Infantile pyloric stenosis

Infantile

Pyloric

A REVIEW CLIFFORD D. BENSON, M.D.

AND

OF

In 1957 we reported a survey of 707 infants operated upon for pyIoric stenosis from 1940 through 1955 [2]. In the present report, we have extended the study to incIude an additiona1 group of 413 infants treated for infantiIe pyIoric stenosis from 1936 to 1963, making a total of 1,120 patients during a twenty-three year period on the SurgicaI Service at the ChiIdren’s HospitaI of Michigan and on our private service at Harper HospitaI. Of the 413 infants in the 1956 to 1963 period, 412 were operated upon. One infant was admitted in a moribund state and died without responding to preoperative preparation.

NFAN’I’ILE pyIoric stenosis for some years has been and continues to be the most common Iesion requiring abdomina1 surgery during the first six months of life. This entity, first described by Hirschsprung just three quarters of a century ago, and having been subject to direct surgica1 intervention by means of the Fredet-Ramstedt pyh~romotomy for just half a century, was once a dread attended by an aImost scourage of infancy, negIigibIe survival rate [I]. However, during the past quarter of a century, it has become attended by an ahnost negIigibIe mortaIity. One of the principa1 factors contributing to this improved outIook has been the progressively earlier recognition and more prompt surgica1 management of the Iesion. With earIier diagnosis, fewer infants are being admitted to our hospitaIs in a state of severe aIkaIosis and malnutrition. This has brought about a dramatic shortening of the time required for preoperative preparation and an even more dramatic reduction in the postoperative hospita1 stay. (Figs. I and 2.)

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2

3

n

2

3.5

I :I

r

CASES

JAMES R. LLOYD, M.D., Detroit, Michigan

From tbe Surgical Services of tbe Children’s Hospital of Michigan and tbe Harper Hospital, Detroit, Michigan.

4

1,120

Stenosis

ETIOLOGY The exact etioIogic cause of congenitat pyIoric stenosis remains unknown despite a voIuminous research effort, the majority of which has been directed toward the eIucidation of a genetic factor or the demonstration of an histopathoIogic abnormaIity. However, there may be a simpIe physioIogic expIanation as proposed by Lynn [2]: miIk curds, propuIsed against a pyIoric cana in spasm, resuIt in edema of the pyIoric mucosa and submucosa

3

1940-45 1945-50 1950-55

m

1955-60 1961-62

1950-55 1965-60

F~ti. I. Comparison of the average time required for preoperative preparation during each of the five periods studied.

1961-62

FIG. 2. Comparison of the average postoperative hospital stay during each of five periods studied.

429

American

Journal

of Surgery.

Volume

IO,.

March

1964

Benson and LIoyd fact that one or more sibIings had been operated upon for pyIoric stenosis in infancy. In four families, the patient was the third chiId invoIved and in one instance, the patient was the fourth chiId to undergo pyIoromyotomy. Eight fathers and six mothers had undergone surgery for pyIoric stenosis in infancy. InterestingIy enough, the three sets of twins operated upon between 1956 and 1963 consisted of one set of premature identica1 maIe infants, one set of premature identica1 Negro femaIe infants, and one set of fraterna1 twins, a maIe and a female. In tweIve instances, uncIes or cousins had previousIy undergone pyIoromyotomy for pyIoric stenosis in infancy. SYMPTOMS

FIG. 3. Upper gastrointestina1 study demonstrating the typica narrowing and eIongation of the pyIoric cana (string sign) and marked gastric retention.

which narrows the pyIoric canaI. There is a concomitant response of work hypertrophy of the pyIoric and gastric muscuIature, setting up a vicious circIe, progressing to high grade obstruction of the pyIoric cana1. This seems IogicaI as we have examined infants admitted as having possibIe pyIoric stenosis at the first sign of vomiting, in whom initiaIIy we have not been abIe to palpate a tumor. After two or three days of carefu1 observation, an unmistakabIe pyIoric tumor wouId suddenIy appear. PresumabIy, the process suggested by Lynn was taking pIace. INCIDENCE

Of the I, I 20 infants in this series, I ,o I 5 were white and 105 were Negro. As in nearIy a11 series, maIe infants greatIy predominate; in this group of infants, 882 were maIe and 238 were femaIe, a ratio of approximateIy four to one. Thirty-six infants were premature and a11 survived operation. There were twenty-two sets of twins, and in five sets both required pyIorotomy. In the remaining seventeen sets, onIy one twin was invoIved. The famiIy history of thirty-two of these patients discIosed the 430

AND

SIGNS

The average age for the onset of vomiting in this study and in nearIy a11 reported series is about three weeks, aIthough the onset may be much earIier. In this series forty-one infants had vomiting from birth. BiIious vomiting was absent in a11 I, 120 patients, but occasionaIIy a history of vomiting coffee-ground materia1 apparently caused by hemorrhagic gastritis or superficia1 mucosa1 erosions was ascertained. In our previous study of 707 patients, 68 per cent of the tumors were paIpated. This percentage has increased to 78 per cent in the Iast 413 patients, presumabIy as a resuIt of a more meticuIous and more patient abdomina1 examination. In actuaIity, this percentage varied considerabIy with the individua1 examiner. Upper gastrointestina1 series were performed in 181 or 25.6 per cent of the first 707 patients. In the Iast 413 patients this percentage increased to 48 per cent. We as surgeons do not advocate routine upper gastrointestina1 studies when the tumor is paIpabIe; however, nearIy a11 of the x-ray examinations had been performed at the request of the referring physician before surgica1 consuItation was obtained. (Fig. 3.) Recent reports in the Iiterature have caIIed attention to the occasiona association of jaundice with pyIoric stenosis [4]. In the first 707 patients in this study, jaundice was apparentIy not a prominent part of the cIinica1 picture, or if present was not considered a significant factor. At Ieast, no direct reference was made to jaundice. Ten babies in the Iast 413 patients dispIayed clinica evidence of jaundice, with a biIirubin IeveI ranging to 18.7 mg. per cent. Exchange

Inf:kntiIe

PyIoric

Stenosis

t ra nafu>ic 11)s were not deemed necessary in and the jaundice subail!. of thcsc patients, sided from tM o to five days after p>-Iorom.);otorny. \Ve can not be certain whether this or response \VBS a resuIt of pyIoromyot.omy adequate h,vdration during the preoperative preparation. WC tend to favor the latter suggestion. PREOPERATIVE

PREPARATION

With earlier diagnosis of pyIoric stenosis, we are confronted with significantly fewer patients in :I state of severe dehydration, aIkaIosis or maInutrition. As pointed out by Lynn [3], infants admitted to the hospita1 with a minima1 or moderate degree of dehydration and alkalosis can be prepared by the ora 5 per cent route aIone. In our institution, dextrose in 0.33 per cent norma saIine soIution in amounts up to 3 ounces is offered every two hours. When a satisfactory state of hydration is reached, 3 to 4 mEq. of potassium chIoride is added to every second or third bottIe, not to exceed 4 mEq. per kiIogram per day. We have used this regimen in a Iimited number of patients and it has proved quite satisfactory. However, the preoperative preparation in the great majority of patients in this series has been managed by the administration of intravenous fIuids. The stomach is emptied of curds and barium by carefu1 saIine gavage and ora fluids are withheId. RoutineIy 5 per cent dextrose soIution in 0:.33 per cent norma saline solution is administered, usualiy by a scaIp vein. The amount of infusion varies from 60 to go cc. per pound of body weight per day, depending on the degree of dehydration. When adequate urinary output has been IO mEq. of potassium chIoride is estabIished, added to every 250 cc. of soIution. We never exceed a concentration of 40 mEq. per Iiter unless there is eIectrocardiographic evidence of a severe potassium deficiency. Laboratory data obtained on admission are usuaIIy Iimited to a compIete bIood count, urinaIysis, serum chIoride and carbon dioxide determination. These serve as a vaIuabIe aid in preoperative evaluation. If the initia1 CO2 is 37 mEq. or higher, we check the patient’s progress by obtaining a second reading in twenty-four hours. From a practica1 standpoint, those patients with minima1 or moderate dehydration and miId alkaIosis (CO2 below 37 mEq. per Iiter and chIorides above go mEq. per Iiter)

can usuaIIy be prepared for operation within twenty-four hours of admission, while those in a more se\.ere state of eIectroIytc imbalance and dehydration can almost always be adequateIv prepared within forty-eight hours after admisiion. AIthough sixth moIar ammonium chIoride was previousIy advocated as an acIjunct to the repair of more severe nlkalotic states [6,7], we have not found it necessary to resort to the use of this agent to correct eIevated CO2 IeveIs since we have begun using adequate amounts of potassium chIoride in our repair fluids. OPERATIVE

PROCEDURE

The pyIoromyotomies reported in this series were performed in part by the attending staff surgeons of our institutions and in part by surgica1 residents under staff supervision. The operative procedure is simiIar to that described in a previous communication [2] with onIy some minor changes. For cosmetic reasons, we now nearIy aIways empIoy a transverse skin incision but continue to spIit the rectus muscIe in a IongitudinaI direction. The posterior rectus sheath and peritoneum are incised in a transverse direction, thereby having not onIy the advantage of a gridiron incision, but also the convenience of a muscIe spIitting incision as weI1. The skin is closed with subcuticuIar No. 4-o plain catgut, and a thin Mm of fTexibIe coIIodian is used for a dressing. We continue to be satisfied with the use of the previousIy described pyIoric spreader (Fig. 4), beIieving that this instrument enabIes one to separate effectiveIy the circuIar muscIe of the pyIorus with minima1 bleeding and fragmentation of the muscIe fibers. (Fig. 5.) The fine point and curved hemostats are no Ionger used in our cIinic because these instruments are not designed for this maneuver; they are more hazardous insofar as perforation is 43’

Benson

and LIoyd that the pyIoromyotomy was incompIete or the infant has had a recurrence. We are strongIy opposed to reoperation in Iess than two weeks and in nearIy a11 instances, vomiting wiI1 have ceased by that time. COMPLICATIONS

Perforation of the duodenum can be a tragic technica error in the performance of pyIoromyotomy if the opening is not recognized and cIosed. In our previous study of 707 patients, onIy three instances of perforation were recorded. Two were recognized and cIosed with no morbidity, but in one instance a smaI1 perforation was overIooked and resuIted in fata peritonitis. In the Iast 412 patients operated upon, perforation occurred in twenty instances and was recognized and cIosed with no mortaIity. This increase in the incidence of perforation in performing pyIoromyotomy in the Iast group of 412 patients is most IikeIy due to the fact that some surgeons carry the serosa1 incision into or beyond the white Iine (pyIoric vein) which onIy invites this compIication. As has been emphasized before, one shouId onIy incise to the white Iine which obviates this compIication but stiI1 permits compIete pyIoromyotomy to be performed. Minor wound infections occurred in 5.9 per cent of 707 patients in the earIier study and have decreased to an incidence of 3.1 per cent in the Iast 412 patients. One wound disruption and one incisiona hernia occurred in the former group and none have occurred in the Iatter group of patients. There were four recurrences in the first 707 patients. Two of these infants underwent the origina surgery eIsewhere. AI1 four underwent successfu1 secondary pyIoromyotomy. In the Iast 412 patients, four recurrences occurred. Three of these infants had undergone the origina pyIoromyotomy eIsewhere and two of these were successfuIIy reoperated upon. The other two died of compIications before reoperation couId be accompIished. Death was attributed to inanition and severe eIectroIyte imbaIance in one of these babies and the other died from septic emboIi and renaI faiIure.

Frc. 3. PyIoric spreader in use. concerned and offer Iess insurance against the performance of an incompIete operation. Prior to 1950 IocaI anesthesia was empIoyed in 35 of 506 pyIoromyotomies. Since that time genera1 anesthesia has been used excIusiveIy. The avaiIabiIity of skiIIed pediatric anesthesioIogists in our institutions has been of considerabIe benefit to both surgeon and patient by enhancing the efficiency and safety of this operative procedure. POSTOPERATIVE

TREATMENT

The administration of ora feedings is begun six hours after operation if the infant is aIert and has a good sucking refIex. Oral feedings consist of I0 per cent sugar water ahernating with one-haIf strength evaporated miIk formuIa every two hours, starting with IO cc. and increasing IO cc. every hour unti1 2 ounces are taken and retained at one feeding. The evaporated miIk formuIa is then changed to fuII strength, the time interva1 is Iengthened to three hours and the voIume of the feedings is graduaIIy increased. By the time these infants are discharged from the hospita1, they are usuaIIy taking and retaining 3 or more ounces every four hours. In the infant who was severeIy dehydrated before operation, intravenous fluids are usuaIIy continued for the first twenty-four hours after operation. About one haIf the babies wiI1 spit up a smaI1 amount of formuIa during the earIy postoperative feeding routine and rareIy an infant wiII vomit persistentIy. This is frequently corrected by gastric gavage with warm saIine soIution, thereby ridding the stomach of an accumuIation of thick, tenacious mucus, and vomiting ceases. When the infant continues to vomit beyond the fifth or sixth postoperative day, the surgeon is pIagued with the thought

MORTALITY In our previous study of 707 patients (1940 to 1955) four deaths occurred. Three of the deaths were the resuIt of postoperative diarrhea 432

Infantile

Pyloric

Stenosis

2. ‘I‘he classic Ramstedt pvIorc)m~c)tolrl~ was performed in 1,119 infants with ;I mortalit! of 0.s~ per cent. 3. .Se;;en deaths occurred in 1,120 patients for an over-all mortality of 0.02 per cent. LL Seven hundred seven Datients lverc examined between the years 1.940 and 1056 and 413 patients between the years I9j6 and 1963. Certain comparisons between these two groups of patients are anaIyzed and discussed. 5. Age, sex and coIor incidence are presented. 6. CompIications, recurrence and mortaIity are reviewed.

;~nd the fourth was attributed to :t fulmin:rnt prritl jnitis from an overIooked duodenal perforation. In the last l13 patients (1956 to 1963) three tlc;rth occurred. Postmortem lindings disc,losed severe inanition, dehydration and electrolytc imbalance in one of these infants, renal infarction and extensive Iiver necrosis of unknown ctiologic cause in the second infant, and dehydration, septic emboIi and renaI failure in the third patient. In summarizing the mortaIity of the entire group of infants with pJ;Ioric stenosis, I, I 19 were operated upon with SIX deaths, an operative mortality of 0.54 per cent. One patient, who did not undergo surgery, was admitted in a moribund state and faiIed to respond to intensive corrective fluid and electrolyte therapy. The operation for pyIoric stenosis continues to be one of the most gratifying surgica1 procedures empIoyed today. AIthough the etioIogic cause of the disease is as yet unknown, this does not seem to inffuence adverseIy the outIook for these infants. We have not had any deaths in the Iast 403 consecutive patients operated upon for infantile pyIoric stenosis and at present the surviva1 rate is 99.38 per cent.

REFEREi’iCES I. MACK, H. C. A history of hypertrophic pyIoric stenosis and its treatment. Bull. Hi.ct. Med., 12: 465. 595, 666, 1942. 2. BEXSON, C. D. and WARDEN, hl. J. Seven hundred and seven cases of congenita1 hypertrophic pyIoric stenosis. Surg. Gynec. ti Obst., 103: 348, 354, 1957. 3. LYNN, H. B. The mechanism of pyloric stenosis and its relationship to preoperative preparation. Arch. Surg., 81: 453, 1960. 4. ARIAS, I., SCHORR, J. B. and FKAAD, L. M. Clinical conference: congenital hypertrophic pyloric stenosis with jaundice. Pediatrics, 24: 338, ,959. 5. RHEA, W. G., JR., HEADRICK,J. R. and STEPHENSON, S. E., JR. Hgpertrophic pyIoric stenosis with jaundice. Surgkr), 51:.687, ;662. 6. BENSON, C. D. and ALPERN, E. B. Preoperative and postoperative care of congenita1 ovIoric stenosis. A&b. Surg., 75: 877, 195; I LI 7. BENSON, C. D., MUSTARD, W. T., RAVITCH, M. K., SNYDER, W. H., WELSH, K. J. Pediatric Surgery. Chicago, 1962. Year Book Publishers. Inc.

SUMMARY

In a twenty-three year period (1940 to I, 120 infants were treated for infantiIe pyIoric stenosis. I.

1963)

433