구순열과 구개열의 발생요인 및 치료 경향

윤천주,유선열,Yoon, Chun-Ju,Ryu, Sun-Youl 대한악안면성형재건외과학회 2006 Maxillofacial Plastic Reconstructive Surgery Vol.28 No.4

The present study was aimed to evaluate the incidence, etiological factors, and management of cleft lip and palate. Two hundred and twenty patients with cleft lip and/or cleft palate who were treated at Department of Oral and Maxillofacial Surgery, Chonnam National University Hospital, during the period between January 1994 and December 2003 were reviewed. The ratios of cleft lip : cleft lip with cleft palate : and cleft palate were 0.4:1.1:1. Males were more common than females in cleft lip (1.3:1) and cleft lip and palate (2.5:1), while females were more common than males in cleft palate (1:1.3). In the cleft side, left clefts were more prevalent than right clefts (cleft lip 1.3:1, cleft lip and palate 1.6:1). Unilateral clefts were more common than bilateral clefts in cleft lip (79:21). Cleft lip and cleft palate were more common in those with blood type A (34.5%) than those with other types. There was no significant relationship between birth season and frequency of clefts. The clefts were common in the first-born (48.8%), and in mothers aged between 25 and 29 (51.7%). Medication (24.7%) and stress (16.7%) during the first trimester were noted. Positive familial history was noted in 13 cases (5.9%). Thirty-two cases (15%) were associated with other congenital anomalies, in which tonguetie (40.6%) and congenital heart disease (21.9%) were most common. Among 100 patients with cleft palate, 77 patients had middle ear disease (77%), which occurred predominently in the incomplete cleft palate. Seventy-six among the 77 patients received myringotomy and ventilation tube insertion, and the remaining one received antibiotic medication only. Cleft lips were treated primarily at 3 to 6 months, and cleft palates were at 1 to 2 years. Treatment regimens included modified Millard method mainly in the cleft lip, and Wardill V-Y, Dorrance method, and Furlow method in the cleft palate. The percentage of palatal lengthening as type of cleft palate was greater in the incomplete cleft palate group (11.2%) than in the complete cleft palate group (9.6%). The percentage of palatal lengthening as operating method was no difference between the Furlow method (10.9%) and the push back method (10.7%). As postoperative complications, hypertrophic scar was most frequent in the cleft lip, and oronasal fistula in the cleft palate. In summary, it was shown that medication and stress during the first trimester of pregnancy were frequently associated with cleft lip and cleft palate, adequate timing and selection of method of operation are important factors to obtain morphologically and functionally good results. Furthermore prevention and treatment of middle ear disease are important in cleft palate patients because of its high co-occurrence.

구순열 및 구개열 환자 673례에 대한 임상적 분석

장도명,위성신,강윤제,임풍,이종건 大韓成形外科學會 1996 Archives of Plastic Surgery Vol.23 No.4

Cleft lip, with or without cleft palate, is one of the most common congenital malformations in Korea. Improper management of the patients with cleft lip and/or palate may result in facial maldevelopment, speech disturbance, social maladjustment and psychological stress together with their parents. For the past 16 years, from 1978 through 1994, we have treated 1502 patients with cleft lip and/or palate in Catholic Medical Center. The medical records and laboratory data were reviewed in order to secure the information for the retrospective study but valuable data were available in 673 cases. The present paper deals with an analysis of them to report the statistical and basic clinical data of cleft lip and/or palate. The results were as follows; 1. The rate of the associated anomalies was 4.1% and congenital heart disease was the most frequent one. 2. The ratio of cleft lip, cleft lip with palate and cleft palate alone was 1.5 : 1.3 : 1.0. 3. The ratio of left, right and bilateral cleft lip was 2.3 : 1.4: 1.0 and that of cleft lip with palate was 1.5 : 1.0 : 1.01. 4. The incomplete cleft lip occurred 2.01 times more than complete cleft lip and complete cleft lip with palate occurred 2.95 times more than incomplete cleft lip with palate, and the ratio of complete, incomplete and submucose cleft palate was 2.5 : 8.0 : 1.0. 5. Male was affected 1.4 times more than female in cleft lip and 2.3 times in cleft lip with palate and female was affected 2.3 times more than male in cleft palate. 6. Most of the patients was operated between the age of 3 months to 1 year in cleft lip(62%) and between 1 to 2 years in cleft palate(50%), 7. Before 1990, the Tennison-Randall method was widely used but after that, the Millard methods( ⅠⅡ) were undergone predominantly in cleft lip repair and most of the patients with cleft palate (91%) was treated by push-back palatoplasty, except submucose type which was corrected by double reversing Z-palatoplasty. 8. Almost all the patients with cleft palate(95%) were treated by myringotomy due to the middle ear disease (serous otitis media)at the same time.

점막하구개열 환자에서 구개인두조영술을 이용하여 계측한 두개기저부와 연구개인두부의 구조 및 운동에 대한 연구

정재호,나동균,이승헌,서진석,박병윤 大韓成形外科學會 1998 Archives of Plastic Surgery Vol.25 No.7

Submucous cleft palate is defined as a bifid uvula, palatal muscle diastasis, and a notch in the posterior surface of the hard palate. Since submucous cleft palate is presently felt to be of clinical relevance only in the presence of velopharyngeal inadequacy, the evaluation and diagnosis of occult submucous cleft palate are only pursued if the patient has velopharyngeal incompetency. The age for surgical correction of submucous cleft palate is one of the most important factors determining the speech outcome of surgical treatment. The available evidence suggests that earlier repairs yield better speech results than late repairs and that later treatment is associated with a higher rate of velophryngeal incompetency. But, treatment of patients with submucous cleft palate depend on the diagnosis of the velopharyngeal incompetency and therefore nearly always occurs later than the time for optimal treatment. Twenty submucous cleft palate patients aged 5 to 12 years with normal hearing and intelligence who visited Yonsei university plastic and reconstructive surgery department from January 1993 to January 1996 were evaluated as an experimental group and nineteen children aged 7 to 12 years with normal hearing and intelligence who randomly selected from the Hyosung elementary school were evaluated as a control group. In this study we analyzed the cranial base and soft tissue structures in submucous cleft palate using velopharyngogram. The results are summarized as follows: 1. The survey of the nasopharynx with submucous cleft palate indicated that the angle of cranial base was outside the range toward the obtusity considering analysis of covariance. 2. The pharyngeal angle(Ba-S-PNS) exceeds normal limits and the ratio of anteroposterior distance of nasopharynx with submucous cleft palate is larger in submucous cleft palate group considering analysis of covariance. 3. The patients with submucous cleft palate have relatively short soft palate in the neutral state and during phonation. 4. The soft palate of submucous cleft palate patients has lessened mobility in submucous cleft palate group. 5. The distance between both lateral pharyngeal wall shows shorter in submucous cleft palate group only during "Su" phonation. From these result, the submucous cleft palate patients have relatively obtuse cranial base and wide nasopharynx of deficient velum with limited mobility, so velopharyngeal incompetency is inevitable.

점막하 구개열 환자 공명장애의 스펙트럼 특성 연구

김현철,임대호,백진아,신효근,김오환,김현기,Kim, Hyun-Chul,Leem, Dae-Ho,Baek, Jin-A,Shin, Hyo-Keun,Kim, Oh-Hwan,Kim, Hyun-Ki 대한악안면성형재건외과학회 2006 Maxillofacial Plastic Reconstructive Surgery Vol.28 No.4

Submucosal type cleft palate is subdivision of the cleft palate. It is very difficult to find submucosal cleft, because when we exam submucosal type cleft palate patients, it seems to be normal. But in fact, there are abnormal union of palatal muscles of submucosal type cleft palate patients. Because of late detection, the treatment - for example, the operation or the speech therapy - for the submucosal type cleft palate patient usually becomes late. Some patients visited our hospital due to speech disorder nevertheless normal intraoral appearance. After precise intraoral examination, we found out submucosal cleft palate. We evaluated the speech before and after surgery of these patients. In this study, we want to find the objective characteristics of submucosal type cleft palate patients, comparing with the normal and the complete cleft palate patients. Experimental groups were 10 submucosal type cleft palate patients and 10 complete cleft palate patients who got the operation in our hospital. And, the controls were 10 normal person. The sentence patterns using in this study were simple 5 vowels. Using CSL program we evaluated the Formant, Bandwidth. We analized the spectral characteristics of speech signals of 3 groups, before and after the operation. In most cases, the formant scores were higher in experimental groups (complete cleft palate group and submucosal type cleft palate group) than controls. There were small differences when speeching /a/, /i/, /e/ between experimental groups and control groups, large differences when speeching /o/, /u/. After surgery the formant scores were decreased in experimental groups (complete cleft palate group and submucosal type cleft palate group). In bandwidth scores, there were no significant differences between experimental groups and controls.

한국인 성인 남자에게 구순열 및 구개열의 유병률에 관한 연구

백형선,김재훈,김동준 대한치과교정학회 2001 대한치과교정학회지 Vol.31 No.1

구순열 및 구개열은 두개 악안면 부위에서 가장 자주 발생되는 선천성 기형으로 적절한 의료진의 확보와 의료 정책의 수립을 위해서 정확한 환자 수를 파악하는 것이 필요하다. 그러나, 기존의 국내 발생률 추정 연구는 표본선정의 문제점과 이 질환이 갖는 높은 유산율과 사산을 및 다양한 형태로 인한 분류의 어려움과 한국 내에서의 특수성 중 하나인 장애아들의 해외 입양으로 인해 정확한 환자 수의 파악이 어려웠다. 따라서, 한국인에서의 체계적인 유병률 연구가 필요하리라 사료되며, 대부분의 구순열 및 구개열 환자에서 필요한 교정 치료의 수혜 정도를 파악할 필요성이 있다고 사료된다. 이에 본 연구에서는 한국 성인 남자에서 구순열 및 구개열 환자에 대한 유병률과 이들에 대한 교정 치료 수혜율을 조사하고자, 1998년도 병무청 징병 검사자 중 1979년도에 출생한 자만을 연구대상으로 하여 서울, 광주, 대구, 부산의 성인 남자 218,322명에 대해 3단계 검사를 실시한 후 다음과 같은 결과를 얻었다. 1. 1979년 생 한국 성인남자에서 구순열 및 구개열 환자의 유병률은 1000명당 0.65명이었다. 2. 전후방적 심도에 따라 1000명당 각각의 유병률은 구순열이 0.26명, 구순구개열이 0.36명, 구개열이 0.03명이었다. 구순열과 구순구개열은 비슷한 정도의 유병률을 보였으나, 구개열은 이에 비해 낮은 유병률을 보였다. 3. 횡적 심도에 따라 1000명당 각각의 유병률은 좌측 파열이 0.35명, 우측 파열이 0.16명, 양측 파열이 0.12명이었다. 좌측 파열은 우측 파열에 비해 매우 뚜렷하게 높은 유병률을 보였으며, 양측 파열은 편측 파열에 비해 낮게 나타났다. 4. 구순열 및 구개열 환자 중 성인남자에서의 한국 내 교정치료 수혜율은 35%였으며, 구순열, 구개열, 구순구개열의 순으로 28%, 29%, 67%였다. 구순열이나 구개열과 같이 단독 발생된 경우보다 파열의 심도가 심한 구순구개열의 경우에서 교정치료 수혜율이 높게 나타났다. Cleft lip and/or palate(CLP) is the most common congenital deformity in the craniofacial region. For a practical public health system for these patients it is necessary to have an understanding on the prevalence of CLP. However, it was difficult to estimate the exact number of CLP patients due to problems in sample selection, higher miscarriage and still birth rate, difficulty in classification, and adoptions to foreign countries. Therefore this study was to estimate the prevalence of CLP and the rate of orthodontic treatment, which is usually necessary in cleft lip and/or palate patients. The samples consisted of 218,322 Korean male adults from Seoul, Kwangju, Taegu and Pusan, all born in 1979. The screening method for recognizing the CLP patients was proceeded in steps and the results are as follows. 1. The prevalence of cleft lip and/or palate in Korean male adults born in 1979 was 0.65 out of the 1000 samples. 2. In the anteroposterior aspect of the 1000 samples, the prevalence of cleft lip, cleft lip and palate, and cleft palate was 0.26, 0.36 and 0.03 respectively. 3. In the transverse aspect of the 1000 samples, the prevalence of left, right and bilateral cleft was 0.35, 0.16, 0.12 respectively. The cleft in the left showed a much higher prevalence than in the right, while bilateral cleft showed a lower prevalence than unilateral cleft. 4. The orthodontic treatment rate of Korean male adults among cleft lip and/or palate patients was 35%, and it was in the order of cleft lip and palate, cleft lip, and cleft palate, being 67%, 29% and 29% respectively. The orthodontic treatment rate in patients with the more severe cleft lip and palate was higher than in patients solely haying cleft lip or cleft palate.

구개열의 연구개 근육에 대한 조직 병리학적 연구(II)

김현철,이윤호,김석화,정두현,김진환 大韓成形外科學會 1992 Archives of Plastic Surgery Vol.19 No.4

Anatomic abnormalities of palatal muscles in the cleft palate have been already described in many papers by several authors, that is, the levator palatini muscle is abnormally inserted onto the posterior margin of the hard palate and cleft margin instead of being insterted into the medial raphe of the soft palate. The muscle fibers have irregular arrangements and are gypoplastic in the cleft margin, and the degree of hypolasia of the muscle is directly proportional to the severity of the palatal cleft. But there are no studies based on histochemical and ultrastructural analyses of soft palate muscles in the cleft palate. Pathophysiology of cleft lip muscles was reported by Schendel et al in 1989, who demonstrated a nonneurogenic muscle atrophy and mitochondrial myopathy. We assumed that the above findings might be present at cleft palate muscles also. In twenty patients, two muscle biopsy specimens were obtained in each person at the time of primary palatoplasty : one from the cleft margin and the from the center of the soft palate muscle. Enzyme histochemical stains(ATPase, NADH-TR, modified Gomori trichrome stains) and electron microscopy were performed. Variable degree of nonneurogenic muscle atrophy was seen in ever specimen and modified Gomori trichrome stain demonstrated "ragged red" fibers, typical of mitochondrial myopathy. Such findings were present in both specimens of cleft margin(specimen "A")and muscle proper(Specimen "B"), and the degree of mitochondrial myopathy of "A" was slightly more severe than "B". The degree of mypathy of the levator was inversely proportional to the severity of the palatal cleft : mitochondreal myopathy is more severe in the incomplete cleft than in the complete cleft. Electron microscopy confirmed abnormally large mitochondria and accumulation of mitochondria distorting fibrils. We suggest that the intrinsic abnormality of the cleft palate muscles may be one of the important causes of the unfavorable speech in the cleft palate patients even after intravelar veloplasty.

입술갈림증 태아에서 동반된 입천장갈림증의 예후적 의미

정지예 ( Ji Ye Jung ),홍준석 ( Joon Seok Hong ),김혜진 ( Hye Jin Kim ),손유경 ( Yoo Kyung Sohn ),심순섭 ( Soon Sup Shim ),박중신 ( Joong Shin Park ),전종관 ( Jong Kwan Jun ),윤보현 ( Bo Hyun Yoon ),신희철 ( Hee Chul Syn ) 대한주산의학회 2006 Perinatology Vol.17 No.2

목적 : 본 연구의 목적은 입술 및 입천장갈림증의 유형에 따른 임신 결과 및 생후 장애를 비교하고, 입천장갈림증의 유무에 따른 위험도를 평가하는 것이다. 방법 : 990년 1월부터 2004년 7월까지 서울대학교병원에서 분만, 유산, 또는 임신중절된 입술 및 입천장갈림증 96례를 후향적으로 분석하였다. 입술 및 입천장갈림증의 분류는 Nyberg 등의 방법을 중심으로 입천장갈림증 단목을 추가하였다. 유형별로 동반 기형, 염색체 이상의 빈도를 평가하고, 임신 중절, 자연유산, 자궁 내 태아사망, 영아사망의 빈도를 비교하였다. 생존아 중 장기 추적이 가능한 경우에 중이 환기관 삽입, 청각장애, 언어발달 장애를 비교하였다. 입천장갈림증이 있는 경우에 입술갈림증 단독 군보다 어느 정도 위험이 증가하는지 평가하였다. 결과 : 동반 기형은 입술갈림증만 있는 경우 23.7% (9/38)이었고, 입천장갈림증이 있는 경우는 55.2% (32/58)이었다. 동반 기형으로는 심장기형이 가장 많았고 중추신경계, 근골격계, 위장관계, 비뇨생식기계 기형도 흔하였으며, 67.4% (29/43)의 증례에서 다발성 기형으로 관찰되었다. 입천장갈림증이 있는 생존아의 경우 입술갈림증만 있는 경우보다 중이 환기관 삽입 (70.0% [14/20] vs 3.1% [1/32])과 언어발달 장애 (42.1 % [8/19] vs 10.7% [3/28])의 발생이 유의하게 많았다. 입천장갈림증이 있는 경우에 입술갈림증만 있는 군보다 동반기형(OR 3.97), 임신중절(OR 5.20), 영아사망(OR 12.96), 중이 환기관 삽입 (OR 72.33), 언어발달 장애 (OR 6.06) 발생의 위험이 증가하였다. 결론 : 입천장갈림증을 동반한 군이 입술갈림증만 단독으로 가진 군에 비하여 임신 결과, 중이 질환, 언어발달 장애에 있어서 불량한 예후를 보이고 있다. 따라서, 산전 정밀초음파 검사시에 입술갈림증 뿐만 아니라 입천장갈림증 유무의 진단에도 주의를 기울여야 한다. Objective : The objective of this study is to compare the pregnancy outcomes and postnatal developmental problems according to the types of cleft lip and/or palate, and to evaluate the increase of the risk of the presence of cleft palate. Methods : Retrospective analysis was performed in 96 cases of cleft lip and/or palate that were delivered, aborted, or terminated at Seoul National University Hospital from January 1990 to July 2004. The cases of cleft lip and/or palate were categorized based on the Nyberg`s classification, and cleft palate alone was added to it. According to the types, the frequencies of associated anomaly and chromosomal anomaly were assessed, and the frequencies of termination, spontaneous abortion, fetal death in utero, and infant death were compared. When long-term follow-up was possible among the surviving infants, we compared the frequencies of ventilatory tube insertion into the middle ear, hearing problem, and speech-language problem. We evaluated the increase of the risk in the presence of cleft palate. Results : Associated anomalies were present in 23.7% (9/38) of cases with cleft lip alone and in 55.1 % (32/58) of those with cleft palate. The most common associated anomaly was cardiac anomaly. The surviving infants with cleft palate showed significantly higher rates of ventilatory tube insertion (70.0% [14/20] vs 3.1% [1/32]) and speech-language problem (42.1 % [8/19] vs 10.7 % [3/28]) than those with cleft lip alone. The presence of cleft palate increased the risk of occurrence of associated anomaly (OR 3.97), termination (OR 5.20), infant death (OR 12.96), ventilatory tube insertion (OR 72.33), and speech-language problem (OR 6.06). Conclusion : Associated cleft palate in patients with cleft lip has a poor impact on pregnancy outcomes, middle ear disease, and speech-language problem. Therefore, during the prenatal screening ultraso-nography, the careful examination should be directed for the detection of cleft palate in cases with cleft lip.

선천성 구순열 및 구개열 환자 895례의 임상적 분석

김석권,박지훈,이근철,박정민,김정태 대한성형외과학회 2002 Archives of Plastic Surgery Vol.29 No.6

Cleft of the lip and palate is one of the most common congenital anomalies. Considerable epidemiologic work has been done in different parts of the world. The predisposing cause of these anomalies are multifactorial. Heredity, environment, nutrition and infection have been reported to be the predisposing cause. The aim of this study is to examine clinical aspect of cleft lip and palate by investigating nine items such as incidence, type of cleft, associated anomaly and familial history in last 10 years. From 1991 to 2000, clinical analysis of patients with cleft lip and palate was carried out in Dong-A university hospital. Eight hundred ninety-five patients were operated during that period and we investigated clinical aspect through chart review retrospectively. The result of clinical analysis were as follows. Of the 895 patients, 24% had cleft lip only, 44% had cleft lip and palate and 32% had cleft palate only. In cases of cleft lip with or without cleft palate, the most commonly affected side was the left(55%). Cleft lip and cleft lip with palate were more frequent in the male patients, while cleft palate were more frequent in the female patients. Associated anomalies were present in 8.5% of cases. A positive familial history was found in 5% of cases. 75 of 895 mothers(8.4%) had a history of disease and 98 of 895 mothers(11%) had a history of taking drugs during the first trimester. The significant percentage of patients(48%) was the first baby. Maternal age of affected children was most frequently in the range of 25 to 29. The operation was performed most frequently while the baby was 3 to 5 months old in cleft lip patients and 6 months to 1 year old in cleft palate.

선천성 구순열 빛 구개열 환자에 대한 임상적 연구

최준,오재욱 인제대학교 1993 仁濟醫學 Vol.14 No.2

당병원에서 출생한 신생아 및 일차로 수술받은 선천성 구순열 및 구개열 환자를 대상으로 그 발생요인, 형별발생 빈도, 남녀비, 가족력, 타기형 동반 유무, 수술방법 등을 임상 기록을 토대로 추적조사하여 이에 대한 통계적 관찰을 시행하였다. The authors reviewed 5,967 deliveries and 152 patients with cleft lip and cleft palate who were admitted and treated at Inje University Medical college, Paik hospital during 6 years from Apr. 1983 to March. 1988 in order to evacuate the incidence, sex ratio, type, etiologic factors, combined anomalies, operation methods and so on. The results are as follows : 1.13 babies of cleft lip and cleft palate were born among 5967 deliveries, so the incidence of all clefts were 1 : 452. 2.The rates of cleft lip, cleft lip with cleft palate, and cleft palate were 1 : 1 : 1.4. 3.Males were affected 1.8 times more than females in cleft lip and 4.1 times in cleft lip with cleft palate and 1 : 1 in cleft palate. 4.Incomplete types were 2.8 times more common than complete types in cleft lip and 11 times more common in cleft palate. 5.Clefts in the 1st born group were most common and mothers in the group aged between 26 and 30 years were most frequent. 6.In the prenatal history during the first trimester, herb medication was the most frequent administrated drug, and common cold was the most common disease. 7.10 cases(6.6%) showed positive family history. 8.13 cases(8.6%) had combined anomalies. 9.There were no significant relationship between season and blood type with clefts. 10.Rotation-advancement method was used more than triangular flap method in cheiloplasty, and in almost of allcases, V-Y push back method was used in palatoplasty. 11.14 cases(7%) showed post-operative complications and average period of hospitalization was 11∼15 days.

Early outcomes of cleft and palatal width following anterior palate repair (vomerine flap) in infants with wide cleft lip and palate

Arman Zaharil Mat Saad,Koh Siang Chai,Wan Azman Wan Sulaiman,Siti Fatimah Noor Mat Johar,Ahmad Sukari Halim 대한성형외과학회 2019 Archives of Plastic Surgery Vol.46 No.6

Background Anterior palatal repair performed during cleft lip repair using a vomerine flapmay assist in recruiting additional soft tissue for subsequent completion of palatoplasty, especiallyin patients with a wide cleft. We present our early results in the hope of triggering are-evaluation of this technique regarding its advantages for maxillary growth through furtherstudies of patients with a wide cleft. Methods A retrospective analysis of patients with complete unilateral and bilateral cleft lipand palate was performed, including cleft and palatal measurements taken during initial surgery(lip repair together with anterior palate repair) and upon completion of palatoplasty. Results In total, 14 patients were included in this study, of whom nine (63.3%) had unilateralcleft lip and palate and five (37.5%) had bilateral cleft. All patients had a wide cleft palate. Lip and anterior palate repair was done at a median age of 3 months, while completion ofpalatoplasty was done at a median age of 10.5 months. Measurements taken upon completionof palatoplasty showed significant cleft width reduction in the mid-palate and intertubercleregions; however, the palatal arch distances at nearby landmarks showed non-significantmarginal changes. Conclusions Anterior palate repair using a vomerine flap significantly reduced the remainingcleft width, while the palatal width remained. Further research is warranted to explore thelong-term effects of this technique in wide cleft patients in terms of facial growth.