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A de novo deletion in the C1 inhibitor gene in a case of sporadic hereditary angioneurotic edema.

A sporadic case of hereditary angioneurotic edema (HANE) is reported here. The patient was a 15-year-old girl who for 4 years had suffered recurrent episodes of urticaria-like erythema, followed by vomiting with abdominal pain. She was diagnosed as having Sjögren syndrome by results of sialography and serological studies, and moreover, it was also observed that the C1 inhibitor (C1-INH) activity in her plasma was very low during these episodes of urticarial erythema. Diagnosis of acquired angioneurotic edema (AANE) was excluded because the patient's plasma had no inhibitory effect on the C1-INH activity of normal individuals. Although neither of her parents had a history of HANE, we were able to show that the patient had HANE by Southern blot analysis using C1-INH cDNA as a probe. One of the patient's C1-INH gene alleles was revealed to have at least a 17-kb-length deletion including exons 5-8. Neither her parents nor her healthy brother showed any abnormalities on Southern blot analysis. The parent-child relationship in this family was confirmed by an HLA-typing study of all family members.

Adolescent↗

Submandibular gland with multiple ducts.

A morphological peculiarity was observed in the form of a submandibular gland having three ducts. These ducts opened separately into the oral cavity. Only the upper and the largest of these three ducts was in the usual position and had the expected relation to the lingual nerve. Such a condition is to be kept in mind by a surgeon or a radiologist performing sialography. Unless each of the duct in instilled he may miss pathological findings.

Adult↗

Development of Sjögren's syndrome during treatment with recombinant human interferon-alpha-2b for chronic hepatitis C.

A 63-year-old woman with type C chronic active hepatitis developed Sjögren's syndrome after being treated with recombinant interferon-alpha-2b. After 3 months' interferon-alpha administration, serum levels of gamma-globulin (4.5 g/dl) and titers of antinuclear and anti-SS-A antibodies were greatly increased, anti-SS-B antibody appeared, and the erythrocyte sedimentation rate was elevated. Although no xerostomia was exhibited, the patient experienced conjunctival dryness. Schirmer's test showed reduced lacrimal gland function and a gum test showed reduced salivary gland function. Sialography revealed scattered pools of retained contrast media with a diameter of around 1-2 mm. Based on these findings, a diagnosis of Sjögren's syndrome was made. This present case may provide important information regarding the pathogenesis of Sjögren's syndrome.

Biopsy, Needle↗

[Unusually long latency before the appearance of Frey's syndrome after parotidectomy].

We report on an unusual case of Frey's syndrome. A 45 year old male patient presented with gustatory sweating 14 years after parotidectomy. The sialography showed a post-parotidectomy status without any pathologic findings. The MRI also showed a normal rest-parenchyma of the parotid gland without any pathologic findings. Minor's starch iodine test [14], confirmed the gustatory sweating in the left preauricular area. Frey's syndrome can appear with a latency of 14 years postoperative or post-trauma. The pathogenic mechanism and the curative therapy for this auriculotemporal-syndrome are still unsolved.

Adaptation, Physiological↗

Carotid sheath tumor--a diagnostic challenge.

The tumor within the carotid sheath described in this article is unusual in that its clinical presentation mimicked a deep-lobe parotid tumor. Despite extensive investigation with conventional sialography, technetium pertechnetate scintigraphy, and computerized axial tomography, the correct preoperative diagnosis was not established. The value of CAT scanning in the diagnosis of parapharyngeal tumors is demonstrated.

Adenoma, Pleomorphic↗

Sjögren's syndrome in the adolescent. Report of four cases.

We report three cases of Sjögren's syndrome and a case with a probable diagnosis of Sjögren's syndrome at the ages of 16 (2 cases), 17, and 18 years. Two patients were affected by systemic lupus erythematosus. All four patients showed periductal lymphocytic infiltration in the labial glands. Two of the three patients underwent parotid sialography that revealed punctate or globular sialectasis. A decrease in stimulated parotid flow rate was noted in three of the cases compared with age-matched healthy females, whereas only one patient was diagnosed as having keratoconjunctivitis sicca.

Adolescent↗

Neurogenic tumours of the parapharyngeal space in the paediatric age group.

Two cases of neurogenic tumours of parapharyngeal space in children of 7 years old are presented. Difficulties during the diagnosis, investigations and management are discussed along with a review of the available literature. CAT scan with sialography, carotid angiography during surgery, angle mandibulectomy and liberal incision to expose the great vessels for better control of bleeding are stressed to minimise the peri-operative complication and to assist in complete removal of the tumours.

Child↗

Xeroradiography in relation to a Stafne bone cavity.

A symptomless radiolucency was noted to be lying between the inferior dental canal and the lower border of the left side of the mandible. Sialography of the left submandibular gland showed the existence of salivary gland tissue in relation to this radiolucency. However, xeroradiography revealed that this radiolucency was formed by an infolding of the lower border of the mandibular cortex, thus supporting the hypothesis that a Stafne bone cavity arises from the incorporation of salivary tissue into the developing mandibular cortical bone.

Bone Cysts↗

A radiolucency of the ascending ramus of the mandible associated with invested parotid salivary gland material and analogous with a Stafne bone cavity.

A 60-year-old man presented with a well demarcated radiolucency in the posterior border of the left ascending ramus of the mandible, lying below the neck of the condyle. This area appeared to have the characteristic features of a bone cavity as previously described by Stafne. Although such cavities are now frequently recognized and generally accepted to represent entrapment of the submandibular, or more rarely sublingual, salivary gland tissues; Stafne (1942) only described their location as being, 'situated below the mandibular canal in the region as far forward from the angle as the third molar'. Developmental bone cavities have not been reported previously in the region of the ascending ramus of mandible, nor in relation to the parotid salivary gland. In the case described the radiolucency in the ramus appeared to be developmental in origin and was later shown by sialography, to be closely related to salivary gland tissues of the parotid gland.

Bone Cysts↗

Extraoral parotid sialolithotomy.

The extraoral approach to duct surgery for the removal of parotid stones can be a simple procedure once the stone is accurately located in relation to the skin surface. The combination of sialography and sonography can provide this information. A case report demonstrates the step-by-step approach to diagnosis, localization, and surgery for the management of such extraglandular sialoliths.

Aged↗

Treatment of iatrogenic submandibular sialocele with botulinum toxin. Case report.

We report the case of a 40-year-old woman with a submandibular sialocele (diagnosed by ultrasonography and magnetic resonance sialography) after sialoadenectomy for sialolithiasis. Type A botulinum toxin was injected percutaneously under colour Doppler ultrasonographic guidance into the sialocele and the residual salivary gland. Five months later the submandibular swelling had gone, and we gave a second injection of botulinum toxin to block any residual secretory activity. There were no side effects. This is, as far as we know, the first published report of the use of botulinum toxin to treat an iatrogenic submandibular sialocele.

Adult↗

Submandibular sialogram performed through a Wharton's duct fistula: technical adaptation of conventional technique.

We report a case of submandibular duct fistula in which a sialogram was performed through the fistulous opening after attempt of sialography through the Wharton's duct had failed. To our knowledge, this technical modification in obtaining a sialogram has not been described previously in scientific literature. Blunt-tipped needles and polythene catheters are commonly used for cannulation of the submandibular duct orifice for obtaining a sialogram. However, in this case, a larger gauge metallic cannula was employed to opacify the ductal system through the fistula.

Catheterization↗

Sonographic diagnostic criteria for screening Sjögren's syndrome.

OBJECTIVE: The objective of this study is to establish readily applied sonographic diagnostic criteria for Sjögren's syndrome. STUDY DESIGN: Sonographic images of 79 cases of previously suspected Sjögren's syndrome (including 43 actual cases) were analyzed retrospectively for the following characteristic features: (1) multiple hypoechoic areas, (2) multiple hyperechoic lines or spots, (3) multiple hypoechoic areas surrounded with hyperechoic lines or spots, and (4) obscuration of the gland configuration. Logistic regression analysis was used to extract valuable sonographic findings. Sonographic images of 80 prospective patients (of whom 48 proved to have Sjögren's syndrome) were scored prospectively using selected features to verify the usefulness of the established criteria. RESULTS: Three sonographic findings in parotid and submandibular glands were selected by logistic regression analysis and retrospective and prospective patients compared. Experienced observers could differentiate positive cases of Sjögren's syndrome from negative controls to a highly significant degree. Findings correlated very well with sialographic grading. CONCLUSION: Sonography can be substituted for sialography when applying the selected criteria in screening for Sjögren's syndrome.

Female↗

Computed tomography and ultrasonography in submandibular tumours.

The value of computed tomography (CT) and ultrasound (US) for assessing submandibular tumours has been assessed. Thirty-five patients with 23 benign submandibular gland tumours and 12 malignant tumours have been investigated. In 33/35 patients, 18 plain CT (PCT), 19 CT sialography (CTS), and eight intravenous contrast-enhanced CT (CECT) procedures were performed. PCT did not allow clear delineation of 78% of the lesions, due to the similar density of the tumour and normal submandibular gland parenchyma. The tumours were easily recognized as low-echogenic or low-density masses by US, CTS and CECT. Benign lesions were usually sharply demarcated and had smooth borders on US, CTS and CECT. In addition, lobulation was often recognized in pleomorphic adenomas. Malignant tumours were poorly defined with irregular borders in 11/12 cases on US, CTS and CECT. CECT was superior to PCT and CTS in providing images of the tumour and its involvement of adjacent structures. It was concluded that US is the most useful primary examination method for submandibular tumours, while CECT provides the best evaluation of extension to the surrounding structures and/or adjacent lymph nodes. CTS seems to be unnecessary, if a submandibular tumour can be visualized by US and CECT.

Adenoma, Pleomorphic↗

Sequential quantitative scintigraphy of parotid glands with chronic inflammatory diseases.

A practical, time-saving procedure for sequential quantitative scintigraphy is introduced and 4 parameters chosen from 12 parameters by discriminant analysis are used to evaluate the function of the parotid gland. The examination was performed in 120 cases, including 16 cases with recurrent parotitis in childhood, 33 with chronic obstructive parotitis (COP), 37 with Sjögren's syndrome (SS), 4 with sialadenosis, and 30 normal controls. The scintigraphic findings were analyzed and compared with the histologic findings. The diagnostic value of this method was investigated and scaling for differential diagnosis of COP and SS was established. Scintigraphy is considered to be a useful method for evaluation of parotid function and as a diagnostic aid for SS and COP, especially in patients in whom sialography cannot be performed.

Adolescent↗

Sialoendoscopy: three years' experience as a diagnostic and treatment modality.

PURPOSE: This article describes the use of endoscopy for treating sialolithiasis and compares this diagnostic method with other diagnostic methods. PATIENTS AND METHODS: Forty-six major salivary glands suspected for obstructive pathology (26 males and 20 females aged 12 to 65 years) were treated using a rigid mini endoscope. The indications for sialoendoscopy were 1) screening the ductal system for any residual calculi after sialolith removal, and 3) determining the status of the major duct lumens. RESULTS: Of the 46 endoscopies attempted, 5 were immediate failures as a result of technical problems. Of the remaining 41 patients, 32 had salivary stones, and nine had sialadenitis without evidence of sialolith formation. Within the former group (22 submandibular and 10 parotid), there were four patients in whom sialolithotomy was unsuccessful (three submandibular and one parotid). Of the 22 patients with submandibular sialolithiasis, seven (32%) were undetected by imaging methods (conventional radiography, sialography, and ultrasound). In the 10 parotid ducts, seven (70%) sialoliths were undetected. In the 28 patients who underwent successful calculus removal, no major post-operative complications were noted. Interesting findings noted during endoscopy were a sphincter-like mechanism in the ductal system, evidence of ductal wall changes associated with the presence of salivary stones, peculiar connection between calculi and the ductal wall, a salivary stone forming around a hair inclusion, and the presence of polyps projecting into the duct lumen. CONCLUSIONS: Endoscopy is a minimally invasive technique for removal of calculi from the salivary glands as well as an excellent diagnostic procedure.

Adolescent↗

Changes in the parotid salivary gland of beta-thalassemia patients due to hemosiderin deposits.

2 patients afflicted with beta-thalassemia major presented with pain and swelling of the parotid salivary gland. In 1 patient, the symptoms were of long standing and particularly severe, with diminished and highly viscous salivation. In the other patient, the parotid glands had only recently become painful, while salivary output and quality remained normal. The parotid anatomy in both patients, as demonstrated by sialography, and the clearance of opaque material were normal. Tc99 scintigraphy revealed reduced uptake and rapid clearance of the isotope from the parotid serous glands into the oral cavity, in contrast to the increased uptake by the sublingual mucous glands. Iron deposits were demonstrated in the parotid secretory serous cells. It is postulated that the impaired function of the parotid is linked to iron deposits in the serous cells of this gland, as a sequella of beta-thalassemia.

Adolescent↗

Posterior lingual mandibular bone depression.

An unusual case of posterior lingual mandibular bone depression in a 39-year-old Japanese man is reported. The bilocular type of this condition was composed of 2 large ovoid radiolucent areas which were adjacent to each other. The diagnosis of this condition was made by sialography and tomography. The present case was compared with other unusual cases reported in the Japanese literature.

Adult↗