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Anorectal and colonic manifestations of schistosoma mansoni infestation (intestinal bilharzia)
ANORECTAL AND COLONIC MANIFESTATIONS SCHISTOSOMA MANSONI INFESTATION (INTESTINAL BILHARZIA)*
OF
CASE REPORT BENJAMIN NEW
W.
WARNER,
YORK,
S
mansoni is an endemic tropical and subtropica parasitic disease caused by a bIood or fluke worm (Schistosoma mansoni) which spends part of its intermediate Iife cycIe in a snail. It gains access to its human host in its IarvaI stage from infected water. The larvae penetrate the skin or buccal mucous membrane and then enter the periphera1 veins; after passing through the puImonary circuIation they arrive in the systemic circuIation and are carried into the superior mesenteric artery and. its branches and then into the porta venous system; they feed and grow in the intrahepatic branches of the porta vein. In their mature form the parasites migrate to the termina1 tributaries of the superior and inferior mesenteric veins where copuIation and egg-laying occurs. The extrusion of the eggs from the venules produces IocaI pathoIogic tissue changes and systemic toxic manifestations. The disease is endemic in Egypt, CentraI and West Africa, the East Coast of South America, the West Indies and especiaIIy in Puerto Rico. With increased trave1, popuIation shifts, the establishment of miIitary bases in subtropica and tropica cIimates, parasitic diseases endemic in those areas wiI1 be brought to our attention with increasing frequency.
M.D.
YORK
the gynecology clinic of Beth David Hospital in February, 1941, complaining of sterility and intermittent pain in her left Iower quadrant, unreIated to menstruation. A mass in the Ieft adnexa was palpated. In June, 1941, after severa months of observation she was hospitalized on Dr. Mortimer Hyam’s service. The routine Iaboratory findings were as folIows: hemogIobin 65 per cent, erythrocytes 4,670,000, Ieucocytes 8,600 with 78 per cent poIymorphonucIears and 22 per cent Iymphocytes. There were no eosinophiIes. UrinanaIysis was negative. BIood Wassermann: aIcoho1 antigen I plus, choIestero1 antigen 2 PIUS. * A Iaparotomy was performed by Dr. Moses Lobsenz. A Ieft tubo-ovarian mass the size of a smaI1 peach was found and a Ieft saIpingo-oophorectomy and appendectomy were performed. The pathoIogic salpingo-oophoritis, diagnoses were : chronic corpus Iutean cyst of the ovary, atrophy and fibrosis of the appendix. In the appendix sIight cosinophiIic ceI1 infiltration of the muscuIaris was noted. ConvaIescence was uneventfu1. Upon her discharge from the hospita1 she returned to the gynecoIogy out-patient department for follow-up care and compIained of sIight rectal bIeeding, pain and itching. She was then referred to the rectal clinic. Recta1 examination reveaIed an infiamed, tender posterior ana crypt with a posterior fissure-in-an0 and slightly enIarged interna hemorrhoids. Sigmoidoscopy reveaIed a sessile, eIevated pea-sized noduIe, diagnosed preoperatively as an adenoma, in the mucous membrane of the posterior recta1 waI1 about four inches above the anus and muItipIe smaI1 papiIIomas in the vicinity of the rectosigmoida1 junction.
CHISTOSOMIASIS
CASE
NEW
REPORT
Mrs. D. S., age twenty-six, was born in Puerto Rico and lived there in a rura1 area, Bonito, unti1 six years ago when she came to New York City. She had been married for five years and had no chiIdren. She was first seen in
* It was Iearned Iater that she had been treated for Iues severa years previously by a private physician who had found a four plus Wassermann reaction.
* From the ProctoIogy Service of the Department of Surgery, Beth David Hospital, New York City. Dr. Frederic W. Bancroft, Director of Surgery, Dr. I. M. Brenner, Attending in Proctology. Read before the Beth David HospitaI CIinicaI Society November 17, 1941.
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There was a smaI1 area of granuIation tissue 3 mm. in diameter on the anterior recta1 waI1. The sigmoid above the rectosigmoida1 junction appeared normal.
FIG. I. Section of noduIe showing inflammation with mononucIear and polymorphonuclear Ieucocytes and eosinophiles. Two oval-shaped ova of Schistosoma mansoni with a typical Iateral spine are seen. (High power.)
was hospitalized again on The patient 12, 1941. Laboratory findings were September as foIIows: hemogIobin 97 per cent, erythroIeucocytes 8,460 with 72 per cytes 4,7~0,000, 27 per cent Iymphocent poIymorphonucIears, cytes and I per cent monocytes; urinanaIysis was negative. StooI examination was not done. Under IocaI anesthesia the sessiIe noduIe was removed with the eIectric cutting Ioop and the smaIIer papiIIomas coagulated; the posterior anal crypt and fissure were excised. Routine histoIogic examination of the tissue by Dr. Milton HeIpern was reported as foIIows: “There is a moderate infkimmation in the mucosa and submucosa; the inffammatory ceIIs are mononucIear and poIymorphonucIear Ieucocytes and eosinophiIes. The sub-mucosa contains diIated venuIes. In some section there are oval-shaped parasitic ova possessing a typical IateraI spine. In one section a group of such ova is surrounded by numerous eosinophiles. “Diagnosis: chronic inflammation of anal mucosa resuhing from infestation with parasitic ova of Shistosoma mansoni. (Schistosomiasis mansoni.)” (Figs. I and 2.) With the finding of the ova of Schistosoma mansoni the histoIogic sections of the tube, ovary and appendix previousIy removed were
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re-examined but no ova were found. UnfortunateIy, the gross specimens had been discarded so that more sections couId not be made. The appendix is a fairIy frequent site of invoIvement
FIG. 2. Section of anal crypt showing infIammation and many distorted ova of Schistosoma mansoni. (High power.)
and cases of faIIopian tube infestation’ have been reported. The patient’s convaIescence was uneventful She was discharged from the hospita1 in five days and returned to the cIinic. The posterior anocutaneous wound deveIoped excess granuIation tissue which was curetted and examined histoIogicaIIy but ova were not found. Sigmoidoscopy reveaIed the mucous membrane of the rectum and sigmoid studded with punctate bIeeding areas which were interpreted as points of rupture or escape of the ova into the Iumen of the bowe1. Repeated stoo1 and direct recta1 smears reveaIed an occasiona distorted ovum. A blood count on Octover IO, 1941, revealed: hemogIobin 97 per cent, erythrocytes 4,g2o,ooo, Ieucocytes I 1,900 with 59 per cent polymorpho7 per cent nucIears, 28 per cent Iymphocytes, 3 per cent monocytes, 2 per cent eosinophiIes, basophiIes. This was the first appearance of eosinophiIia. At this time there were numerous bIeeding areas in the mucous membrane of the rectum and sigmoid. There was no diarrhea. A barium enema did not reveal any additiona1 findings in the coIon. A chest x-ray on this date reveaIed multiple smaI1 areas of infiItrations at the right apex which were absent on a subsequent x-ray one month later. It is probabIe that these infiItra-
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tions resulted from a Schistosoma invasion of the lungs. At the present writing the patient feels well
FIG. 3. TypicaI Iateral-spined ovum. (From Faust, after Cort; courtesy of the Univ. of CaIifornia Press.)
and has no compIaint other than slight peri-anal itching. ObjectiveIy, the sigmoidoscopic findings persist. The spIeen is just paIpabIe but the Iiver is not. Treatment with fuadin (sodium antimonylll biscatecho1 2.4 disuIfonate of sodium) intramuscuIarIy has been started. The cause of human Schistosomiasis invoIving the urinary bIadder with hematuria as its outstanding symptom was recognized and described by Dr. BiIharz in Egypt in 185I and this disease bears his name. For many years all types of human Schistosomiasis were caIIed BiIharzia unti1 attention was caIIed, in 1893, by Dr. Manson to the morphoIogic difference in the ova from an essentiaIIy intestina1 type of the disease. In this type ova with a IateraI instead of a termina1 spine were identified. Schistosoma producing the IateraI-spined ova have a different geographic distribution and produce a different viscera1 invoIvement and carry the name of Manson. (Fig. 3.) The Iife cycIe2 of the parasites in the vesica1 and the intestina1 type of Schisto-
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somiasis are simiIar differing onIy in their fina IocaIization in the veins of the porta system; both types produce simiIar pathoIogica1 changes in the host but in different viscera. Schistosoma mansoni eggs are discharged from their human host in feces, occasionaIIy in urine. CareIess toiIet habits and fauIty sewage disposal resuIt in the contamination of bodies of water which are aIso the suitabIe environment for a specific species of snail (P. guadaIoupensis) which acts as an intermediate host. The ovum when discharged in the water Iiberates a miracidium which enters the snaiI’s viscera1 mass where it muItipIies and grows, giving rise to another intermediate IarvaI stage, the cercariae, which possess a Iong tai1. The Iarvae in the cercaria1 stage Ieave the snai1 after four weeks and move activeIy in the water; they can survive from one to three days if they do not find an appropriate human host. On making contact with the exposed skin or bucca1 membrane of their human host, they penetrate the skin or mucous membrane, their taiIs drop off and within one-haIf to one hour they penetrate to the deeper Iayers. Infestation cannot occur by ora ingestion as the Iarvae cannot survive the action of gastric juice. From the deeper Iayers of the skin the Iarvae enter the periphera1 venuIes, are carried to the right heart and thence to the puImonary capiIIaries. “The Iarvae having reached the puImonary capiIIaries squeeze their way through, enter the Ieft heart, systemic circuIation, abdomina1 aorta and superior mesenteric artery. OnIy those Iarvae survive which enter the superior mesenteric artery. Thence they are carried through the capiIIaries to the superior mesenteric vein and enter the intrahepatic radicIes of the porta vein where they feed on red bIood ceIIs, become sexuaIIy differentiated and mature. On reaching maturity, the aduIt worms move against the porta bIood stream to the iIeocoIic and coIic branches of the superior mesenteric vein and wander into the anastomoses of the venous circuIation toward the Iower branches of the inferior
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mesenteric vein especiaIIy in the hemorrhoida pIexus in the Mansoni type and in the vesica1 pIexus of veins in the Bilharzia type. This growth and migration of the aduIt worm takes about six weeks from the time of entrance of the Iarvae into the skin. In the venous pIexus copuIation occurs and the femaIe then makes her way into the smalIest venuIe which becomes distended and there deposits her IateraI-spined eggs one at a time from one to severa hundred per day, moving to a new site for each egg. The distended vein tends to return to norma caliber; the spine of the egg penetrates its waI1 and by both mechanica and lytic digestive action perforates the vein and is either extruded into the Iumen of the viscus, the sub-mucosa or the peritonea1 surface or peri-rectal tissues. The eggs discharged into the Iumen of the bIadder or rectum are excreted in the urine and feces and if a body of water containing an appropriate species of snai1 is contaminated, the snaiIs become infected and the cycle begins again in the intermediate host.“2 The entrance of the Iarvae into the skin is accompanied by an intense pruritus spoken of by the natives of Puerto Rico as “piquina.” Th is may Iast several hours to severa days. There is a type of Schistosoma in snaiIs in resort Iake regions of Michigan and Minnesota which penetrates the skin of bathers and sets up an intense skin rash with itching caIIed “swimmer’s itch”3,* but fortunateIy the larvae in these cases are destroyed in the deeper Iayers of the skin and do not enter the circuIation. There are no reports of cases in residents of the United States who have never Ieft this country. An infection is reported5 in a resident of the United States who dipped his hand into a home aquarium which contained a species of the snai1 transmitting the Egyptian type of Schistosomiasis. With promiscuous distribution of human excreta and improper sewage disposa1 in the tropics and especiaIIy in Puerto Rico where the rura1 areas are studded with irrigation ditches with stagnant water and
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sIow moving rivers, as we11 as Iime stone ~001s offering a suitabI5 environment for the appropriate species of snai1, the incidence of Schistosomasis mansoni in the genera1 popuIation is about 12 per cent, whiIe in certain endemic areas the incidence of infestation reaches 50 to 60 per cent. The cIinicopathoIogic picture and symptomatoIogy are cIassified by Craig and Faust2 into three stages: “ (I) invasion and incubation period-six to seven weeks; (2) egg deposition and extrusion; (3) tissue proIiferation and repair.” A diagnosis is seIdom made in the first stage aIthough there may be symptoms of miId toxemia and occasionally diarrhea of supposedIy toxic origin. It is during the second stage of egg-Iaying and extrusion which occurs from five to seven weeks after exposure that IocaI abdominal or intestina1 and systemic toxic symptoms make their appearance. There may be a sudden onset with temperature, chiIIs and sweating, abdomina1 pain with tenderness aIong the coIon, dysentery with passage of bIood and mucus with IateraI-spined ova in the stoo1, dry cough and even parenchymatous infiItration especiaIIy of the apices, urticaria, enIargement and tenderness of the Iiver and Iater spIenic enIargement. Th ere is Ieucocytosis and eosinophiIia.2,6a7 With subsidence of this acute phase which may have varying degrees of severity, proIiferative and reparative tissue changes occur. These granuIomatous infIammatory changes depend upon the Iocation of the aduIt worm and the site of egg extrusion and may appear cIinicaIIy as superficia1 uIcerations with granuIations, papiIIomas and pseudo-adenomas (sessiIe or poIypoid). The ova may be extruded to the peritonea1 surface of the bowe1 producing pseudotubercIes or into the perirecta1 tissues with resuItant fistuIas. FistuIization may occur from any part of the invoIved boweI. The granuIomas in the Iumen of the coIon and rectum may become poIypoid and produce the same sequence of symptoms and compIications common to a11 poIyps. MaIignant degeneration in the
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granuIomas is not rare.2 If the poIypoid granuIomas are Iocated in the rectum or ana cana1, they may proIapse through the anus. The cryptitis is due to the presence of the ova; hemorrhoida vein engorgement may resuIt from obstruction of the venuIes by the aduIt parasites and from interference with Iymphatic and venous circuIation due to IocaI inffammatory infltration and obstruction of the porta circuIation where Iiver enIargement and subsequent cirrhosis occurs. In this connection a report by a Cairo surgeon, M. Samy,2 on “BiIharziaI PiIes and AnaI Fissure” is interesting: “In the routiae examinations and treatment of piIes the author has met with a pecuIiar kind of piIes which deserve a specia1 treatment. The patients suffering from this kind of piIes are mostIy from the country and suffer from a11 the usua1 symptoms of piIes. Some inteIIigent patients may give a history of a severe fever, sometimes amounting to 40 degrees c. or more, accompanied by an urticaria1 rash, acute abdomina1 pain, rigors symptoms of puImonary congestion and profuse sweating. This may be accompanied by some diarrhea. After about three months from the fever the IocaI symptoms of piIes begin to appear. If they are operated on they recur unIess systemic treatment is instituted.” The diagnosis of Schistosomiasis mansoni infestation is made from the history of travel in an endemic area, finding the ova in the stoo1 or peri-ana or recta1 swabs,2 and tissue biopsy. The seroIogic tests are not deveIoped suffIcientIy to be of vaIue for genera1 usage as diagnostic aids. In the acute or open stage of active egg extrusion the eosinophiIia, urticaria and dysentery may indicate the diagnosis but it is aIso at this stage that typhoid, tubercuIosis and maIaria are frequentIy incorrectIy diagnosed. In the proIiferative stage sigmoidoscopy may be of great vaIue with biopsy of any granulomatous tissue seen. As the
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infection becomes more chronic, eggs in the feces graduaIIy decrease in number.2 SUMMARY
A case of Schistosomiasis mansoni infestation in a native Puerto Rican, now Iiving in the United States, withpredominant anorecta1 manifestations is reported. Physicians shouId be on the aIert to suspect this infestation in soIdiers and workers who return from their stations in Puerto Rico and other endemic areas and present anorecta1 and intestina1 symptoms with or without enIargement of the Iiver and spIeen. EosinophiIia is inconstant and usuaIIy absent in the Iate stages. PuImonary infiItrations may be confusing unIess the infestation is suspected. WhiIe mention of a third type of pathogenic human Schistosomiasis was not intended for this report, it is now pertinent to make note of Schistosoma Japonicum infestation which is endemic in and about China and Japan. This Schistosome invoIves the smaI1 bowe1 with severe systemic toxic symptoms. I am gratefu1 to Dr. MiIton HeIpern and Dr. AIfred Gudemann of the PathoIogy Department of Beth David HospitaI for their kind assistance in preparing this report. REFERENCES
MICHAEL. A note on the cIinica1 features of bilharzia salpingitis. South African M. J., February 22, 1941. CRAIG and FAUST. Clinical ParasitoIogy. 2d ed. PhiIadeIphia, Lea Br Febiger. CORT, WILLIAM. Schistosome dermatitis in the United States. J. A. M. A., 90: 13, 1928. CHRISTENSON,R. 0. and GREENE, W. P. Studies on biologic and medical aspects of “swimmer’s itch.” Minnesota Med. Sys;, I I : 573, 1928. SULLIVAN. S. J. Schistosoma hematobium--sDoradic case. J. A. M. A., 98: 1642, I 93’2. Manson’s Tropical Diseases. 10th edition.,BaItimore, WilIiam Wood & Co. PONS, J. A. and HOFFMAN, W. A. FebuIe phenomena in schistosomiasis mansoni with iIIustrated cases. Puerto Rico J. Pub. Health 4p Trop. Med., 9: r-17, 1933. SAMY, M. BiIharziaI piIes and anal fissure. J. Egyptian Med. Ass., 2: 65-71, 1936.
I. GELFAND,
2. 3. 4.
/4. 6. 7.
8.
OF
CASE REPORT BENJAMIN NEW
W.
WARNER,
YORK,
S
mansoni is an endemic tropical and subtropica parasitic disease caused by a bIood or fluke worm (Schistosoma mansoni) which spends part of its intermediate Iife cycIe in a snail. It gains access to its human host in its IarvaI stage from infected water. The larvae penetrate the skin or buccal mucous membrane and then enter the periphera1 veins; after passing through the puImonary circuIation they arrive in the systemic circuIation and are carried into the superior mesenteric artery and. its branches and then into the porta venous system; they feed and grow in the intrahepatic branches of the porta vein. In their mature form the parasites migrate to the termina1 tributaries of the superior and inferior mesenteric veins where copuIation and egg-laying occurs. The extrusion of the eggs from the venules produces IocaI pathoIogic tissue changes and systemic toxic manifestations. The disease is endemic in Egypt, CentraI and West Africa, the East Coast of South America, the West Indies and especiaIIy in Puerto Rico. With increased trave1, popuIation shifts, the establishment of miIitary bases in subtropica and tropica cIimates, parasitic diseases endemic in those areas wiI1 be brought to our attention with increasing frequency.
M.D.
YORK
the gynecology clinic of Beth David Hospital in February, 1941, complaining of sterility and intermittent pain in her left Iower quadrant, unreIated to menstruation. A mass in the Ieft adnexa was palpated. In June, 1941, after severa months of observation she was hospitalized on Dr. Mortimer Hyam’s service. The routine Iaboratory findings were as folIows: hemogIobin 65 per cent, erythrocytes 4,670,000, Ieucocytes 8,600 with 78 per cent poIymorphonucIears and 22 per cent Iymphocytes. There were no eosinophiIes. UrinanaIysis was negative. BIood Wassermann: aIcoho1 antigen I plus, choIestero1 antigen 2 PIUS. * A Iaparotomy was performed by Dr. Moses Lobsenz. A Ieft tubo-ovarian mass the size of a smaI1 peach was found and a Ieft saIpingo-oophorectomy and appendectomy were performed. The pathoIogic salpingo-oophoritis, diagnoses were : chronic corpus Iutean cyst of the ovary, atrophy and fibrosis of the appendix. In the appendix sIight cosinophiIic ceI1 infiltration of the muscuIaris was noted. ConvaIescence was uneventfu1. Upon her discharge from the hospita1 she returned to the gynecoIogy out-patient department for follow-up care and compIained of sIight rectal bIeeding, pain and itching. She was then referred to the rectal clinic. Recta1 examination reveaIed an infiamed, tender posterior ana crypt with a posterior fissure-in-an0 and slightly enIarged interna hemorrhoids. Sigmoidoscopy reveaIed a sessile, eIevated pea-sized noduIe, diagnosed preoperatively as an adenoma, in the mucous membrane of the posterior recta1 waI1 about four inches above the anus and muItipIe smaI1 papiIIomas in the vicinity of the rectosigmoida1 junction.
CHISTOSOMIASIS
CASE
NEW
REPORT
Mrs. D. S., age twenty-six, was born in Puerto Rico and lived there in a rura1 area, Bonito, unti1 six years ago when she came to New York City. She had been married for five years and had no chiIdren. She was first seen in
* It was Iearned Iater that she had been treated for Iues severa years previously by a private physician who had found a four plus Wassermann reaction.
* From the ProctoIogy Service of the Department of Surgery, Beth David Hospital, New York City. Dr. Frederic W. Bancroft, Director of Surgery, Dr. I. M. Brenner, Attending in Proctology. Read before the Beth David HospitaI CIinicaI Society November 17, 1941.
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There was a smaI1 area of granuIation tissue 3 mm. in diameter on the anterior recta1 waI1. The sigmoid above the rectosigmoida1 junction appeared normal.
FIG. I. Section of noduIe showing inflammation with mononucIear and polymorphonuclear Ieucocytes and eosinophiles. Two oval-shaped ova of Schistosoma mansoni with a typical Iateral spine are seen. (High power.)
was hospitalized again on The patient 12, 1941. Laboratory findings were September as foIIows: hemogIobin 97 per cent, erythroIeucocytes 8,460 with 72 per cytes 4,7~0,000, 27 per cent Iymphocent poIymorphonucIears, cytes and I per cent monocytes; urinanaIysis was negative. StooI examination was not done. Under IocaI anesthesia the sessiIe noduIe was removed with the eIectric cutting Ioop and the smaIIer papiIIomas coagulated; the posterior anal crypt and fissure were excised. Routine histoIogic examination of the tissue by Dr. Milton HeIpern was reported as foIIows: “There is a moderate infkimmation in the mucosa and submucosa; the inffammatory ceIIs are mononucIear and poIymorphonucIear Ieucocytes and eosinophiIes. The sub-mucosa contains diIated venuIes. In some section there are oval-shaped parasitic ova possessing a typical IateraI spine. In one section a group of such ova is surrounded by numerous eosinophiles. “Diagnosis: chronic inflammation of anal mucosa resuhing from infestation with parasitic ova of Shistosoma mansoni. (Schistosomiasis mansoni.)” (Figs. I and 2.) With the finding of the ova of Schistosoma mansoni the histoIogic sections of the tube, ovary and appendix previousIy removed were
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re-examined but no ova were found. UnfortunateIy, the gross specimens had been discarded so that more sections couId not be made. The appendix is a fairIy frequent site of invoIvement
FIG. 2. Section of anal crypt showing infIammation and many distorted ova of Schistosoma mansoni. (High power.)
and cases of faIIopian tube infestation’ have been reported. The patient’s convaIescence was uneventful She was discharged from the hospita1 in five days and returned to the cIinic. The posterior anocutaneous wound deveIoped excess granuIation tissue which was curetted and examined histoIogicaIIy but ova were not found. Sigmoidoscopy reveaIed the mucous membrane of the rectum and sigmoid studded with punctate bIeeding areas which were interpreted as points of rupture or escape of the ova into the Iumen of the bowe1. Repeated stoo1 and direct recta1 smears reveaIed an occasiona distorted ovum. A blood count on Octover IO, 1941, revealed: hemogIobin 97 per cent, erythrocytes 4,g2o,ooo, Ieucocytes I 1,900 with 59 per cent polymorpho7 per cent nucIears, 28 per cent Iymphocytes, 3 per cent monocytes, 2 per cent eosinophiIes, basophiIes. This was the first appearance of eosinophiIia. At this time there were numerous bIeeding areas in the mucous membrane of the rectum and sigmoid. There was no diarrhea. A barium enema did not reveal any additiona1 findings in the coIon. A chest x-ray on this date reveaIed multiple smaI1 areas of infiItrations at the right apex which were absent on a subsequent x-ray one month later. It is probabIe that these infiItra-
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tions resulted from a Schistosoma invasion of the lungs. At the present writing the patient feels well
FIG. 3. TypicaI Iateral-spined ovum. (From Faust, after Cort; courtesy of the Univ. of CaIifornia Press.)
and has no compIaint other than slight peri-anal itching. ObjectiveIy, the sigmoidoscopic findings persist. The spIeen is just paIpabIe but the Iiver is not. Treatment with fuadin (sodium antimonylll biscatecho1 2.4 disuIfonate of sodium) intramuscuIarIy has been started. The cause of human Schistosomiasis invoIving the urinary bIadder with hematuria as its outstanding symptom was recognized and described by Dr. BiIharz in Egypt in 185I and this disease bears his name. For many years all types of human Schistosomiasis were caIIed BiIharzia unti1 attention was caIIed, in 1893, by Dr. Manson to the morphoIogic difference in the ova from an essentiaIIy intestina1 type of the disease. In this type ova with a IateraI instead of a termina1 spine were identified. Schistosoma producing the IateraI-spined ova have a different geographic distribution and produce a different viscera1 invoIvement and carry the name of Manson. (Fig. 3.) The Iife cycIe2 of the parasites in the vesica1 and the intestina1 type of Schisto-
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JULY,
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somiasis are simiIar differing onIy in their fina IocaIization in the veins of the porta system; both types produce simiIar pathoIogica1 changes in the host but in different viscera. Schistosoma mansoni eggs are discharged from their human host in feces, occasionaIIy in urine. CareIess toiIet habits and fauIty sewage disposal resuIt in the contamination of bodies of water which are aIso the suitabIe environment for a specific species of snail (P. guadaIoupensis) which acts as an intermediate host. The ovum when discharged in the water Iiberates a miracidium which enters the snaiI’s viscera1 mass where it muItipIies and grows, giving rise to another intermediate IarvaI stage, the cercariae, which possess a Iong tai1. The Iarvae in the cercaria1 stage Ieave the snai1 after four weeks and move activeIy in the water; they can survive from one to three days if they do not find an appropriate human host. On making contact with the exposed skin or bucca1 membrane of their human host, they penetrate the skin or mucous membrane, their taiIs drop off and within one-haIf to one hour they penetrate to the deeper Iayers. Infestation cannot occur by ora ingestion as the Iarvae cannot survive the action of gastric juice. From the deeper Iayers of the skin the Iarvae enter the periphera1 venuIes, are carried to the right heart and thence to the puImonary capiIIaries. “The Iarvae having reached the puImonary capiIIaries squeeze their way through, enter the Ieft heart, systemic circuIation, abdomina1 aorta and superior mesenteric artery. OnIy those Iarvae survive which enter the superior mesenteric artery. Thence they are carried through the capiIIaries to the superior mesenteric vein and enter the intrahepatic radicIes of the porta vein where they feed on red bIood ceIIs, become sexuaIIy differentiated and mature. On reaching maturity, the aduIt worms move against the porta bIood stream to the iIeocoIic and coIic branches of the superior mesenteric vein and wander into the anastomoses of the venous circuIation toward the Iower branches of the inferior
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mesenteric vein especiaIIy in the hemorrhoida pIexus in the Mansoni type and in the vesica1 pIexus of veins in the Bilharzia type. This growth and migration of the aduIt worm takes about six weeks from the time of entrance of the Iarvae into the skin. In the venous pIexus copuIation occurs and the femaIe then makes her way into the smalIest venuIe which becomes distended and there deposits her IateraI-spined eggs one at a time from one to severa hundred per day, moving to a new site for each egg. The distended vein tends to return to norma caliber; the spine of the egg penetrates its waI1 and by both mechanica and lytic digestive action perforates the vein and is either extruded into the Iumen of the viscus, the sub-mucosa or the peritonea1 surface or peri-rectal tissues. The eggs discharged into the Iumen of the bIadder or rectum are excreted in the urine and feces and if a body of water containing an appropriate species of snai1 is contaminated, the snaiIs become infected and the cycle begins again in the intermediate host.“2 The entrance of the Iarvae into the skin is accompanied by an intense pruritus spoken of by the natives of Puerto Rico as “piquina.” Th is may Iast several hours to severa days. There is a type of Schistosoma in snaiIs in resort Iake regions of Michigan and Minnesota which penetrates the skin of bathers and sets up an intense skin rash with itching caIIed “swimmer’s itch”3,* but fortunateIy the larvae in these cases are destroyed in the deeper Iayers of the skin and do not enter the circuIation. There are no reports of cases in residents of the United States who have never Ieft this country. An infection is reported5 in a resident of the United States who dipped his hand into a home aquarium which contained a species of the snai1 transmitting the Egyptian type of Schistosomiasis. With promiscuous distribution of human excreta and improper sewage disposa1 in the tropics and especiaIIy in Puerto Rico where the rura1 areas are studded with irrigation ditches with stagnant water and
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sIow moving rivers, as we11 as Iime stone ~001s offering a suitabI5 environment for the appropriate species of snai1, the incidence of Schistosomasis mansoni in the genera1 popuIation is about 12 per cent, whiIe in certain endemic areas the incidence of infestation reaches 50 to 60 per cent. The cIinicopathoIogic picture and symptomatoIogy are cIassified by Craig and Faust2 into three stages: “ (I) invasion and incubation period-six to seven weeks; (2) egg deposition and extrusion; (3) tissue proIiferation and repair.” A diagnosis is seIdom made in the first stage aIthough there may be symptoms of miId toxemia and occasionally diarrhea of supposedIy toxic origin. It is during the second stage of egg-Iaying and extrusion which occurs from five to seven weeks after exposure that IocaI abdominal or intestina1 and systemic toxic symptoms make their appearance. There may be a sudden onset with temperature, chiIIs and sweating, abdomina1 pain with tenderness aIong the coIon, dysentery with passage of bIood and mucus with IateraI-spined ova in the stoo1, dry cough and even parenchymatous infiItration especiaIIy of the apices, urticaria, enIargement and tenderness of the Iiver and Iater spIenic enIargement. Th ere is Ieucocytosis and eosinophiIia.2,6a7 With subsidence of this acute phase which may have varying degrees of severity, proIiferative and reparative tissue changes occur. These granuIomatous infIammatory changes depend upon the Iocation of the aduIt worm and the site of egg extrusion and may appear cIinicaIIy as superficia1 uIcerations with granuIations, papiIIomas and pseudo-adenomas (sessiIe or poIypoid). The ova may be extruded to the peritonea1 surface of the bowe1 producing pseudotubercIes or into the perirecta1 tissues with resuItant fistuIas. FistuIization may occur from any part of the invoIved boweI. The granuIomas in the Iumen of the coIon and rectum may become poIypoid and produce the same sequence of symptoms and compIications common to a11 poIyps. MaIignant degeneration in the
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granuIomas is not rare.2 If the poIypoid granuIomas are Iocated in the rectum or ana cana1, they may proIapse through the anus. The cryptitis is due to the presence of the ova; hemorrhoida vein engorgement may resuIt from obstruction of the venuIes by the aduIt parasites and from interference with Iymphatic and venous circuIation due to IocaI inffammatory infltration and obstruction of the porta circuIation where Iiver enIargement and subsequent cirrhosis occurs. In this connection a report by a Cairo surgeon, M. Samy,2 on “BiIharziaI PiIes and AnaI Fissure” is interesting: “In the routiae examinations and treatment of piIes the author has met with a pecuIiar kind of piIes which deserve a specia1 treatment. The patients suffering from this kind of piIes are mostIy from the country and suffer from a11 the usua1 symptoms of piIes. Some inteIIigent patients may give a history of a severe fever, sometimes amounting to 40 degrees c. or more, accompanied by an urticaria1 rash, acute abdomina1 pain, rigors symptoms of puImonary congestion and profuse sweating. This may be accompanied by some diarrhea. After about three months from the fever the IocaI symptoms of piIes begin to appear. If they are operated on they recur unIess systemic treatment is instituted.” The diagnosis of Schistosomiasis mansoni infestation is made from the history of travel in an endemic area, finding the ova in the stoo1 or peri-ana or recta1 swabs,2 and tissue biopsy. The seroIogic tests are not deveIoped suffIcientIy to be of vaIue for genera1 usage as diagnostic aids. In the acute or open stage of active egg extrusion the eosinophiIia, urticaria and dysentery may indicate the diagnosis but it is aIso at this stage that typhoid, tubercuIosis and maIaria are frequentIy incorrectIy diagnosed. In the proIiferative stage sigmoidoscopy may be of great vaIue with biopsy of any granulomatous tissue seen. As the
JULY.1942
Parasites
infection becomes more chronic, eggs in the feces graduaIIy decrease in number.2 SUMMARY
A case of Schistosomiasis mansoni infestation in a native Puerto Rican, now Iiving in the United States, withpredominant anorecta1 manifestations is reported. Physicians shouId be on the aIert to suspect this infestation in soIdiers and workers who return from their stations in Puerto Rico and other endemic areas and present anorecta1 and intestina1 symptoms with or without enIargement of the Iiver and spIeen. EosinophiIia is inconstant and usuaIIy absent in the Iate stages. PuImonary infiItrations may be confusing unIess the infestation is suspected. WhiIe mention of a third type of pathogenic human Schistosomiasis was not intended for this report, it is now pertinent to make note of Schistosoma Japonicum infestation which is endemic in and about China and Japan. This Schistosome invoIves the smaI1 bowe1 with severe systemic toxic symptoms. I am gratefu1 to Dr. MiIton HeIpern and Dr. AIfred Gudemann of the PathoIogy Department of Beth David HospitaI for their kind assistance in preparing this report. REFERENCES
MICHAEL. A note on the cIinica1 features of bilharzia salpingitis. South African M. J., February 22, 1941. CRAIG and FAUST. Clinical ParasitoIogy. 2d ed. PhiIadeIphia, Lea Br Febiger. CORT, WILLIAM. Schistosome dermatitis in the United States. J. A. M. A., 90: 13, 1928. CHRISTENSON,R. 0. and GREENE, W. P. Studies on biologic and medical aspects of “swimmer’s itch.” Minnesota Med. Sys;, I I : 573, 1928. SULLIVAN. S. J. Schistosoma hematobium--sDoradic case. J. A. M. A., 98: 1642, I 93’2. Manson’s Tropical Diseases. 10th edition.,BaItimore, WilIiam Wood & Co. PONS, J. A. and HOFFMAN, W. A. FebuIe phenomena in schistosomiasis mansoni with iIIustrated cases. Puerto Rico J. Pub. Health 4p Trop. Med., 9: r-17, 1933. SAMY, M. BiIharziaI piIes and anal fissure. J. Egyptian Med. Ass., 2: 65-71, 1936.
I. GELFAND,
2. 3. 4.
/4. 6. 7.
8.