Archives of Disease in Childhood 1997;77:359–363 359

REGULAR REVIEW

Recurrent parotitis

V V Chitre, D J Premchandra

Recurrent parotitis is defined as recurrent glands in patients with unilateral disease.4 This parotid inflammation, generally associated with suggests that those with low salivary flow rates non-obstructive sialectasis of the parotid might be predisposed to suVer from repeated gland.1 Also known as juvenile recurrent ascending infections. This relation to salivary parotitis,23 this disease is characterised by flow rates could also explain the familial recurring episodes of swelling and/or pain in tendency that has been reported.389 the parotid gland, usually accompanied by The histological picture includes lym- fever and malaise. It usually aVects children, phocytic infiltration around the intralobular but may persist into adulthood.(Arch Dis Child ducts, and Patey and Thakray proposed that 1997;77:359–363) It is a rare condition, and its aetiology this lymphocytic infiltration damages the duct remains an enigma. Its natural history is wall reticulum, allowing extravasation of secre- tions into the gland parenchyma, and thus variable, and in adults more aggressive inter- 10 vention is often needed. In addition, there is no exacerbating the inflammation. This was sup- 41112 satisfactory explanation for its usual tendency ported by Hemenway and others. to resolve spontaneously after puberty. All this The fragmentation of connective tissue sup- has resulted in considerable uncertainty con- porting the intralobular ducts was also impli- cerning its appropriate management. This cated by these authors in the production of the review was undertaken to collate all the characteristic punctate sialectasis. They pro- information available on this uncommon and posed that the dye used for sialography distressing condition. Diagnosis and manage- ruptured the already weakened duct walls, pro- ment of this condition is also discussed. ducing the appearance of punctate sialectasis. This theory therefore neatly explained the presence of sialectasis in the absence of Aetiology demonstrable distal obstruction. Its cause remains unknown despite several The situation, however, is not as simple. studies. Though the aVected glands demon- Punctate sialectasis is seen in totally asympto- strate sialectasis of the distal ducts, there seems matic glands of aVected individuals in up to to be no element of obstruction in most cases. 70% of cases.13 Further, detailed histopatho- Several theories of causation have been put logical studies have confirmed the presence of forward over the years. duct dilatation and cystic cavities associated Traditionally, ascending infection from the with a chronic inflammatory process.214 And oral cavity has been considered the primary more recently, ultrasonography consistently event, with sialectasis being a secondary revealed hypoechoic areas that corresponded change. Maynard proposed that the recurrent to the punctate sialectases demonstrated by episodes of parotid swelling was the end result 15 of a sequence of events4: sialography. It therefore would appear that x There is first a low grade inflammation of the sialectases are actually present, and are not the gland and duct epithelium, possibly caused merely artefacts produced by the radio-opaque by a low salivary flow rate due to dehydration dye. and debility. As long ago as 1945, Hamilton Bailey x This results in distortion and stricturing of proposed the presence of a congenital abnor- the distal ducts, and metaplasia of the duct mality of the ductal system, and drew a parallel 16 epithelium. with bronchiectasis. He pointed out that x The metaplasia results in excessive mucus bronchiectasis could be congenital as well as Department of secretion. acquired, and in both cases, the end result was Otolaryngology, James These changes, along with possibly a further secondary infection of the bronchioles and Paget Hospital, Great reduction in salivary flow rate, then predispose alveoli. He has been subsequently supported by Yarmouth, Norfolk to recurrent parotid inflammations. several others.1–3 14 According to this argument, V V Chitre D J Premchandra A reduced salivary flow rate may result from punctate dilatation of the small distal ducts glandular damage caused by the primary infec- results in stasis and ascending infection, giving Correspondence to: tion. However, it may be a primary factor as rise to the recurrent acute attacks. Though no Mr V V Chitre, Flat 6, well. Several workers showed low salivary flow evidence has so far emerged in favour of a con- Rosemont, 80/81 Mount 4–7 Ephraim, Tunbridge Wells, rates, and the significant finding was that the genital abnormality, it is still possible that Kent TN4 8BS. flow rate was reduced in even the unaVected genetic factors may prove important. 360 Chitre, Premchandra

Given this state of incomplete knowledge, The number of attacks vary individually, the present consensus is towards a multifacto- with attacks every three to four months being rial approach. Thus Kono and Ito concluded the commonest pattern.13Mandel and Kaynar that the sialectasis is both the cause and the state that attacks tend to occur one to five times result of recurrent parotitis.14 Their histological a year.23 The frequency rate peaks during the studies detected dilated cavities consistent with first year at school, but otherwise remains fairly true sialectasis, as well as a few areas of constant for each individual until puberty. extravasated dye which mimicked sialectasis on After puberty, the symptoms usually subside, the sialogram. Similarly, a detailed study of and may disappear completely.1–4 14 17 25 clinical, radiological, immunological, bacterio- Geterud et al reported that 84% of their logical, and histological findings in 20 aVected patients had recovered by the time they children concluded that the cause was probably attained puberty.1 Further, another 8% of their a combination of a congenital malformation of patients were considered cured by the time portions of the salivary ducts and infections they reached the age of 22. Galili and Yitzhak ascending from the mouth after dehydration of proposed two possible ways by which this the aVected children.2 However, it must also be spontaneous recovery might occur: total atro- acknowledged that juvenile recurrent parotitis phy with consequent lack of symptoms, or can occasionally occur without sialectasis.11417 regeneration of the gland from surviving ductal One child has suVered repeated attacks of system.29 The authors favoured regeneration as parotitis secondary to repeated chewing of the the likely mechanism. However, there are also Stenson’s duct orifice.18 persistent cases.1–3 13 20 23 29 30 The actual Many associations have been proposed in the proportion of ‘persisters’ is debatable, though past; these include immunodeficiency, allergy, most researchers agree that the numbers are upper respiratory infections, mumps, etc. None small.1341114 of these, however, has been conclusively shown The painful swelling is usually associated to have any bearing on this disease.2 Friis et al19 with fever.2 There is typically an absence of pus and others14 17 proposed an autoimmune ori- despite the pyrexia and malaise,3 though gin, but the self limiting nature of recurrent Geterud et al noticed a few drops of mucopu- parotitis and the absence of detectable auto- rulent secretion on palpating the parotid antibodies makes this unlikely.2131420 There gland.1 The swelling lasts from several days to have been reports of sensitivity to upper respi- two weeks,23 and resolves spontaneously, inde- ratory tract infections21421; these infections pendent of any treatment. may set oV attacks of sialadenitis merely by causing dehydration in a child with sialectasis.2 Investigations The higher rate of secretion in the sub- (1) SIALOGRAPHY mandibular gland compared with the parotid The mainstay of diagnosis is sialography, gland may protect it from infections2; also, the though its role is now becoming secondary to submandibular gland secretion is relatively ultrasonography. In 1971, Hemenway classi- richer in mucus, which has antiseptic proper- fied sialectasis into (a) large duct sialectasis, due ties. In fact, no report was found in the to obstruction of the main duct, and (b) small literature implicating the submandibular duct/punctate/terminal sialectasis, in which there gland. are multiple small round opacities at the termi- nation of the smaller ducts.11 Later, Gates31 and Clinical features Noyek et al32 reviewed the classification of Recurrent parotitis presents as a recurrent punctate sialectasis, and further classified this painful swelling during mastication and/or into (a) pruned tree appearance—in the early swallowing.22 The disease usually starts in a stages, the ducts are stretched, tapered, and child between 3 and 6 years of age,131323 but decreased in number; (b) punctate sialectasis— earlier and later occurrence has been the peripheral ducts demonstrate punctate observed.214172425Ericson et al followed up 20 dilatation <1 mm diameter, and the intraglan- children and the age of onset in their series dular ductal system is stretched and tapered; ranged from 3 months to 16 years.2 (c) globular sialectasis—the ducts are between 1 Most studies report a sex distribution mm and 2 mm in size and they may be partially favouring males.1232426Seventy two per cent of non-visualised or irregular, giving rise to a patients were male in the series of Geterud et al ‘mulberry pattern’ fruit laden tree appearance; of 25 patients.1 However, Watkin and Hobsley (d) cavitatory sialectasis—coalescence of cystic found from a study of 68 patients, of whom 26 lesions produces a cavitatory appearance; and were children, that the sex distribution was (e) destructive sialectasis—there is a bizarre pat- equal in childhood, and actually aVected tern of pooling, possibly with stones in the females more than males (7.5:1) in patients gland.33 where the onset of symptoms was after the age The typical changes in recurrent parotitis are of 16 years.13 punctate and globular sialectasis, which are The symptoms are usually unilateral; when scattered throughout the gland; cavitatory and bilateral, the symptoms are more prominent on destructive sialectasis are not seen.2 one side.232327In a series of 25 patients, symp- These changes are usually bilateral even if toms were unilateral in 18 patients and bilateral the presentation is unilateral.12131423Ericson et in seven patients (28%).1 Katzen and Du Ples- al found eight out of 12 clinically symptomless sis, in 1964, reported a higher incidence of glands demonstrated sialectasis.2 The lesions bilateral presentations than that observed by were however smaller and fewer in the more recent studies.28 symptom-free glands. Watkin and Hobsley Recurrent parotitis 361

found punctate sialectasis in 69% of asympto- abnormality, sialography is performed only if matic glands.13 On the other hand, sialographic symptoms recur. changes are not always bilateral, as had been 1 previously believed. Several authors report (4) CYTOLOGY that these changes tend to diminish and some- Salivary smears of normal children are acellu- times disappear after the disease becomes lar. In contrast, saliva in the presence of quiescent.3142329However, Geterud et al found sialectasis revealed large amounts of granulo- that only two out of 16 patients demonstrated a cytes, some lymphocytes, and in about 50% of significant reduction in the sialectasia and fur- cases, bacteria.2 The bacteria were mixed, and ther, these two patients had only minimal included aerobic and anaerobic cocci. primary changes.1 In glands with sialectasis, peripheral intrag- (5) HISTOLOGY landular ducts are invisible,125132730indicating Dilated interlobular ducts with lymphocyte that the changes aVect the peripheral parts of infiltration in the surrounding tissues is seen2; the ductal tree. The main ducts may be affected the lymphocytes tend to form lymphoid as well. Ericson et al found slight to moderate follicles. The duct epithelium shows hyperpla- dilatation of the main duct in 25% of sympto- sia and metaplasia, with a pseudostratified matic glands,2 while Geterud et al reported cylindric pattern being common. severe main duct changes in 8% of their patients.1 However, the presence of main duct dilatation did not influence the clinical course Treatment of these patients, as had previously been Uncertainty about its aetiology has hampered proposed.4 Other findings on sialography the development of a universally accepted include acinar and ductal atrophy, with im- treatment strategy for this condition. The paired glandular function.3 problem is compounded by the rarity of this There is some controversy whether sialo- disease and its uncertain natural history. graphic changes correlate with clinical symp- Treatment of the acute episode aims to toms. Ericson et al found a strong relationship deliver relief of symptoms and to prevent dam- between clinical symptoms and multiple age to the gland parenchyma. Analgesics and sialectases.2 However, Geterud et al reached antibiotics have been found to be rapidly eVec- exactly the opposite conclusion.1 tive in relieving the pain and swelling.213 Though co-amoxiclav may be used, penicillin is considered adequate, as the infecting agent is (2) DIGITAL SUBTRACTION SIALOGRAPHY not usually a staphylococcus.2 Most workers This has been recommended as superior to agree in practice, but many question whether conventional sialography for the recognition of antibiotics really change the natural course of inflammatory changes and chronic 128 34 the disease, and wonder if resolution of sialolithiasis. symptoms could merely reflect the natural pro- gression of this disease.23 In addition to antibi- (3) ULTRASONOGRAPHY otics and analgesics, other treatments include Ultrasonography consistently revealed hypo- sialagogic agents to increase salivary flow, echoic areas that corresponded to the punctate warmth and massage, and duct probing.223 sialectases demonstrated by sialography.15 The The logic behind probing is questionable1 as authors therefore recommended ultrasonogra- duct dilatation and not duct stenosis is the phy as the primary investigation for diagnosis, underlying pathology. The treatment seems to in addition to follow up. An earlier study had be eVective, however, and may help by clearing also found ultrasonography useful,35 though a the plugs of mucus and cells that form in the direct comparison between conventional acute phase. Bailey recommended duct cannu- sialography, digital subtraction sialography, lation and lavage with 1% mercurochrome.16 and ultrasonography had found ultrasonogra- Steroids may reduce swelling, but will not pre- phy less sensitive than the other two for inflam- vent recurrences.13 23 matory changes and sialolithiasis.34 It is possi- Prevention of recurrences is diYcult, but ble that improvements in radiological skills and preventing dehydration and prescribing a equipment may have now rendered ultrasonog- prophylactic course of penicillin/co-amoxiclav raphy as reliable as sialography. during winter may help; no studies are Ultrasonography may completely replace available, however, to substantiate this belief. sialography in the near future. Murrat et al have The treatment of repeated attacks is more recently proposed a protocol for the investiga- diYcult, and several diVerent methods have tion of investigating a case of intermittent pain been tried. and/or swelling of the salivary glands, and they 36 recommend an initial ultrasound. If this (1) RADIOTHERAPY investigation reveals calculi/duct dilatation/ This was used for several years,1 despite it cysts/gland enlargement, they then proceed to being considered useless by several researchers sialography. Duct dilatation having been al- more than 30 years ago.37 In fact, the literature ready revealed by ultrasonography, the role of contains virtually no evidence in favour of this sialography would be to rule out duct stenosis method of treatment, and yet radiotherapy was or obstruction. If, on the other hand, ultra- being used as the sole treatment at several sound reveals a solid mass, computed tomogra- centres.13 Its popularity probably rested on its phy or magnetic resonance imaging is indi- perceived eYcacy in reducing the mortality cated. If the ultrasonography reveals no from acute bacterial parotitis by about 50% in 362 Chitre, Premchandra

11 the 1930s, though the author rightly pointed Ultrasound Solid CT/MRI out that the improved prognosis of that condi- mass tion was probably due to improved medical care. Calculi/duct dilatation/gland enlargement (2) DUCT LIGATION Normal This was popularised by Diamant and Enfors.38 It has been used successfully by others,1 though at least one study had described varying Sialography results.12 Geterud et al recommend it as a sim- To rule out duct Sialography only if ple and eVective treatment. stenosis/obstruction symptoms recur

36 (3) PAROTIDECTOMY Figure 1 Investigations (proposed by Murrat et al ); CT = computed tomography, MRI = magnetic resonance Parotidectomy has always been the gold stand- imaging. ard for obtaining permanent relief. With this operation, however, one is faced with the risk of TREATMENT OF ACUTE EPISODES facial nerve injury; this is especially relevant in Aim: to relieve symptoms and prevent damage recurrent parotitis, where repeated infections to gland parenchyma. result in fibrosis of the gland. x Analgesics x Antibiotics—penicillin/co-amoxiclav (4) TYMPANIC NEURECTOMY x Sialogogic agents This procedure has recently been recom- x Warmth and massage 22 mended as an eVective procedure, with good x (? Probing of duct and ? steroids for severe results in 70% cases. Its aim is to destroy secre- attacks). tomotor fibres to the parotid gland thus abolishing/reducing its secretion. The authors PREVENTING REPEATED ATTACKS recommend extensive interruption of the (Preventing repeated attacks is usually unsuc- secretomotor fibres by thoroughly drilling into cessful.) the hypotympanum and below the basal turn of x Prophylactic antibiotics—penicillin/co- the cochlea. Several others have also reported amoxiclav 39 40 similar results. Nerve regeneration and x Prevent dehydration. incomplete sectioning are probably responsible for the early and late failures; it is therefore RECURRENT ATTACKS CONTINUING INTO necessary that the procedure is performed by 22 ADULTHOOD an experienced otologist. x Duct ligation—simple operation, but with variable results (5) OTHER METHODS x Parotidectomy—good results, but with a Various other treatment options have been small risk of facial nerve damage studied. Bowling et al proposed intraductal tet- x Tympanic neurectomy—good results in racycline instillation as an eVective, low risk the hands of experienced otologists. treatment.41 They hypothesised that tetracy- cline produced acinar atrophy and demon- strated encouraging results in rabbits. No stud- Summary ies have been conducted on human subjects. (1) Recurrent parotitis is probably caused by Some studies found that performing a sialo- a congenital abnormality of the salivary gland graphic study itself resulted in significant ducts with recurrent attacks of ascending improvement of symptoms.33442 The hypoth- infection, perhaps aided by dehydration. The esis was that improvement resulted as a result parotid gland is predominantly aVected prob- of the flushing, dilating, and antiseptic actions ably because of its lower rate of secretion com- of the iodine containing dye.23 pared with the submandibular gland. Finally, the fact that many patients seem to (2) The condition mainly aVects children recover spontaneously has led researchers to between the ages of 3 and 6, with males being support a conservative approach. Watkin and more commonly aVected. The symptoms peak Hobsley found that 56% adults and 64% chil- in the first year of school, and usually, but not dren recovered with only symptomatic treat- invariably, begin to subside at puberty. By the ment, over a five year period.13 Similarly, age of 22, most patients are completely Geterud et al reported that symptoms disap- symptom-free. When the disease starts after peared by the age of 22 in 23 of 25 patients.1 A puberty, females are predominantly aVected. conservative approach therefore is recom- (3) Ultrasound is the appropriate initial mended for children, with more aggressive investigation, and is usually supplemented by treatment being reserved for the 40% adults sialography. The sialography may itself cause a and 4% of children7 whose symptoms persist or resolution of symptoms. worsen. (4) Treatment is conservative in the first instance, and an expectant policy is indicated. More aggressive treatment is justified only for A suggested management plan those adults with persistent problems. This INVESTIGATIONS may be parotid duct ligation, parotidectomy, or The investigations to perform if there is a clini- tympanic neurectomy, depending upon the cal suspicion of recurrent parotitis are shown in preference and experience of the treating phy- fig 1. sician. Recurrent parotitis 363

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