선천성 구개열의 임상적 고찰

Abstract

[한글]

선천성 구개열은 선천성 기형중 구순열과 함께 그 발생빈도가 높은 것으로 알려져 있다. 그리고 많은 학자들이 구개열의 정확한 발생원인을 규명하려 했으나 아직 완전히 규명하지 뭇하고 있다. 저자는 발생원인 치료방법 그리고 수술중 수혈의 의의 및 수술후 언어

의 호전성 유무를 규명하고자 1970년부터 1977년까지 만 8년간 연세의료원에서 일차 교정술을 받은 선천성 구개열 환자 362명의 임상기록을 검토하여 다음과 같은 결론을 얻었다.

1.만 8년동안 연세의료원의 총신생아 15,7497명중 27명이 구순 혹은 구개열로 그 발생빈도는 585:1이었다.

2.구개열은 여자에서 남자보다 1.5배 더 많았고 구순구개열은 남자에서 여자보다 4배가랑 더 많은 빈도를 보였다.

3.구개열환자의 월별 발생빈도 출산순서볕 발생빈7도 그리고 부모외 연령별 분포를 분석해 본 결과 별 의의가 없었다.

4 .환자의 모친의 임신초기의 태생기역은 질환이 상기도감염으로 가장 많았고 약물복용 중에는 한약복용이 가장 많았다.

5.구개열 환자중 가족력이 있었던 에는 19예로 전체의 5.2%에서 볼 수 있었다.

6. 혈액형별 빈도는 A경, 0형, B헝 그리고 AB형 순으로 많았다.

7. 타기형 동반예는 12예 (3.3%)에 불과했으며 동반된 기형중 Pierre-Robin syndrome이 가장 많았다 .

8.수술의 치적기인 생후 1세에서 2세 사이에 수술받은 환자는 107명 (27.6%) 에 해당했다.

9.수술을 2세이하의 최적기에 받은 환자는 86.6 % 이상의 우수한 언어 명료도의 호전을 보였고 3세이하에서 수술받은 환자도 100 % 양호한 결과를 얻었으며 늦게 수술하면 할수록 언어의 호전도가 빈약함을 관찰할 수 있다.

10.수술중 수혈받은 환자는 87명 (21.3 % )으로 평균 체중 1 kg당 8.6㎖의 혈액을 수혈받았으며 수술중 수혈에 따라 수술전후의 Hb.과 Hct.의 변화는 통계학적으로 별 의의가 없음을 관찰했다 .

11.수술후 병균 재원기간은 96.4 %가 6일에서 15일간이었다.

12. 수술후 사망한 예는 한 예도 업었으며 합병증은 30예 (8.3%)에 불과했으며 U.R.I. 비구강누공, 폐렴, 출혈 그리고 소화기 장애 등을 볼 수 있었다.

[영문]

Cleft palate, with cleft lip, is one of the most common congenital anomalies of the head and neck in a man. And patients with cleft palate must face a diversity of difficulties from birth onward, which were parental emotional reaction, mechanical

difficulties in feedings, airway obstruction, chronic otitis media, hearing impairment, and cosmetic, speech problems.

The etiologic factor of this anomalies have been experimatally and statistically studied by many scholars. But, unfortunnately, little is known regarding the etiology and embryology of this malformation, and there is even disagreement as to their frequency of occurrance and their relatioship to such elements as heredity and environmental factors.

So the author retrospectively reviewed 362 patients with cleft palate who were admitted and treated at Yonsei-Medical center during the past 8 years period from Jan. 1970 to Dec. 1977 in order to evaluate the incidence of cleft palate, sex ratio, etiologic factors, post operative hospitalization, relationship of transfusion and Hb. Hct. Changes, time of operation, and speech evaluation after operation.

The following results were obtained ;

1. 27 babies of cleft lip and/or palate were born among 15749 deliveries, and so cleft birth rate was 1:585.

2. The cleft lip birth rate was 1:1449, cleft palate birth rate was 1:3125 and cleft lip with palate birth rate was 1:1449 as same as cleft lip.

3. Females were affected 1.5 times more than males in cleft palate alone, but in cleft palate and cleft lip, male was affected 4 times more than female

4. Incidence was as follow in the order of frequency, complete cleft palate with cleft lip, incomplete cleft palate, submucous cleft palate, and complete cleft palate alone.

5. Left side of lip was affected 1.6 times more than right side in complete cleft palate with cleft lip. And bilateral cleft palate was 26 cases(7.2%).

6. Cleft palate birth rate by incidence of month of birth, parity, and parental age was not statistically able to evaluate. And the distribution was insignificant in etiology of cleft palate.

7. In the incidence of prenatal history of cleft palate patients was only 19 cases and 5.2% of total numbers. Among the 19 cases, 11 cases were observed in complete cleft palate with cleft lip.

9. In blood type of cleft palate patients, A type was 37% O type was 32% B type was 25%, and 뮤 type was 6%.

10. Number of total associated anomalies of cleft palate. was 12 cases(3.3%). :

Pierre-Robin syndrome(4 cases), congenital Heart disease(3 cases), Tongue-tie(2 cases), Ear deformity(2 cases) and club foot(1 case).

11. In complete and submucous cleft palate, Palatolasty with 2 flap was applied.

and especially in bilateral type, palatoplasty with 4 flap was applied. And rarely,

on other department, Von-Langen-beck palatoplasty was done.

12. 10 cases among 362 cases of cleft palate was operated at the age of proper time which was between 1yr. and 2yr. And 60 cases was operated at the age of over 10yr.

13. The patients who were operated at adequateime of operation, was revealed excellent post-operative speech evaluation, but poor post-operative result was obtained at aged patients.

l4. The total number of transfused group during operation was 87 cases(4.3%), and the mean amount of transfused blood web about 8.6ml/kg. And change of Hb. and Hct. was statistically insignificant in pre-operative data and postoperative data.

15. The average period of hospitalization was from 6 days to 15 days and the percentage was revealed to 96.4%. Within 5 days of period was noted in 4.9% of total cases.

16. Mortality was none and complications were 30 cases(8.3%); These were upper respiratory infection. Oronasal fistula, preumonia, bleeding and gastro-intestinal diseases.