IgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: a case report

Background IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis. Case presentation A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made. Conclusions IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient’s previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility.

Treatment of juvenile recurrent parotitis with irrigation therapy without anesthesia

Purpose No standardized treatment regimen exists for juvenile recurrent parotitis (JRP). The investigators hypothesized that irrigation with saline only without local anesthesia will be an effective and beneficial option. Methods Using a retrospective study design, a series of children with typical symptoms of JRP who were treated with at least one irrigation therapy were evaluated. This treatment consisted of irrigation of the affected gland with 3–10 ml saline solution without any type of anesthesia. The outcome variables were patient/parent satisfaction, frequency and duration of acute JRP episodes, and the need for antibiotics before and after irrigation therapy. Results The case series was composed of six boys aged 3.3–7.7 years who experienced one to eight sessions of irrigation therapy. The period of follow-up was 9–64 months. We observed a total resolution of symptoms in two children and an improvement in the other four. No relevant side effects were seen. Conclusion Our results suggest that irrigation therapy is a reasonable, simple, and minimally invasive treatment alternative for JRP. In contrast to sialendoscopy or sialography, there is no need for general anesthesia or radiation exposure.

Deep Sedation for Pediatric Parotid Sialendoscopy in Juvenile Recurrent Parotitis

Sialendoscopy is a minimally invasive diagnostic and therapeutic tool for juvenile recurrent parotitis (JRP); the procedure is under general anesthesia, but local anesthesia has been used for sialendoscopy in children >8 years. Based on the experience in children with sedation for gastrointestinal endoscopy, we investigated the reliability and safety of deep sedation for sialendoscopy in JRP. Six children (3 females, 6–13 years) with episodes of parotid swelling underwent interventional (duct dilation and steroid irrigation) sialendoscopy with intravenous bolus of 1 mg/kg propofol and 1 mcg/kg fentanyl, and continuous infusion of 2 mg/kg/h propofol. Sialendoscopy under deep sedation was successfully performed in all the patients; the procedure was well tolerated, without any adverse effects. One event of full awakening was registered and promptly solved without needing to interrupt the procedure. Effectiveness of sialendoscopy under deep sedation was subjectively attested by high positive scores obtained at post-operative standardized questionnaires administered to the patients and their parents, and objectively by the lack of clinical recurrences during the follow-up. The combination of propofol and fentanyl seems to be a reliable and safe means of sedating children with JRP undergoing sialendoscopy.

Salivary Gland Disorders in Children and Adolescents: A 15-year Experience

Introduction Diseases of the salivary glands are rare in children and adolescents, with the exception of viral-induced infections. Objective To determine the clinical course of the disease, the diagnostic procedures, the treatment and the outcome of all children and adolescents affected with salivary gland diseases at our clinic over a period of 15 years. Methods A retrospective chart review including a long-term follow-up was conducted among 146 children and adolescents treated for salivary gland disorders from 2002 to 2016. Results Diagnosing acute sialadenitis was easily managed by all doctors regardless of their specialty. The diagnosis of sialolithiasis was rapidly made only by otorhinolaryngologists, whereas diagnosing juvenile recurrent parotitis imposed difficulties to doctors of all specialties – resulting in a significant delay between the first occurrence of symptoms and the correct diagnosis. The severity-adjusted treatment yielded improvements in all cases, and a full recovery of 75% of the cases of sialolithiasis, 73% of the cases of juvenile recurrent parotitis, and 100% of the cases of acute sialadenitis. Conclusions Due to their low prevalence and the lack of pathognomonic symptoms, salivary gland diseases in children and adolescents are often misdiagnosed, resulting in an unnecessarily long period of suffering despite a favorable outcome following the correct treatment.