Case of Suspected Sialodochitis Fibrinosa (Kussmaul’S Disease)
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Bull Tokyo Dent Coll (2016) 57(2): 91–96 Case Report doi:10.2209/tdcpublication.2015-0028 Case of Suspected Sialodochitis Fibrinosa (Kussmaul’s Disease) Kamichika Hayashi1), Takeshi Onda1), Hitoshi Ohata1), Nobuo Takano2) and Takahiko Shibahara1) 1) Department of Oral and Maxillofacial Surgery, Tokyo Dental College, 1-2-2 Masago, Mihama-ku, Chiba 261-8502, Japan 2) Oral Cancer Center, Tokyo Dental College, 5-11-13 Sugano, Ichikawa, Chiba 272-8513, Japan Received 14 September, 2015/Accepted for publication 12 January, 2016 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. Key words: Sialodochitis fibrinosa — Kussmaul’s disease — Fibrin clot — Allergy — Salivary gland Introduction terized by episodic swelling of the salivary glands, which then discharge clots of fibrin First reported in 1879, Kussmaul’s disease, into the oral cavity5). Although firm diagnostic or sialodochitis fibrinosa, is rare. It is charac- criteria remain to be established, a number 91 92 Hayashi K et al. of studies have listed the following as typical findings in this disease: (1) episodic swelling of the salivary glands; (2) discharge of plugs containing numerous eosinophils from the main ducts; (3) elevated levels of eosinophils and IgE; (4) concomitant presence of other allergic disorders; (5) stenosis and irregular dilatation of the main duct evident on ptya- lography; and (6) hypertrophy of the main ducts, edema, and lymphocyte infiltration of the interstitium surrounding the salivary 3,4,9,11) ducts . Fig. 1 Gelatinous plug discharged from parotid papilla Here we report a case of suspected sialo- Pressure on parotid glands bilaterally induced discharge dochitis fibrinosa. of gelatinous plugs from parotid papillae. Case Report (Fig. 2). The patient was an 80-year-old man who Magnetic resonance imaging (MRI) showed presented at our hospital in October 2012 segmental dilatation of the main ducts of the with the chief complaint of bilateral pain in bilateral parotid ducts. There was no sign of the parotid gland area. The patient had first displacement due to sialoliths or tumors, or noticed swelling and tenderness in the area of abnormal leakage of saliva (Fig. 3). Based of the left parotid gland only. Subsequently, on these results, we reached a tentative he had experienced pain bilaterally in this clinical diagnosis of sialodochitis fibrinosa. area when eating. His previous medical history revealed allergic rhinitis, an enlarged prostate gland, surgical intervention for Treatment and Course thyroid cancer, and mumps in childhood. His temperature on the initial visit was 36.0°C. At his initial visit, the patient complained A blood test showed the following results: of bilateral pain in the parotid gland area. He CRP, 0.01 mg/dl; WBC, 5,500/dl (neutrophils had no difficulty in opening his mouth. Mild 65.2%, eosinophils 1.3%, basophils 0.3%, swelling and tenderness were observed bilat- monocytes 5.0%, lymphocytes 28.2%); RBC, erally in the parotid gland area. Indurations 5×106/dl; and platelet count, 25×104/dl. could be felt around the parotid papillae A physical examination revealed that the bilaterally. Pressure on the parotid glands patient had no difficulty in opening his bilaterally induced discharge of gelatinous mouth. There was mild swelling and tender- plugs from the parotid papillae (Fig. 1). No ness bilaterally in the parotid gland area. discharge of pus was observed. Therefore, the Indurations could be felt around the parotid bilateral parotid gland area was investigated papillae bilaterally. Pressure on the parotid in more detail by panoramic X-ray (Fig. 2) glands bilaterally resulted in discharge of and MRI (Fig. 3). The results showed no gelatinous plugs from the parotid papillae obvious focus of dental infection, nor any (Fig. 1). No discharge of pus was observed tumorous lesions in the buccal or parotid and palpation revealed no hard objects. areas on either side. Sialoliths were also A panoramic X-ray revealed no obvious absent. Retrograde oral bacterial infection tooth that might have served as a focal point via the parotid papillae was suspected, but of infection. There was no calcification in the administration of 750 mg/day amoxicillin region corresponding to the parotid glands for 2 weeks did not improve the symptoms. Sialodochitis Fibrinosa 93 Fig. 2 Panoramic X-ray on initial examination No clear focal point of dental infection was evident. No calcification was observed in region corresponding to parotid glands. Tangential to right parotid gland Tangential to left parotid gland Fig. 3 MRI on initial examination Segmental dilatation of main ducts of bilateral parotid ducts. No signs of displacement due to sialoliths or tumors, or of abnormal leakage of saliva were observed. Swelling of the bilateral parotid gland area gland improved, and by the 60th day, the subsequently recurred, and pressure on the pain had disappeared. On the right side, parotid glands induced discharge of gelati- however, although improving somewhat, the nous plugs from the parotid papillae. On pain persisted. the 15th day, the patient was switched to On the 100th day (February 2013), the 200 mg/day clarithromycin and instructed to patient noticed worsening of the pain in the be conscientious in massaging the parotid right parotid gland area. The area around glands. The pain was alleviated with discharge the right parotid papilla was tender, and of the gelatinous plugs, but reappeared indurations could be felt in that region. at approximately weekly intervals. On the Detailed examination of the bilateral parotid 45th day, the symptoms in the left parotid gland area by computed tomography (CT) 94 Hayashi K et al. Fig. 4 CT from February 2013 Fig. 5 FDG-PET/CT from February 2013 No clear sign of dental infection, tumorous lesion, or No clear sign of any tumorous lesion or pressure on pressure on parotid glands due to such a lesion was parotid glands due to such a lesion was observed. observed; sialoliths were also absent. (Fig. 4) and FDG-PET/CT (Fig. 5) revealed Postoperatively, the pain and swelling of the no dental infection, tumorous lesion, or parotid gland and discharge of gelatinous pressure on the parotid glands due to such a plugs improved, with no recurrence on either lesion; sialoliths were also absent. Retrograde side as of March 2014. oral bacterial infection via the parotid papillae The differential diagnoses for sialodochitis was suspected, and 750 mg/day amoxicillin fibrinosa include mumps, sialolithiasis, recur- was administered for 2 weeks. Although the rent parotiditis, purulent parotiditis, Sjögren’s symptoms improved somewhat, the tenderness syndrome, and periparotid tumor. In the did not resolve. Salivary outflow from the present case, blood tests revealed no indi- right parotid papilla was poor, and a lacrimal cations of inflammation, ruling out mumps, duct bougie was therefore inserted. This recurrent parotiditis, purulent parotiditis, resulted in discharge of gelatinous plugs. No and Sjögren’s syndrome. Sialolithiasis, parotid pus was discharged. tumor, and periparotid tumor were ruled out On the 130th day (March 2013), the based on the CT (Fig. 4), MRI (Fig. 3), and area around the right parotid papilla was PET/CT (Fig. 5) findings. Bilateral sialodo- anesthetized with 1.2 ml of 1% lidocaine chitis fibrinosa was diagnosed based on the containing 1/100,000 epinephrine. A lacrimal recurrent swelling of the parotid glands, the duct bougie was then inserted via the parotid discharge of gelatinous plugs from the parotid papilla and a sharp-pointed scalpel used to papillae (Fig. 1), the segmental dilatation of resect the distal portion of the parotid duct the parotid ducts evident on MRI (Fig. 3), immediately above the bougie, enlarging the and the patient’s history of allergic rhinitis. opening of the parotid duct into the mouth. Clarithromycin was administered for 2 weeks postoperatively at 200 mg/day; salivation was