Hypertrophic pyloric stenosis in twins

HYPERTROPHIC P Y L O R I C S T E N O S I S IN T W I N S THOMAS A. CARD, M.D. RIVERSIDE, CALIF. pylorie stenosis in twins has been reported in the H YPERTROPHIC literature in only a few instances although there are numerous cases of this defect recorded in singly born babies. The r a r i t y of this condition in twins justifies this report. There is some evidence that congenital hypertrophic stenosis was recognized as early as 1627. Osler noted that Bearesley of New Itaven, Conn., gave one of the most accurate descriptions of the condition that is extant in the literature. Since then there have appeared in the literature numerous instances of this disease in infants. Our modern concept of this condition dates from the time tIirschsprung described it as a medical surgical entity. Finklestein in 1896 described the presence of a palpable epigastric tumor which suggested the possibility of surgical intervention in these cases. The incidence of mortality remained high. Ramstedt performed the pylorop]asty now ]r by his name. This operative procedure was the means of lowering the mortality rate and has become accepted as the procedure of choice. The etiology of this condition is clouded in the haze of uncertainty although there is much in the literature which strongly suggests a congenital factor and a distinct tendency to increased incidence in males. As reported by Wallace and Wevill, there were 125 males i~ 145 cases of hypertrophic pyloric stenosis. It is difficult to u n d e r s t a n d why hypertr0phie pyloric stenosis should show such a marked preponderance in males. The etiology of py]orospasm can be demonstrated theoretically only. The fact that the great majority of patients in these cases show clinical symptoms from the second week to the sixth week of life has brought out various theories to account for the condition. One of these is that overdistention of the gastric musculature causes gastroenterospasm which leads to hypertrophy of the musculature, but this has never been demonstrated. Another theory suggests the presence of a central reflex arc of the vagus supplying the motor fibers to the stomach while acting as an inhibition on the pyloric sphincter. Ma]development of the are would result in a contraction in the sphincter against gastric peristalsis, thus theoretically resulting in hypertrophy. I{ecently there l~ave appeared in the l i t e r a t u r e instances which suggest strongly a genetic basis for pyloric stenosis although the way in which it is inherited is still uncertain, and an environmental factor, in addition to a genetic one, may be necessary for its production.

658

CARD:

HYPERTROPHIC

PYL,ORIC S,TEI'r

IN TWINS

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B r i n d l e r e p o r t s t h e ease of a b a k e r ' s f a m i l y l i v i n g in t h e c o u n t r y u n d e r f a v o r a b l e c i r c u m s t a n c e s . I n t h i s f a m i l y t h r e e b r o t h e r s a l l had. severe p y l o r i e o b s t r u c t i o n , t w o d y i n g f o l l o w i n g o p e r a t i o n f o r relief. A n u n c l e of t h e c h i l d r e n on t h e m a t e r n a l side h a d likewise suffered f r o m p y l o r o s p a s m . H e also r e p o r t s a l a b o r e r ' s f a m i l y of t e n c h i l d r e n , of w h o m five d i e d of p y l o r o s p a s m . The c h i l d r e n of t h e r e m a i n i n g t w o sons, a b o y a n d a g i r l , w e r e n o r m a l ; b u t a l l t h e m a l e c h i l d r e n of t h e t h r e e n o r m a l d a u g h t e r s w e r e affected. H e c o n c l u d e d t h a t in p y l o r o s p a s m one h a s to d e a l n o t o n l y w i t h i n h e r i t e d d i s p o s i t i o n s b u t w i t h a specific h e r e d i t a r y f a c t o r . C o e k a y n e (1934) r e p o r t e d the o c c u r r e n c e of p y l o r i c stenosis in t w i n s who were first cousins, a n d t h e d i a g n o s i s was v e r i f i e d i n all f o u r b a b i e s a t o p e r a t i o n . H e c o n c l u d e s t h a t t h e s e two a u t h e n t i c a t e d p e d i g r e e s p r o v i d e d f u r t h e r e v i d e n c e t h a t c o n g e n i t a l p y l o r i e stenosis is a n i n h e r i t e d defect. V a r d e n (1933) r e p o r t s a ease of h y p e r t r o p h i c p y l o r i e stenosis in monozygotie twin boys with recovery following operation, t i e coneludes t h a t t h e r e is m o r e evidence in f a v o r of a c o n g e n i t a l t h e o r y t h a n a g a i n s t it, b u t m o r e e v i d e n c e m u s t be r e c o r d e d b e f o r e a definite conclusion can be r e a c h e d . I n t h e eases to be r e c o r d e d t h e f o l l o w i n g d a t a r e l a t i v e to t h e m a r e of i n t e r e s t . T h e p a r e n t s w e r e Mexican, t h e b a b i e s b o r n a t t e r m in A u g u s t , 1937. T h e r e were f o u r o t h e r c h i l d r e n , two m a l e s a n d two females, all s i n g l e births. T h e r e was no h i s t o r y of s t o m a c h t r o u b l e , in a n y of t h e c h i l d r e n or p a r e n t s or in t h e f a m i l i e s of t h e g r a n d p a r e n t s . T h e o b s t e t r i c i a n who delive*.ed t h e m r e p o r t e d t h e y were a p p a r e n t l y i d e n t i c a l single o v u m t w i n s h a v i n g one p l a c e n t a a n d one ehorion. T h e p l a c e n t a w a s - n o t o t h e r wise u n u s u a l . T h e b a b i e s were in t h e h o s p i t a l f o r t h r e e d a y s a n d exh i b i t e d l~o g a s t r i c d i s t u r b a n c e s . S u b s e q u e n t c a r e was g i v e n a t home b y t h e m o t h e r , a n d i t w a s n o t u n t i l one m o n t h l a t e r t h a t one of the t w i n s was b r o u g h t to t h e h o s p i t a l f o r care. F i v e d a y s l a t e r t h e second t w i n was b r o u g h t i n t o t h e h o s p i t a l s u f f e r i n g in a s i m i l a r m a n n e r . Case 1.--l-IospitM No. 30107. A. M., aged 28 days, Mexican, male twin, baby was delivered spontaneously in a hospital. He was fed breast milk plus supplementary feeding of evaporated mi]k mixture without supervision after the third day. On discharge from the hospital the baby was apparently normM. When the child was 28 days old, he was returned to the hospital acutely ill from malnutrition. The abdomen was moderately distended, and the mother staged that food had not been retained for over a week. Examiuatioit showed a malnourished child, with abdomen distended and a palpable epigastric tumor. A barium meal was passed into the stomach and observa$ion under the fluoroscope failed to show any passing through the pylorus. Regurgitation of the stomach contents was of the forceful type. A diagnosis of congenital hypertrophic pyloric stenosis was mmde, and operation was advised.

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The findings at operation were typical of tile above disease. The pylorus was greatly hypertrophied, and the stomach wall showed chronic dilatation. Following the technique of iBamstedt, an incision was made through the hypertrophied pyloric sphincter, and the abdomen was closed. The immediate condition seemed good but circulatory failure develope.d, and the baby died seven days later. CASE 2.--Hospital No. 30164. A. M.~ aged 33 days, twin brother of baby in Case 1, was brought to the hospital with a history of vomiting~ inability to retain food, and rapid loss of weight. A physical examination revealed a moderately undernourished and dehydrated infant. There was some distention of the abdomen, and a definitely palpable persistent tumor was present to the right of the epigastrium. The Xahn blood test was negative. Barium in small quantity was placed in the stomaeh~ mad under the fluoroscope it was found that none had passed through the pylorus even after two hours' observation. A diagnosis of hypertrophic pyloric stenosis was made, and immediate operation was done. The child was anesthetized with ether and the findings were as in Case 1. l~amstedt operation was performed. Recovery was uneventful. Food was retained early and general improvement was noted within twenty-four hours. He was dismissed from the hospital five weeks later having made a satisfactory weight increase and in normal condition.

We are unable to show a genetic factor in these cases. However, they were without doubt identical twins and the occurrence of pylorospasm in each suggests a congenital developmental factor possibly of neurological origin. SUMMARY P y l o r i c s t e n o s i s in t w i n s is of r a r e is n o t as y e t f u l l y d e t e r m i n e d .

occurrence;

the etiology

of i t

S o m e cases a r e c i t e d to s h o w t h e p o s s i b i l i t y o f a n i n h e r i t e d d e f e c t as t h e f a c t o r i n p r o d u c i n g c o n g e n i t a l p y l o r i c s t e n o s i s . A r e p o r t is g i v e n of c o n g e n i t a l p y l o r i c s t e n o s i s in t w i n m a l e b a b i e s w i t h o p e r a t i o n . T h e p o s s i b i l i t y of a d e v e l o p m e n t a l c o n g e n i t a l d e f e c t of n e u r o l o g i c a l o r i g i n is s u g g e s t e d . REFERENCES 1. 2. 3. 4.

Symposimu Section, International Medical Digest 26: 2~45~ 19~5= Brindle, E.: Monatschr f. Kinderh. 68: 295, 1937. Coekayne, E . A . : Lancet 1:8985 1934. Varden, A. E.: J. PEmA~~. 3: 493, 1933. 3616 MAIN ST~Em'