New insights into juvenile parotitis

AIM: We inquired about the possibility of a familial trend in juvenile parotitis and evaluated the role of SPINK1 mutations in juvenile parotitis. METHODS: The clinical records of all children admitted to the Helsinki University Hospital during 1995 to May 2003 because of swelling in the parotid gland were reviewed. A questionnaire on possible recurrences and on familial cases was mailed. As disturbances in trypsin inhibition might be involved in the pathogenesis, we assessed the SPINK1 gene encoding for Kazal-type trypsin inhibitor in voluntary patients. The study group comprised 133 children (boys 82 girls 51) with juvenile parotitis. The median age at presentation of first symptoms was 6.0 y (range 1-19 y). RESULTS: Recurrent symptoms in the parotid gland were common (57%), and 29% of the children (38/133) had suffered from four or more episodes. A young age at the first episode of symptoms increased the likelihood of recurrences (p<0.0001). Familial cases of parotid swelling were common (22%; response rate 67%). A total of 47 patients (35%) agreed to testing for SPINK1 status. Four children had a major mutation (N34S or P55S), corresponding to an 8.5% (4/47) prevalence, but this was not different from the controls (5%).CONCLUSION: It is likely that inherited factors are involved in the manifestation of juvenile parotitis in a subset of patients. It is tempting to speculate that disturbed proteolytic balance may play a role in the development of symptoms.     

Suppurative parotitis caused by anaerobic bacteria in newborns.

Staphylococci are the usual bacterial etiology of suppurative parotitis in newborns. This report describes for the first time recovery of anaerobic bacteria from aspirates of the infected gland in two infants with suppurative parotitis. Peptostreptococcus intermedius and Prevotella melaninogenica were isolated from one child and Prevotella intermedia from the other patient. Complete recovery occurred after 4 weeks of antimicrobial therapy.     

小儿流行性腮腺炎并发急性胰腺炎——附23例报道

目的总结和探讨小儿流行性腮腺炎并发急性胰腺炎临床诊治特点。方法回顾性分析23例（男13例，女10例，年龄5～14岁）小儿流行性腮腺炎并发急性胰腺炎的临床资料。全部病例检查血尿淀粉酶及腹部CT。经TPN、生长抑素、奥美拉唑及阿昔洛韦治疗，全组治愈。结果全组血尿淀粉酶增高，CT显示16例胰腺水肿增大，7例正常。23例患儿均为腮腺炎病毒感染所致；均为急性水肿性胰腺炎，给予保守治疗，治愈。结论儿童期急性胰腺炎病因与成人不同，多由于病毒感染引起，保守治疗效果较好。     

IMMUNOLOGICAL STUDIES IN TWO CHILDREN WITH RECURRENT PAROTITIS

ABSTRACT. In two children with recurrent parotitis, labial salivary gland biopsies showed chronic sialoadenitis. Immunofluorescence studies disclosed deposits of immunoglobulins and complement in juxta-acinar small vessels. Case 1 had gluten enteropathy, IgA deficiency and high titres of antinuclear antibodies (ANA), and in vivo fixation of ANA to nuclei of different cells in lip, skin and jejunum was present. Case 2 showed deposition of IgM in the dermo-epidermal junction of the skin. These findings suggest that autoimmune reactivity and immune complexes may play a role in the pathogenesis of this disorder.     

Intrathyroidal Branchial Cleft-like Cyst with Heterotopic Salivary Gland-type Tissue

A rare case is described of intrathyroidal branchial cleft-like cyst associated with unusual heterotopic tissues including the salivary gland type tissue, fat, and cartilage. This coexistence in the thyroid gland has not been described previously, to our knowledge. The patient was a 7-year-old girl with a growing mass in the left lateral neck. The ultrasonography revealed a cystic lesion in the left thyroid. Histologically, the cyst was lined by squamous or respiratory-type epithelium resting on the fibrous tissue containing lymphoid tissues with follicle formation and solid cell nests (SCNs). This cyst was intimately associated with heterotopic tissues including lobules of well-differentiated seromucinous salivary glands, mature fat tissue, and islands of the cartilage. This association of branchial cleft-like cyst with SCNs and unusual heterotopic tissues in the normal thyroid suggests a possible origin from the SCN as ultimobranchial vestigial structures.     

Diabetic ketoacidosis following mumps

A-13-year-old girl presented with diabetic ketoacidosis with convincing clinical signs of parotitis (fever, drooling of saliva, inability to swallow with development of bilateral parotid swelling) and pancreatitis (fever, abdominal pain and vomiting), along with high serum amylase and positive mumps IgM titer. This suggests that mumps virus may have been the causative factor, probably as a result of concomitant involvement of the pancreas.     

Selective IgA deficiency in children with recurrent parotitis of childhood

Recurrent parotitis of childhood is defined as the relapsing form of juvenile (idiopathic) parotitis and represents a rare inflammatory disorder of the parotid gland with potentially significant morbidity. We reviewed the charts of patients who were diagnosed with inflammatory parotid diseases in our institution between 1992 and 2002. There were 91 patients presenting with juvenile parotitis (1 of 6117 of all clinical visits). Of these 91 cases, 23 patients (28%) had the relapsing form of juvenile parotitis, and the median number of episodes was 5 (range, 2-20). Laboratory investigations revealed that 5 patients had selective IgA deficiency. The prevalence (22%) is different from the cumulative prevalence of IgA deficiency in a healthy population (0.3%; P < 0.001).     

Isolated Langerhans cell histiocytosis of the thyroid in a female infant

Langerhans cell histiocytosis (LCH) involving the thyroid is very rare. It can be easily confused with far more common benign goiters or thyroid neoplasms. We report on a 5-month-old female patient presenting with progressive enlargement of an anterior neck mass. This patient underwent left subtotal thyroidectomy following which a diagnosis of isolated LCH involving the thyroid gland was confirmed. A course of chemotherapy was administered, and oral thyroxine replacement was initiated. Eighteen months after this treatment, the patient remained in complete remission. Following a thorough review of the literature, as best we are aware, this patient is the youngest individual suffering LCH who has been reported in the literature. In conclusion, isolated LCH of the thyroid is rare, and its diagnosis can be challenging for a clinician and typically requires appropriate awareness. Local excision is the treatment of choice, and prolonged follow-up is recommended.     

Facial palsy associated with mumps parotitis.

Peripheral facial nerve paralysis is relatively common in the pediatric age group. However, facial palsy rarely has been documented in patients with mumps parotitis. This case report describes the findings of a 3-year-old Japanese boy who developed facial palsy immediately after mumps parotitis. This work calls attention to a possible association between mumps parotitis and facial palsy.     

Acute suppurative parotitis caused by anaerobic bacteria: report of two cases.

Staphylococcus aureus is the predominant pathogen in acute suppurative parotitis. Although anaerobic bacteria are prevalent in the normal oral and upper respiratory tract flora and frequently are involved in oral and facial infections, they have seldom been isolated from patients with suppurative parotitis. It may be that a role for anaerobes in parotitis has not been documented because of inadequate specimen transport or anaerobic culture techniques. We describe two cases of acute suppurative parotitis in which cultures yielded anaerobic bacteria. In one case, no aerobes were isolated; in the other, the anaerobe was the predominant organism numerically.     

Clinical and microbiological analysis of six children with acute suppurative parotitis

Six children including two neonates with acute suppurative parotitis are described. They presented typical symptoms and signs of fever (4/6) and swelling (6/6), tenderness (6/6), erythema (4/6) and local warmness (4/6) of the parotid gland affected. Leucocytosis and an elevated erythrocyte sedimentation rate and serum amylase level may be seen. The presence of purulent discharge from the Stensen duct when the parotid gland is externally compressed is pathognomonic of the disease, and the microbiological diagnosis can be made by culture of the pus. Microbiologically, this series highlights the polymicrobic nature and importance of Streptococcus viridans in paediatric suppurative parotitis, indicating, therefore, that the causative bacteria entered the gland from the oral cavity. Our data also suggest that initial antibiotic therapy for such patients should provide adequate coverage for streptococci and staphylococci as well as for anaerobic bacteria.     

Brunner's gland adenoma: a rare cause of vomiting

Upper gastrointestinal system adenoma is generally seen amongst elderly patients and quite rarely seen during the childhood. A 14-y-old female patient was referred to our hospital with complaints of vomiting and abdominal pain. She had been followed up for 6 y with the diagnosis of familial intermittent fever and chronic renal failure due to amyloidosis. Endoscopic examination of the upper gastrointestinal system revealed mild hyperaemia in the corpus and antrum, and a polyp of 0.5x0.5 cm with an ulcerated and pedunculated top in the bulbus. Brunner's gland adenoma was diagnosed by the histopathological examination of the lesion following polypectomy. CONCLUSION: Brunner's gland adenoma is usually asymptomatic; however, it may reveal clinical findings such as obstruction, bleeding or intussusception, especially in uraemic patients. Thus, we would like to emphasize that, in patients with chronic renal failure and acute onset vomiting and abdominal pain, Brunner's gland adenoma should be considered in the differential diagnosis.     

Reactive Follicular Hyperplasia of Intraparotid Lymphoid Tissue Presenting as a recurrent Parotid Enlargement

A patient who had reactive lymphoid hyperplasia of intraparotid lymphoia tissue, clinically presenting as a recurrent parotitis is described. The case offers a new etiologic mechanism for recurrent parotitis.     

Primary Gougerot-Sj?gren syndrome in a 13-year-old girl]

Sj?gren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sj?gren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sj?gren's syndrome, revealed by recurrent parotitis. CASE REPORT: This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sj?gren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sj?gren's syndrome. Diagnosis of Sj?gren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.     

Salivary Gland Tumors in Children: A Retrospective Clinical Review

Seven patients with salivary gland tumors who underwent between 1972 and 2012 were retrospectively evaluated. The age of the patients ranged from 6.3 to 13 years old; five were females and two were males. Five patients had stage IVa, one patient had stage I, and one patient had stage II disease. The surgical margin was found to be positive in five cases. There were three adenoid cystic carcinoma, two adenocarcinoma, one anaplastic carcinoma, and one mucoepidermoid carcinoma. There were five parotid, one lacrimal gland, and one palatal involvement. Three patients who had low stage tumors were treated with surgery alone. Four of the cases received adjuvant radiation and chemotherapy. One patient with parotid tumor died with progressive disease. One case with lacrimal gland neoplasm was alive 48 months after discontinuation of treatment. The other palate case was lost to follow-up 1 month after the beginning of the treatment. Surgery is the primary treatment, with radiotherapy and chemotherapy used as adjuvant treatments. The treatment options need to be selected and planned for each individual patient.     

MRI and MR sialography of juvenile recurrent parotitis

Background  

Juvenile recurrent parotitis (JRP) is the second most common inflammatory salivary gland disease of childhood, after mumps. Diagnosis of JRP is usually based on clinical history of recurrent unilateral or bilateral parotid swelling and demonstration of sialectasis. Conventional sialography, digital sialography, US, MRI and sialoendoscopy have been used as investigative tools for the diagnosis of JRP. MR sialography is increasingly recognized as a useful supplement to sialography in salivary duct disorders.     

Simultaneous pediatric kidney transplantation and ureterocystoplasty in a 20-month-old boy

Kidney transplantation (KT) in children with end-stage renal disease and an abnormal bladder poses a complex management challenge. Ureterocystoplasty (UC) has been previously reported in older children with non-compliant bladders, but the timing and technique of repair are controversial. This case reports the youngest patient, a 20-month-old boy to undergo successful single-stage UC and living-related KT. UC was performed because of a fibrotic, non-compliant bladder. A temporary vesicostomy was placed to provide adequate drainage in the presence of urethral stenosis. The patient developed a single episode of pyelonephritis within the first six months post-operatively, but there were no other urologic complications. At 13 months, the renal function is excellent with a mean glomerular filtration rate of 100 mL/min/1.73 m(2) and no clinical evidence of rejection. This case demonstrates that simultaneous UC and KT can be safely performed even in infants with non-compliant bladders and renal failure.     

Plunging (cervical) ranula

A plunging ranula is a cyst containing extravasated saliva originating from the sublingual gland. It presents as a swelling in the submandibular region of the neck, often without any abnormality in the oral cavity. Excision of the sublingual gland without neck dissection usually is curative. We report a 16-year-old patient with a plunging ranula and review the aetiology, diagnosis, and management of this infrequent condition, which often is misdiagnosed.     

Recurrent mumps parotitis following natural infection and immunization

Mumps is presumed to confer permanent immunity. Three patients previously known to possess mumps serum antibodies subsequently developed clinical parotitis and showed a diagnostic fourfold rise in mumps V antibody. A review of the literature disclosed several cases of parotitis, either recurrent or postimmunization, but the preparotitis immune status of the patient was unknown or the cause of the mumps was not verified. Case reports of parotitis due to parainfluenza 3 and coxsackie viruses A and the heterotypic serological responses among the myxoviruses are reviewed. Parotitis, especially after prior immunization, requires definitive diagnosis by viral isolation and/or serology before it can be attributed to mumps virus.     

Thyroglossal duct cyst or ectopic thyroid gland?

Abstract: We report a 16-year-old boy with an anterior midline neck mass clinically diagnosed as a thyroglossal duct cyst. An ultrasound scan performed prior to surgery suggested a normally located thyroid gland. Following surgical excision of the presumed thyroglossal duct cyst the patient became hypothyroid. A radioisotope scan confirmed no active thyroid tissue. A pitfall in the ultrasonographic identification of the thyroid gland is discussed.