Juvenile recurrent parotitis (JRP) can be a debilitating illness in children. Knowing how to recognize and diagnose it for early treatment avoids recurrences that could lead to significant destruction of the glandular parenchyma. This article discusses the various therapeutic modalities proposed in the literature (medical treatment or sialendoscopy) and describes the authors’ treatment of choice of combining antibiotics and iodinated oil sialography.
Juvenile recurrent parotitis (JRP) is a nonspecific sialadenitis with recurrent inflammation of parotid glands in children. JRP is the second most common cause of parotitis in childhood, only after paramyxovirus (the mumps). Furthermore, chronic enlargement of the parotid gland with recurring infection may lead to mistaking this disease for other differential diagnoses such as Godwin’s benign lymphoepithelial lesion, chronic punctate sialectasis, Mikulicz disease, and Sjögren syndrome.
Clinical symptoms of JRP include recurrent parotid inflammation with swelling or pain associated with fever. This pathology is usually unilateral, but bilateral can occur with symptoms usually more prominent on one side. The particular natural history of this disease is its recurrence. The first episode typically occurs between the age of 1 and 2 years and is most often not diagnosed, goes unnoticed, or is mistaken for the mumps, otitis, or pharyngitis.
The diagnosis, often made after the third or fourth episode, is suggested from the history of the disease, the clinical examination, and ultrasonographic findings. The interval between two acute episodes is variable, with an average from 15 days to 2 months, but the disease always recurs. The main criteria for establishing the severity of JRP is the frequency of the recurrences.
The true severity of this disease is its inexorable progression leading to the destruction of the glandular parenchyma with a diminution of its functionality by 50% to 80%. Ultrasonographic findings then show vacuoles, a dilatation of Stensen’s canal, and a wide-open ostium. The challenge is, thus, to diagnose JRP as early as possible, to provide treatment, and to avoid the ultimate destruction of the gland.
Many causes have been described as being responsible for JRP. The present consensus favors a multifactorial origin. However, the main cause is decreased salivary production with an insufficient salivary outflow through the ductal system, which favors ascending salivary gland infections via the oral cavity. Partial obstruction of the ducts is gradually followed by retention and duct dilatation. Thus, further infection is facilitated by sialostasis. Microbiological studies show the same mixed streptococcal and staphylococcal pathogens as found in the oral cavity.
A diminution of local or general immunity has been suggested by several studies on genetic factors and immunoglobulin A deficiency. Allergy has also been incriminated, but not confirmed, as a predisposing factor.
Treatment of JRP
The treatment modalities range from conservative observation to invasive surgical procedures. Indeed, some investigators advocate abstention because of the habitually spontaneous disappearance of the signs in 95% of the cases before or at puberty. These investigators use preventive therapy against recurrences such as massages, encouragement of fluid intake, warmth, and use of chewing gum or sialogogues. Antibiotic treatment is often proposed but with varying regimens. Cohen and colleagues recommended long-term, low-dose prophylactic antibiotics when an immunoglobulin A deficiency is observed. Antibiotics, analgesics, mouth rinses, and sialogogues are considered the first line of treatment.
Minor surgical procedures have been described. One is ligation of Stensen duct to create a pressure-induced atrophy of the secretory acinar cells. However, this method is rarely used because of frequent sialocele or abscess formation. According to the literature, another method of facilitating acinar cell atrophy is through denervation of the parasympathetic supply to the parotid by transecting Jacobson nerve in the middle ear cleft. Some investigators have proposed transecting the chorda tympani nerve, but the results have been unsatisfactory with recurrence of the salivary flow.
Major surgical approaches include superficial, subtotal, and total parotidectomy. Usually only radical methods resolve symptoms, but they are known to be associated with complications such as a high rate of facial nerve damage, Frey syndrome, earlobe numbness, traumatic neuroma of the greater auricular nerve, and unsatisfactory aesthetic results. Only total parotidectomy resolves symptoms, not partial parotidectomy.
Sialography and Sialendoscopy
Many different types of intraglandular medical treatment have been described. Ductal injection of normal saline (0.9%) solution and manipulation with a lacrimal probe via the ostium of Stensen duct has been recommended. Tetracycline instillation into the parotid duct has also been described as having effective results through its sclerotic and cytotoxic effects in the gland. Wang and colleagues proposed intraductal injections of methyl violet, which induced widespread fibrosis and reduction of gland activity with resolution of clinical symptoms. However, it has been established that these dyes are capable of carcinogenic activity, so their intraglandular use should be discontinued.
Galili and Marmary were the first to use lavage to treat JRP with sialography, with good results in 13 of 15 children with unilateral swelling. Symptoms persisted in five of seven children with bilateral disease. Nahieli and colleagues treated JRP by dilatation and abundant flushing (60 mL) under endoscopic control in a series of 26 cases (between 1993 and 2002) with a resolution of symptoms in 92% of the cases with a follow-up of 36 months.
Patients who are candidates for interventional endoscopic treatment are those who suffer from more than one acute episode per year. The justification of this treatment was the sialographic substantiation of multiple strictures of the ducts and the need to dilate them. Furthermore, endoscopic findings show multiple mucous plaques that may be washed away. Lavage with isotonic saline solution under hydrostatic pressure causes dilatation of the strictures and removal of the debris and mucus plaques through the ostium of Stensen duct. Nahieli and colleagues described an adjunct to the procedure by introducing a high-pressure sialoballoon (2.5 French; 18 bars) to dilate the strictures. After the procedure, they injected hydrocortisone solution intraductally and, when possible, introduced a sialostent for 4 weeks to prevent recurrence of the strictures. The postoperative treatment was based on antibiotics, analgesics, and water intake. Sialendoscopy was always bilateral and performed under general anesthesia.
If the result seems to be effective, these reports do not comment on complications. Furthermore, this type of treatment requires general anesthesia and is thus invasive in children. Finally, there is no information regarding the evaluation of glandular function after puberty, and intraglandular high pressure and invasive dilatations may definitively damage glandular function.
Quenin and colleagues, and Faure and colleagues, published preliminary reports of 10 cases using sialendoscopy to treat children with symptomatic JRP. They observed resolution of the symptoms in 89% of the cases with a follow-up of 11 months. Sialendoscopy was performed for patients suffering from at least two episodes of parotitis within a 6-month period. The procedure was bilateral in 7 of the 10 cases and performed under general anesthesia (average time of sialendoscopy 57 minutes) with a hospitalization of 24 hours. The investigators followed a protocol similar to that described by Nahieli and colleagues. They concluded that the diagnosis of JRP is more sensitive with sialendoscopy than with ultrasonography and that sialendoscopy should be used regularly as a diagnostic tool. Concerning the therapeutic effects, they found that high-pressure saline solution was as effective as the sialoballoon for dilatation of the strictures. The main risk described was the possibility of swelling of the pharyngeal portion of the parotid gland, with partial upper airway obstruction, which may be avoided by performing unilateral sialendoscopy only. The investigators also described technical difficulties with endoscope diameter, which limits exploration only to the first branches of the salivary duct (seven cases).
According to the literature, sialendoscopy would seem to provide satisfactory results in the treatment of JRP. However, these reports are of relatively small cohorts (36 in 2 reports). The authors prefer a less invasive treatment method used in over 800 cases, described below.
Sialography with Iodinated Oil
After the diagnosis is made, we always begin treatment with a prescription of antibiotics: macrolides (spiramycin) and nitroimidazoles (metronidazole) by mouth, with antispasmodics (phloroglucinol), with dose according to the weight of the child. Treatment is prescribed for at least 15 days. Corticosteroids (prednisolone, 1 mg per kilo per day) are associated with this treatment.
After this initial treatment, once the infection, inflammation, and pain cease, we perform sialography on an outpatient basis using iodinated oil (Lipiodol Ultra-Fluid ©Guerbet ® ) without any anesthesia of Stensen papilla. The procedure is atraumatic and painless with a very slow injection of only 0.1 to 0.2 mL of Lipiodol without applying any pressure.
We follow the patients in the clinic with clinical examination and ultrasound. In cases of recurrence, we perform the same procedure a second time, but with only one radiograph during the sialography, at a dose of 0.02 millisirven to limit radiation exposure.