Primary malignant melanoma of the oral mucosa.

PURPOSE: The occurrence of primary oral melanoma is very rare. Large clinical series suggesting appropriate treatment modalities are lacking; the clinician has to rely on case reports to gain insight into the management of this tumor, which is much more aggressive than its skin counterpart.Patients and methods The cases of 5 patients with primary oral melanoma during a 6-year period were retrospectively reviewed. Four patients presented with tumors located in the maxilla. Preoperative workup included microscopic examination of incisional biopsy specimens for the confirmation of the diagnosis and thorough imaging of the body to rule out distant metastases. Histologically significant vertical invasion was found in all patients. No patient had distant metastases initially, and only 1 patient presented with neck disease. Primary treatment included wide local excision in 4 patients and therapeutic modified radical neck dissection in the 1 patient with neck node involvement on presentation. One patient was treated primarily with radiation therapy due to medically compromised status. Adjuvant immunochemotherapy (DAV protocol) was administered to 4 patients. Radiation therapy was used in an adjuvant fashion in 3 patients. RESULTS: All of the patients developed distant metastases to the lung and liver and eventually died of their disease. Survival ranged from 14 to 38 months (mean, 25.6 months). Local recurrence occurred in 1 patient and was attributed to positive surgical margins. Contralateral neck disease developed in the patient who underwent therapeutic neck dissection. CONCLUSIONS: The prognosis of primary oral melanoma remains poor despite adequate locoregional control of the disease. It seems that diagnosis is made late in the course of the disease when the primary tumor has already shed microscopic distant metastases.     

Acidic pH Is Required for the Multilamellar Assembly of Skin Barrier Lipids In Vitro

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Signs and Symptoms of Temporomandibular Joint Disorders Related to the Degree of Mouth Opening and Hearing Loss

Background

The temporomandibular joint is a unique bi-condylar joint involved in mastication and speech. Temporomandibular joint disorders (TMD) have a range of symptoms, including aural symptoms, and are present in approximately 75% of normal populations. The present study examined the relationship between signs and symptoms of TMD and mouth opening, gender, joint and aural symptoms, and hearing loss.

Methods

The study involved 464 healthy Greek university students (156 men and 308 women) with a mean age of 19.6 years. Age, gender and maximum mouth opening was recorded. Mouth opening was measured using Vernier calipers. An anamnestic questionnaire was used to stratify the subjects into four groups based on TMD severity. Aural symptoms and an audiogram were recorded for each subject too. Data were analyzed using multifactor ANOVA, chi-square, t-test, Mann-Whitney and Kruskal-Wallis tests.

Results

The overall incidence of TMD signs and symptoms was 73.3%. The incidence and severity was greater in females than males (p-value 0.0001 < 0.05). The number of aural symptoms was associated to the TMD severity (p-value 0.0001 < 0.05) as well as maximum mouth opening (p-value 0.004 < 0.05). Audiometry showed that moderate and severe TMD was associated with hearing loss of median and low tones respectively (p-value 0.0001 < 0.05). TMJ pain (p-value 0.0001 < 0.05), TMJ ankylosis (p-value 0.0001 < 0.05), bruxism (p-value 0.0001 < 0.05) and ear itching (p-value 0.0001 < 0.05) were also found to be statistically different between TMD and non-TMD subjects.

Conclusions

TMD signs and symptoms were more common and severe in females than males. TMD severity is correlated with the degree of mouth opening and the number of aural symptoms. The absence or presence of mild TMD are associated with normal audiograms while moderate and severe TMD are related to hearing loss in median and low tones respectively. Bruxism, joint ankylosis, joint pain and ear itching were more common in TMD than non-TMD patients.     

Food allergy-related paediatric constipation: the usefulness of atopy patch test

The aims of this study were to evaluate the implication of food allergy as a cause of paediatric constipation and to determine the diet period needed to tolerate the constipation-causing foods. Fifty-four children aged 6 months to 14 years (median, 42 months) suffering from chronic constipation (without anatomic abnormalities, cοeliac disease or hypothyroidism), unresponsive to a 3-month laxative therapy, were prospectively evaluated. All participants were evaluated for allergy to cow's milk, egg, wheat, rice, corn, potato, chicken, beef and soy, using skin tests (SPT), serum specific IgE and atopy patch test (APT). A withdrawal of the APT-positive foods was instructed. Thirty-two children had positive APT; 15 were positive to one; six, to two and 11, to three or more food allergens, wheat and egg being the commonest. After withdrawing the APT-positive foods for an 8-week period, constipation had improved in 28/32 children, but a relapse of constipation was noticed after an oral food challenge, so they continued the elimination diet. Tolerance to food allergens was achieved in only 6/28 after 6 months, compared to 25/28 after 12 months and to all after a 2-year-long elimination. Food allergy seems to be a significant etiologic factor for chronic constipation not responding to treatment, in infants and young children. APT was found to be useful in evaluating non-IgE allergy-mediated constipation, and there was no correlation of APT with IgE detection. Tolerance was adequately achieved after 12 months of strict food allergen elimination.     

Use of annual ABPM, and repeated carotid scan and echocardiography to monitor cardiovascular health over nine yr in pediatric and young adult renal transplant recipients

In adult hypertensive patients, increased cIMT and LVH are independent risk factors for cardiovascular events. We have previously observed that in pediatric RTRs with tight control of BP, cIMT did not progress over time. This investigation is an extension of the aforementioned study aimed at re-examining cIMT and also reporting serial echocardiography results. Twenty-two RTRs aged 9.4 ± 3.3 yr at their baseline carotid scan underwent two additional vascular ultrasounds during a follow-up of 9.1 ± 0.9 yr. Carotid scan and echocardiography examinations were carried out simultaneously with ABPM. Antihypertensive therapy was determined according to the recipient's ABPM results, which were performed at yearly intervals. Baseline cIMT was significantly greater in RTRs than in healthy controls. There was no statistical evidence of systematic changes in cIMT over time. At the last examination, 14 of 17 RTRs with treated hypertension had controlled hypertension (prevalence 82%; 95% CI, 56.5-96.2), and the overall prevalence of LVH was 4.5% (95% CI, -0.01 to 23.5). The lack of progression of cIMT over time and the low prevalence of LVH might reflect the effect of long-standing BP control.     

Inner ear anatomy in Waardenburg syndrome: Radiological assessment and comparison with normative data

Objective

As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations.

Methods

A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values.

Results

Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others.

Conclusions

Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided.     

Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

Purpose

To evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.

Methods

We retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.

Results

Five consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).

Conclusion

Embolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.     

Arthroscopic treatment of recurrent acetabulum osteoid osteoma

In this case report, arthroscopic treatment of a recurrent osteoid osteoma in the posterior column of the pelvis extending to the acetabular fovea in a young adolescent is being presented. Level of evidence IV.     

Assessment of the skeletal status by MR relaxometry techniques of the lumbar spine: comparison with dual X-ray absorptiometry

Purpose: To measure lumbar spine T2*, T2, T2′ and T1 MR relaxometry parameters and compare them with lumbar spine bone mineral density (BMD) in a group of postmenopausal women. Materials and methods: Lumbar spine T2*, T2, T2′ and T1 MR relaxometry parameters and BMD values were assessed in 101 postmenopausal women (mean age: 61.8±7.1 (1 S.D.) years); of them 63 referred to as control subjects (group A, BMD T-scores ≥ −2.5 S.D.) and 38 as osteoporotic (group B, BMD T-scores < −2.5 S.D.). All magnetic resonance imaging (MRI) examinations were performed on an 1.5 T imaging system using: (a) a 2D single slice multi echo (32 echoes) gradient echo (MEGRE) sequence (TR/TE1/TE32/FA: 160/2.7/74.93 ms/25°) for the T2* measurement, (b) a respiratory gated 2D single slice Multi Echo (16 echoes) Spin Echo (MESE) sequence (TR/TE1/TE16/FA: 2000–2500/22.5/360 ms/90°) for the T2 measurement and (c) a 2D single slice multi TI (18 repeats) turbo Fast Low Angle Shot (turbo FLASH) sequence (TR/TE/TI1/TI16/FA: 11/4.2/10/5000 ms/10°) for the T1 measurement. T2′ was calculated from its definition equation: (1/T2′ = 1/T2* − 1/T2). Lumbar spine BMD was assessed using DXA. Results: All measured parameters showed statistically significant differences between groups A and B (from P<0.05 to <0.001). All parameters showed significant associations with subject’s age ranging from r=0.245 (P<0.05) for the T2 up to r=0.377 (P<0.001) for the T2*. All parameters showed significant associations with subject’s BMD measurements ranging from r=−0.184 (P<0.05) for the R1 = (1/T1) up to r=−0.345 (P<0.0005) for the T2. Conclusion: Among the MR relaxometry parameters studied, T2* and T2 showed better discrimination of patients with osteoporosis from control subjects.