Duplication of the Entire Small Intestinewith Massive Melena *

THEODoRE C. JEWETr, JR., M.D.

From the Children's Hospital of Buffalo and the Department of Surgery,University of Buffalo School of Medicine

IntroductionDUPLICATIONS of the intestinal tract are

now being recognized with increasing fre-quency as a cause of melena in childhood.The recent surge of interest in pediatricsurgery in the past few years has familiar-ized many more physicians with these un-usual anomalies of the intestinal tract, andas a consequence these pathologic lesionsare now being encountered more com-monly than in the past. Since massive gas-tro-intestinal tract bleeding due to duplica-tion is amenable to surgical treatment, it isof the utmost importance to recognize thislesion early so that corrective measures maybe undertaken. While the diagnosis oftencannot be made before laparotomy, it isnecessary for the surgeon to realize thatduplications can cause melena and to rec-ognize the lesion at the time of operation.The blood loss suffered in duplications of

the intestinal tract is due to one of threeetiologic factors: (1) peptic ulceration, (2)impairment of intestinal blood supply or(3) intussusception (Fig. 1). Peptic ulcera-tion may occur in the normal bowel mucosaat the site where it communicates with theduplication which contains gastric mucosaor even occasionally in the duplication it-self. The more distal in the small intestinethis type of duplication is found the morelikely is the chance of ulcer formation withhemorrhage, since it is now generally recog-nized that there is a progressive increasein susceptibility to peptic ulceration in thesmall intestine from the ligament of Treitz

to the ileocecal valve. Another rarer cause ofmelena due to duplication is necrosis of thenormal intestinal wall from interferencewith blood supply because of the space-occupying effect of duplications which haveno communication with the normal gastro-intestinal tract. This type of duplicationgradually increases in size as the mucosalsecretions accumulate in the blind loop,thus causing venostasis in the mesentericveins by compressing them as they courseover the duplication. The same series ofevents then occurs as in any obstruction inwhich the arterial blood continues to bepumped into the bowel and there is noescape for the venous blood through normalchannels. A third cause of melena asso-ciated with duplication is actually the re-sult of the duplication being a lead pointin an intussusception resulting in the typicalcurrant jelly bleeding seen from that lesion.

Duplications involving almost the entiresmall bowel are extremely rare. In review-ing the literature for the past ten years, itwas discovered that ones involving the en-tire colon are not too uncommon. Therewere also several reports of one-half to two-thirds of the small intestine being involvedby this entity, but none similar to either ourpatient or the case reported by Thompson,Wilson and Cunningham.3 Their child hada duplication of the mediastinum as well asa second one involving the entire smallintestine with the exception of a small por-tion of the terminal ileum. At the upperjejunal end of the duplication there waspeptic ulceration which was the source ofthe massive hemorrhage, with an apparent

239

v Submitted for publication June 28, 1957.

JEWETT Annals of SurgeryFebruary 1958

B Duplicatton'%% l

A

IULCERFIG. 1. a. Gastro-intestinal tract bleeding due to venous obstruction from a distended dupli-

cation within the leaves of the mesentery. b. Gastro-intestinal tract bleeding due to the duplica-tion being the lead point of an intussusception. c. Melena due to peptic ulceration from aduplication with gastric mucosa (artist's conception of resected specimen in our case).

communication at the lower end with thelumen of the normal ileum. Excision of theulcerated area was performed with an end-to-end anastomosis of the normal boweland then turning in the proximal part of theduplication as a blind end. This patient didwell postoperatively with no evidence ofany hemorrhage at a later date. A secondcase was reported by Ripstein 2 which in-volved a large portion of the small intestine,starting in the lower part of the jejunumand extending the entire length of theileum. In this child a local excision of thedistal end of the duplication where it en-

tered the normal lumen of the intestine wasperformed for massive gastro-intestinalbleeding, with recurrent hemorrhage tendays later. This necessitated excision of theentire duplication with adjacent normal

bowel. This child gradually went downhilland died from inanition and nutritionaldeficiency due to the large portion of smallintestine removed.

Duplications with gastric mucosa whichcause massive bleeding due to peptic ulcerpresent an extremely interesting problemin therapy. This anomaly can be easilyhandled in those children who have onlya short segment of the small intestine in-volved, as local excision of both the dupli-cation and the adjacent bowel can be per-

formed with end-to-end anastomosis of theremaining small intestine. However, whena duplication involves the major portion ofthe small bowel and is causing melena dueto ulceration of the mucosa of the normaladjacent bowel from peptic digestion, itposes an extremely difficult problem. Local

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DUPLICATION OF ENTIRE SMALL INTESTINE

excision of the ulcerated area where theduplication enters the normal small bowelwith re-anastomosis is not the solution,since this only removes the local pathologybut not the etiological cause of the ulcera-tion, and the process of ulceration recursagain at the site of the anastomosis. Exci-sion of the duplication and leaving the nor-mal adjacent small bowel intact is impos-sible due to the common blood supplyderived by the two structures. Conse-quently, any attempt to remove the duplica-tion results in impairment of the blood sup-ply to the adjacent bowel with consequentnecrosis. Excision of the entire area is notpracticable because of the large segmentsof normal small bowel that must be re-moved with duplications of this magnitude,since resections of major portions of thesmall intestine invariably lead to a gradualdownhill course of the patient with inani-tion due to nutritional deficiencies andeventually death. It is because of theseproblems that we are presenting this case

report with the surgical treatment used tosolve this child's difficulty.

Case ReportB. W., a five-month-old male infant, was first

admitted to the Buffalo Children's Hospital on

September 18, 1955, with a two-day history ofpassing bloody stools. The admission hemoglobinwas 7.4 Gms. Following 24 hours of observation,during which time there was no evidence of anyfurther active bleeding, x-ray studies of the entiregastro-intestinal tract incltuding the esophagus andcolon were done. These revealed a normal intes-tinal tract. Sigmoidoseopic examination and hema-tologic studies were also done and were found tobe within normal limits. Two transfusions were

given which increased the hemoglobin to 12.5Gms. and the patient was then discharged afterno further evidence of bleeding.

He was readmitted on October 24, 1955, whenhe again began to pass currant jelly type stool.Hemoglobin on this admission was 9.3 Gms. Phys-ical examination again was essentially negativeexcept for dark red blood on the tip of the exam-

ining finger. Repeat x-rays of the gastro-intestinaltract and a sigmoidoscopic examination were doneand revealed no source for the bleeding. The infant

FIG. 2. Artist's conception of operative findings.Upper drawing shows proximal blind end of dupli-cation. Lower drawing shows duplication at itsdistal end where it communicates with the lumenof the terminal ileum.

was given one transfusion of whole blood andlaparotomy then performed. At the time of opera-

tion a duplication was found which involved theentire small bowel except the distal 10 cm. ofileum (Fig. 2). At its distal end the duplicationand normal ileum were in such close proximityto each other that they appeared to be a single nor-

mal loop of bowel on external examination. How-ever, in the lower jejunal area a line of demarca-tion could be seen between these two structures,and from this point proximally the duplicationbecame increasingly farther away from the ad-jacent bowel as it descended between the leaves ofthe mesentery. There was marked inflammation andedema where the duplication communicated withthe terminal ileum. This inflammatory area wasresected and an end-to-end anastomosis done be-tween the double-lumened proximal ileum andthe single-lumened distal ileum. The resected por-

tion of bowel was reported several days later by

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242 !EWETT

Cross section

FIG. 3. Artist's conception of the final operativeprocedure. This was an end-to-end ileo-ileostomy,turning in the distal end of the duplication as ablind loop and then making a window between thedistal end of the duplication and the stomach.

the pathologist as showing a peptic ulceration inthe normal ileal mucosa just adjacent to the entryof the duplication. The microscopic appearance ofthe mucosal duplication was typical of gastricglands.

The child began to pass normal brown stoolson the second postoperative day. However, on thethird day following surgery, he again began topass currant jelly stools and his hemoglobin fell to9.5 Gms. On the fifth day after the initial surgicalprocedure, the abdomen was reopened because ofcontinued melena and the previous anastomoticarea resected. The distal end of the duplicationwas then tumed in as a blind stump and end-to-end anastomosis done between the proximal anddistal loops of ileum (Fig. 3). Following this, theterminal ileum with the distal end of the duplica-tion was swung up to the stomach where side-to-side anastomosis was performed between the du-plication and the stomach, thus draining the distalend of the duplication into the stomach. The re-sected surgical specimen again showed an ulcerat the site where the duplication and ileum hadbeen previously anastomosed to the terminal ileum.The patient's postoperative course this time wascompletely uneventful and there was no evidenceof any gastro-intestinal bleeding either grossly orby guaiac test. A barium swallow one week post-operatively revealed a small trickle of bariumpassing from the stomach through the anastomosisinto the duplication. The remaining barium emp-tied from the stomach in a normal manner throughthe duodenum. The barium which had entered theduplication showed complete evacuation one hourlater. He was discharged on the tenth postopera-tive day.

Annals of SurgeryFebruary 1958

Follow up on this patient to the present date,which is now one and one-half years postopera-tively, reveals the child to be growing and develop-ing normally, gaining weight and showing no evi-dence of any acute or chronic blood loss.

DiscussionThe ideal therapy for duplications in-

volving the intestinal tract would be sur-gical excision of this pathologic entity.However, it is now well accepted that thisis anatomically impossible because of thedual blood supply to the duplication andthe adjacent bowel. Consequently, the nextprocedure of choice would be excision ofthe duplication with the adjacent intestinewhich, in most cases, is done. However, thisis not always feasible, because of either thelocation of the duplication or the extensiveinvolvement of a major portion of the in-testinal tract. A good example of a duplica-tion which is unresectable because of itslocation is one lying in the area of theduodenum in which it would be necessaryto remove not only the duodenum andduplication but also the head of the pan-creas with re-implantation of the pancreaticand common ducts. This is an extremelyformidable procedure for a benign condi-tion. The simple solution to a duplicationof this nature has been the creation of awindow between the duplication and theadjacent duodenum. When a duplicationwhich involves an extensive portion of gas-tro-intestinal tract is found, it poses an en-tirely new problem. In this situation, totalexcision of this lesion and the adjacentintestinal tract is impossible because of thenutritional deficiencies which follow. In-ternal drainage into the adjacent intestineis an adequate form of therapy if the dupli-cation is causing symptoms purely due tothe space-occupying effect of the duplica-tion. However, if the symptomatology isthat of massive bleeding due to pepticulceration, then an entirely different situa-tion presents itself, for certainly here localresection of the involved area with internal

DUPLICATION OF ENTIRE SMALL INTESTINE

drainage would only lead to a similar cycleof events and new ulceration in the area ofthe entrance of the duplication into thesmall intestine. This proved to be the situa-tion in our particular case following the firstoperative procedure. It then becomes ap-

parent that in a situation such as this it isnecessary to not only divert the stream ofgastric juice produced by the duplicationfrom the sensitive small intestinal mucosa,

but also to institute some form of drainageso that the duplication may empty itself.If this latter procedure were not done, one

would be faced with the new problem ofgradual enlargement of the duplicationwith the space-occupying effects of eitherobstruction to the adjacent bowel or gastro-intestinal tract bleeding from the venous

impairment produced. Thus the only twolocations into which this duplication couldbe safely drained were either to the outsideby marsupialization or to the only place inthe gastro-intestinal tract resistant to pepticdigestion, namely, the stomach. It was feltthat marsupialization was not the accept-able procedure because of the severe ex-

coriation and digestion of the abdominalwall that would result. Consequently, theoperation of internal drainage of the dupli-cation into the stomach by creation of a

window was felt to be the operation ofchoice.One remaining problem, however, had to

be faced first, and this was the solution towhether the duplication had gastric mucosainvolving its entire length or just locallywhere it emptied into the ileum, as if thelatter were the case a local resection shouldsuffice. Nothing could be found in the liter-ature about this during the short periodof time we had between the primary pro-cedure and the second operation, and, con-

sequently, it was felt that the operativeprocedure described should be performed.However, since then correspondence withDr. John Craig,' pathologist at the Chil-dren's Medical Center in Boston, revealed

that Dr. John L. Bremer, the consultant inembryology at this institution, feels that ingeneral a duplication usually has the epi-thelium of the adjoining intestine. However,the blind end in such duplications, if theyjoin the intestine, quite frequently containgastric mucosa even though the remainderof the duplication may have normal in-testinal epithelium. In this fashion, theduplication mimics a Meckel's diverticulum.This undoubtedly was the situation in thepatient described by Thomson, Wilson andCunningham.3 However, in our case, thegastric mucosa was not at the blind end ofthe duplication but at the end that com-

municated with the normal intestinal tract.Therefore, the operative procedure per-

formed was most likely the only solution,since both resected specimens whichamounted to approximately 20 to 30 cm.

of the intestinal tract showed only gastricmucosa. Thus it could be assumed that theremaining portion of the duplication con-

tained similar type mucous membrane.Consequently, the second operative pro-

cedure performed on this child theoreticallysatisfied all the criteria to prevent recur-

rence of symptoms from the duplication.By isolating it from the small intestine, no

further peptic ulceration with hemorrhagecould occur. Yet, the duplication couldempty itself through the opening into thestomach, thus sidestepping the problemsinitiated when a duplication increases insize because of the lack of a passageway

through which the mucosal secretions can

escape. The absence of any further gastro-intestinal tract hemorrhage for one and a

half years since the last operation bears outthe efficacy of this operation in regard tocorrection of the cause of the melena. Nor-mal growth and development of this boyindicates that this procedure in no way

interfered with the normal physiologicfunctions of digestion and absorption of theintestinal tract.

Volume 147Number 2

244 WAnnals of Surgery244 WIlTFebruary 19S8

Conclusions1. A case of gastric mucosal-lined dupli-

cation involving almost the entire small in-testine causing massive hemorrhage due topeptic ulceration is presented.

2. The literature concerning similar casesis reviewed.

3. The problems associated with surgicaltherapy in this type of case is discussed.

4. The surgical solution used in this caseis presented.

Bibliography1. Craig, J. M.: Personal communication.2. Ripstein, C. B.: Duplication of Small Intestine.

Am. J. Surg., 78:847, 1949.3. Thomson, T. J., J. H. Wilson and G. L. W.

Cunningham: Gastrointestinal Tract Duplica-tion: Massive Hemorrhage from Ulcers. Gas-troenterology, 26:774, 1954.

Editorial ...

The Role of the Internal Jugular Vein and Sternocleidomastoid Musclein Cervical Node Dissection

THE PURPOSE of this editorial is to drawattention to the fact that an adequate

radical cervical lymph node dissection does notrequire resection of the internal jugular vein orthe sternocleidomastoid muscle or both in allinstances. This is true whether there is meta-static carcinoma in cervical lymph nodes orwhether preventive node dissection is beingcarried out.

There are those who believe that the veinand muscle must be resected if the operationis to be a radical node dissection. This opera-tion is technically easier to perform and con-sumes less time.

Preservation of the internal jugular vein andsternomastoid muscle does not mean that theoperation is less thorough. Good cancer surgerytechnic and meticulous sharp dissection are themeans of assuring thoroughness.Many surgeons trained by the late George

H. Semken have passed on to the youngergeneration his teaching and principles and thetechnical steps involved. Probably other sur-geons here and abroad have followed andtaught similar principles.

Anatomic studies of the cervical lymphatics,both vessels and nodes, made by such author-ities as Hunter, Mascagni, Cruikshank, Sappey,Hyrtl, Teichmann, Gerota, Kuttner, Doren-dorff, Poirier-Cuneo, Most, Bartels and othermore recent authors are the basis for this con-tention. Lymph nodes lie on the sheath of theinternal jugular vein, on the medial side, infront and on the lateral side. They do not lie

behind the vein or within the carotid sheath.Only in far advanced metastases may thesheath and adventitia of the vein be involvedand then the lumen of the vein is actuallyinvaded.

Therefore, if the sheath of the vein is incisedand resected by sharp dissection the lymphnodes will come away with the sheath. Theglistening adventitia of the vein remainsbehind.

It has been the experience of many surgeonsthat patients thus treated remain free of localreappearance of the disease.The principles underlying an adequate rad-

ical cervical lymph node dissection are similarto those followed in an adequate axillary orgroin dissection.

In the axilla or groin the axillary vein andfemoral vein are usually preserved. Resectionis performed only if the lymph nodes are verylarge and carcinoma has extended into the veinwall. Why then should the jugular vein beresected in every instance? Why should a cer-vical node dissection be considered inadequateand not radical unless the jugular vein is re-sected when the same principle is not followedin the axilla or groin? I believe that cervicalnode dissection with preservation of the jug-ular vein is just as radical as an axillary dissec-tion with preservation of the axillary vein.The next point refers to routine resection of

the stemocleidomastoid muscle in radical cer-vical node dissection.

(Continued on page 250)