Vocal fold hyalinosis in Urbach-Wiethe disease, a rare cause of hoarseness.

BACKGROUND: Lipoid proteinosis is an autosomal recessive disorder characterized by hyalin deposits in the skin and mucosa of the upper aerodigestive tract; currently, no treatment exists. Nearly all patients experience hoarseness and speech difficulties, due to hyalin deposition in the vocal folds and diminished mobility in infiltrated lips, tongue, and palate. METHODS: We describe a patient with extensive hyalin plaques on the vocal folds, which resulted in near-aphonic hoarseness. Hyalin deposits in the vocal folds and skin were treated with laser resection. RESULTS: Both the vocal folds and skin improved in appearance, with smoother surface epithelium. However, the patient's speech remained impaired, due to extensive hyalin plaques in the mouth, tongue, and lips. The voice improved only temporarily. CONCLUSIONS: Laser resection of hyalin plaques in the vocal folds and skin is a feasible treatment for lipoid proteinosis. However, speech may remain severely limited, due to impaired tongue and lip movement.

Surgical management of deep lobe parotid tumours with and without involvement of the parapharyngeal space.

The diagnosis and treatment of deep lobe parotid tumours is challenging because of the complex surgical accessibility. There is a lack of studies describing the differences between deep lobe tumours that do and do not occupy the parapharyngeal space (PPS). Patients treated for deep lobe tumours occupying the PPS (PPS group) and not occupying the PPS (non-PPS group) were analysed retrospectively. A total of 227 patients were treated surgically for deep lobe parotid tumours between 1990 and 2019. Sixty patients (26.4%) presented with tumours that involved the PPS (PPS group), while 167 (73.6%) presented with tumours that did not occupy the PPS (non-PPS group). The majority of the PPS group tumours were removed using a transcervical or transcervical-transparotid approach. PPS group tumours were larger (P < 0.001), and tumour spill occurred more frequently in this group (benign tumours: P = 0.002; malignant tumours: P = 0.033). Complication rates did not differ between the PPS and non-PPS groups. A transcervical or transcervical-transparotid approach is the preferred method for the management of deep lobe parotid tumours that occupy the PPS in our practice. Tumour spill occurred more frequently in the PPS group, which is most probably due to the larger tumour size and more complex accessibility.

Development of the Drooling Infants and Preschoolers Scale (DRIPS) and reference charts for monitoring saliva control in children aged 0-4 years.

OBJECTIVES: To develop and validate a parent questionnaire to quantify drooling severity and frequency in young children (the Drooling Infants and Preschoolers Scale - the DRIPS). To investigate development of saliva control in typically developing young children in the age of 0-4 years. To construct sex-specific reference charts presenting percentile curves for drooling plotted for age to monitor the development of saliva control in infancy and preschool age. STUDY DESIGN: The DRIPS was developed consisting of 20 items to identify severity and frequency of drooling during meaningful daily activities. Factor analysis was performed to test construct validity. A piecewise logistic regression was followed by a piecewise linear regression to construct sex-specific reference charts. RESULTS: We obtained 652 completed questionnaires from parents of typically developing children. The factor analysis revealed four discriminating components: drooling during Activities, Feeding, Non nutritive sucking, and Sleep. To illustrate the development of saliva control, eight sex-specific reference curves were constructed to plot the scores of the DRIPS by age group, at the 15th, 50th, 85th and 97th percentile. About 3-15% of the preschoolers in our cohort did not acquire full saliva control at the age of 4 years. CONCLUSIONS: With the DRIPS it is possible to validly compare and visualize the development of saliva control in an individual infant or preschooler and allow clinicians to timely initiate individually targeted interventions if children outperform.

[Diagnostic image (342). A woman with a fistula at the edge of the jaw]. / Diagnose in beeld (342). Een vrouw met een fistel aan de kaakrand.

A 17-year-old girl had a persistent ulcerating external submandibular fistula which radiographically originated from a periapical abscess of the left lower second molar.

The efficacy of hyperbaric oxygen therapy related to the clinical stage of osteoradionecrosis of the mandible.

This study aimed to evaluate the success of hyperbaric oxygen therapy (HBOT) and surgery in the treatment of mandibular osteoradionecrosis (ORN) in relation to the extent of the ORN. Twenty-seven patients with ORN were identified from a total of 509 patients with a history of primary oral or base of the tongue cancer; these patients had been treated with radiation therapy with curative intent between 1992 and 2006, with a radiation dose to the mandible of ≥50Gy. The ORN was staged according to the classification of Notani et al. The time from completion of radiation therapy to the development of ORN varied (median 3 years). Forty HBOT sessions were offered. After HBOT alone, 3 of 11 stage I lesions, 0 of 8 stage II lesions, and 0 of 8 stage III lesions had healed (P=0.0018). An absolute incidence of 5.3% ORN was found in this population. Of all sites irradiated in this study, the floor of the mouth was most associated with ORN (8.6%), whereas the cheek was least associated (0%). Based on the results of this study, HBOT can be recommended for stage I and II ORN and for selected cases of stage III ORN.

Effect of postoperative radiotherapy on the functional result of implants placed during ablative surgery for oral cancer.

The purpose of this retrospective study was to evaluate the survival of dental implants placed during ablative surgery in the interforaminal region of the original edentulous mandible in patients with squamous cell carcinoma of the oral cavity in relation to postoperative radiotherapy. Forty-eight patients treated in 1996-2003 with surgery alone or in combination with postoperative radiotherapy were analysed. In all patients, 2 to 4 Brånemark Mk II/III 2-phase implants were placed during tumour resection. A total of 139 implants were placed of which 61 (21 patients) received postoperative radiotherapy: 60-68 Gy as a boost dose on the primary tumour site and 10-68 Gy on the symphyseal area. No difference was found in percentage of functional dentures on implants between the radiated and non-radiated groups. The success rate of osseointegration was 97% in the postoperative irradiated group and 100% in the non-irradiated group. The prosthetic success rate (75%) was lower because in 12 of the 48 patients (34 implants) a functional denture could not be fitted due to tumour recurrence or metastasis (7 patients, 22 implants) or for psychological reasons (4 patients, 12 implants), independent of whether radiotherapy was administered. Postoperative radiotherapy does not affect the osseointegration of dental implants placed during tumour ablation and the ultimate number of functional dentures. Primary implant placement in edentulous mandibles may have advantages over secondary implant placement in patients with oral squamous cell carcinoma.

Compliance, quality of life and quantitative voice quality aspects of hands-free speech.

CONCLUSIONS: With the use of a new automatic stoma valve (ASV) it appears possible to rehabilitate patients who have previously been unsuccessful in acquiring hands-free speech. As well as making daily ASV use possible for an additional group of patients, this new device was also appreciated by many patients as an additional rehabilitation tool for specific occasions. Despite statistically significant improvements in aspects of voice and breathing using this novel ASV, improvement of peristomal adhesion is probably the main factor needed to further increase success rates. Nevertheless, our results show that it makes sense to keep trying to achieve hands-free speech, even if previous attempts have failed. OBJECTIVE: To make a long-term (6 months) assessment of compliance and aspects of voice, breathing and quality of life using a new ASV: the Provox FreeHands heat and moisture exchanger (HME). MATERIAL AND METHODS: This was a prospective clinical multicentre trial in 79 laryngectomized patients (8 regular ASV users, 58 previously unsuccessful users and 13 new users). Data were collected at baseline and after 1 and 6 months by means of European Organization for Research and Treatment of Cancer Quality of Life questionnaires and specific structured questionnaires concerning compliance, skin adhesion, voicing and pulmonary aspects. An objective assessment of voice parameters (maximum phonation time, maximum phonation time while counting, dynamic loudness range and number of pauses in a standard read-aloud text) was made for comparison of different stoma occlusion methods (digital occlusion via an HME and two different ASVs). A subjective assessment of overall voice quality was made. RESULTS: After 6 months, 19% of patients used the new ASV on a daily basis (mean 5 h/day), while 57% used it on an irregular basis as an additional rehabilitation tool for special occasions. Two-thirds of the study group indicated that they would continue to use the new ASV after the study period. With respect to the objective parameters, statistically significantly better maximum phonation times and dynamic loudness ranges were observed with the new ASV compared to the Blom-Singer ASV. However, the best results for all the objective parameters were obtained with digital occlusion via the Provox HME.

[Diagnostic image (223). A woman with a bleeding tongue]. / Diagnose in beeld (223). Een vrouw met een bloedende tong.

An 81-year-old woman suffered from bleeding of the tongue due to vascular malformations accompanying Rendu-Osler-Weber disease.

[Rendu-Osler-Weber disease]. / De ziekte van Rendu-Osler-Weber.

Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT) is a multisystem autosomal dominant hereditary disorder. The disorder is manifested by multiple dysplasia of blood vessels of the skin and mucous membranes. This results in recurrent and sometimes severe bleeding, of which epistaxis is the most common. Cardial, pulmonary and cerebral manifestations can be responsible for complications. A patient is presented with Rendu-Osler-Weber disease followed by a review of literature.

Patterns of proliferation related to vasculature in human head-and-neck carcinomas before and after transplantation in nude mice.

PURPOSE: The predictive potential of tumor cell kinetic parameters may be improved when they are studied in relation to other microenvironmental parameters. The purpose of this investigation was to quantitatively categorize human tumor samples according to proliferation patterns. Second, it was examined whether these characteristics are retained after xenotransplantation. METHODS AND MATERIALS: Fifty tumor samples from head-and-neck cancer patients were immunohistochemically stained for Ki-67 and vessels. Also, parts of the samples were transplanted into nude mice. Tumors were categorized according to previously described patterns of proliferation. Vascular and proliferation patterns were analyzed using an image processing system. RESULTS: The 50 tumors were categorized into four patterns of proliferation by visual assessment: marginal (6), intermediate (10), random (21), and mixed (12). One tumor could not be classified. These patterns were quantified by calculating the Ki-67 labeling index in distinct zones at increasing distance from vessels yielding good discrimination and significant differences between patterns. The probability of growth after xenotransplantation was significantly higher for tumors with a labeling index and vascular density above the median value compared to tumors with both parameters below the median (82% vs. 35%). Fifty percent of the tumors retained their proliferation patterns after xenotransplantation. CONCLUSION: The categorization by proliferation pattern previously described by others was reproduced quantitatively and spatially related to the vascular network using a computerized image processing system. The combination of quantitative and architectural information of multiple microenvironmental parameters adds a new dimension to the study of treatment resistance mechanisms. Tumor models representative of the various patterns can be used to further investigate the relevance of these architectural patterns.

Accelerated radiotherapy with carbogen and nicotinamide (ARCON) for laryngeal cancer.

BACKGROUND AND PURPOSE: Tumor hypoxia and tumor cell repopulation are known factors determining radiation response. Accelerated radiotherapy as a method to counteract cellular repopulation was combined with carbogen (95% O2 + 5% CO2) breathing and oral administration of nicotinamide as a means to improve tumor perfusion and oxygenation. The feasibility, toxicity and clinical effectiveness of this approach as a voice-preserving treatment for carcinoma of the larynx was assessed in a prospective study. PATIENTS AND METHODS: Sixty-two patients with stage III-IV laryngeal carcinoma were treated with a schedule of accelerated radiotherapy. The total radiation dose to the primary tumor was 64 Gy and that to the metastatic nodes was 68 Gy delivered in fractions of 2 Gy over 35-37 days. Radiotherapy was combined with carbogen breathing in the initial 11 patients and with both carbogen and nicotinamide administration in the subsequent 51 patients. RESULTS: After a median follow-up of 24 months, the actuarial local control rate at 2 years was 92%. This is higher than any previous report in the literature for this category of patients. Five patients had a local tumor recurrence and underwent laryngectomy. There was one regional recurrence. Including salvage surgery the loco-regional control rate was 100%. Four patients developed distant metastases and died. The actuarial overall survival rate at 2 years was 85%. Toxicity was increased relative to conventional radiotherapy but was considered as acceptable. One patient underwent laryngectomy for radiation-induced cartilage necrosis. CONCLUSION: These preliminary results indicate that advanced laryngeal cancer can be controlled in a high proportion of patients when treated with accelerated radiotherapy combined with carbogen and nicotinamide. This approach offers excellent possibilities for larynx preservation.

Supraomohyoid neck dissection with frozen section biopsy as a staging procedure in the clinically node-negative neck in carcinoma of the oral cavity.

A retrospective analysis was performed to evaluate the efficacy of elective supraomohyoid neck dissection in 57 newly diagnosed patients with squamous cell carcinoma of the oral cavity. The protocol included sampling of both the most suspicious and the largest node in the jugulodigastric region (if present) and the most distal jugulo-omohyoid lymph node (if present) for frozen section examination. In 10 cases, frozen section biopsy revealed metastatic disease, and surgery was continued using standard or modified radical neck dissection en bloc with the primary tumor. In another 10 cases, histologic examination of the supraomohyoid neck dissection specimens revealed occult nodal disease at other sites. In the histologically proven absence of metastatic disease in the supraomohyoid neck dissection specimens, disease recurrence in the neck occurred in only three cases (7%), all in the presence of local failure. The results of our analysis support the conclusion that elective supraomohyoid neck dissection with frozen section biopsy appears to be a valid staging procedure and a valuable approach to the management of the clinically node-negative neck in squamous cell carcinoma of the oral cavity.

The value of computed tomography scan versus an explorative surgical approach for removal of squamous cell carcinoma of the posterior pharyngeal wall.

BACKGROUND: The prevertebral fascia plays a key role in surgery of posterior pharyngeal wall tumors. Invasion of the prevertebral muscles determines the irresectability of the tumor and accurate diagnosis of invasion posteriorly is a prerequisite for major ablative surgery. METHODS: A retrospective study was performed to define the value of CT scanning versus open neck exploration with regard to the resectability of posterior pharyngeal wall carcinoma. The predictive value of preoperative CT scans was assessed and compared with the outcome of open neck exploration and resectability of the primary tumor, using the final histopathology report as a gold standard. RESULTS: Nineteen patients with 20 tumors were included in this study. Overall the CT scan was correct concerning prevertebral muscle status in 4 out of 20 (20%), whereas open neck exploration was correct in 18 tumors (90%). CONCLUSION: The predictive value of a suspicious CT scan in determining prevertebral muscle invasion is extremely low in this study. Open neck exploration seems to be superior for determining resectability of posterior wall carcinoma.

The guide wire dilating forceps technique of percutaneous tracheostomy.

BACKGROUND: Prospective evaluation of the percutaneous tracheostomy by the guide wire dilating forceps (GWDF) technique. METHODS: In 50 selected patients percutaneous tracheostomy with fiberscopic control was performed and evaluated. RESULTS: Most percutaneous tracheostomies were performed without any adverse effect. No life-threatening complications or deaths were related to the procedure. The procedure was successful in 49 of 50 patients (98%). In 1 patient the procedure was converted to an open tracheostomy because significant bleeding occurred. Five perioperative complications, including this significant bleeding and four minor complications, occurred in 50 patients (10%). Early complications occurred in 6 of 48 patients (13%), including one significant bleeding and five minor complications. A subglottic stenosis occurred in 2 of 36 successfully decannulated patients (6%). In one case this was certainly due to prolonged endotracheal intubation. CONCLUSIONS: The GWDF technique is a safe and efficient bedside alternative to open tracheostomy. Fiberscopic control is recommended to increase the safety of the procedure. Although studies of late complications are necessary, it appears to be justifiable to consider percutaneous tracheostomy for patients who require tracheostomy.

A surgical technique to prevent tracheostomal stenosis after laryngectomy.

Tracheostomal stenosis after total laryngectomy is a frequently occurring nuisance to both the patient and the surgeon. Although many causes may contribute to the development of tracheostomal stenosis, operative technique seems to play a major role. The authors describe and explain their method of stomal construction. Over a 10-year period they have used the described method on 260 patients undergoing a total laryngectomy, with an incidence of tracheostomal stenosis of 6%.

Analysis of the mechanical behavior of the Nijdam voice prosthesis.

The valveless Nijdam prosthesis is a new voice prosthesis for laryngectomized patients using tracheoesophageal speech. An "umbrella-like hat" covers the esophageal side of the tracheoesophageal fistula and is deformed during speech by air pressure. To decrease pressure loss during speech, a good understanding of the mechanical behavior is essential. In the present study, the Finite Element Method (FEM), used in engineering to analyze the mechanical behavior of complex structures, was applied to analyze eight possible improvements of the Nijdam prosthesis. This study found that, during speech, deformation of hat and soft tissue occur. Distinct differences in the hat's deformation of the eight models also were found. It is concluded that complex structures like the Nijdam prosthesis can be analyzed by FEM. An optimal model was found to decrease pressure loss while stresses in the device remain safe.

A decade of postlaryngectomy vocal rehabilitation in 318 patients: a single Institution's experience with consistent application of provox indwelling voice prostheses.

OBJECTIVE: To assess long-term results with consistent use of indwelling voice prostheses (Provox; Atos Medical AB, Hörby, Sweden) for vocal rehabilitation after total laryngectomy. DESIGN: Retrospective clinical analysis. SETTING: Comprehensive national cancer center. PATIENTS: Three hundred eighteen patients (261 men and 57 women; mean age, 62 years) from November 1988, through May 1999. INTERVENTION: Standard wide-field total laryngectomy (287 patients) or total laryngectomy with circumferential pharyngeal resection (31 patients), and 2700 prosthesis replacements. Prostheses remained in situ during 364,339 days (1000 patient-years). MAIN OUTCOME MEASURES: Device lifetime, indications for replacement (device or fistula related), adverse events, and voice quality. RESULTS: Median patient-device follow-up was 67 months. Mean actuarial device lifetime for all indications for replacement was 163 days (median, 89 days). Main indications for replacement were device-related, ie, leakage through the prosthesis (73%) and obstruction (4%), or fistula-related, ie, leakage around the prosthesis (13%), and hypertrophy and/or infection of the fistula (7%). Adverse events occurred in 11% of all replacements in one third of the patients, mostly solvable by a shrinkage period, or adequate sizing and/or antibiotic treatment. Definitive closure of the tracheoesophageal fistula tract occurred in 5% of the patients. Significant clinical factors for increased device lifetime were no radiotherapy (P =.03), and age older than 70 years (P<.02). Success rate with respect to voice quality (ie, fair to excellent rating) was 88%, which was significantly influenced by the extent of surgery (P<.001). CONCLUSION: The consistent use of indwelling voice prostheses shows a high success rate of prosthetic vocal rehabilitation, in terms of the percentage of long-term users (95%), and of a fair-to-excellent voice quality (88% of patients).