Atlas of Esophageal Atresia By Dietrich Kluth (Translated by Michael W. L. Gruderer) TYPE I
Definition: Belonging to Type I are all the cases of esophageal atresia in which a distal esophagus cannot be demonstrated as a hollow viscus above the diaphragm.
Ph~'~~l'irOel"~~iil BrL?-~~Br ~,0 I1 Sonderland (1820) Lozach (1816)
12 Brodie ( 1810) Anders (cited by Fischer, 1926)
i 1I 13 Durston (1670)
14 Vancryck (1824) Pinard (1873)
~176 Oe I Oel A
Oe2 ~
~ Is Cleaver (1892) Kraus (1902)
Ma Ie R~derer (1754)
Ir
DeMuth et al. (1957)
From the Department of Surgery of the Children's Hospital, Bremen, Germany. Address for reprints." Dr. Dietrich Kluth, Waetjenstr. 43, D2800, Bremen, Germany. 9 1976 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 11, No. 6 (December),1976
901
902
DIETRICH KLUTH
SUBTYPE I1--Agenesis of the entire esophagus. Br--Bronchus, LawLarynx, Ma--Stomach, Ph--Pharynx, Tr--Trachea SUBTYPE 12--Agenesis of the distal part of the esophagus. Oel--Proximal esophageal blind pouch. SUBTYPE 13--Extremely long proximal blind pouch. Agenesis of the distal esophagus. SUBTYPE 14--Total atresia of the esophagus. Ph--Pharynx, A--Esophagus in the form of an atretic strand. SUBTYPE 15--Atresia of the distal esophagus (A). Oel--Proximal esophagus. SUBTYPE 16--Atresia of the distal esophagus (A). The stomach (Ma) is missing. Oel--Proximal esophagus. SUBTYPE 17--Agenesls of the midportion of the esophagus. The distal esophagus (Oe2) is present as an atretic structure.
TYPE H
Definition: Belonging to Type II (of the esophageal malformations) are all the cases in which there are proximal and distal segments without a fistula.
Oel Fi
II 1 Pagenstecher (1829) Steele (1888)
112 Daum (1970)
113 Mason (1855) Jlott (cited by Kreuter, 1905)
Cy A
Mu Oe~
II4 Keith ( 1910)
II s
Matsumoto et al. (1972)
ATLAS
903
SUBTYPE II 1- Agenesis of the midportion of the esophagus. Oei--Proximal esophagus. Oe2--Distal esophagus. SUBTYPE 112--Same as Subtype II 1. Between the two segments there is a cyst (Cy) that communicates with the trachea through a fistula (Fi). SUBTYPE 113--The midportion of the esophagus is atretic (A), SUBTYPE 114--Same as Subtype II 3. A muscular strand (Mu) is present between the trachea and the diaphragm. SUBTYPE 115--Same as Subtype II 3 with a cyst (Cy) located in the atretic strand.
T Y P E Ilia
Definition: Belonging to Type IIIa are the atresias of the esophagus with tracheoesophageal fistula of the proximal segment only.
JFi
0
Oe ~ $ t
Ilia 1
Ilia 2
Ilia 3
Vogt (1929)
Dudgeon et al. (1972)
Mahour et al, (1971)
SUBTYPE Illal--Esophageal atresia with fistula (Fi) of the upper segment (Oel). Oe2--Distal segment. SUBTYPE Illa2--Same as Subtype Ilia 1 with an additional fistula (Fi I ) of the proximal segment. SUBTYPE IIla3--Same as Subtype Ilia 1. The distal segment of the esophagus exhibits a stenotic (St) a rea.
904
DIETRICH KLUTH
T Y P E Illb
Definition: Belongingto Type IIIb are the atresias of the esophagus with tracheoesophageal fistula of the distal segment only.
Oe~--~~I
~
~i~
~ e 2
Oe~
e~
IIIb 1 Gibson (1697) Martin ( 1821 )
IIIb2 Schiller (1838)
- Fi Oe,
Oe,
Oe,--~!~--Oe=
I
IIIb5 Rehbein (1972)
IIIb3 Howston (1830) Vincent (1887)
I
Oe~
~
jr" IIIb4 Holcomb & Daniel (1966) Bienayme (1967)
O~
Fi
Oe~ IIIb6 Dafoe & Ross(1960)
e,
IIIb7 Waterston et al (1962) Koop & Hamilton
IIIb8 Overton & Creech (1958)
(1968)
i
Fi, Cy
IIIb9 Tuqan (1962) Koop & Hamilton (1968)
IIIbl0 Humphreys et al. (1956)
Fi~
Cy IIIbl 1 Koop & Hamilton (1968)
IIib12 Roberts (1958)
ATLAS
905
SUBTYPE IIIb I --Esophageal atresia with fistula of the lower segment (Oe 2) with a short distance between the segments. (Oe 1)--Proximal esophagus. SUBTYPE I I I b 2 --Atresia as in Subtype IIIb 1 with a long distance between the segments. There are fibromuscular strands (Mu) that connect the segments (Mu) (can also happen in Subtype IIIb 1). SUBTYPE IIIb 3 --The proximal blind pouch (Oel) ends quite high. The fistula from the lower segment (Oe2) ends high on the trachea. There is no overlapping of the segments. SUBTYPE IIIb 4 - - T h e fistula from the distal segment (Oe2) ends high on the trachea. There is overlapping of the segments. SUBTYPE IIIb s --Same as Subtype IIIb 4. The fistula (Fi) is a long atretic strand. SUBTYPE I I I b 6 --Long proximal esophagus (Oe 1). The fistula from the distal segment ends at the usual (normal) site. There is overlapping of the segments. SUBTYPE I I I b 7 --Same as Subtype IIIb 1. The fistula (Fi) is closed at its connection w i t the trachea. SUBTYPE I I I b s --Same as Subtype IIIb 7 with an additional membranous atresia (Me) of the distal segment (Oe2). SUBTYPE nlb 9 --Same as Subtype lllb 1 with an additional stenosis (St) of the distal esophagus (Oe2). SUBTYPE IIIbl0--1nstead of a distal esophagus and a fistula, multiple cysts (Cy) are found. These are connected to each other by a fibretic band and are suspended between the cardia and the trachea. SUBTYPE IIIb11--Same as Subtype IIIb 1 with multiple cysts (Cy) present at the distal esophagus. SUBTYPE IIIb12--Esophageal atresia with two fistulas arising from the distal esophagus, both reaching the trachea. (Fi 1 and Fi2)
Oe, / Du--
Oe,
A
Oe
IIib13 Devens & Ritz (1965)
IIib14 Holden & Wooler (1970)
IIIbl s Keep & Hamilton (1968)
L a 9A
IIib16 Sayre & Hall (1954)
La Oe~
Tr
B
e2
Oe,
Tr. St Br
IIIbl 7 Sayre & Hall (1954)
IIIbl s Franklin & Graham (1953)
IIIblv Welch & Husain (1958)
Br A
IIIb20 De Buse & Morris (1973)
906
DIETRICH KLUTH
SUBTYPE IIIb13--Esophageal atresia with two fistulas arising from the distal segment. The first one (Fi 1) enters the trachea, the other one (Fi2) enters the right main bronchus. SUBTYPE IIIb14--The external continuity of the esophagus is maintained but the lumina are separated from each other by an atretic segment (A). The fistula arises below the upper end of the distal esophagus. SUBTYPE IIIbls--Same as Subtype IIIb 1 with the proximal esophagus existing as duplication (Du). SUBTYPE IIIb16--Same as Subtype IIIb 1 with stenosis of the larynx (La St). SUBTYPE IIIb17--Same as Subtype IIIblwith atresia of the larynx (La A). SUBTYPE IIIbls--Same as Subtype IIIb 1 with additional tracheal stenosis (Tr St) below the entrance of the fistula in the trachea. SUBTYPE IIIb19--High atresio of the esophagus (Oel). Between the larynx and the stomach there is a tube with tracheal (Tr) and esophageal (Oe2) characteristics. From this tube arise two normal bronchi (Br). SUBTYPE IIIb20--Esophageal atresia with fistula from the distal segment and additional atresia of both main bronchi (Br A) as well as agenesis of both lungs.
TYPE Illc
Definition: Belonging to this group are the cases (of esophageal atresia) with fistulas from the proximal and distal segments.
Oe, [ Oe
FLFi:A?
Oe~
IIIc 1 V. D. Water (1857) B. Fischer (1905)
IIIc2 Krediet (1955)
••'•F
~
IIIc4 Rehbein (1973)
FI2
=~F,1
FL ' FOel~i 2//.,~ ___.Fi, Oe~,~
IIIc3 Holden & Waoler (1970)
Oe,
--
La.A
--
Fi~
--
Fi~
Oe~
IIIcs Heineken (1952)
ATLAS
907
SUBTYPE IIIcl--Esophageal atresia in which there is a fistula from the proximal esophagus (Oe 1) and the distal esophagus (Oe2) , each one communicating with the trachea. (Fi 1 and Fi2). SUBTYPE IIIc~--Between the upper esophagus (Oe 1) and the lower esophagus (Oe2) there is a short atretic esophageal segment (A). Both segments have a fistula (Fi 1 and Fi2) communicating with the trachea. SUBTYPE IIIc3--Same as Subtype Illc 1 except for the proximal fistula (Fil) that exhibits an atretic segment. SUBTYPE IIIc4--Same as Subtype IIIc 3 with an additional atretic segment in the distal fistula (Fi2). SUBTYPE IIIcs--Besides the atretic Subtype IIIcl, there is a laryngeal atresia (La A).
T Y P E IV." M e m b r a n o u s atresias
Definition: The esophagus exhibits external continuity, but its lumen is divided into a proximal and a distal segment by a transverse or oblique, purely mucous, m e m brane.
V
Me
Fi Me-~
Fi Me
M
i1
J IV 1 Abel (1928) Schwartz (1962)
IV 2 Haight (1957)
Me
Me~ Fi
IV s Matsumoto et al. (1972) Azima & O'Hara (1973)
IV 3 Kate, ten (1952)
Fi, rl\~
IV 6 Goldenberg (1960)
IV4 Lister (1963)
Me Fi
Fi~
|V 7 Touloukian (1973) Rehbein (1973)
908
DIETRICH KLUTH
SUBTYPE IV1--The lumen of the esophagus is interrupted by a transverse membrane (Me). There are no fistulas. SUBTYPE IV2--The esophagus is closed by a transverse membrane (Me). Proximal to the closure is a stenosis (St). SUBTYPE IV3--The mucous membrane does not occlude the esophagus completely. Its ventral part is pulled upwards providing a fistulous tract (Fi) between the proximal and distal segments. SUBTYPE IV4--The proximal and distal esophagus are divided by a moderately oblique membrane. The distal esophagus communicates with the trachea through a fistula just below the ventral insertion of the membrane. SUBTYPE IVs--The fistula (Fi) ends relatively high in the trachea. The membrane has a very oblique course (Me). SUBTYPE IV6--There are two fistulous tracts (Fi 1 and Fi2) to the trachea. One of the tracts is proximal and the other one is distal to the membrane (Me). SUBTYPE IV7--The membrane (Me) is at the level of the tracheo-esophageal fistula (Fi). It occludes the lumen of the esophagus and penetrates the fistulous tract. Both segments communicate through the fistula.
T Y P E V: Complete and partial duplication of the esophagus.
Atresia with intramural duplication. Definition: Besides the patent esophagus, there is a second one that may show a variable length and is in communication with the main lumen or the stomach. The atresia with intramural duplication is morphologically similar to the atresia by membrane, but the dividing wall between the segments also contains a layer o f muscularis.
Du
Dul
v~
Gi~rup (1933) Butler & Ende (1950)
Du
v~ Blasius (1674) Frank & Paul (1949)
Du
Mu
v3
v4
Kathe (1907) Ciechanowski & Glinski (1910)
Maier (1957)
ATLAS
909
v
i
Oe~_ _
Oe~ j Vs Yahr et al. (1962)
Oe~ ~ Mu Fi -Oe~ Oe~-~ ~-St
V6 Minnis et al. (1962) Wiersema (1956)
Vz Bienayme (1967)
SUBTYPE V i - - T h e esophageal duplication is present throughout the whole length of the esophagus (Du). SUBTYPE V2--Only a part of the esophagus is duplicated (Du). SUBTYPE V3--The duplication (Du) is a submucous tract that is present in the ventral part of the esophagus. SUBTYPE V4--Only a short segment of the esophagus is duplicated. The duplication is a structure resembling a diverticulum but it does not have the characteristics of a traction or pulsion diverticulum. SUBTYPE Vs--There is a fairly long overlapping of the proximal blind pouch (Oel) and the distal esophagus (Oe2). Along practically the whole segment of duplication, both segments share a common muscular wall (Mu). SUBTYPE V6--There is a muscular septum (Mu) between the proximal (Oel) and the distal (Oe2) segments of the esophagus. The fistula arising from the distal segment ends high on the trachea. SUBTYPE VT--This Subtype is similar to the preceding one, but the fistula (Fi) is not patent and the muscular membrane is pierced, forming a stenosis (St).
910
DIETRICH KLUTH
TYPE
111."Esophagobronchial communications.
a - - E s o p h a g o b r o n c h i a l fistulas. b--Bronchoesophageal fistulas. Definition (a): The fistula coming from the esophagus reaches a main bronchus or a secondary (sequential) bronchus. It may also communicate with an intra or extra lobar pulmonary sequestration. It may even replace a main bronchus.
Fi
Oe~F~ .lli~o~i~
,~.~ Fi
Vial Heiderich
Via 2 Moscarella & Wylie
(1916)
Via 3 Ellerbroek (1922)
Via 4 Gans & Potts
(1968)
(1951)
Morton et al. (1950)
Fi
~'.A Via s Nikaidoh & Swenson ( ! 971 )
Tr.St
agLu~
T~
~
Via 6 Toyama (1972)
Eu Via 7 Hanna (1964)
~ Vla$ Kee|ey & Schairer (1960)
SUBTYPE V i a l - - T h e fistula (Fi) enters the main or lobar bronchus a short distance from the esophagus. SUBTYPE Vla2--The fistula (Fi) enters isolated "ectepic" pulmonary tissue (Lu). SUBTYPE Vla3--Esophageal atresia in which the proximal esophagus (Oel) ends with a fistulous tract (Fil) in a main bronchus. The distal esophagus (Oe2) originates with its fistula from the trachea. SUBTYPE Vla4--Total sequestration of the lef t upper lobe, which is also aplastic (ap. Lu). This sequestered segment communicates with the esophagus via a fistula. SUBTYPE Vlas--"Ectopic origin" of the right aplastic lung (ap. Lu) from the esophagus by a fistula (Fi) with atresia of the right main bronchus (Br.A). SUBTYPE Vla6--Esophageal atresia with "ectopic origin" of the right aplastic lung (ap. Lu) from the distal esophagus (Oe2). There is also a tracheal stenosis (Tr.Sto) below the entrance of the fistula and atresia of the right main bronchus (Br.A).
ATLAS
911
SUBTYPE VlaT--"Ectopic origin" of the right lung from a fistula (Fi). The trachea ends directly in the left main bronchus (Br.) without a bifurcation. SUBTYPE Vlas--Esophageal atresia with "ectopic origin" of the right lung from the distal esophagus (Oe2). There is a long segmental stenosis of the trachea (Tr.St,) which ends directly in the left main bronchus without a bifurcation.
Definition (b): Esophageal atresia with origin of the distal esophagus from a bronchus.
Tr Oe~ ~ Oe~--
Oe~ Fi
~
r -
O
e
Oe~
Cy
Br
=
9
e
~"
VIb] Levy (1845)
Vlb2 Roe & Nobis (1963)
Vlb3 Willich & Rehbein (1965)
VIb4 Rehbein
SUBTYPEVlbl--The proximal blind pouch (Oel) ends at the level of the tracheal bifurcation. The distal esophagus (Oe2) originates as a fistula (Fi) from the right main bronchus. SUBTYPEVIb2--The proximal esophageal pouch (Oel) is longer than usual. The distal esophagus (Oe2) originates from the left main bronchus (Br). SUBTYPEVIh3--Esophageal atresia with the right main bronchus dividing into a cystic structure (Cy) and the beginning of the distal esophagus (Oe2). Fi--fistula between the right main bronchus and the distal esophagus. (La--Larynx; Tr--Trachea). SUBTYPEVIb4--Esophageal atresia in which the right main bronchus (Br) goes directly in the distal esophagus (Oe2). Br.Oe--brenchoesophageal transition.
T Y P E VII: H Fistulas
Definition: Fistula (Fi) between the esophagus and the trachea. No atresia.
Fi
Fi~ ~- FJ2 ~L~Fi~ ~Fi~ St
Fi
Vll I
Vl12
Vll 3
Vll 4
Lamb (1873) Pinard ( ! 873)
H~Jbner (1943) Leven et al. (1952)
Eckstein & Somasundaram (1966)
Moyson (1970) Stephens (1970)
9t2
DIETRICH KLUTH
St
~/~! -- Fi
Di
VII s Brenner (1892)
Fi
---Fi
Di
VII 6 Knox ( 1951 )
VII 7 Robb (1952)
VII1--One tracheoesophageal fistula (Fi) between esophagus and trachea. VII2--Two tracheoesophageal fistulas (Fi 1 and Fi2). VII3--Three tracheoesophageal fistulas (Fil, Fi2 and Fi3). VII4--Besides a tracheoesophageal fistula (Fi), there is a stenosis of the esophagus (St) distal to the fistula. SUBTYPE VIIs--The esophageal stenosis (St) is proximal to the fistula. SUBTYPE VII6--The esophagus presents an outpauching (Du) at the level of the fistula (Fi). SUBTYPE VIIT--The esophagus has a double lumen distal to the H-fistula (Fi). The dorsal lumen (Di) ends blindly. SUBTYPE SUBTYPE SUBTYPE SUBTYPE
T Y P E VIII: Stenoses
Definition: Narrowing of the esophageal lumen of congenital origin. No evidence of signs of inflammation.
Me St
j
~,, St
VIII 1 Bonilla & Bowers (1959)
Oe,
St ~
VIII 2 Budgen & Delmonico (1956) Adler (1963)
\
VII| 3 Handousa Bey (1949) Greenough (1964)
~
St~
I
VIII 4 Jewsbury (1971)
ATLAS
913
Cy St
VIii s Vargas (1956)
Vii| 6 Soave & Mezzano ( 1965 ) |shida et al. (1969)
VIii 7 Hecker (1968)
SUBTYPE Villi--Segmental stenesis. The esophagus is stenotic (St) over a certain segment. SUBTYPE Vlil2--Esophageal stenosis produced by a ring or a semicircular mucous membrane (St). If this membranous stenosis is in the lower third of the esophagus it is called "lower esophageal web." SUBTYPE Vl|13--There are two simultaneous stenoses. One is segmental (St) and the other one is membranous (Me). SUBTYPE Vill4--1n this form there is a morphological similarity to the Type Inb. There is a communication between the upper and the lower esophageal segments which is considerably narrower than these segments, acting as a stenesis. (St)--stenetic communicating segment. SUBTYPE VIIIs--Stenosis above the cardia produced by muscular hypertrophy (Mu). This is also known as "lower esophageal ring." SUBTYPE VIII 6 - Stenosis produced by inclusion of trachea-bronchial rests (St). SUBTYPE VIIIT--The lumen ef the esophagus is narrowed by a submucosal cyst (Cy), preducing a stenosis. (St)
T Y P E IX: Tracheal atresias.
Definition: Agenesis or atresia of the trachea combined with tracheoesophageal fistula.
r. A
iX~ Devenis & Otis (1957) Josh| (]969)
|X 2 Fritz (1933) Sand|son (1955)
Fi
IX 3 Peison et al. (1920)
Tr. A
IX4 Walcher (1928) Floyd et al, (1962)
914
DIETRICH KLUTH
SUBTYPE IXl--Total agenesis of the trachea. The larynx (La) is normal. Both main bronchi (Br) are normal and form a bifurcation that communicates with the esophagus by a fistula (Fi). SUBTYPE IX2--Tracheal atresia in which this organ sometimes can be identified as an atretic strand (Tr.A) orbital to a normal larynx. Distally the trachea (Tr) is normal, dividing into two main bronchi. Between the esophagus and the normal segment of the trachea there is a fistula. SUBTYPE IX3--Similar to Subtype IX 2 with additional atresia of the larynx. (La.A) SUBTYPE IX4--Agenesis of the trachea. It is possible that there is tracheal tissue (Tr.A) below the normal larynx (La). Both main bronchi arise from the esophagus. (Br)
TYPE X." Fissure formation. The forms of Type X are divided in four groups according to the depth of the fissures (after Pettersson, 1969). Group Xa--Fissure formation at the level of the larynx with and without esophageal atresia. GroupXb Fissure formation at the level of the larynx and the proximal trachea with and without esophageal atresia. Group Xc--Total fissurization, involving the larynx and the trachea down to the level of the bifurcation. Group Xd Fissure at the distal level of the trachea. The larynx and the proximal trachea are normal. Group Xa:
JSp
Sp
d
L Xa! Finlay (1949)
I Xa 2 Cameron & Williams (1962) Delahunty & Cherry (1969)
Xa 3
Jahrsdoerfer (1967)
ATLAS
915
La.St Sp Oe~
Oe_~_="~Sp
Oe2 Xa 4 Cameron & Williams (1962) Delahunty & Cherry (1969~
XQ5 Delahunty & Cherry (1969)
SUBTYPE X a l - - T h e fissure (Sp) between the esophagus and the larynx is not complete. The arytenoid incisura is caudally deepened and leads to the formation of the fissure~ SUBTYPE Xa2--Simple laryngeal fissure (Sp) i The formation of the fissure does not reach the level of the trachea. SUBTYPE Xa3--Same as Subtype Xa 2. There is esophageal musocal tissue in the fissure (Oe.Sc) SUBTYPE Xa4--Same as Subtype Xa 2. In addition there is esophageal atresia of the Subtype IIIb 1. SUBTYPE Xas--Same as Subtype Xa 2. In addition there are esophageal atresia of the Subtype II 1 and a subglottic stenosis of the larynx (La.St).
Group XO:
liSp [~~SpOe,/SPi="~ Oe~~~~FI' ~L~Br'St Oe~-~l~. !!
Xbl Imbrle & Doyle (1969) Geiger (1970)
Xb 2 Blumberg et al. (1965)
Xb 3 Oaum et al. (1965) Donahoe & Hendren (1972)
sp
Xb 4 Mahour et al. (1973)
SUBTYPE X b i - - T h e fissure (Sp) goes beyond the limits of the larynx into the upper level of the trachea. SUBTYPE Xb2--Same as Subtype Xb I. In addition there is a stenosis of both main bronchi (Br.St). SUBTYPE Xb3--1n addition to the fissure (Sp) that extends into the upper level of the trachea, there is an atresia of the Subtype I|lbl, with fistula of the lower segment. SUBTYPEXb 4 - Corresponds to the Subtype Xb 3. In addition to the fissure formation and the atresia of the esophagus with fistula (Fi3) from the lower segment, there are two more fistulas (Fi I and Fi~) from the proximal segment.
916
DIETRICH KLUTH
Group Xc:
- -
~
Sp
Sp
~
N.I_
J Xcl Frates (1967) F~disch & Sauer (1972)
Xc2 Stolze (1952)
SUBTYPE Xcl--The fissure (Sp) goes from the larynx to the carina. There is therefore a complete fissure between the esophagus on one side and the larynx and the trachea on the other side. SUBTYPE Xc2 - Besides the complete fissure (Sp) there is an accessory lung (N.L) that communicates with the alimentary tract by a fistula to the stomach (Ma).
Group Xd:
Xd~ Rehbein (1974) SUBTYPE Xd~--In the lower segment of the trachea there is a large communication with the esophagus (Sp). Proximal to this fissure there is a fistula (Fi).
ATLAS
917 REFERENCES
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Surgical management of near-total esophageal atresia. AMA Arch Surg 75:813-816, 1957 23. Devenis AM, Otis RD: Tracheal atresia associated with tracheoesophageal fistula. J Thorac Surg 34:405-408, 1957 24. Devens K, Ritz O: Eine bisher unbekannte Form der ()sophagusatresie. Z Kinderchir 2:484-486, 1965 25. Donahoe PK, Hendren WH: The surgical management of laryngotracheoesophageal cleft with tracheoesophageal fistula and esophageal atresia. Surgery 71:363 368, 1972 26. Dudgeon DL, Morrison CW, Woolley, MM: Congenital proximal tracheoesophageal fistula. J Ped Surg 7:614-619, 1972 27. Durston W: cited by Kreuter, E 28. Eckstein HB, Somasundaram K: Multiple tracheoesophageal fistulas without atresiareport of a case. J Ped Surg 1:381-383, 1966 29. Ellerbroek N: Ein Beitrag zur Oesophagusatresia. MUnch Med Wschr 69~.591, 1922 30. Finlay HVL: Familial congenital stridor in infancy. Arch Dis Childh 24:219 223, 1949 31. Fischer B: Cited by Fischer, W 32. Fischer W: Die MiBbildungen der SpeiseriShre in Henke F, und Lubarsch O: Handbuch der speziellen pathologischen Anatomic und Histologie, vol 1 Springer, Berlin, 1926, p 84 33. Floyd J, Campbell DC, Dominy DE: Agenesis of the trachea. Amer Rev Resp Dis 86:557-560 (1962) 34. F~Sdisch H J, Sauer H: Die ~Ssophagolaryngotracheale L~ingspalte. Zblatt f Allg Path 116:372 379, 1972 35. Frank RC, Paul LW: Congenital reduplication of the esophagus. Radiology 33:417419, 1949 36. Franklin RH, Graham AJP: Atresia of the esophagus with an abnormal tracheal fold. Thorax 8:102 103, 1953 37. Frates RE: Roentgen signs in laryngotracheoesophageal cleft. Radiology 88:484-486, 1967 38. Fritz E: Eine seltene Mi/$bildung der oberen Luftwege. Virch Arch Path Anat 289: 264-269, 1933 39. Gans SL, Potts W J: Anomalous lobe of lung arising from the esophagus. J Thorac Surg 21:313-318, 1951 40. Geiger JP, O'Conell T J, Carter SC, Gomez AC, Aronstam EM: Laryngotrachealesophageal cleft. J Thoracic Cardiovasc Surg 59:330-334, 1970
918
41. Gibson T: cited by Cautley E 42. Gjt~rup E: Un cas d'oesophage double et estomac double. Acta Paed Scand 15:91 98, 1933 43. Goldenberg IS: An unusual variation of congenital tracheoesophageal fistula. J Thoracic Cardiovasc Surg 40:114 116, 1960 44. Greenough WG: Congenital esophageal strictures. Amer J Roentgenol 92:994-999, 1964 45. Haight C: Some observations on esophageal atresias and tracheo-esophageal fistulas of congenital origin. J Thorac Surg 34:141-172, 1957 46. Handousa Bey AS: Some rare anomalies of the oesophagus with discussion of their aetiology. J Laryngol Otol 63:217 224, 1949 47. Hanna ED: Bronchoesophageal fistula with total sequestration of the right lung. Ann Surg 159:599-603, 1964 48. Hecker W Ch: Osophagusstenosen im Kindesalter und ihre Therapie. Mtinch Med Wschr 36:2026 2033, 1968 49. Heiderich: Cited by Mullard M 50. Heineken H: Beitrag zur angeborenen Kehlkopfatresie. Frankf Zzchr Path 63:30-39, 1952 51. Holcomb WH, Daniel RA: Unusual tracheoesophageal fistulas with membranous atresia of the middle or distal esophagus. Surgery 59:1112-1119, 1966 52. Holden MP, Wooler GH: Tracheooesophageal fistula and atresia: Result of 30 years experience. Thorax 25:406 412, 1970 53. Howston: cited by Kreuter, E 54. Hiibner O: Doppelte angeborene Oesophago-Trachealfistel ohne Oesophagusatresie Zblatt Allg Path pathol Anat 80:358-362 (1943) 55. Humphreys GH, Hogg BM, Ferrer J: Congenital atresia of esophagus J Thorac Surg 32:332-343 (1956) 56. Imbrie JD, Doyle PJ: Laryngotracheoesophageal cleft Laryngoscope 79:12521274 (1969) 57. Ishida M, Tsuchida Y, Saito S, Tsunoda A: Congenital esophageal stenosis due to tracheobronchial remnants J Ped Surg 4:339-345 (1969) 58. Jahrsdoerfer RA, Kirchner JA, Thaler, SU: Cleft larynx Arch Otolaryng 86:82-87 (1967) 59. Jewsbury P: An unusual case of congenital oesophageal stricture Brit J Surg 58:475476 (1971) 60. Jlott: cited by Kreuter, E
DIETRICH KLUTH
61. Joshi VV: Tracheal agenesis Amer J Dis Child 117:341-343 (1969) 62. Kate J: Congenital diverticulum of the oesphagus Arch Chir Neerl 4:277-282 (1952) 63. Kathe H: Partielle Verdopplung der SpeiseriShre Virch Arch Path Anat 190:78-92 (1907) 64. Keeley JL, Schairer AE: The anomalous origin of the right main bronchus from the esophagus Ann Surg 152:871-874 (1960) 65. Keith A: Obstructions and occlusions of the alimentary tract of congenital or obscure origin Brit Med J 1:301-305 (1910) 66. Knox G: Congenital tracheo-esophageal fistula without atresia Surgery 30:1016-1020 (1951) 67. Koop CE, Hamilton JP: Atresia of the esophagus: factors affecting survival in 249 cases Z Kinderchir 5:319-329 (1968) 68. Kraus F: cited by Kreuter, E 69. Krediet H: A case of congenital atresia of the oesophagus with two oesophago-tracheal fistulas Arch Chit Neerl 7:265-268 (1955) 70. Kreuter E: Die angeborenen VerschlieBungen und Verengungen des Darmkanals im Lichte der Entwicklungsgeschichte Deutsche Zschr Chir 79:1-89 (1905) 71. Lamb DS: A fatal case of congenital tracheo-esophageal fistula Med Times 3:705 707 (1873) 72. Leven NL, Varco RL, Lannin BG, and Tongen LA: The surgical management of congenital atresia of the esophagus and tracheoesophageal fistula Ann Surg 136:701-719, 1952 73. Levy C: Zwei F~ille yon sackfOrmiger Verschlie/~ung NZ Geburtsk 18:436-449 (1845) 74. Lister J: An unusual variation of oesophageal atresia Arch Dis Childh 38:176179 (1963) 75. Lozach: cited by Cautley, E 76. Mahour GH, Johnston PW, Gwinn JL, Hays DM: Congenital esophageal stenosis distal to esophageal atresia Surgery 69:936-939 (1971) 77. Mahour GH, Cohen SR, Woolley MM: Laryngotracheoesophageal cleft associated with esophageal atresia and multiple tracheoesophageal fistulas in a twin J Thoracic Cardiovasc Surg 65:223-226 (1973) 78. Maier HC: Intramural duplication of the esophagus Ann Surg 145:395-403 (1957) 79. Martin: cited by Kreuter, E. 80. Mason: cited by Kreuter, E 81. Matsumoto Y, Ogawa K, Yamamoto T, Kimura K, Kawai Y: Extremly rare types of esophageal atresia: Two case reports of mem-
ATLAS
branous atresia and multiple atresia of the esophagus Surgery 71:795-800 (1972) 82. Minnis JF, Burko H, Brevetti G: Segmental duplication of the esophagus associated with esophageal atresia and tracheoesophageal fistula Ann Surg 156:271 275 (1962) 83. Morton DR, Osborne JF, Klassen KP: An apparently congenital bronchoesophageal fistula persistent to adult life J Thorac Surg 19:811-816 (1950) 84. Moscarella AA, Wylie RH: Congenital communication between the esophagus and isolated ectopic tissue J Thoracic Cardiovasc Surg 55:672 676 (1968) 85. Moyson F: St6nose oesophagienne cong6nitale et fistula trach6o-oesophagienne cervicale Ann Chir In fant 11:179-183 (1970) 86. Mullard KS: Congenital tracheoesophageal fistula without atresia of the esophagus J Thorac Surg 28:39-54 (1954) 87. Nikaidoh H, Swenson O: The ectopic origin of the right main bronchus from the esophagus J Thoracic Cardiovasc Surg 62:151160 (1971) 88. Overton RC, Creech O: Unusual esophageal atresia with distant membranous obstruction of the esophagus J Thorac Surg 35:674-677 (1958) 89. Pagenstecher: cited by Kreuter, E. 90. Peison B, Levitzky E, Sprowls J J: Tracheoesophageal fistula associated with tracheal atresia and malformation of the larynx J Ped Surg 5:464-467 (1970) 91. Pinard: cited by Kreuter, E 92. Rehbein F: personal communication 93. Robb D: Congenital tracheo-oesophageal fistula without atresia but with large oesophageal diverticulum Austr New Zeal J Surg 22:120-122 (1952) 94. Roberts KD: Congenital oesophageal atresia and tracheo-oesophageal fistula Thorax 13:116-129 (1958) 95. Roe BB, Nobis PD: Congenital tracheoesophageal fistula Amer J Dis Child 106:489491 (1963) 96. ROderer: cited by Kreuter, E 97. Sandison AT: Partial absence of the trachea with live birth Arch Dis Child 30:475477 (1955) 98. Sayre JW, Hall EG: Anomalies of the larynx associated with tracheo-oesophageal fistula Pediatrics 13:150-154 (1954) 99. SchiSller: cited by Kreuter E. 100. Schwartz SI: Congenital membranous obstruction of esophagus Arch Surg 85:480 482 (1962)
919
101. Soave F, Mezzano A: Kongenitale Oesophagusstenose durch Knorpeleir~schlu~3 in der Wand Z Kinderchir 2:487-492 (1965) 102. Sonderland: cited by Kreuter, E 103. Steele C: Case of deficient oesophagus Lancet 1888:764 104. Stephens HB: H-type tracbeoesophageal fistula complicated by esophageal stenosis J Thoracic Cardiovasc Surg 59:325-329 (1970) 105. Stolze E: Beitrag zur Pathogenese der intrathorakalen Nebenlunge Zblatt Allg Path 88:417 427 (1952) 106. Touloukian R J: Membranous esophageal obstruction simulating atresia with a double tracheoesophageal fistula in a neonate J Thoracic Cardiovasc Surg 65:191-194 (1973) 107. Toyama WM: Esophageal atresia and tracheoesophageal fistula in association with bronchial and pulmonary anomalies J Ped Surg 7:302-307 (1972) 108. Tuqan NA: Annular stricture of the esophagus distal to congenital tracheoesophageal fistula Surgery 52:394-395 (1962) 109. Van Cryck: cited by Kreuter, E 110. Vargas LL, Britton RC, Goodman EN: Congenital esophageal stenosis. Report of a case of annular muscle hypertrophy at the ~sophagogastricjunction. New Engl J Med 255: 1224-1227 (1956) 111. Vincent: cited by Kreuter E 112. Vogt EC: Congenital esophageal atresia Amer J Roentgenol 22:463-465 (1929) 113. Walcher K: cited by Joshi VV 114. vd Water:cited by Kreuter E 115. Waterston D J, Carter REB, Aberdeen E: Oesophageal atresia: Tracheo-oesophageal fistula. A study of survival in 218 infants Lancet 1962:819-822 116. Welch RG, Husain OAN: Atresia of the ~oesophagus with common tracheooesophageal tube Arch Dis Childh 33:367-370 (1958) 117. Wiersema JS: Partial reduplication of the oesophagus with oesophageal atresia and oesophago-tracheal fistula Arch Chir Neerl 8:97-101 (1956) 118. Wiltich E: Lungenaplasie rechts mit Situs inversus totalis, Osophagusatresie und Abgang des unteren Osophagussegmentes aus dem rechten Hauptbronchus Z Kinderchir 2:120-121 (1965) 119. Yahr WC, Azzoni AA, Santulli TV: Congenital atresia of the esophagus with tracheo-esophageal fistula: an unusual variant Surgery 52:937-941 (1962)
Definition: Belonging to Type I are all the cases of esophageal atresia in which a distal esophagus cannot be demonstrated as a hollow viscus above the diaphragm.
Ph~'~~l'irOel"~~iil BrL?-~~Br ~,0 I1 Sonderland (1820) Lozach (1816)
12 Brodie ( 1810) Anders (cited by Fischer, 1926)
i 1I 13 Durston (1670)
14 Vancryck (1824) Pinard (1873)
~176 Oe I Oel A
Oe2 ~
~ Is Cleaver (1892) Kraus (1902)
Ma Ie R~derer (1754)
Ir
DeMuth et al. (1957)
From the Department of Surgery of the Children's Hospital, Bremen, Germany. Address for reprints." Dr. Dietrich Kluth, Waetjenstr. 43, D2800, Bremen, Germany. 9 1976 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 11, No. 6 (December),1976
901
902
DIETRICH KLUTH
SUBTYPE I1--Agenesis of the entire esophagus. Br--Bronchus, LawLarynx, Ma--Stomach, Ph--Pharynx, Tr--Trachea SUBTYPE 12--Agenesis of the distal part of the esophagus. Oel--Proximal esophageal blind pouch. SUBTYPE 13--Extremely long proximal blind pouch. Agenesis of the distal esophagus. SUBTYPE 14--Total atresia of the esophagus. Ph--Pharynx, A--Esophagus in the form of an atretic strand. SUBTYPE 15--Atresia of the distal esophagus (A). Oel--Proximal esophagus. SUBTYPE 16--Atresia of the distal esophagus (A). The stomach (Ma) is missing. Oel--Proximal esophagus. SUBTYPE 17--Agenesls of the midportion of the esophagus. The distal esophagus (Oe2) is present as an atretic structure.
TYPE H
Definition: Belonging to Type II (of the esophageal malformations) are all the cases in which there are proximal and distal segments without a fistula.
Oel Fi
II 1 Pagenstecher (1829) Steele (1888)
112 Daum (1970)
113 Mason (1855) Jlott (cited by Kreuter, 1905)
Cy A
Mu Oe~
II4 Keith ( 1910)
II s
Matsumoto et al. (1972)
ATLAS
903
SUBTYPE II 1- Agenesis of the midportion of the esophagus. Oei--Proximal esophagus. Oe2--Distal esophagus. SUBTYPE 112--Same as Subtype II 1. Between the two segments there is a cyst (Cy) that communicates with the trachea through a fistula (Fi). SUBTYPE 113--The midportion of the esophagus is atretic (A), SUBTYPE 114--Same as Subtype II 3. A muscular strand (Mu) is present between the trachea and the diaphragm. SUBTYPE 115--Same as Subtype II 3 with a cyst (Cy) located in the atretic strand.
T Y P E Ilia
Definition: Belonging to Type IIIa are the atresias of the esophagus with tracheoesophageal fistula of the proximal segment only.
JFi
0
Oe ~ $ t
Ilia 1
Ilia 2
Ilia 3
Vogt (1929)
Dudgeon et al. (1972)
Mahour et al, (1971)
SUBTYPE Illal--Esophageal atresia with fistula (Fi) of the upper segment (Oel). Oe2--Distal segment. SUBTYPE Illa2--Same as Subtype Ilia 1 with an additional fistula (Fi I ) of the proximal segment. SUBTYPE IIla3--Same as Subtype Ilia 1. The distal segment of the esophagus exhibits a stenotic (St) a rea.
904
DIETRICH KLUTH
T Y P E Illb
Definition: Belongingto Type IIIb are the atresias of the esophagus with tracheoesophageal fistula of the distal segment only.
Oe~--~~I
~
~i~
~ e 2
Oe~
e~
IIIb 1 Gibson (1697) Martin ( 1821 )
IIIb2 Schiller (1838)
- Fi Oe,
Oe,
Oe,--~!~--Oe=
I
IIIb5 Rehbein (1972)
IIIb3 Howston (1830) Vincent (1887)
I
Oe~
~
jr" IIIb4 Holcomb & Daniel (1966) Bienayme (1967)
O~
Fi
Oe~ IIIb6 Dafoe & Ross(1960)
e,
IIIb7 Waterston et al (1962) Koop & Hamilton
IIIb8 Overton & Creech (1958)
(1968)
i
Fi, Cy
IIIb9 Tuqan (1962) Koop & Hamilton (1968)
IIIbl0 Humphreys et al. (1956)
Fi~
Cy IIIbl 1 Koop & Hamilton (1968)
IIib12 Roberts (1958)
ATLAS
905
SUBTYPE IIIb I --Esophageal atresia with fistula of the lower segment (Oe 2) with a short distance between the segments. (Oe 1)--Proximal esophagus. SUBTYPE I I I b 2 --Atresia as in Subtype IIIb 1 with a long distance between the segments. There are fibromuscular strands (Mu) that connect the segments (Mu) (can also happen in Subtype IIIb 1). SUBTYPE IIIb 3 --The proximal blind pouch (Oel) ends quite high. The fistula from the lower segment (Oe2) ends high on the trachea. There is no overlapping of the segments. SUBTYPE IIIb 4 - - T h e fistula from the distal segment (Oe2) ends high on the trachea. There is overlapping of the segments. SUBTYPE IIIb s --Same as Subtype IIIb 4. The fistula (Fi) is a long atretic strand. SUBTYPE I I I b 6 --Long proximal esophagus (Oe 1). The fistula from the distal segment ends at the usual (normal) site. There is overlapping of the segments. SUBTYPE I I I b 7 --Same as Subtype IIIb 1. The fistula (Fi) is closed at its connection w i t the trachea. SUBTYPE I I I b s --Same as Subtype IIIb 7 with an additional membranous atresia (Me) of the distal segment (Oe2). SUBTYPE nlb 9 --Same as Subtype lllb 1 with an additional stenosis (St) of the distal esophagus (Oe2). SUBTYPE IIIbl0--1nstead of a distal esophagus and a fistula, multiple cysts (Cy) are found. These are connected to each other by a fibretic band and are suspended between the cardia and the trachea. SUBTYPE IIIb11--Same as Subtype IIIb 1 with multiple cysts (Cy) present at the distal esophagus. SUBTYPE IIIb12--Esophageal atresia with two fistulas arising from the distal esophagus, both reaching the trachea. (Fi 1 and Fi2)
Oe, / Du--
Oe,
A
Oe
IIib13 Devens & Ritz (1965)
IIib14 Holden & Wooler (1970)
IIIbl s Keep & Hamilton (1968)
L a 9A
IIib16 Sayre & Hall (1954)
La Oe~
Tr
B
e2
Oe,
Tr. St Br
IIIbl 7 Sayre & Hall (1954)
IIIbl s Franklin & Graham (1953)
IIIblv Welch & Husain (1958)
Br A
IIIb20 De Buse & Morris (1973)
906
DIETRICH KLUTH
SUBTYPE IIIb13--Esophageal atresia with two fistulas arising from the distal segment. The first one (Fi 1) enters the trachea, the other one (Fi2) enters the right main bronchus. SUBTYPE IIIb14--The external continuity of the esophagus is maintained but the lumina are separated from each other by an atretic segment (A). The fistula arises below the upper end of the distal esophagus. SUBTYPE IIIbls--Same as Subtype IIIb 1 with the proximal esophagus existing as duplication (Du). SUBTYPE IIIb16--Same as Subtype IIIb 1 with stenosis of the larynx (La St). SUBTYPE IIIb17--Same as Subtype IIIblwith atresia of the larynx (La A). SUBTYPE IIIbls--Same as Subtype IIIb 1 with additional tracheal stenosis (Tr St) below the entrance of the fistula in the trachea. SUBTYPE IIIb19--High atresio of the esophagus (Oel). Between the larynx and the stomach there is a tube with tracheal (Tr) and esophageal (Oe2) characteristics. From this tube arise two normal bronchi (Br). SUBTYPE IIIb20--Esophageal atresia with fistula from the distal segment and additional atresia of both main bronchi (Br A) as well as agenesis of both lungs.
TYPE Illc
Definition: Belonging to this group are the cases (of esophageal atresia) with fistulas from the proximal and distal segments.
Oe, [ Oe
FLFi:A?
Oe~
IIIc 1 V. D. Water (1857) B. Fischer (1905)
IIIc2 Krediet (1955)
••'•F
~
IIIc4 Rehbein (1973)
FI2
=~F,1
FL ' FOel~i 2//.,~ ___.Fi, Oe~,~
IIIc3 Holden & Waoler (1970)
Oe,
--
La.A
--
Fi~
--
Fi~
Oe~
IIIcs Heineken (1952)
ATLAS
907
SUBTYPE IIIcl--Esophageal atresia in which there is a fistula from the proximal esophagus (Oe 1) and the distal esophagus (Oe2) , each one communicating with the trachea. (Fi 1 and Fi2). SUBTYPE IIIc~--Between the upper esophagus (Oe 1) and the lower esophagus (Oe2) there is a short atretic esophageal segment (A). Both segments have a fistula (Fi 1 and Fi2) communicating with the trachea. SUBTYPE IIIc3--Same as Subtype Illc 1 except for the proximal fistula (Fil) that exhibits an atretic segment. SUBTYPE IIIc4--Same as Subtype IIIc 3 with an additional atretic segment in the distal fistula (Fi2). SUBTYPE IIIcs--Besides the atretic Subtype IIIcl, there is a laryngeal atresia (La A).
T Y P E IV." M e m b r a n o u s atresias
Definition: The esophagus exhibits external continuity, but its lumen is divided into a proximal and a distal segment by a transverse or oblique, purely mucous, m e m brane.
V
Me
Fi Me-~
Fi Me
M
i1
J IV 1 Abel (1928) Schwartz (1962)
IV 2 Haight (1957)
Me
Me~ Fi
IV s Matsumoto et al. (1972) Azima & O'Hara (1973)
IV 3 Kate, ten (1952)
Fi, rl\~
IV 6 Goldenberg (1960)
IV4 Lister (1963)
Me Fi
Fi~
|V 7 Touloukian (1973) Rehbein (1973)
908
DIETRICH KLUTH
SUBTYPE IV1--The lumen of the esophagus is interrupted by a transverse membrane (Me). There are no fistulas. SUBTYPE IV2--The esophagus is closed by a transverse membrane (Me). Proximal to the closure is a stenosis (St). SUBTYPE IV3--The mucous membrane does not occlude the esophagus completely. Its ventral part is pulled upwards providing a fistulous tract (Fi) between the proximal and distal segments. SUBTYPE IV4--The proximal and distal esophagus are divided by a moderately oblique membrane. The distal esophagus communicates with the trachea through a fistula just below the ventral insertion of the membrane. SUBTYPE IVs--The fistula (Fi) ends relatively high in the trachea. The membrane has a very oblique course (Me). SUBTYPE IV6--There are two fistulous tracts (Fi 1 and Fi2) to the trachea. One of the tracts is proximal and the other one is distal to the membrane (Me). SUBTYPE IV7--The membrane (Me) is at the level of the tracheo-esophageal fistula (Fi). It occludes the lumen of the esophagus and penetrates the fistulous tract. Both segments communicate through the fistula.
T Y P E V: Complete and partial duplication of the esophagus.
Atresia with intramural duplication. Definition: Besides the patent esophagus, there is a second one that may show a variable length and is in communication with the main lumen or the stomach. The atresia with intramural duplication is morphologically similar to the atresia by membrane, but the dividing wall between the segments also contains a layer o f muscularis.
Du
Dul
v~
Gi~rup (1933) Butler & Ende (1950)
Du
v~ Blasius (1674) Frank & Paul (1949)
Du
Mu
v3
v4
Kathe (1907) Ciechanowski & Glinski (1910)
Maier (1957)
ATLAS
909
v
i
Oe~_ _
Oe~ j Vs Yahr et al. (1962)
Oe~ ~ Mu Fi -Oe~ Oe~-~ ~-St
V6 Minnis et al. (1962) Wiersema (1956)
Vz Bienayme (1967)
SUBTYPE V i - - T h e esophageal duplication is present throughout the whole length of the esophagus (Du). SUBTYPE V2--Only a part of the esophagus is duplicated (Du). SUBTYPE V3--The duplication (Du) is a submucous tract that is present in the ventral part of the esophagus. SUBTYPE V4--Only a short segment of the esophagus is duplicated. The duplication is a structure resembling a diverticulum but it does not have the characteristics of a traction or pulsion diverticulum. SUBTYPE Vs--There is a fairly long overlapping of the proximal blind pouch (Oel) and the distal esophagus (Oe2). Along practically the whole segment of duplication, both segments share a common muscular wall (Mu). SUBTYPE V6--There is a muscular septum (Mu) between the proximal (Oel) and the distal (Oe2) segments of the esophagus. The fistula arising from the distal segment ends high on the trachea. SUBTYPE VT--This Subtype is similar to the preceding one, but the fistula (Fi) is not patent and the muscular membrane is pierced, forming a stenosis (St).
910
DIETRICH KLUTH
TYPE
111."Esophagobronchial communications.
a - - E s o p h a g o b r o n c h i a l fistulas. b--Bronchoesophageal fistulas. Definition (a): The fistula coming from the esophagus reaches a main bronchus or a secondary (sequential) bronchus. It may also communicate with an intra or extra lobar pulmonary sequestration. It may even replace a main bronchus.
Fi
Oe~F~ .lli~o~i~
,~.~ Fi
Vial Heiderich
Via 2 Moscarella & Wylie
(1916)
Via 3 Ellerbroek (1922)
Via 4 Gans & Potts
(1968)
(1951)
Morton et al. (1950)
Fi
~'.A Via s Nikaidoh & Swenson ( ! 971 )
Tr.St
agLu~
T~
~
Via 6 Toyama (1972)
Eu Via 7 Hanna (1964)
~ Vla$ Kee|ey & Schairer (1960)
SUBTYPE V i a l - - T h e fistula (Fi) enters the main or lobar bronchus a short distance from the esophagus. SUBTYPE Vla2--The fistula (Fi) enters isolated "ectepic" pulmonary tissue (Lu). SUBTYPE Vla3--Esophageal atresia in which the proximal esophagus (Oel) ends with a fistulous tract (Fil) in a main bronchus. The distal esophagus (Oe2) originates with its fistula from the trachea. SUBTYPE Vla4--Total sequestration of the lef t upper lobe, which is also aplastic (ap. Lu). This sequestered segment communicates with the esophagus via a fistula. SUBTYPE Vlas--"Ectopic origin" of the right aplastic lung (ap. Lu) from the esophagus by a fistula (Fi) with atresia of the right main bronchus (Br.A). SUBTYPE Vla6--Esophageal atresia with "ectopic origin" of the right aplastic lung (ap. Lu) from the distal esophagus (Oe2). There is also a tracheal stenosis (Tr.Sto) below the entrance of the fistula and atresia of the right main bronchus (Br.A).
ATLAS
911
SUBTYPE VlaT--"Ectopic origin" of the right lung from a fistula (Fi). The trachea ends directly in the left main bronchus (Br.) without a bifurcation. SUBTYPE Vlas--Esophageal atresia with "ectopic origin" of the right lung from the distal esophagus (Oe2). There is a long segmental stenosis of the trachea (Tr.St,) which ends directly in the left main bronchus without a bifurcation.
Definition (b): Esophageal atresia with origin of the distal esophagus from a bronchus.
Tr Oe~ ~ Oe~--
Oe~ Fi
~
r -
O
e
Oe~
Cy
Br
=
9
e
~"
VIb] Levy (1845)
Vlb2 Roe & Nobis (1963)
Vlb3 Willich & Rehbein (1965)
VIb4 Rehbein
SUBTYPEVlbl--The proximal blind pouch (Oel) ends at the level of the tracheal bifurcation. The distal esophagus (Oe2) originates as a fistula (Fi) from the right main bronchus. SUBTYPEVIb2--The proximal esophageal pouch (Oel) is longer than usual. The distal esophagus (Oe2) originates from the left main bronchus (Br). SUBTYPEVIh3--Esophageal atresia with the right main bronchus dividing into a cystic structure (Cy) and the beginning of the distal esophagus (Oe2). Fi--fistula between the right main bronchus and the distal esophagus. (La--Larynx; Tr--Trachea). SUBTYPEVIb4--Esophageal atresia in which the right main bronchus (Br) goes directly in the distal esophagus (Oe2). Br.Oe--brenchoesophageal transition.
T Y P E VII: H Fistulas
Definition: Fistula (Fi) between the esophagus and the trachea. No atresia.
Fi
Fi~ ~- FJ2 ~L~Fi~ ~Fi~ St
Fi
Vll I
Vl12
Vll 3
Vll 4
Lamb (1873) Pinard ( ! 873)
H~Jbner (1943) Leven et al. (1952)
Eckstein & Somasundaram (1966)
Moyson (1970) Stephens (1970)
9t2
DIETRICH KLUTH
St
~/~! -- Fi
Di
VII s Brenner (1892)
Fi
---Fi
Di
VII 6 Knox ( 1951 )
VII 7 Robb (1952)
VII1--One tracheoesophageal fistula (Fi) between esophagus and trachea. VII2--Two tracheoesophageal fistulas (Fi 1 and Fi2). VII3--Three tracheoesophageal fistulas (Fil, Fi2 and Fi3). VII4--Besides a tracheoesophageal fistula (Fi), there is a stenosis of the esophagus (St) distal to the fistula. SUBTYPE VIIs--The esophageal stenosis (St) is proximal to the fistula. SUBTYPE VII6--The esophagus presents an outpauching (Du) at the level of the fistula (Fi). SUBTYPE VIIT--The esophagus has a double lumen distal to the H-fistula (Fi). The dorsal lumen (Di) ends blindly. SUBTYPE SUBTYPE SUBTYPE SUBTYPE
T Y P E VIII: Stenoses
Definition: Narrowing of the esophageal lumen of congenital origin. No evidence of signs of inflammation.
Me St
j
~,, St
VIII 1 Bonilla & Bowers (1959)
Oe,
St ~
VIII 2 Budgen & Delmonico (1956) Adler (1963)
\
VII| 3 Handousa Bey (1949) Greenough (1964)
~
St~
I
VIII 4 Jewsbury (1971)
ATLAS
913
Cy St
VIii s Vargas (1956)
Vii| 6 Soave & Mezzano ( 1965 ) |shida et al. (1969)
VIii 7 Hecker (1968)
SUBTYPE Villi--Segmental stenesis. The esophagus is stenotic (St) over a certain segment. SUBTYPE Vlil2--Esophageal stenosis produced by a ring or a semicircular mucous membrane (St). If this membranous stenosis is in the lower third of the esophagus it is called "lower esophageal web." SUBTYPE Vl|13--There are two simultaneous stenoses. One is segmental (St) and the other one is membranous (Me). SUBTYPE Vill4--1n this form there is a morphological similarity to the Type Inb. There is a communication between the upper and the lower esophageal segments which is considerably narrower than these segments, acting as a stenesis. (St)--stenetic communicating segment. SUBTYPE VIIIs--Stenosis above the cardia produced by muscular hypertrophy (Mu). This is also known as "lower esophageal ring." SUBTYPE VIII 6 - Stenosis produced by inclusion of trachea-bronchial rests (St). SUBTYPE VIIIT--The lumen ef the esophagus is narrowed by a submucosal cyst (Cy), preducing a stenosis. (St)
T Y P E IX: Tracheal atresias.
Definition: Agenesis or atresia of the trachea combined with tracheoesophageal fistula.
r. A
iX~ Devenis & Otis (1957) Josh| (]969)
|X 2 Fritz (1933) Sand|son (1955)
Fi
IX 3 Peison et al. (1920)
Tr. A
IX4 Walcher (1928) Floyd et al, (1962)
914
DIETRICH KLUTH
SUBTYPE IXl--Total agenesis of the trachea. The larynx (La) is normal. Both main bronchi (Br) are normal and form a bifurcation that communicates with the esophagus by a fistula (Fi). SUBTYPE IX2--Tracheal atresia in which this organ sometimes can be identified as an atretic strand (Tr.A) orbital to a normal larynx. Distally the trachea (Tr) is normal, dividing into two main bronchi. Between the esophagus and the normal segment of the trachea there is a fistula. SUBTYPE IX3--Similar to Subtype IX 2 with additional atresia of the larynx. (La.A) SUBTYPE IX4--Agenesis of the trachea. It is possible that there is tracheal tissue (Tr.A) below the normal larynx (La). Both main bronchi arise from the esophagus. (Br)
TYPE X." Fissure formation. The forms of Type X are divided in four groups according to the depth of the fissures (after Pettersson, 1969). Group Xa--Fissure formation at the level of the larynx with and without esophageal atresia. GroupXb Fissure formation at the level of the larynx and the proximal trachea with and without esophageal atresia. Group Xc--Total fissurization, involving the larynx and the trachea down to the level of the bifurcation. Group Xd Fissure at the distal level of the trachea. The larynx and the proximal trachea are normal. Group Xa:
JSp
Sp
d
L Xa! Finlay (1949)
I Xa 2 Cameron & Williams (1962) Delahunty & Cherry (1969)
Xa 3
Jahrsdoerfer (1967)
ATLAS
915
La.St Sp Oe~
Oe_~_="~Sp
Oe2 Xa 4 Cameron & Williams (1962) Delahunty & Cherry (1969~
XQ5 Delahunty & Cherry (1969)
SUBTYPE X a l - - T h e fissure (Sp) between the esophagus and the larynx is not complete. The arytenoid incisura is caudally deepened and leads to the formation of the fissure~ SUBTYPE Xa2--Simple laryngeal fissure (Sp) i The formation of the fissure does not reach the level of the trachea. SUBTYPE Xa3--Same as Subtype Xa 2. There is esophageal musocal tissue in the fissure (Oe.Sc) SUBTYPE Xa4--Same as Subtype Xa 2. In addition there is esophageal atresia of the Subtype IIIb 1. SUBTYPE Xas--Same as Subtype Xa 2. In addition there are esophageal atresia of the Subtype II 1 and a subglottic stenosis of the larynx (La.St).
Group XO:
liSp [~~SpOe,/SPi="~ Oe~~~~FI' ~L~Br'St Oe~-~l~. !!
Xbl Imbrle & Doyle (1969) Geiger (1970)
Xb 2 Blumberg et al. (1965)
Xb 3 Oaum et al. (1965) Donahoe & Hendren (1972)
sp
Xb 4 Mahour et al. (1973)
SUBTYPE X b i - - T h e fissure (Sp) goes beyond the limits of the larynx into the upper level of the trachea. SUBTYPE Xb2--Same as Subtype Xb I. In addition there is a stenosis of both main bronchi (Br.St). SUBTYPE Xb3--1n addition to the fissure (Sp) that extends into the upper level of the trachea, there is an atresia of the Subtype I|lbl, with fistula of the lower segment. SUBTYPEXb 4 - Corresponds to the Subtype Xb 3. In addition to the fissure formation and the atresia of the esophagus with fistula (Fi3) from the lower segment, there are two more fistulas (Fi I and Fi~) from the proximal segment.
916
DIETRICH KLUTH
Group Xc:
- -
~
Sp
Sp
~
N.I_
J Xcl Frates (1967) F~disch & Sauer (1972)
Xc2 Stolze (1952)
SUBTYPE Xcl--The fissure (Sp) goes from the larynx to the carina. There is therefore a complete fissure between the esophagus on one side and the larynx and the trachea on the other side. SUBTYPE Xc2 - Besides the complete fissure (Sp) there is an accessory lung (N.L) that communicates with the alimentary tract by a fistula to the stomach (Ma).
Group Xd:
Xd~ Rehbein (1974) SUBTYPE Xd~--In the lower segment of the trachea there is a large communication with the esophagus (Sp). Proximal to this fissure there is a fistula (Fi).
ATLAS
917 REFERENCES
1. Abel AL: Congenital oesophageal obstruction. Brit Med J II:46-49, 1928 2. Adler AO: Congenital esophageal webs. J Thoracic Cardiovasc Surg 45:175 185, 1963 3. Anders HE: cited by Fischer, W 4. Azimi F, O'Hara E: Congenital intramural mucosal web of the esophagus with tracheoesophageal fistula. Amer J Dis Child 125: 92-95, 1973 5. Bienaym6 J: Deux observations de malformations oesophagiennes rares; atr6sie avec duplication et atr6sie maiqu6e. Ann Chit Infant 8: 34-43, 1967 6. Blasius G: Observata Anatomica. Leyden and Amsterdam 1674 7. Blumberg JB, Stevenson JK, Lemire RJ, Boyden EA: Laryngotracheal cleft, the embryologic implications: review of the literature. Surgery 57:559-566, 1965 8. Bonilla KB, Bowers WF: Congenital esophageal stenosis. Amer J Surg 97:772-776, 1959 9. Brenner: cited by Fischer, W 10. Brodie: cited by Kreuter, E 11. Budgen WF, Delmonico JE: Lower esophageal web. J Thorac Surg 31:1 15, 1956 12. Butler CL, Ende M: Double esophagus with carcinoma in one. Report of a case with autopsy. AMA Arch Path 49:605-611, 1950 13. Cameron AH, Williams TC: Cleft larynx: a cause of laryngeal obstruction and incompetence. J Laryngol Otol 76:381 387, 1962 14. Cautley E: Malformation of the oesophagus. Brit J Child Dis 16:1-10, 1917 15. Ciechanowski S, Glinski LK: Fistulae oesophago-oesophageales congenitae. Virch Arch Path Anat 199:420-454, 1910 16. Cleaver: Lancet I:, 1892, p 805 17. Dafoe CS, Ross CA: Tracheo-esophageal fistula and esophageal atresia. Dis Chest 37: 42-51, 1960 18. Daum R, Hecker W Ch, Rossner JA, Wenz W: Kongenitale oesophago-laryngo-tracheale Kommunikationen, ein Beitrag zur Differentialdiagnose der oberen Oesophagotrachealfisteln. Z Kinderchir 2:314-325, 1965 19. Daum R: Formen der Oesophagusfehlbildungen. Z Kinderchir 8:39 44, 1970 20. DeBuse PJ, Morris G: Bilateral pulmonary agenesis, oesophageal atresia and the first arch syndrome. Thorax 28:526-528, 1973 21. Detahunty JE, Cherry J: Congenital laryngeal cleft. Ann Oto178:96-106, 1969 22. DeMuth WE, Ames MD, Hoffman DA:
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918
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DIETRICH KLUTH
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ATLAS
branous atresia and multiple atresia of the esophagus Surgery 71:795-800 (1972) 82. Minnis JF, Burko H, Brevetti G: Segmental duplication of the esophagus associated with esophageal atresia and tracheoesophageal fistula Ann Surg 156:271 275 (1962) 83. Morton DR, Osborne JF, Klassen KP: An apparently congenital bronchoesophageal fistula persistent to adult life J Thorac Surg 19:811-816 (1950) 84. Moscarella AA, Wylie RH: Congenital communication between the esophagus and isolated ectopic tissue J Thoracic Cardiovasc Surg 55:672 676 (1968) 85. Moyson F: St6nose oesophagienne cong6nitale et fistula trach6o-oesophagienne cervicale Ann Chir In fant 11:179-183 (1970) 86. Mullard KS: Congenital tracheoesophageal fistula without atresia of the esophagus J Thorac Surg 28:39-54 (1954) 87. Nikaidoh H, Swenson O: The ectopic origin of the right main bronchus from the esophagus J Thoracic Cardiovasc Surg 62:151160 (1971) 88. Overton RC, Creech O: Unusual esophageal atresia with distant membranous obstruction of the esophagus J Thorac Surg 35:674-677 (1958) 89. Pagenstecher: cited by Kreuter, E. 90. Peison B, Levitzky E, Sprowls J J: Tracheoesophageal fistula associated with tracheal atresia and malformation of the larynx J Ped Surg 5:464-467 (1970) 91. Pinard: cited by Kreuter, E 92. Rehbein F: personal communication 93. Robb D: Congenital tracheo-oesophageal fistula without atresia but with large oesophageal diverticulum Austr New Zeal J Surg 22:120-122 (1952) 94. Roberts KD: Congenital oesophageal atresia and tracheo-oesophageal fistula Thorax 13:116-129 (1958) 95. Roe BB, Nobis PD: Congenital tracheoesophageal fistula Amer J Dis Child 106:489491 (1963) 96. ROderer: cited by Kreuter, E 97. Sandison AT: Partial absence of the trachea with live birth Arch Dis Child 30:475477 (1955) 98. Sayre JW, Hall EG: Anomalies of the larynx associated with tracheo-oesophageal fistula Pediatrics 13:150-154 (1954) 99. SchiSller: cited by Kreuter E. 100. Schwartz SI: Congenital membranous obstruction of esophagus Arch Surg 85:480 482 (1962)
919
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