R O E N T G E N M A N I F E S T A T I O N S OF I N F A N T I L E H Y P E R T R O P H I C PYLORIC STENOSIS ]~ERBERT ~r 0LNICK, M.D., AND II. STEPIIEN WEENS,
M.D.
ATLANTA, GA. INTRODUCTION
E S P I T E the p r o m i n e n t rote of r a d i o l o g y in the recognition of gastrointestinal disorders, c o m p a r a t i v e l y few pediatricians, surgeons, and radiologists fully a p p r e c i a t e the value of r o e n t g e n methods in the diagnosis of infantile h y p e r t r o p h i c pyloric stenosis. This m a y be explained b y the f a c t t h a t certain r o e n t g e n criteria p r o p o s e d m a n y y e a r s ago h a v e been f o u n d unreliable for this condition. ~, 2 Also, there are m a n y clinicians who are hesitant to utilize the p r o c e d u r e in infants for f e a r of possible h h r m f u l effects. 3, 4
D
The purpose of the following s t u d y is twofold. F i r s t , newer, more specific r o e n t g e n signs are emphasized and their value d e t e r m i n e d in an unselected series of cases in the m a n i f e s t stage of infantile h y p e r t r o p h i c pyloric stenosis. Second, i n f o r m a t i o n as to the presence of residual pyloric changes was ohrained b y roentgenologic follow-up studies r a n g i n g from five months to t w e n t y two y e a r s a f t e r successful surgical t r e a t m e n t b y the F r e d e t - R a m s t e d t operation. CLINICAL CONSIDERATIONS
The clinical features of h y p e r t r o p h i c pyloric stenosis have been adequately described in m a n y excellent reports and will not be elaborated upon here. '~ I t m a y suffice to state that palpation of the pyloric t u m o r is generally considered to be the most i m p o r t a n t clinical sign. Though experienced observers have claimed that this m a y be possible in practically all cases, ~, ~ other qualified clinicians have been unable to demonstrate a palpable mass in more t h a n twothirds of cases2, 7 I t has been our observation that in some instances competent physicians were at variance in their opinion as to the presence or absence of a tumor. Also cases have been reported in which a mass was thought to be present clinically, but in which a normal pylorus was subsequently demonstrated at operation. ~ The occurrence of these cases makes it desirable to utilize methods which m a y improve the accuracy of clinical diagnosis. ROENTGENOLOGIC CONSIDERATIONS
E a r l i e r r o e n t g e n studies of h y p e r t r o p h i c pylorie stenosis placed m u c h emphasis on such signs as gastric hyperperistalsis, dilatation, and r e t e n t i o n as i m p o r t a n t f e a t u r e s in the establishment of a correct diagnosis, s, 9 These signs cannot be considered as specific since a n y of t h e m have been f o u n d to occur F r o m the D e p a r t m e n t of l%oentgenology, E m o r y U n i v e r s i t y School of Medicine, G r a d y ]Y[emori'al Hospital, and E m o r y U n i v e r s i t y Hospital. 720
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P Y L O R I C STEi~70SIS
F i g . 1.
Fig.
2.
Fig. 3,
Fig. 1 . - - T h e p a t h o l o g i c c h a n g e s of h y p e r t r o p h i c p y l o r i c stenosis. Marked obstruction p r e v e n t s filling of t h e p y l o r i c c h a n n e l . :Fig. 2 . - - A s m a l l ' a m o u n t of b a r i u m h a s e n t e r e d t h e n a r r o w e d p y l o r i e c h a n n e l , t h e "string sign." :Fig. 3 . - - T h e p y l o r i c t u m o r e n c r o a c h e s u p o n t h e l u m e n of t h e p y l o r i e c h a n n e l a n d p r o t r u d e s into t h e b a s e of t h e d u o d e n a l cap.
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in such conditions as gaseous distention of the intestines, 1~ severe infection, cerebral trauma, and duodenal atresia. The modern criteria for the radiologic diagnosis of infantile hypertrophic pyloric stenosis were introduced by Meuwissen and Slooff in 1932.1~ Subsequent articles iu the European and American literature have served to corroborate their findings. 12, ~3 In all these studies an attempt was made to demonstrate the radiologic equivalent of the underlying anatomic changes.
Fig. 4.--Typical "string sign."
It will be remembered that the pathology of this condition consists of a hypertrophy of the pyloric muscle which enlarges to involve not only the true pylorus but also the prepyloric segment of the stomach, the so-called "canalis egestorius." Mucosal thickening further diminishes the caliber of the lumen. These anatomic changes result in the formation of a narrow canal at the junction of:the stomach and duodenum. The direct demonstratidn by barium studies of this narrowed segment forms the basis of current x-ray diagnosis. On proper filling the stenotie canal will be outlined by a thin streak of barium measuring usually 2 to 3 era. in length. This radiologic pattern is commonly r'eferred to as the "string sign" (Fig's. 2 and 4). To our knowledge, this radiologie Sign has not been observed in any other gastric lesion of infancy and should be considered as pathognomonie. In markedly obstructed cases it may be difficult to demonstrate the stenotic canal, but by proper manual compression its. most proximal portion can frequently be visualized at the height of a peristaltic wave. A short streak of barium, having the contour of a bird's bill, may then protrude from the distal part of the pyloric antrum, the so-called "pyloric beak" (Fig. 5). A second valuable radiologic sign is the indentation of the base of the duodenal cap (Figs. 3 and 6). The structural basis for this feature is the cervixlike protrusion of the pyloric tumor into the lumen of the proximal
O L N I C K AND W E E N S :
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723
duodenum. C o n t r a r y to the findings of Miller a n d 0 s t r u m 1~ t h a t changes in the d u o d e n a l cap were not observed b y them, we h a v e f o u n d i n d e n t a t i o n o~ the base of the duodenal cap to be of a p p r e c i a b l e value.
~'ig. 5 . - - A m i n i m a l a m o u n t of b a r i u m h a s p e n e t r a t e d into t h e p r o x i m a l p o r t i o n of t h e p y l o r i c channel, the "pyloric beak."
Fig. 6 . - - S , S t r i n g s i g n ; I, I n d e n t a t i o n of p r o x i m a l d u o d e n u m .
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TECHNIQUE OF ROENTGEN EXAMINATION Previous writers have expressed f e a r as to the effects of tile oral administration of barium in these infants. 2, ~ I t has been stated t h a t b a r i u m might impact in the pylorus, completing the obstruction, or t h a t b a r i u m m i g h t be aspirated into the lungs. I n our experience the procedure has been f o u n d to be entirely harmless.
Fiff.
7.--Collapsible
elevating
board
utilized
in
fluoroscopy
of
infants.
The use of a stomach tube has simplified the e x a m i n a t i o n considerably. A f t e r withholding food for a few hours, a sterile gavage tube is inserted into: the stomach u n d e r fluoroscopic control. S t r u c t u r a l changes in the esophagus m a y be detected b y difficulty in the passage of the tube. A n y retained fluid and air are aspiration f r o m the stomach and a p p r o x i m a t e l y 1 to 2 ounces of a thin b a r i u m mixture is instilled. A f t e r the completion of the examination residual barium may be withdrawn. It is very important to examine the infant in the oblique and lateral positions in order to visualize properly the pyloric channel. Spot films taken during fluoroscopic examination are used extensively to record fluoroscopic impressions. In our studies we thought it advisable to use a collapsible 7-inch elevating board, placed on the x-ray table top (Fig. 7). The increased "tube-to-patient distance serves two purposes. The sharpness of the image of spot roentgenograms is improved, and at the same time the radiation exposure of the patient is reduced.
0LNICK
AND
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725
STENOSIS
RESULTS
A. Preoperative Studies.--The results of o u r f i n d i n g s i n p a t i e n t s e x a m i n e d d u r i n g the m a n i f e s t stage of the disease are r e c o r d e d i n T a b l e I. I n n i n e t e e n out of twenty-t~vo cases positive r o e n t g e n evidence was o b t a i n e d . A l l these cases were p r o v e d b y o p e r a t i o n except f o r one w h i c h was t r e a t e d medically. I n the t o t a l of n i n e t e e n positive cases, all showed the " s t r i n g s i g n " a n d seven showed, as a n a d d i t i o n a l finding, a n i n d e n t a t i o n of the base of the d u o d e n a l cap. TABLE I. X-I~AY STUDIES ])UIglNG MANUo'EST STAGE OP DISEASE .
X - r A Y SIGNS STRING SIGN
INDENTATION OF CAP
0
NO.
NAME
RACE
SEX
SURGICAL PROOF
1
P.C.
w
F
+
0
+
2
C.S.
W
M
+
o
+
o
3 4 5
P.R. It.G. J.M.
W W W
M M F
+ + +
+ + 0
+ + +
0 0 +
6
J.B.
W
1Y~
+
0
+
0
7
J.J.
W
M
+
+
+
+
8
PALPABLE lY[ASS
B.B.
W
M
+
+
0
0
9 10
McK. L.
W W
M IV[
+ +
+ +
0 +
11 12 13 14 15 16 17 18 19 20 2J 22
L.
W
3/[
+ Not operated on +
+
+
0
M.C.
IV
F
+
+
+
0
W.P. C.M. J.C. J.M. F.S. W.B. E.S. E.W. W.H. R.M.
N N 2,I N N N N N N N
F I~ M M IV[ M M M M M
+ + + + + + + + + +
0 0 0 0 0 + 0 + + 0
+ + + + 0 0 + + + +
+ 0 0 + 0 0 + 0 0 +
I n the r e m a i n i n g t h r e e eases the s e v e r i t y of the o b s t r u c t i o n p r e v e n t e d filling of the n a r r o w e d p y l o r i e c h a n n e l , so t h a t a specific diagnosis could n o t be m a d e ( F i g . 1). I t is conceivable t h a t a d m i n i s t r a t i o n of a n t i s p a s m o d i c s m i g h t have f a e i l i t a t e d v i s u a l i z a t i o n of the i n v o l v e d g a s t r i c segment. W i t h p r o p e r a t t e n t i o n to the p a t h o g n o m o n i e f e a t u r e s of i n f a n t i l e h y p e r t r o p h i c stenosis, the r a d i o l o g i s t m a y i m p r o v e his accuracy. D u r i n g the past five y e a r s o u r r a d i o l o g i e a l serviee has s t u d i e d more t h o r o u g h l y the c h a r a c t e r istic f e a t u r e s of this disease a n d has d e m o n s t r a t e d the specific radiologie signs with increasing frequency. C o n c u r r e n t l y the e l i n i e i a n s have placed g r e a t e r confidence i n r o e n t g e n diagnosis a n d have r e f e r r e d p r a c t i c a l l y all suspected eases f o r s t u d y . A n i n t e r e s t i n g s i d e l i g h t of o u r sInall series of t w e n t y u n s e l e c t e d eases at G r a d y M e m o r i a l H o s p i t a l i n the p a s t f o u r y e a r s is the h i g h i n c i d e n c e of the disease i n t h e N e g r o r a c e ( e l e v e n Negro, n i n e w h i t e ) . M a n y e a r l i e r r e p o r t s on this s u b j e c t stress the r a r i t y of this e o n d i t i o n i n the Negro race. 14 O u r obs e r v a t i o n s t e n d to confirm the f i n d i n g s of MeGahee, 1~ who n o t e d t h a t i n f a n t i l e h y p e r t r o p h i c stenosis o c c u r r e d n o t i n f r e q u e n t l y a m o n g Negro i n f a n t s .
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B. Follow-up Studies.---In s i x t e e n cases follow-up g a s t r o i n t e s t i n a l e x a m i n a t i o n s were p e r f o r m e d at i n t e r v a l s of f r o m five m o n t h s to t w e n t y - t w o y e a r s followi n g successful t r e a t m e n t b y o p e r a t i o n . The f i n d i n g s of these studies are summ a r i z e d i n T a b l e I I . I n all b u t one case (No. 8, aged 1 8 9 years) p e r s i s t e n t c h a n g e s i n the most d i s t a l p o r t i o n of the s t o m a c h were observed, c o n s i s t i n g of v a r y i n g degrees of n a r r o w i n g as well as d i m i n i s h e d or a b s e n t p e r i s t a l s i s ( F i g s . 8, 9, 10, 11). S e v e r a l of these p a t i e n t s d e m o n s t r a t e d b a s i l a r i n d e n t a t i o n of the d u o d e n a l cap. S i m i l a r f i n d i n g s were observed i n one p a t i e n t t r e a t e d m e d i c a l l y w h e n e x a m i n e d five m o n t h s a f t e r c l i n i c a l recovery. W e were i m p r e s s e d t h a t despite the radiologic c h a n g e s p r o p e r gastric e m p t y i n g was a r e g u l a r o c c u r r e n c e i n all these cases. S i m i l a r studies were p r e v i o u s l y u n d e r t a k e n b y R u n s t r o m ~6 a n d also A n d r e s e n , ~ who n o t e d t h a t a py]oric d e f o r m i t y m a y persist f o r m a n y
F i g . 8. Fig. 8.--Hypertrophic N o t e m a r k e d n a r r o w i n g of F i g . 9 . - - C a s e 5, f o u r nel w i t h r e a p p e a r a n c e of
Fig.
TABLE :[I.
FOLLOW-UP ]~INDINGS I
NO.
NAME
9.
pyloric stenosis tire m,onths following Fredet-l=tamstedt procedure. p y l o r i c c h a n n e l a n d i n d e n t a t i o n of d u o d e n a l c a p . y e a r s f o l l o w i n g s u r g e r y . N o t e r e s i d u a l d i s t o r t i o n of p y l o r i c c h a n m u c o s a l folds.
RACE
SEX
] 2 3 4 5 6 7
I. tt. V.M. C. D.C. W.P. W. :B. P.C.
W W W W N N W
M M M M F M F
8 9
F.S. It, G.
N W
M M
:[0 1] 12 13 :[4 :[5 16
C.S. P.R. E.S. E. St. C.M. E.W. W.H.
W W N ~V N N N
M M M M F M M
LENGTH OF FOLLOW-UP INTERVAL
22 :[3 10 6 4 31~ 2l~ :[6 :[3 10 :[0 7 6 6 5 5
years years years years years years years months months months months months months months months months
POSITIVE FINDINGS
+ + + + + + + 0 + + + + + + + +
OLNICK AND WEENS:
Fig.
10.--Case
3, t e n y e a r s
Fig, 11.--Case
following operation.
1, t w e n t y - t w o
years
727
PYLORIC STENOSIS
after
Residual
narrowing
successful surgical
of p y l o r i c s e g m e n t .
treatment.
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years. These radiologic observations suggest that there are, following operative procedures, residual changes in tone or structure at the site of the h y p e r t r o p h i c pyloric stenosis. These residual changes are of importance to the roentgenologist who occasionally encounters adolescent or a d u l t patients with n a r r o w i n g or stiffening of the prepyloric or pyloric portion o:f the stomach. Conceivably, some of these patients m a y have had h y p e r t r o p h i c pylorie stenosis in infancy. S U ~ A a Y AND CO~Cr,USmNS The r o e n t g e n criteria of infantile h y p e r t r o p h i c pylorie stenosis are briefly e n u m e r a t e d and a technique of radio]ogle e x a m i n a t i o n is described. R o e n t g e n observations of an unse]ected series of cases in the active stage of infantile h y p e r t r o p h i c pyloric stenosis revealed positive signs in nineteen out of t w e n t y - t w o eases. These changes consisted of (a) n a r r o w i n g and stiffening of the stenotic canal in the pyloric t u m o r ( " s t r i n g s i g n , " "pyloric b e a k " ) and (b) i n d e n t a t i o n of the base of the duodenal cap. These specific signs occur in association with other nonspecific signs such as delayed emptying, gastric hyperperistalsis, and dilatation. I n fifteen out of sixteen cases examined a f t e r complete r e c o v e r y following a F r e d e t - R a m s t e d t operation, residual py]oric d e f o r m i t y was observed consisting of v a r y i n g degrees of n a r r o w i n g and diminished or absent peristalsis. G e n e r a l l y the p o s t o p e r a t i v e x - r a y changes were less p r o n o u n c e d t h a n those seen d u r i n g the m a n i f e s t stage of the disease. I n one p a t i e n t distortion of the pyloric segment of the s t o m a c h was still p r e s e n t t w e n t y - t w o years following operation. I n spite of these radiologic changes, gastric e m p t y i n g was entirely normal. T h o u g h in the m a j o r i t y of cases of infantile h y p e r t r o p h i c stenosis the diagnosis m a y be established on the basis of the clinical studies, there r e m a i n m a n y cases in which x - r a y e x a m i n a t i o n will m a t e r i a l l y assist in the establishm e n t of a correct diagnosis. REFERENCES
1. Findlay, L.: Radiology in the Diagnosis of Hypertrophic Pyloric Stenosis, Arch. Dis. Childhood 13: 145~ 1938. 2. Nafe~ C. A.: Congenital Hypertrophic Py]oric Stenosis, Arch. Surg. 54: 555~ 1947. 3. Robertson~ D.E.: Congenital Pyloric Stenosis, Ann. Surg. 112: 687, 1940. 4. Schaefer, A. A., and Erbes, J.: Hypertrophic Py]oric Stenosis, Surg., Gynee. & Obst. 86" 45, 1948. 5. Szilagyi, D. E, and ]~d[cGraw, A.B.: The Problems of Infantile Py]oric Stenosis With Particular l%ference to Surgical Treatment, Surgery 13" 764, 1943. 6. Ladd, W. E, Ware, P. F., and Pickett~ L.K.- Congenital Hypertrophic Pylorie Stenosis, 5. A. 1VLs 131: 647, 1946. 7. l%invik, R.: Investigations o n Congenital Stenosis of the l~ Acta paedlat. 27: 296, 1939-40. 8. Abt, I. A, and Strauss, A.A.: Clinical Study of 221 Operated Cases o2 Hypertrophic Congenital Py]orle Stenosis, Med. C]in. North America 9: 1305, 1926. 9. Akin, J. T, Jr., and Forbes, G.B.: Congenital Hypertrophic Pylorie Stenosis, Surgery 21: 512, 1947. 10. l~iller, R. F., and Ostrum, H. W.: Hypertrophic Pylorle Stenosls in Infants~ Am. J. l%entgeno]. 54: 17, 1945.
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AND WEENS:
PYLORIC STENOSIS
729
ii. Meuwlssen, T., an/[ Slooff, J.: Roentgen Examinatio~ of l~yloric Canal of Infants With Congenital 14ypertrophlo Pylorie Stenosis, Am. J. Dis. Child. 48: ]304, 1934. ]2. I-Iefke, I-I. ~V.: Roentgen Diagnosis of ~Iypertrophie Pylorlc Stenosis in Infants, Radiology 43: 267, 1944. ]3. Andresen, ~[.: Roentgeno]ogic Follow-up Examination in Congenital Pyloric Stenosis After the Manifest Stage, Acta paediat. 27: 334, 1940. 14. Conte, A. l~I., and i%/ioNally, J. T.: Congenital }Iypertrophic Pyloric Stenosis, Arch. Pediat. 63: I, ]946. ]5. IV.[oGahee, !~. C.: Congenital ]-lypertrophlc l~ylorio Stenosis~ J. l~/[ed. Assoc. Ga. 26: 89, 1937. ]6. Runstrom~ G.: On lqoentgen-anatomical Appearance of Congenital Fylorie Stenosis During and After IV[anifest Stage of the Disease, Acta pediat. 26: 383, ]939.