Urbach-Wiethe disease in a young woman: A case report.

Urbach-Wiethe disease (lipoid proteinosis) is an autosomal recessive disorder that is characterized by a general thickening of the skin and mucous membranes. We report the case of a 22-year-old woman with lipoid proteinosis who presented with hoarseness, poor dentition, and skin lesions, and we discuss the management of this rare disease.

Cervical hemorrhage due to spontaneous rupture of the superior thyroid artery: Case report and review of the literature.

BACKGROUND: Beneath the different reasons for cervical masses, a spontaneous hemorrhage presents a rare and life-threatening condition. METHODS AND RESULTS: We present the rare case of a 62-year-old man who was presented with a dramatically enlarging cervical mass causing respiratory distress because of upper airway compression. An endotracheal intubation was lifesaving and avoided tracheotomy. A CT scan revealed a hematoma in the region of the left external carotid artery. An emergency angiography embolized a ruptured branch of the superior thyroid artery and surgery evacuated the hematoma. We discuss the rarity of the condition, reasons for a spontaneous rupture of the artery, and the diagnostic and treatment strategy. In addition, we review the literature on spontaneous thyroid artery hemorrhages, which, up to now, have been described only for the inferior thyroid artery. CONCLUSION: We conclude that the optimal management for cases of cervical hematoma is intubation, diagnosis, and angiography before surgery.

An analysis of surgical complications, morbidity, and cost calculation in patients undergoing multimodal treatment for operable oropharyngeal carcinoma.

OBJECTIVE/HYPOTHESIS: Tumor control and survival are considered the most important measures of treatment efficacy for patients with primary oropharyngeal squamous cell carcinoma. Furthermore, multimodal treatment protocols should be judged by their complication rates, morbidity, and therapy costs. STUDY DESIGN: The results of a combined approach of primary surgery and neck dissection with postoperative radio(chemo)therapy were analyzed in retrospective chart review. METHODS: Two hundred eleven patients' records were analyzed for surgical complications, therapeutic morbidity, and treatment costs. RESULTS: The rate of postoperative hemorrhage was 4.7%. We observed no fatal complications. Ten percent of our patients required nutrition through percutaneous endoscopic gastrostomy (PEG). Twelve percent of all patients required long-term tracheostomy. The rates of PEG and tracheostomy were significantly higher in patients operated by the transcervical approach. The costs for the combined approach ranged from 10,587 euros (13,377 dollars) to 24,531 euros (30,996 dollars). CONCLUSIONS: The presented multimodal approach provides a low rate of surgical complications and a tolerable morbidity. Considering the excellent oncologic results, this extensive and more cost-intensive multimodal approach is justified for patients with oropharyngeal cancer.

Lymphatic malformations of the head and neck: introduction of a disease score for children, Cologne Disease Score (CDS).

OBJECTIVE: A clinical staging system for children with lymphatic malformations that would allow for a standardized comparison of disease and treatment outcomes. METHODS: We developed an examination sheet for the determination of a disease score ranging from 0 (worst) points to 10 (best) points, Cologne Disease Score (CDS). Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement towards progression were contributing to CDS. Each parameter yielded two, one or zero points. Two points were given when no limitation was seen in the patient concerning the respective item. One point was given at mild limitation and zero points were given when considerable limitation in the respective item could be observed. We evaluated 26 patients with lymphatic malformations of the head and neck retrospectively by completing one examination sheet for each patient-visit. RESULTS: Four patients had an initial CDS value of three or less points (severe disease-group). After therapy the mean value of their score increased only slightly to 3.9+/-2.6 points. Six patients had an initial CDS value of four, five or six points (advanced disease-group). After treatment their mean score value increased dramatically to 9.0+/-1.5 points (P<0.001). Sixteen patients initially had seven points or higher (moderate disease-group), they had 9.8+/-0.4 points after treatment. CONCLUSIONS: The evaluation of the CDS was easy and expeditious. The score itself was a good predictor in view of the outcome. This new disease score for paediatric patients might be appropriate to evaluate therapeutic trials in paediatric patients.

Proinflammatory effects of Staphylococcus aureus exotoxin B on nasal epithelial cells.

OBJECTIVE: The aim of this study was to evaluate the effects of Staphylococcus aureus exotoxin B (SE-B) on proinflammatory cytokine/chemokine releases in primary nasal epithelial cell cultures (NECC) of subjects with and without chronic rhinosinusitis (CRS). STUDY DESIGN AND SETTING: NECC (CRS: n = 14; CONTROLS: n = 11) were stimulated with SE-B. Protein concentrations of interleukin-(IL)-1beta, IL-6, and IL-8 levels were measured in NECC supernatants by ELISA before (T0) and after 24 hr stimulation with SE-B (T1). RESULTS: T0: supernatants of the NECC of CRS patients contained significant lower levels of IL-8 (2.1 ng/ml) compared to CONTROLS (IL-8: 6.2 ng/ml; P < 0.01). T1: SE-B induced a significant increase of IL-6 in NECC (P < 0.001). IL-1beta was not detectable. CONCLUSIONS: This is the first study evaluating the effects of exotoxins on NECC. SE-B showed proinflammatory effects on NECC. SIGNIFICANCE: Our data suggest that resident NECC are involved in immunological responses to Staphylococcus aureus toxins, supplementing the so-called "superantigen hypothesis" in CRS.

Nasal colonization with Staphylococcus aureus is not associated with the severity of symptoms or the extent of the disease in chronic rhinosinusitis.

OBJECTIVES: There is an increasing knowledge that the severity of perennial allergic rhinitis is associated with nasal carriage of Staphylococcus aureus (S. aureus). The aim of this study was to evaluate the rate of bacterial colonization with S. aureus in the nose of subjects with and without chronic rhinosinusitis (CRS) and to correlate these findings with the severity of symptoms and the extent of the disease. STUDY DESIGN AND SETTING: Open, prospective controlled trial. METHODS: 190 subjects with CRS and 42 subjects with septal deviation without sinusitis (control subjects) were included in this study. Swabs were taken endoscopically from the middle meatus and bacteria were cultured and identified. Airway symptoms were assessed by subjects in standardized questionnaires and frequencies of respiratory tract infections were noted. The rhinosinusitis extent was graded by CT scan assessment. Analysis of variance, chi-square test, and Pearson's correlation test were applied for statistical analyses. RESULTS: The S. aureus carriage rate was 25.5% in CRS and 31.4% in control subjects. Further facultative pathogens were cultured in 20.6% of subjects with CRS and in 8.5% of controls. 73.8% of S. aureus were ampicillin-resistant, multiresistant strains were cultured in 5.8%. Most airway symptoms and the frequencies of respiratory tract infections were significantly higher in the CRS group compared with control subjects. In post hoc comparison between the subgroups with and without S. aureus colonization, no significant differences were found between the extent of rhinosinusitis and the severity of airway symptoms. CONCLUSIONS: Facultative pathogens were detected in more than 40% of CRS patients without acute respiratory tract infections. In contrast to the findings in atopic dermatitis and perennial allergic rhinitis, the colonization rate with S. aureus is not increased and S. aureus carriage is not a risk factor for the severity or the extent of rhinosinusitis.

Primary parotid malignoma surgery in patients with normal preoperative facial nerve function: outcome and long-term postoperative facial nerve function.

OBJECTIVES/HYPOTHESIS: Most patients with primary parotid cancer present with normal facial nerve function. The common surgical strategy for these patients is to perform a parotidectomy with facial nerve preservation. Nevertheless, the functional outcome for the facial nerve and oncological outcome is unclear. STUDY DESIGN: Medical records of 211 patients treated from 1986 to 2000 in an university hospital were reviewed. One hundred seven patients with primary surgery for primary parotid cancer and long-term follow-up were analyzed retrospectively. METHODS: The characteristics, treatment, and oncological outcome were evaluated using hospital chart data. All patients with postoperative facial paresis were had follow-up with electromyography until recovery or permanent paresis became apparent. RESULTS: Ninety-one patients had a normal preoperative function. Facial nerve preservation during surgery by total parotidectomy was possible in 79 patients, whereas in 28 patients a radical parotidectomy was necessary. Otherwise, the oncological characteristics of both groups were not different. Directly after total parotidectomy, half of the patients presented a facial paresis but only two patients (2%) developed a permanent partial paresis. The 5-year disease-free rate and the 5- and 10-year survival rates were 65%, 83%, and 54%, respectively. After radical parotidectomy, the results were not significantly different. The 5-year disease-free rate and the overall 5- and the 10-year survival rates were 56%, 62%, and 42%, respectively. CONCLUSION: Treatment of primary parotid cancer with preoperatively normal facial nerve function by standardized parotidectomy and precise microsurgical preservation of the facial nerve is often possible. This approach demonstrates favorable oncological results with a low level of long-term facial nerve morbidity.

Impact of functional endoscopic sinus surgery on symptoms and quality of life in chronic rhinosinusitis.

OBJECTIVES: Chronic rhinosinusitis restricts the quality of life of millions of involved patients. The aim of the study was to evaluate how functional endoscopic sinus surgery modifies patients symptom profiles and quality of life. STUDY DESIGN: Open prospective clinical trial. METHODS: Questionnaires were given to 279 patients included in the series, who underwent sinus surgery at the Department of Otorhinolaryngology--Head and Neck Surgery, University of Cologne (Cologne, Germany) from 1995 to 1999. Patients assessed typical chronic rhinosinusitis--associated symptoms and restricted quality of life preoperatively and postoperatively using ranking scales (scales ranging from no to intolerable complaints). Statistical analyses were performed with the Wilcoxon test and Spearman rank correlation coefficient. RESULTS: Quality of life was restricted by chronic rhinosinusitis in 94% of all patients preoperatively and ranked as severe or intolerable in 74%. Leading symptoms of chronic rhinosinusitis were nasal obstruction in 92% and postnasal drip in 87%. Furthermore, patients reported dry upper respiratory tract syndrome in 68%, hyposmia in 66%, headache in 64%, and asthmatic complaints in 34%. After a mean postoperative follow-up of 31.7 months, an amelioration of quality of life was achieved in 85%, no change in 12%, and a deterioration in 3%. The ranking of restricted quality of life improved from "severe" to "mild" (P <.01) in the mean. Mainly responsible for this improvement was the postoperative decrease of nasal obstruction (84%), headache (82%), and postnasal drip (78%) (all P <.01), which correlated significantly with nasal obstruction (r = 0.59), headache (r = 0.39), and postnasal drip (r = 0.55), respectively (all P <.01) with better quality of life. CONCLUSIONS: The leading complaints within the symptom profile of patients with chronic rhinosinusitis are airway obstruction and postnasal drip. The restriction of quality of life in patients with chronic rhinosinusitis is mainly caused by these symptoms, which can be improved in excellent fashion by functional endoscopic sinus surgery in the majority of patients, achieving better quality of life in the long term.