Case of Suspected Sialodochitis Fibrinosa (Kussmaul’s Disease)

Abstract

Here we report a case of Kussmaul's disease, or sialodochitis fibrinosa. This rare disease is characterized by recurrent swelling of the salivary glands, which then discharge clots of fibrin into the oral cavity. An 80-year-old man with a history of allergic rhinitis visited our department with the chief complaint of pain in the bilateral parotid gland area on eating. An initial examination revealed mild swelling and tenderness in this region, and indurations could be felt around the bilateral parotid papillae. Pressure on the parotid glands induced discharge of gelatinous plugs from the parotid papillae. No pus was discharged, and there were no palpable hard objects. Panoramic X-ray showed no obvious focus of dental infection, and there was no calcification in the parotid gland region. Magnetic resonance imaging revealed segmental dilatation of the main ducts of both parotid ducts, with no signs of displacement due to sialoliths or tumors, or of abnormal saliva leakage. Two courses of antibiotic therapy resulted in no improvement. During treatment, gelatinous plugs (fibrin clots) obstructing the left parotid duct were dislodged by massage, which prevented further blockage by encouraging salivary outflow. The obstruction persisted in the right parotid duct, however. Therefore, the distal portion of the right parotid duct was partially resected and the opening into the mouth enlarged, which, in combination with massage, prevented further obstruction. The pain and swelling of the parotid gland and discharge of gelatinous plugs improved, with no further recurrence at 12 months postoperatively. This case is presented along with a review of the relevant literature.

Sialodochitis fibrinosa (kussmaul disease) report of 3 cases and literature review

“…In 11 cases, an invasive procedure was required such as instrumental dilation of the parotid ducts and/or partial gland resection. [3] …”

“…[2] Although diagnostic criteria has not been defined, the typical findings of described cases are: (a) recurring episodes of parotid/submandibular gland swelling, (b) discharge of mucofibrinous plugs with a high content of eosinophils, (c) elevated levels of IgE and/or eosinophils in blood, (d) presence of concomitant allergic processes such as asthma or allergic rhinitis, (e) irregular dilation of the main salivary ducts as seen by imaging studies (sialography or MRI), (f) glandular biopsy showing lymphocytic infiltrates and abundant eosinophils within the interstium surrounding the salivary ducts. [3,4] The etiology of this disease remains unknown although the most accepted hypothesis is that it is an allergic process. [4–6] Treatment varies according to the severity of the disease, nevertheless initial supportive treatment with massages and hydration is recommended in conjuncture with the use of antihistamines and/or corticosteroids.…”

“…In refractory cases, corticosteroid infiltration and dilation of the parotid duct has been done. [3] The following presents 3 cases of sialodochitis fibrinosa seen in a third-level hospital with a description of their clinical presentation, diagnostic workup, and management.…”

Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

“…We defined 'eosinophilic sialodochitis' as recurrent painful major salivary gland swelling associated with eosinophil-rich mucus plugs or the histopathologic finding of sialodochitis with a periductal eosinophilic infiltrate. This definition excluded cases of recurrent salivary gland swelling associated with fibrinous mucus plugs and ductal abnormalities, but lacking evidence for eosinophilic infiltration of the major salivary glands (Londe and Pelz, 1933;Ammar-Khodja, 1971;Laudenbach et al, 1985;Ericson et al, 1991;Ray et al, 2015;Hayashi et al, 2016). These cases have been described in children, often in association with atopic illness, and in adults without atopy (Laudenbach et al, 1985).…”

Sialodochitis fibrinosa managed with staged bilateral total parotidectomy

“…4,5 For those with massive parotid enlargement or where conservative measures fail, such as in our patient, parotidectomy can be offered to provide a curative outcome. 6,7 • Sialodochitis fibrinosa should be considered a potential cause of recurrent parotid or submandibular swelling after systemic and infectious causes have been excluded • Sialodochitis fibrinosa should initially be managed conservatively, with operative management reserved for cases refractory to treatment • Investigation into sialodochitis fibrinosa should include a detailed history of the patient's diet, and indicators of allergy or atopy • Staged approach surgery may be a better way of managing the condition, to avoid simultaneous facial nerve injury and allow time for facial nerve rehabilitation…”