Intussuscepting Hemangiomas of the Gastro-Intestinal Tract: *Report of a Case and Review of the Literature

EUGENE C. WEINSTEIN, M.D."* CHARLES G. MOERTEL, M.D.,***JOHN M. WAUGH, M.D.f

From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota

HEMANGIOMAS of the gastro-intestinaltract have interested physicians since thereports of Phillips and Gascoyen. How-ever, intussuscepting hemangiomas of theintestine occur so rarely that no one authorhas collected enough personal cases to at-tempt to standardize their treatment. It isthe purpose of this article to report a casedealing with this unusual entity, to reviewthe literature concerning previous reportsand, finally, to emphasize some importantpoints in the handling of these tumors.

Report of CaseThe patient was first seen at the Mayo Clinic

on June 6, 1941, at the age of seven weeks be-cause of a slowly growing tumor on the flexorsurface of the left foot present at birth (fig. 1).The tumor was purplish and firm and was diag-nosed as a hemangioma. Other hemangiomas werenoted on the hands, face, back and right foot.There was no familial history of similar lesionselicited on close questioning of the parents. Sincethe tumor on the left foot had been increasingin both size and firmness, radium was applied.

The patient was re-examined on four separateoccasions between September 8, 1941, and De-cember 15, 1942, with radium being applied tothe slowly growing hemangioma on three of thesevisits.

She was next seen on November 1, 1955, at14 years of age with a two-week history of fa-tigue, constant headache, and aching in her armsand legs. Since her last visit an infection had de-veloped in the hemangioma of her left foot ne-

* Submitted for publication April 27, 1962.* Fellow in Surgery.

*** Section of Medicine.+ Section of Surgery. Dr. Waugh died August

12, 1962.

cessitating amputation below the knee. She alsohad been treated for anemia for many years priorto this visit.

Physical examination revealed multiple bluishhemangiomas on the neck, left side of the tongue,pharynx, sole of the right foot, and left anteriorpart of the chest (Fig. 2). She was extremelypale.

Pertinent laboratory examination showed thefollowing values: hemoglobin, 2.8 Gm.; erythro-cytes, 1,320,000; reticulocytes, 1.3 per cent; plate-lets, 208,000; bleeding time, 3.7 minutes; andcoagulation time, 5.5 minutes. A blood smearshowed profound hypochromic, microcytic anemia.Proctoscopic examination revealed sessile, poly-poid lesions 8.0 cm. and 13 cm. above the analmargin (Fig. 3). Biopsy of one lesion proved itto be a cavernous hemangioma with hyperplasiaof the overlying mucosa. Gastro-intestinal roent-genograms revealed multiple polypoid lesions inthe stomach, small bowel and colon (Fig. 4).

The patient was given a transfusion of 2,000ml. of blood. A course of external irradiation tothe abdomen through both front and back portswas tried in an attempt to arrest the bleeding.

Between January 10, 1956, and August 5,1957, she was seen on three occasions for con-tinued bleeding. Treatment consisted of bloodtransfusions, and a second course of external ir-radiation to the abdomen. Iron for oral intakewas prescribed and she was dismissed from thehospital.

When seen on April 3, 1961, the patient was19 years of age and had been receiving multipletransfusions over the previous three years. Shenow required 500 ml. of blood each week tocorrect her anemia and was having moderatelysevere reactions to each transfusion. The findingson physical examination were essentially un-changed from previous admissions.

Results of laboratory studies of interest wereas follows: hemoglobin, 9.0 Gm.; reticulocytecount, 0.4 per cent; serum iron, 18 Ag.; hypo-

265

266 WEINSTEIN, MOERI

FIG. 1. Cavernous hemangioma of the left footof 7-week-old girl.

chromic microcytic cells with polychromasia onblood smear; stool test for blood guaiac, positive;proctoscopic examination and roentgenograms ofcolon and stomach, unchanged from previous ex-

FIG. 2. Hemangiomas on neck and left ante-rior portion of the chest (a), on pharynx (b)and on sole of right foot (c).

[EL AND WAUCH Annals of SurgeryFebruary 1963

aminations; and roentgenogram of a small bowel,evidence of multiple polypoid lesions throughoutthe entire intestine.

Gastroseopy revealed four or five small antralhemangiomas, none of which were large enoughto be radiologically demonstrable. On the sameday 500 ml. of blood labeled with radioactivechromium (Cr51) was transfused with the thoughtof aspirating the intestinal contents at variouslevels in an attempt to localize the site of thebleeding. Over a three-day period intestinal con-tents were aspirated from the stomach, secondportion of the duodenum, the jejunum one footdistal to the ligament of Treitz, the small intes-tine 5.5 feet from the nose and the small intestineeight feet from the nose. None of the aspirationsshowed any significant radioactivity, thus leadingto the conclusion that blood was not constantlyoozing but rather that bleeding was intermittentand profuse from the larger lesions, perhaps onthe basis of intussusception.On April 19, 1961, after adequate preoperative

preparation with transfusions, exploration of theabdomen was carried out. The spleen, pancreas,uterus and tubes were normal. There was a smallleft parovarian cyst and an adenoma of the gall-bladder. Two or three 1.0-cm. hemangiomas werepalpated in the stomach. The liver contained a5.0-mm. hemangioma; the mesentery of the smallbowel contained one hemangioma, and the je-junum and ileum, 15 hemangiomas ranging indiameter from 5.0 mm. to 1.0 cm. (Fig. 5). Asmall amount of blood was noted in the terminal

FIG. 3. Proctoscopic view of cavernoushemangioma.

Volume 157Number 2

INTUSSUSCEPTING HEMANGIOMAS 267portion of the ileum. Nine of the largest ilealhemangiomas were removed by simple enterotomyand excision; two were the sites of easily reduci-ble intussusceptions. One or two small ileal le-sions and those of the stomach and jejunum werenot removed.

Pathologic examination of the surgical speci-mens revealed nine cavernous hemangiomas meas-uring 0.5 to 1.0 cm. in diameter. They had beensituated in the submucosa of the ileum. Thetwo which had been involved in the intussuscep-tions showed some ulceration (Fig. 6).

Postoperatively the patient did well and wasdismissed from the hospital on May 3, 1961, atwhich time her hemoglobin measured 13.2 Gm. t...;~~~~~~~~~~~~.:-7W.

C~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.....:

FIG. 4c. Evidence of polypoid lesions in colon.

She was seen for re-evaluation of her gastro-intestinal difficulty on October 19, 1961. Heronly symptom at this time was occasional mildfatigue. In the interim she had been fully activeas a college student with many extracurricularactivities. She had not noted melena or hemato-chezia since her dismissal from the hospital, andhad not required blood transfusions. Hemoglobindeterminations had ranged between 10 and 13Gm. per 100 ml. of blood. Laboratory studiesrevealed a value for hemoglobin of 9.3 Gm. and

b

FIG. 4. Gastro-intestinaldence of polypoid lesions.small intestine.

roentgenograms. Evi-a. In stomach. b. In FIG. 5. Multiple hemangiomas of the small intes-

tine as visualized at operation.

WEINSTEIN, MOERI

FIG. 6. Section of an excised cavernous he-mangioma. Note the area of mucosal ulcerationoverlying the multiple vascular spaces locatedsubmucosally (hematoxylin and eosin; X 25).

EL AND WAUGH Annals of SurgeryFebruary 1963

a red cell count of 3,430,000. It was decided thatfor the present time close follow up of her con-dition and the administration of iron orally werethe only treatments indicated.

Review of the Literature

Raiford found three hemangiomas ofthe small intestine among 11,500 necropsiesand 45,000 surgical specimens. Merchantfound three hemangiomas of the small in-testine in 50,775 surgical specimens and7,340 necropsies. Gentry and associates re-viewed the files of the Mayo Clinic throughJune, 1945, and found 38 benign hemangi-omas and 16 malignant vascular tumors.

TABLE 1. Intussuscepting Hemangiomas of the Gastrointestinal Tract

Sex, AgeCase Author (years) Symptoms

Locationof tumor Operation* Type of Hemangioma

M Acute abdominal pain, hiccup,23 eructations, vomiting, consti-

pation

M Recurrent attacks of colicky,27 abdominal pain associated with

vomiting, an occasional mass,two tarry stools

F Vomiting, hemorrhage3 mo.

M Typical chronic intussusception32

Smallintestine

Resection Cavernous

Ileum Resection Cavernous

Jejunum Resection Capillary and cavernous

Cecum Resection Capillary and cavernous

5 McClure and FEllis 30

6 Kaufmann M

62

7 Weber M

48

8 Merchant F67

9 Merchant M

8

10 Browne and FMcHardy 50

1 Hansen M

56

12 Weddle and M

Snyder 7

13 Krause and M

Hanlon 25

14 Antila F44

15 Presentpaper

F19

Anemia, tarry stools Jejunum Reduction

Typical intussusception

Crampy abdominal pain

Nausea, vomiting, pain in rightlower quadrant

Crampy abdominal pain withvomiting

Intermittent colicky epigastricpain, four tarry stools

Crampy pain, upper part ofabdomen, vomiting

Pain in right lower quadrant,vomiting

Weakness, intermittent tarrystools

Anemia

Anemia

Cecum Resection Capillary

Jejunum

Jejunum Reduction

Jejunum Resection Sclerosing

Ileum Resection Capillary

Ileum Resection Cavernous

Jejunum Resection Cavernous

Jejunum Multipleenterotomies,resection

Ileum Multipleenterotomies

Cavernous

Cavernous

* Necropsy performed in case 8.

268

1 Nicoll

2 Shillito

3 Sussig

4 Kayser

I

1

Il

Volume 157 INTUSSUSCEPTING HEMANGIOMAS 269Number 2

In 1960, Rissier summarized a total of 114reported cases of hemangiomatosis of theintestine and added two cases of his own.We reviewed 14 previously reported

cases of intussuscepting hemangiomas ofthe gastro-intestinal tract and their salientfeatures are summarized in the table. In-cluding our patient, there have been re-ports on nine males and six females, rangingin age from three months to 67 years, whohave been known to have this disease. Themost frequent symptoms were crampingabdominal pain, vomiting, and melena.The treatment in most cases was resection(Table).

Comment

It is evident from a review of the litera-ture on this subject that resection of theinvolved intestine has been the procedureof choice for symptomatic hemangiomas.However, in only one other case (Case14) were there multiple hemangiomas suchas we have reported in our case. In Case14 the author' reported that multiple en-terotomies were performed for some tu-mors followed by resection of 70 cm. of thelower part of the ileum where a numberof hemangiomas were concentrated.Our case emphasizes two important

points. The first is the failure of total ab-dominal irradiation in this case to arrestgastro-intestinal bleeding which developedsecondary to discrete hemangiomas. Thepatient received irradiation to her abdomenon two occasions without any regressionof the bleeding tendency.The second important point concerns the

feasibility of multiple enterotomies andexcisions to control bleeding in conditionsof this nature. If multiple hemangiomasare present, the largest tumors seem mostlikely to give rise to intussusception andbleeding. Therefore, at the initial surgicalprocedure, only the larger hemangiomasneed be removed, leaving the smaller le-sions for excision at a later date, if symp-toms recur, when it is possible that the

patient will be in better physical condition.If this procedure is followed, the activebleeder is not unnecessarily subjected to along and extensive surgical procedure, andnutritional problems can be avoided whichmight result from resection of a sizableportion of small intestine.

SummaryA case is reported of a 19-year-old girl

having multiple intussuscepting hemangio-mas of the small intestine. Only 14 casesof this rare condition have been reportedpreviously. Symptoms in general are cramp-ing abdominal pain, vomiting and melena.X-ray treatment did not arrest the gastro-intestinal bleeding. Management of intus-suscepting hemangiomas by means of mul-tiple enterotomies and excisions is feasible,however, if several areas of the intestinaltract are involved.

References1. Antila, L.: Intestinal Hemangiomatosis: A

Case Report. Ann. chir. et gynaec. Fenniae,48:406, 1959.

2. Browne, D. C. and G. McHardy: SolitarySclerosing Hemangioma of the Jejunum:Case Report. Gastroenterology, 8:665, 1947.

3. Gascoyen: Case of Naevus Involving theParotid Gland, and Causing Death FromSuffocation: Naevi of the Viscera. Tr. Path.Soc. London, 11:267, 1860.

4. Gentry, R. W., M. B. Dockerty and 0. T.Clagett: Collective Review: Vascular Mal-formations and Vascular Tumors of theGastrointestinal Tract. Intemat. Abstr. Surg.,88:281, 1949.

5. Hansen, P. S.: Hemangioma of the Small In-testine: With Special Reference to Intussus-ception: Review of the Literature and Re-port of Three New Cases. Am. J. Clin. Path.,18:14, 1948.

6. Kaijser, R.: Uber Hamangiome des TractusGastrointestinalis. Arch. f. klin. Chir., 187:351, 1936-37.

7. Kaufmann, E.: Quoted by Kaijser, Rolf.68. Kayser, 0: Quoted by Rissier, H. L., Jr.159. Krause, A. H. and Hanlon, T. J.: Hemangioma

of the Gastrointestinal Tract With Case Re-port of a Polypoid Cavemous Hemangiomaof the Jejunum. Am. Surgeon, 21:499, 1955.

270 WEINSTEIN, MOERTEL AND WAUGH February 1963

10. McClure, R. D. and S. W. Ellis: Hemangi-omata of the Intestine. Am. J. Surg., 10:241,1930.

11. Merchant, F. T.: Intussusception Due to He-mangioma of the Jejunum. Arch. Surg., 39:1031, 1939.

12. Nicoll, J. H.: On the Removal of a NaevoidTumor of the Intestine. Brit. M. J., 1:843,1899.

13. Phillips, B.: Erectile Tumor of the Anus. Lon-don Med. Gazette, 23:514, 1838-1839.

14. Raiford, T. S.: Tumors of the Small Intestine.Arch. Surg., 25:122, 1932.

15. Rissier, H. L., Jr.: Hemangiomatosis of theIntestine: Discussion Review of the Litera-

ture and Report of Two New Cases. Gastro-enterologia, 93:357, 1960.

16. Shillito, N.: Angiomata of the Small Intes-tines. Pennsylvania M. J., 24:421, 1921.

17. Sussig, L.: Ein Fall von BlastomartosemHamartom des Dunndarmes als Ursacheeiner Invagination im Sauglingsalter. Beitr.klin. Chir., 130:353, 1923-1924.

18. Weber, H.: Invaginatio ileocoecalis, bedingtdurch ein Haemangioma simplex. Centralbl.f. Allg. Path. u. Path. Anat., 66:33, 1936-1937.

19. Weddle, R. H. and C. D. Snyder: Hemangi-omatosis of the Ileum with Intussusception:Case Report. Surgery, 25:749, 1949.

AcknowledgmentA paper entitled: Prognostic Import of Circulating Cancer

Cells after Curative Surgery published in the May 1962 issue ofAnnals of Surgery (J. D. Drye et al.) failed to include notice ofa supporting grant.The acknowledgment should read as follows: This work was

supported by a grant from the United States Public Health Serv-ice, National Institutes of Health, National Cancer Institute,#CA-09652.