Recurrent Parotitis with SialoangiectasisIRVING L. WHITE, M.D., Long Beach

CHRONIC RECURRENT PAROTITIS is reported with in-creasing frequency in children and young adults.Often it is generalized throughout the gland andit may be unilateral or bilateral. Usually sialoadenitis(and rarely fistula formation) is present. Recurrentparotitis in most cases is associated with sialoangiec-tatic changes. Duct or alveolar ectasia may resultfrom acquired blockage5 due to calculus or inflam-matory changes; but the ectasia may also be due toa congenital developmental structural deformity an-tedating the manifestation of sialoadenitis. Since in-fection may complicate congenital sialoangiectasis,it is often impossible to differentiate between pri-mary and secondary disease.

Sialoangiography is an excellent method of dem-onstrating sialoangiectasis, and correlation with his-tologic findings is sometimes possible. As excision ofa biopsy specimen of the parotid gland may be a

Submitted May 31, 1962.

hazardous procedure, it is justifiable only if theinformation anticipated is important enough to out-weigh the danger of damnage to the facial nerve.The author had opportunity to consider the rela-

tionship of ectasia to recurrent infection in thecourge of treating siblings who had chronic recur-rent parotitis and sialoangiectasis. The patients weretwo sisters, in each of whom symptoms of recurrentparotitis with sialoangiectasis developed at the ageof 17. One of them had bilateral disease althoughthe symptoms were unilateral.

REPORT OF CASES

CASE 1. A 21-year-old white married woman wasfirst observed June 14, 1961, with a history of re-current swelling in the right preauricular area forover three years. The swelling would persist forone to three days and could be relieved by exertingpressure over the parotid gland. At the onset, attacksoccurred at intervals of two to four months, butthey had progressively increased in frequency untilat the time the patient was seen by the author they

Figure 1.-(Case 1)-Sialoangiogram of the right parotid area showing extreme and extensive sialoangiectasis ofthe right parotid gland with saccular dilatation of acini as large as 1.5 cm. in diameter. The duct does not appeardistended, nor is there any evidence of ductile obstruction. These changes are characteristic of chronic parotitiswith pronounced saccular dilatations of acini elements, compatible with congenital sialoangiectasis.

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were occurring every two to three weeks. An x-rayof the parotid area a year previouslv had not shownany calculus or radiopaque material in the parotidarea.

There was no history of any previous illness.Tonsilloadenoidectomy at the age of three was theonly surgical treatment she had undergone. Specif-ically there was no history of any swelling orsoreness of the left parotid gland. The patient wasfour months pregnant when first seen.

Extensive questioning about family history ofsymptoms referable to the parotid area elicited areport of swelling of the face in the patient's sister(Case 2, hereafter reported).Upon physical examination the only positive sig-

nificant finding was tender diffuse right preauric-ular swelling over an area some three or fourcentimeters in diameter with involvement of the

Figure 2.-(Case 1)-Portion of salivary gland showingductal ectasia (X5) .

right parotid gland. Light pressure on the enlargedgland expressed a sero-purulent discharge from theright Stensen's duct. Probing of the duct facilitatedmilking of the gland. Sialoangiography showed ex-treme and extensive sialoangiectasis of the right pa-rotid (Figure 1). During the next four weeks, thepatient had two additional attacks of purulentsialoadenitis.

Figure 3.-(Case 1)-Salivary gland with ductal epi-thelium intact but showing some "piling up" of the cells(X200).

Figure 4.-(Case 1) -Sialoangiogram of left parotid area, showing sialoangiectatic changes with saccular dilata-tions adjacent to the non-dilated ducts throughout the parenchyma of the gland.

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Because of the obvious irreversibility of thepathologic process and the increasing frequency ofattacks, it was decided that operation should be donebefore the third trimester of pregnancy. Total paro-tidectomy with preservation of the facial nerve wascarried out. The glandular tissue was nodular witha multitude of cysts on the surface as well asthroughout the parenchyma.Upon microscopic examination pronounced dila-

tation of the duct system, extending to the finestradicals, was noted. The findings were typical ofsialoangiectasis and chronic sialoadenitis. (See Fig-ures 2 and 3.)The postoperative course was completely without

complication. Following normal termination of thepatient's pregnancy, sialoangiographic studies ofthe left parotid gland were made. Although no his-tory of left parotid swelling could be elicited,studies demonstrated extensive sialoangiectaticchanges with saccular dilatations of ducts and inthe parenchyma adjacent to ducts in the left parotidgland (Figure 4).

CASE 2. The patient, a 20-year-old white woman,a sister of the patient of Case 1, was first observedJune 14, 1961, with a history of cystic swellingbehind and below the left ear for over two and a halfyears. During that time incision and drainage hadbeen carried out more than six times, with releaseof bloody purulent discharge.The patient was asked about but could not recall

any swelling of the preauricular areas of eitherside. She had had tonsilloadenoidectomy at age 2.Upon physical examination an inflamed cystic

lesion 2 cm. in diameter behind and below the leftear was observed.A sialoangiogram (Figure 5) showed the parotid

duct posteriorly entered a dilated structure below

and behind the left ear. There were dilated struc-tures along the superior and medial aspects of thecystic structure as well as small but discrete saccula-tions along the duct ramifications, with no dilatationof the duct. The findings were consistent with sialo-angiectasis and fistula formation.

Left total parotidectomy was carried out. Thepostauricular cyst and fistula were excised en masseand the facial nerve was preserved. The pathologicdiagnosis was sialoangiectasis with abscess forma-tion and chronic sialoadenitis.The patient recovered without complication. No

evidence of sialoangiectasis was observed later in asialoangiogram of the right parotid gland.

COMMENT

In reports dealing with pathogenesis of sialoangi-ectasis of the parotid gland, the importance of re-current infection has been stressed. Pathologicstudies done by Jones3 convinced him that dilatationof the finer ducts led to stagnation of secretion,which favored development of infection. Fifteencases of sialoangiectasis, five of wich were bilateral,were reviewed by Bisgard and co-workers.2 In thatseries there was no obstruction to any part of Sten-sen's duct, ruling out dilatation of the duct beingsecondary to ductile obstruction. Perzik6 demon-strated that changes in chronic parotitis associatedwith fistulae, sialoadenitis and sialoangiectasis aresimilar, since all are due to duct and glandularalterations, the main variation being one of degree.He observed that duct blockage, a complicatingfactor in practically all inflammatory processes, maybe partial or complete, intermittent or continuous.

Planka,7 in an exhaustive review of the literature,showed sialoadenitis to be a definite clinical andpathologic entity of the newborn, as well as ofnurslings and very young children.

Figure 5.-(Case 2)-Sialoangiogram of the left parotid area, showing the opaque medium passing through Sten-sen's duct posteriorly to enter the dilated fistulous cyst. There are small but.diserete dilated sacculations along theduct and adjacent to the cystic structure, but no dilatations of the duct.

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Krepler4 reported the case of a 2-year-old boy whoat the time of a primary attack of pyogenic parotitiswas shown by sialoangiography to have ectasia ofthe duct on the diseased side and small alveolardilatation on the clinically normal side. Kepler alsopresented summaries of cases of a 5-year-old boyand a 16-month-old girl, each of whom had unilat-eral acute sialoadenitis for the first time. In thosecases sialoangiography within four weeks after theattacks demonstrated sialoangiectatic changes inboth sides. Krepler. expressed belief that the factthat sialoangiectasis is demonstrable early in thecourse of recurrent sialoadenitis, often with the firstattack, coupled with the young age of these patients,supports the postulation that congenital abnormali-ties are predisposing factors. It was on the basis ofsialoangiographic pictures that he proposed the termcongenital siaooangiectasis of the parotid.

Jones3 reviewed 20 cases of recurrent parotitis inchildhood, including cases in which the mother oftwo siblings with recurrent parotitis had had re-current parotitis as a child.

Becker and co-workers1 observed that in mostcases of chronic parotitis sialoangiograms show sac-cular dilatation of ducts throughout the wholeparotid gland, and that ectasia is always demon-trable in both parotids even though the clinicaldisease may be confined to one.

If the pathologic processes are severe enough towarrant, total parotidectomy with preservation ofthe facial nerve offers an excellent form of manage-ment of sialoangiectasis and sialoadenitis.

SUMMARY

A clinical review of two sisters who had recurrentparotitis with sialoangiectasis is presented with acorrelation of sialoangiographic studies and histo-pathologic findings. One of the patients had fistulaformation behind and below the ear as a complica-tion of the disease. In one of the two cases sialo-angiographic studies showed typical pathologicchanges on both sides although clinically the diseasewas unilateral.The etiologic interrelationship of recurrent paro-

titis and sialoangiectasis is discussed. The evidencesupports the presumption that congenital sialoangi-ectasis may be a basic underlying factor in manycases of recurrent parotitis.

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REFERENCES

1. Becker, W., et al.: Morphology of diffuse saccularduct ectasis of parotid gland, Zeitschrift. fur Laryngologie,Rhinologie, Otolog. e v 39-479, 1960.

2. Bisgard, J. D., et al.: Bilat. sialoangiectasia of parotidgland, Arch. Surg., 71:337, 1955.

3. Jones, H. E.: Recurrent parotitis in childhood, Arch.Dis. in Child., 28:182, 1953.

4. Krepler, P.: Die kongenital sialodochektasie per parot-idia. Ein beitrag zur pathogenese der pyogenen rekur-rierenden parotitis, Zeitschrift f. kinderheilkunde, 793:211, 1957.

5. Lande, S., et al.: Chr. Sialodoche parotitis with re-current subacute exacerbations, 0. Ped., 2:594, 1933.

6. Perzik, S. L.: Parotidectomy for inflammatory lesions,Am. J. Surg., 102:769, Dec. 1961.

7. Plewka, W.: Suppurative parotitis in the newborn,Arch. Kinderheil, 69:297, April 1921.

The Mongolism-Leukemia SyndromeReport of a Case

ROBERT A. BLOSSOM. M.D., Ventura

A NINE-MONTH-OLD NEGRO BOY was admitted to hos-pital because of severe anemia. The obstetrical rec-ord showed that he had been born after 38 weeksof gestation. At birth he weighed 5 pounds 13/4ounces, was 173/4 inches long and was cyanotic andresponded sluggishly. He was considered to be bor-derline premature. By the ninth postpartum day theinfant had intense jaundice and the liver and spleenwere enlarged. Total serum bilirubin reached amaximum of 15.5 mg. per 100 cc. (direct, 7.8 mg.per 100 cc.) by the twentieth postpartum day. Dur-ing this same period the hemoglobin dropped from11.5 gm. to 8.0 gm. per 100 cc. A battery of labora-tory tests were done in an attempt to determine thecause of the jaundice. Among those with negativeresult were: Coombs test (direct and indirect),serial blood cultures, liver function tests includingcholesterol determination, cephalin flocculation andthymol turbidity. Preparations for demonstration ofsickle cells (sodium bisulfite method) were stronglypositive on four occasions. A discharge diagnosisof sickle cell anemia with crisis was made. In theensuing months, after discharge, the child was seenfor infant diarrhea and for upper respiratory infec-tion. No additional icteric episodes were observed,although sickle cell preparations were consistentlypositive.When admitted again at nine months of age, the

child was noted to be decidedly anemic and a mon-goloid diathesis was apparent: Slight slanting ofthe eyes, flattened palpebral fissures with prominentepicanthal folds, bradycephalic head and large de-posits of fat in the cheeks. There was a moderatedisproportion in the size of the upper and lowerjaw. The mouth was small, the tongue large andprotruding. Hypoplasia of the phalangeal bonecaused shortening of the fourth finger. The palmwas square and across it was a single crease run-ning parallel to the line of insertion of the fingers.The entire body musculature was hypotonic and alljoints were easily hyper-extendable.

At this time the sclerae were clinically icteric andthe liver and spleen were palpable. Hemoglobin con-

Submitted August 8, 1962.From the Department of Pathology, University of Southern Cali-

fornia School of Medicine, Los Angeles 33.

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