusion: The findings indicate close relationships between the incidence of OFC and maternal medical history, prenatal factors, and environmental influences.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.23 no.5
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• pp.458-460
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• 2001

A 11-month-old infancy was referred for treatment of cleft palate. The patient's congenital deformities had included oronasal fistula on the soft palate and bifid uvula. Furrow double-opposing Z-plasty was performed under general anesthesia. However, postoperative bleeding developed at ward. Despite the pressure of operative wound, venous oozing continued and hemoglobin concentration and RBC were decreased (Hb: 5.5g/dL, RBC: $1.98{\times}10^6/mm^3$). The patient was pale and confused and transferred to Surgical Intensive Care Unit(SICU) We could not keep the peripheral venous line and there was a high risk of hypovolemic shock. Emergency transfusion was performed through the central femoral venous line and emergency operation for bleeding control was carried out. We explored the operation site and found bleeding point from the posterior pharyngeal wall. Electrocoagulation and surgicel packing were carried out and bleeding was stopped.