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:

E sophagea l A tres ia and H ypertroph ic P y lo ric S tenos is :

S equen tia l C oex is tence o f D isease C ase R epo rt

A rth u r D . M ag iln e r1 2

A d d itio n a l co n g e n ita l a n o m a lie s in a s so c ia tio n w ith e s o ph -

a g e a l a tre s ia a n d

tra che o eso p ha ge a l fis tu la (T E E ) a re w e ll

k n o w n . T he m o st co m m o n ly a ss o c ia te d le s io n s a re o th e r

a tre s ia s o f th e g a s tro in te s tin a l tra c t a n d im p e rfo ra te a n u s ,

b u t o th e r co m b in atio n s o f th e V A T E R (ve rte b ra l d e fe c ts , a n a l

a tre s ia , T E E w ith e s o p ha g e a l a tre s ia , ra d ia l a n d re n a l d y sp la -

s ia ) c om p lex a re n o t u nco m m on [1

].

H yp e rtrp h ic p y lo ric s te -

n o s is co m p lica tin g th e p o s to p e ra tive c o u rse o f tra ch e o e so -

p h a g e a l fis tu la is le ss w e ll kn o w n . T h e re h a ve b e e n 3 5 c a se s

o f th is c o m b in a tio n re p o rte d [2 -4 ]. T h e se h a ve a p p e a re d in

th e su rg ica l a n d p e d ia tr ic lite ra tu re , w ith th e m o s t re c e n t

re p o rts p u b lish e d in 1 97 0 . T w o re ce n t ca se s o f p y lo r ic s te -

n o s is a fte r su rg e ry fo r e s o p h a g e a l a tre s ia a n d T E E fo rm th e

b a s is o f th is re p o rt.

Case

R e p o r t

T h is 4 .1 -kg b oy h a d co rre c tive su rg e ry fo r e so p h a g e a l a tre s ia a n d

T E F o n th e firs t d a y o f life (F ig . 1 ). T h e p o s to p e ra tive co u rs e w a s

u n ev en tfu l, a nd sm a ll fe ed in g s w e re b e gu n on the sev en th da y a fte r

su rge ry . T he p a tien t w as a b ou t to be d isch a rge d w h en , o n th e 18 th

d a y a fte r su rg e ry , h e b e g a n to re gu rg ita te h is fe e d in g s . A b a riu m

sw a llow to e va lua te th e a na s tom os is sh ow e d n o s tric tu re , bu t th e re

w a s m a rke d d e la y in e m p ty in g th e s to m a ch . D e la y e d film s d e m o n -

s tra te d th e ty p ica l a p p e a ra n c e o f h yp e rtro p h ic p y lo ric s te n o s is (F ig .

2 ). S u rg ica l co rre c tio n o f th e p y lo ric s te n o s is w a s p e rfo rm ed , a n d n o

fu rth e r d iff icu lt ie s w ith fe ed in g s d e ve lop e d .

Discuss ion

T h e fre q u e n cy o f a d d itio n a l co n g e n ita l a n o m a lie s in p a -

tie n ts w ith e so p h a g e a l a tre s ia a n d T E E is n e a rly 5 0 [2 ].

W hile th e a sso c ia tio n w ith o th e r a n o m a lie s o f th e g a s tro in te s-

tin a l tra c t is w e ll kn o w n , th e o ccu rre n c e o f p y lo r ic s te n o s is

a s a p o ss ib le co m p lica tin g fa c to r d u r in g th e p o s to p e ra tive

p erio d is le s s w e ll a p p re c ia te d . T h e fre qu e n c y o f p y lo ric

s te n o s is is a p p ro x im a te ly o n e in 4 0 0 b ir th s . H o w e ve r, in

p atie n ts w ith e so p h a g e a l a tre s ia a n d T E E , its fre q u e n cy h a s

b ee n re p o rte d a s ra n g in g fro m 1 to 1 0 [2 -4 ].

Fig .

1 -B ar ium -fi lled p rox im al es op h-

a g ea l p o uc h typical of es ophagea l a tres ia .

F ig . 2 .-A n tra l a n d p ylo ric c ha ng es

charac ter t is t ic o f h yp ertro ph ic p ylo ric s te -

nos is 18 da ys a fte r s u r ge r y .

Rec e iv ed J an ua ry 1 6, 1 9 8 6 ; a cc ep te d a ft er re vis io n M arc h 11 ,1986 .

Depar tmen t o f D ia gn os tic R ad io lo gy . A lb ert E in ste in M ed ic al C en te r, Nor the r n D iv is io n, Y ork a nd T ab or A ds ., P hila de lph ia , P A 19 14 1 . A d dr es s r ep ri nt r eq ue st s

to A . D . M ag ilne r.

2

Depar tmen t o f R a d io l og y ,

Temp le

lk tiv ers ity S ch oo l o f M ed ic in e, 3 40 0 N . B ro ad S t., P hilad elp hia , P A 1 91 40 .

R 147 :329 - 330 , A u g u s t 1 98 6 03 61 -80 3X /86 /1 47 2-0 32 9 C A m er ica n R oe n tgen R ay Soc ie t y

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33 MAGILNER ET AL

A J R:1 47 , A u gu st 1986

W e a ls o s a w a se c o nd ca se , w h ich is n o t de ta ile d in th e

cu rre n t re p o rt. In th is ca s e , th e sym p to m s o f h yp e rtro p h ic

py lo r ic

s te n o s is d id n o t a p p e a r a nd a d ia g n o s is w a s n o t m a d e

un til 6 w e eks a fte r the firs t su rge ry fo r e so ph a ge a l a tre s ia

an d T E E .

M o s t o f th e lite ra tu re su g g e s ts th a t p y lo r ic s te n o s is is a n

ac q u ire d e n tity a n d th a t its o n s e t u s u a lly o cc u rs 3 to 6 w e e ks

afte r b irth . T h is , o f co u rse , is d is tin c tly d iffe re n t fro m m o st

o th e r a n o m a lie s a ss o c ia te d w ith e so p h a g e a l a tre s ia a n d T E E

th a t a re a pp a re n t in th e n e o n a ta l p e r io d .

T h e co m b in a tio n o f d e la ye d on s e t o f sym p to m s a n d th e

te n d e n c y to a ttr ib u te th e vo m itin g to a p o s top e ra tiv e co m p li-

ca tion o fte n o b scu re s th e re a l ca u se o f th e p ro b le m . P y lo r ic

s te n o s is sh o u ld b e co n s id e re d in th e e va lu a tion o f vo m itin g

in an in fa n t w h o ha s h ad re ce n t su rge ry fo r eso p ha ge a l

atres ia

a n d T E E . In a d d itio n to e v a lu a tin g th e e so p h a g e a l a n a s ta -

m o s is a n d e so p h a g e a l m o tility , th e s to m a ch , p y lo ru s , a n d

d uo d e n u m sh o u ld b e s tu d ie d fo r p y lo r ic s te n os is .

R E F E R E N C E S

1

.

Q ua n L , S m ith D W . T h e V A T E R as soc ia tion . V e rte b ra l d e fe c ts ,

an a l a tre s ia , T -E fis tu la w ith e so ph a ge a l a tre s ia , ra d ia l a nd re n a l

dys p las ia : a sp ec tru m o f a sso c ia ted de fec ts . Ped ia t r

1 97 3; 82 : 1 04 -1 07

2 . R affe n s p e rg e r J . G astro in te s tin a l d e fe c ts a sso c ia te d w ith tra ch -

eo es op ha g ea l fis tu la an d eso p ha ge a l a tre s ia . rc h Su rg

1970;101 :2 41 -2 44

3 . A h m e d S . In fa n tile p y lo ric s ten os is as soc ia te d w ith m a jo r an om -

a lie s o f th e ga s tro in te s tin a l trac t. Ped Su rg

1 97 0; 5: 6 60 -6 66

4 . F ra nke n E A , S a ld in o A M . H ype rtro ph ic p y lo ric s ten o s is com p li-

c at in g e so ph ag ea l a tres ia w ith tra ch eo es op ha g ea l fis tu la . m

ur 1 96 9;1 1 7: 64 7-6 49