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Ol-&L.:R· Oleii-&L· ~ or.2.J~ : ~11 i'! ~12 I 1995\1 ~.s;a~. ~9«!' ~~~. ~i.!~. ~.s;a71· ~«!.:r. ~~~. ~~~. ~-11~' ~~~ *~~. ~~.s;a . .2.9~· *9~' ol.!f-~· ol~~' ol~~' ol-&l.:r· olEII-&l· ~9~ ~AI~ . ~ ~~. ~~~. ~~ ~ . ~*~. ZEC~3\f' 2JS~9' ~e~· ~~~. t;.j~9· -5- ~ =Abstract = Esophageal Atresia and Tracheoesophageal Fistula in Korea - A National Sur very of Its Members by the Korean Association of Pediatric Surgeons - WH Park, SI Kwon, SC Kim, SK Kim, WK Kim, IK Kim, JE Kim, HH Kim, KW Park, YS Park, YT Song, JW Yang, SM Oh, SY Yoo, DS Lee, MD Lee, SC Lee, SK Lee, TS Lee, SI Chang, SY Chun, ES Chung, SY Chung, SE Chung. PM Chung, MH Cho, JS Joo, SO Choi, SH Choi, YS Huh, and CHong The Korean Association of Pediatric Surgeons The first national survey on esophageal atresia and tracheoesophageal fistula was conducted to access the current status of its incidence. clinical manifestation, preop­ erative diagnosis and management, type of its anomaly, associated anomalies, and surgical results and course. The 43 members of the Korea Association of Pediatric Surgeons received questionnaires and registration forms to be filled out on each pa­ tient who were born during the three years from January I, 1992 through December 31, 1994. Questionnaires composed of six broad areas which include 1) preoperative diagnosis and management, 2) surgical technic, 3) long gap, 4) postoperative manage­ ment, and 5) complications and courses. A total of 148 cases was returned by 28 members working at 23 institutions and 27 members returped questionnaires. We ob­ tained the following results by analysis of the 148 cases of tracheoesophageal anoma­ lies. The incidence of tracheoesophageal anomaly was about 1/10,000- 11,000 in 1994, which is one third of that of anorectal malformations in Korea and the distribution of the patients was almost proportionate to the size of each province. Both sexes are about equally affected. Majority of the members make diagnosis of tracheoesopha­ geal anomaly by taking a simple infantogram with a radiopaque tube in upper pouch and a little under half(46%) prefers to perform echocardiography as a part of preop­ erative management to identify congenital heart disease and lateralize the aorta. - 149 - - :t.0 l-.21}!j- : ;IJ]l i'! ;IJ] 2 ~ 1995\1 - Esophageal atresia with distal TEF(87.50/0) was by far the most common and threre were pure esopahgeal atresia(5.6%), H-type TEF(2.l%), and so on. About half(49%) of the patients had one or more associated anomalies in addition to tracheoesopha­ geal anomalies. Congenital heart disease was associated in 46 cases(31 %), anorectral malfomations in 19 cases(13%), musculoskeletal anomalies in 15 cases(10%), gen­ itourinary anomalies in 10 cases(7%) and gastrointestinal anomalies in 7 cases(5%). Postoperatively, parenteral nutrition and assisted ventilation were given in 66% and 52% of patients respectively. Ninety three(74%) of 126 cases who underwent sug:ical procedure, experienced one or more complications such as respiratory complication (65%), leak(22%), stricture(21%) and so on. The survival rates related to the Waterston risk categories were 90.2% in grpup A, 71.4-75% in BI, B2, and C groups, and 28% in group C2 and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/ sepsis(12 cases), respiratory failure(l2 cases), and con­ genital heart disease(4 cases). With short term follow-up, 69% of patients have been excellent whereas remainders of the cases have suffered from some sort of morbidi­ ty related to gastroesophageal reflux, recurrent respiratory infection, and esophageal stricure. Index Words: Esophageal atresia, Tracheoesophageal fistula, National survey, Korea (Registration forms) ~ .!i!.t.l1~c}- . ~o} 'g~ rJl->-J ,~ 1992\1 1-%1. 191oj\Al 1994\1 12~ 3191 71\7.\ ~ 3\'1{} 7]-t!-~x7]-&2.Q.£ ~,\~~ ~o}£ -6}';'ctJl 1994\'15:. rJl~5:o}9.j~~§j -&-oa~~§joj] A-l. ~ T~~Jl §j~~ ~0}5:. Y"y 15mc}-. ~-lf-7.]-c Z{ c:jy-2} 7]-t!-~5:.7]-&2oj] -t!-"0 ~~~, ~1±, ~~~-l §j~o] <Q-->-d15}x~ 15}Jl ~o}%~.g. ~~ 1±~ _£ ~], ~]li, ~~-t!-c:j, W~~, ~ 7J~%oj] rJl~ ~l(} 15mc}-. "'-J{E-7.] 57}7.] 5:T-~]£ Y-To-jh1 <Q-->-d~~ ~o]Jl %-~1]~'?l 7J"6J~ ~JlA-l ~~oj] {}AR~ ~ -Cl:l] ::I t.l1%~ .!i!.1i:1 CD ~{i ~1± ~ ~-l~]' @ T §j §j~~ rJl->-J.Q.£ ~A}~ -6}<:x1, ll ~ l- rJl~5:0l-9.j ~~ 7 1. Q) ~{}z:j (long gap), @ ~ ~ ~-l~l. rID ~~§j ~~rJl§j "-T'-~].£-W' ->-]{}oj] ~.R .£9.J-6}7] ~ ~ W~'6 ~ 7J~£ T"'-d~o-j ~c}-. £ inJ~~ c}-. 1:l]Jil~ S~ ~s:.7]-&2oj] -t!-~ .!i!.Jl Table 1. Geographic Distribution ~ ~ 7R'?l01l-J- ~~~9.J .!i!.Jl.!i!.c}-~ ~~iT.£.9.J Area No.of patient Perceni( %) Birth in '93 -lt6jo] .!i!.c}- ~A 7Jj-B-~0]Jl .!i!.Jt!~'tl A}li~ ~ +&- 37 25.0 24.5 % T ~c}-~ 7.J~ol ~.Q.c:j2} '\~Z{~vj, Ef.~ 0]e1 ~::B, 75 7] 39 26.4 24.5 ~ ~Al-~T9.J ~lljj~ 0] oj] g -6}-c §j~!§-9.J ~~ 7J-~ 4 2.7 2.7 oj] '€:t:;l ~ c}-Jl ~ T ~ q. 0] Jtl 7 1%~ 5:.7]-&2 oj] -¥-{]:, 75 '4 23 15.6 17.1 -t!-~ ~Al-oj]A1~ ~c:j42} {i7.]Q;joj] ~-c §j~~o] t:llT,75* 19 12.8 10.3 ~2f-, ~* 4 2.7 3.7 %1ft~ 5Jl -T'-o-jAi ~ ~Al A}lioj] rJl~ ~~5:.~ * ~~ , ~'J 9 6.1 3.7 ~ T ~~c}-Jl '\~Z{¥!q. t:ll~, %'4 4 2.7 3.1 0""1*'" 3 2.0 2.9 ;IJ]2f- 3 2.0 1.2 Not mentioned 3 2.0 0 Total 148 100.0 100.0 rJl~ 5:0l-9.j~~§j :§:j~ 43~oj]71] 7 ]-B-~5:. 7 ]-&2 '4 : 1-1 = 1 : 1.1 oj] -t!- ~ "'-J -If-7.] (Questionnaires) 9-J- ~o} %~7.] - 150- 43"3 ~~ % 23t!J~$.J 30"3°] A}R ~ ..li.41 T Table 3. History of Maternal Polyhydramnios jr:J-. 7]:>&~.£7]~~0l- ;~ A}.li-C 28"30) ..li.4J Polyhydramnios No. of patient Percent( 0/0) r-932.-rlj % 148"3 01 ;:~S:19ir:t. ;:~Al~ ..li.41~ Yes 46 31 ~~ol 'V,': AI~E1 ~~T~ ..li.tB, .+~- 10, i'-~ No 13 9 ), q(-('- 3, /iftJ z, 7J~, 7J.*, -"Lhi-°] 44 <t!- ~ Not clear 89 60 ~2.-.5!.. AJ~A..:12.-.5!.. B]27...:j .2~1l1 *]l..~}.2 'V93q. Total 148 100.0 J.,IYAl ~ 27"3$.J ~~ol .5!..1.J1 "r93t:-j-. %~~AHi. Table 4. Major Presenting Symptoms(N=148) ~ 713':..5!.. 71.i'dA-l.£71~$.J \oIJ-J.~-t, ~}.J-, AJrJ, 7] Sx. No. of case ~$.J *~, q %~71~, ~ ~ ~i;7.l, "Jt!J~, cjH·, Excessive salivation 98 A}OJ~ oJ, ~:i!J-;:~ %AJ ~~.5!.. *~i;}'3.1~o:j AJ~ Respirator}' difficulty 62 AI *J..:j ~:i!J-9} TA1].£$.JAl £..9.1~ A}%~ .::il0,}<>11 Cyanosis 49 Ai T.5!.. t:·P!L93q; Feeding problem as choking 36 A.bdominal distention 17 Associated disease 8 Miscellaneous. 11 Recurrent pnemonia (3) Others (8) Tflble 5. Diagnostic Procedures (N =:= 148) - 143~ojH A(j~ *~ol 7}~"6}S1,~o:j 'ito} 69 Methods No. 0/0 Infantogram with a radioopa· 128 86 ~, cxjo} 73~.2..s. 'it1.-1$.J l:l17} 1: l.l01 9it+. Al~ que NG lube ~ -l't!f~ .!t.1i'1 '?l~ 7cl71 39~ (26.4%), AH: 37 Infantogram with constrast 40 27 ~ (25%), ?g'if 23~ 05.6%), I11~ ?g.Ji1- 19 ¥-{} media in upper pouch (12.8%)tt'- o]9i~vj, ~.::il~ 1993\'15:. ~J.H}-8 l~chocardiography 51 34 ",~ot$.J ~*%4 71=t!-615:.71t§$.J 7,1~~ 1l.J1*%~ Itenal ultrasonogram 51 34 l:ll~~ o,VJ% .!t.S1,t+OE 1). ~.s::.~ 71=t!615:.71t§ I~sophangogram 7 13 9.J ~"'~-{t3l.,': If 29} iJq. Others 5 3 A Table 2, Annual Incidence by Year S:)9i~vj. 9'F J.g. T7J~l:l1% J1}t+, ~*~'Cl. ;<,3 ~~, y*~'Cl~ 9.) tt'-°)':(lqUI 3, 4). ~td.2..£ Year Birth No. Patients Incidence 1992 723,927 39 1/18,562 13JA}-{:l l:l11f-4"'(j 1l'tl- .1- tdtt'-~od~ ~4-7l- 128~1 1993 702,546 43 1/16,338 (86%),6Js:.~03~ol 40~](27%), {l.&g31j-{lA} 1994 (702,546) 66 1/10,645 51~1(34%);:ol ol%soJ':(lr:}(lf 5). 148 1/14,385 * Hirschaprung's disease in 1992: about 190 cases reported in Korea * Imperforate anus in 1993 " 143 caSes reported in -615:.71t§$.J %~ ~ ~~, 6j£4L~~ '3c! '6Pf 7] Korea( estimated in cidence : 1/3,456) W61.s::.T--%(Esophageal atresia with distal tra­ cheoesophageal fistula) 0] 126~ (87.5%), tr-'T ~5:..!j'--%~o] 8~ (5.6%) H-~ 71~-61.s::.T-~ol 3 ~ (2.} %)%$.J tt'-°l~.2..vj, l:ll13AJ?;j~ ~ (unusu­ al type) 3~~ 2t§ t AJlj!·.!j'--tt~ol 9J.::il i;}1jL71~ -151- 6js:.-r-tt 0] .y 7 ]~ A ] ~ A]1fL ~ (carina )oj] ~ ~Jl , Table 7. Incidence of Other Congenita l Anomalies t-j-Pj A] 1 tg ~ "J1fL6J s:.1¥--tt -'6- 1fL~ ~ .y 7] ~ A] ~ Anomaly No. KAPS series Holder series 1fL7} ~%~ (common wa ll) -£- 5:* -6} ~ -r-tt 0 ] \it CHD 46 31 % 19% ~ ~Ell~q(lE 6). Imperforate anus 19 13% 9% 148tg% 73tg(49%)oJ]Ai ~7}A ] o] "J~ 7 ]~0] Musculoskeletal 15 10% 9% Genitourinary 10 7% 10% %It!~ ~:: t~], 11 ::'B"a {l 7.J7] t§ 0] 46 ill] (31 %), "B'J-l!:­ Gastrointestinal 7 5% 13% -3J AJ7]~ 19 ill] (13%), -ctt2j 7 ]~ 15 ill] (10%)% CNS 6 4% 6% 9.J 'i'°]~q(lE 7).
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