Congenital stenosis of the cardia with report of a case in a two-day-old infant

CONGENITAL WITH

REPORT

STENOSIS OF THE CARDIA

OF A CASE IN A TWO,DAY,OLD

J. KNOXSIMPSON,M.D., JACKSONVILLE,

J

INFANT

F.A.C.S.

FLA.

UDGING from the pubIished reports upon the subject, cardiac obstruction in the infant is either rare, or eIse cases of vomiting from this cause are being expIained on simpIer grounds, and this condition i,s being overIooked. At any rate there are few reported cases in the Iiterature. MesseIoff of New York and ShuIman of Boston, in a joint paper pubIished in 1928, reported a case in a chiId ten and one-haIf years of age, and reviewed the Iiterature from 1879 up to that time. They found onIy I I reported cases in chiIdren under tweIve years of age. The youngest was two months of age, reported by Beck, a German surgeon, which case had no x-ray study. This patient was cured by introduction of a catheter from above and feeding through the catheter. The patient whose case Messeloff and ShuIman reported, died foIIowing an esophagoscopic examination. The cause of this condition has been specuIated upon and theorized about at Iength. Some of the more common expIanations are: congenitaIIy defective vegetative nervous system, giving rise to a poorIy baIanced neuromuscuIar swaIIowing reaction, vagotonia or sympatheticotonia; pinching of the esophagus as it passes through the diaphragm; mechanica kinks from pressure, adhesions, or deviation of the norma course of the esophagus, and IastIy psychoneurosis. The morbid anatomy has been described as pure spasm in the region of the cardia; as actuaI narrowing of the submucosa1 fibrous tissue : and as norma tone at the cardia coupIed with a Iack of tone in the esophagus above. In a11 IikeIihood a11 three eIements enter into each case in varying proportions. 319

FIG. I. X-ray fiIm taken when baby was two days old, showing slight amount of barium in stomach, and typica spindle-shaped esophagus, closed at cardia and dilated above. This film was taken one hour after administration of barium, and after administration of atropine to physiologica limit.

CONGENITAL

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At any rate the x-ray findings universaIIy show a definite constriction at or just above the cardia, and a definite diIatation of the esophagus above this area.

FIG. 2. Braasch cystoscope introduced through gastrostomy opening, and cardiac orifice catheterized with a No. 6 Fr. ureteral catheter. Thread is being threaded through end of catheter, to be withdrawn through gastrostomy opening.

T. L. Birnbergl reported the cases

in a comprehensive paper on this subject of three infants with sIight spasm and

1Birnberg, T. L. Cardiospasm

in new-born infant. Am. J. Dis. Cbild., 38: I 183, 1929.

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J. KNOX SIMPSON

deIay of barium in the esophagus. He stated that recognition of the condition in a newborn infant had not be& recorded. It was our good fortune to have had under our care at the

FIG. 3. Continuous

string to which has been tied a soft rubber dilator, puIIed through gastrostomy opening into cardia.

which has been

time this paper was written, a newborn infant with this condition, recognized when the baby was two days oId. It is for the

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purpose of pIacing this case on record, and for discussing some of the probIems arising in its care, that I wish to bring the case to your attention.

FIG. 4. Construction

Baby

Y.,

of smaII hydrostatic

maIe, the second

was born in St. Vincent’s Dr. T. E. Buckman, might

arise,

dilator,

made after plan of a Plummer

chiId of heaIthy

HospitaI,

February

parents,

normaI

deIivery,

17, 1928. Our pediatrician,

was caIIed to take charge of the feeding probIems

and they

proceeded

diIator.

to arise as soon as the baby

which

began

to

take anything into the stomach. As nearIy as one couId judge, there was never from birth any food or water which reached the stomach. Feedings

324

J. KNOX

SIMPSON

would be swaIlowed and after a few moments would be vomited just as taken, and in the same quantity. The vomiting was rather forceful at times. The genera1 physica examination of the chiId was negative.

FIG. 5. Diagrammatic

iIIustration of use of hydrostatic

diIator.

When the chiId was two days oId an x-ray examination of the chest was made in a search for a persistent thymus gIand or some other cause for the apparent obstruction of the esophagus. The first fiIms showed nothing except a shadow near the hiIus of the Ieft Iung which was thought to be an enIarged mediastina1 gIand. The baby was then given a barium mixture with a medicine dropper and a sIight amount trickIed through into the stomach, but the majority of it remained in the esophagus, churning about for an hour, in spite of the administration of atropine to the physioIogicaI Iimit. FinaIIy the barium was vomited. Efforts were then made to

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pass catheters and bougies into the stomach through the esophagus, but this couId not be accomphshed. An impassable obstruction at the cardia was met with, which wouId not give way with as much force as was thought

FIG. 6. Photograph

of patient at two years of age.

wise to exert. Efforts to get the baby to swaIIow a thread to be used as a guide to diIatation were aIso unsuccessfu1. A re-check with barium was made the next day and the same condition found to exist. The baby was becoming somewhat dehydrated, and nothing was passing the cardia, so something had to be done. Two courses were discussed, one was an effort to get by the obstruction with an esophagoscope, and the other to do a gastrostomy and Iater pIan the diIatation of the cardia. The Iatter

326

J. KNOX

SIMPSON

course was chosen as being the Ieast dangerous to the Iife of the chiId, so on the third day of the chiId’s life, a gastrostomy was done. Under IocaI anesthesia a No. 16 catheter was introduced into the stomach and fixed in position. An expIoration of the cardiac end of the stomach and of the diaphragmatic opening at the time of the gastrostomy showed nothing of note except some crescentic bands extending from the fundus of the stomach up to the under surface of the diaphragm, in the region of the cardia. Whether these were a part of the norma reffection of peritoneum or not, I am unabIe to say. Feeding through the tube was begun immediateIy, and the baby began to improve and to gain weight normaIIy. At the end of ten days the tube was removed, a Braasch cystoscope passed into the stomach, and by Iocating the Iesser curvature and foIIowing it up to the region of the cardia, we Iocated it easily. It appeared as a smaI1 dimpIe, about the size of a smaI1 uretera meatus. A No. 6 uretera catheter was passed through it without d&cuIty. As it reached the posterior wall of the pharynx the baby gagged and the end was easiIy picked up with a hemostat and drawn out through the mouth. A siIk fishing Iine was threaded through the end of the catheter with a needIe and the catheter withdrawn. The ends of the thread were tied together, thus making a through-and-through continuous thread, to which couId be attached diIators, or over which couId be threaded bougies. The tube was then replaced for feeding, the string coming out of the opening aIongside of the tube. The next day diIatation of the cardia was begun. This was done with a graduated set of Jackson diIators, made of AexibIe rubber, with a string through the center with a Ioop on each end. The diIators were tied to the end of the continuous thread and puIIed through the gastrostomy opening and on through the cardia until size No. 24 was reached. They were then tied on above and introduced through the mouth and puIIed through the cardia from beIow. FinaIIy we made a hydrostatic diIator of smaI1 size by the use of gIove fingers pIaced over the end of a rubber catheter, as shown in the diagram. We used this, gauging the amount of diIatation by introducing a measured quantity of water into the catheter with a syringe. DiIatation seemed to be somewhat diffrcuIt unti1 we reached a size 24 diIator, after which time diIatation was easy. No manipuIations were accompanied by shock or other untoward reactions, and none seemed to give the baby much discomfort. Within three weeks the baby was taking a formuIa by mouth normaIIy, and a re-check of the cardia showed no deIay at this point. The gastrostomy tube was Ieft in pIace for three weeks more, to be sure there was no return of the cardiac contraction, and was then removed. The gastrostomy wound was sIow in cIosing, so three weeks Iater, under IocaI anesthesia, the

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stomach was freed from the abdomina1 waI1, the opening cIosed, and the stomach dropped back into the abdomen. The wound was closed, heaIing taking pIace without incident. The baby since then has followed the usua1 feeding and growth of a norma chiId, and is now a big husky Iad, nearIy five years old. There have been absoIuteIy no symptoms of any kind referabIe to the gastrointestina1 tract, since the case was finaIIy dismissed.

A narration of the sequence of events in this case at this time, makes everything Iook simpIe, but that is the usual simplicity of hind sight. Confronting the various probIems as they arose at the time, and trying to soIve them without any precedent to guide us, was not so simpIe as it now seems. I trust that a recording of our experiences with this case may be the means of simpIifying the same probIems for someone else, who may have a simiIar experience.