The AMORE protocol as salvage treatment for non-orbital head and neck rhabdomyosarcoma in children.

AIM: To investigate the feasibility and outcome of the AMORE protocol as salvage treatment in paediatric head and neck rhabdomyosarcoma (HNRMS). METHODS: The AMORE protocol is a local treatment regimen, consisting of Ablative surgery, Moulage technique brachytherapy and surgical Reconstruction, scheduled in 1 week. Patients with recurrent or residual non-orbital HNRMS were eligible for AMORE salvage treatment. RESULTS: The procedure was feasible in nine out of 11 eligible patients. Five patients were treated for recurrent or residual parameningeal RMS after prior chemoradiation. Local complete remission was achieved in all five patients and maintained in four. Three patients are without evidence of RMS with a follow-up duration of 4-10 years. Two patients developed a distant relapse, together with a local recurrence in one. Both patients died of their disease. Four patients were included for recurrent non-parameningeal HNRMS. Long-term local control at the site of recurrence was obtained in all four patients (follow-up 5-10 years). CONCLUSIONS: The AMORE protocol is a feasible salvage strategy for non-orbital HNRMS even after external beam radiotherapy. The local salvage rate in this series is promising.

A novel local treatment strategy for advanced stage head and neck rhabdomyosarcomas in children: results of the AMORE protocol.

The AMORE protocol is a local treatment regimen for head and neck rhabdomyosarcomas (HNRMS), consisting of Ablative surgery, Moulage technique brachytherapy and surgical Reconstruction. The aim of AMORE is to intensify local treatment for children with HNRMS and to avoid external beam radiation therapy (EBRT) and its long-term sequelae. All children with primary irresectable, non-orbital HNRMS in whom EBRT was indicated, were evaluated for the feasibility of AMORE. In 20 children, AMORE was performed (15 with parameningeal disease and five with non-parameningeal disease). Complete remission was achieved in all 20 patients. Local complications were limited. 5 patients experienced a local relapse and 1 patient developed distant metastases. Estimated 5-year OS and EFS were 67.5 and 64.1% for the entire group, and 64.2 and 60.0% for the parameningeal subgroup. We conclude that the AMORE protocol is a feasible strategy, with a good local control rate. Long-term sequelae of EBRT might be avoided although, to date, the follow-up is too short for definitive conclusions regarding these sequelae.

Classification of cilio-inhibiting effects of nasal drugs.

OBJECTIVE/HYPOTHESIS: Nasal drug formulations are widely used for a local therapeutic effect, but are also used for systemic drug delivery. In the development of new nasal drugs, the toxic effects on the mucociliary clearance and therefore on the ciliated tissue is of importance. In this study, the effect of nasal drugs and their excipients on the ciliary beat frequency (CBF) is investigated. STUDY DESIGN: Experimental, in vitro. METHODS: CBF is measured by a photograph-electric registration method. Excised ciliated chicken trachea tissue is incubated for 15 minutes in the formulation, followed by a reversibility test. To estimate the ciliostatic potential, a classification is given of all tested formulations. According to the CBF, after 60 minutes every drug or excipient could be classified as follows: cilio-friendly: after 60 minutes the CBF has regained 75% or more of its initial frequency; cilio-inhibiting: after 60 minutes the CBF has regained between 25% and 75% of its initial frequency; or ciliostatic: after 60 minutes the CBF has regained 25% or less of its initial frequency. RESULTS: Most formulations used are cilio-friendly or cilio-inhibiting. Only some are ciliostatic. Preservatives have a major role in the cilio-inhibiting effect of the drug. Also, other additives can contribute to the toxicity profile of nasal drug formulations. CONCLUSION: This classification of the cilio-inhibiting potential of nasal drug formulations is a valuable tool in the design of safe nasal drugs. The number of animal studies in vivo can be reduced substantially by using this in vitro screening technique. This study demonstrates that the effect on ciliary movement of most drug formulations is due to the preservatives and/or additives and mostly not to the drug itself.

First results of the VoiceMaster prosthesis in three centres in the Netherlands.

The VoiceMaster indwelling voice prosthesis was designed and developed for use in laryngectomized patients. The VoiceMaster pre-production model (0-series), tested during 1997 and 1998, proved to be a safe and valid concept. After the refining of a few technical details the currently available VoiceMaster device was introduced in June 1998. The preliminary results obtained with the device were encouraging and led to a multicentre study. The average device life span varies between the participating clinics, ranging up to 4.8 months. As there still are devices in situ, the average life span is still increasing. Factors such as stoma size and fistula position influence the insertion of the device and should be considered before VoiceMaster insertion. The general experience with the VoiceMaster prosthesis has proven it to be a worthwhile new device in prosthetic voice rehabilitation in laryngectomized patients.

Lymphoma in the ear.

BACKGROUND: Malignant tumors of the ear are rare. The most common malignant tumors are squamous cell carcinomas and adenocarcinomas. Lymphoma in the ear is rare. METHODS: We report 2 cases of a primary presentation of a lymphoma of the ear. The literature since 1947 is reviewed. RESULTS: An 83-year-old woman with an anaplastic large cell lymphoma of the skin of the external auditory meatus and a 75-year-old man with a B-cell non-Hodgkin's lymphoma of the mastoid process are presented. The literature review shows that only 16 cases of lymphomas of the ear have been reported so far. CONCLUSIONS: In a case of therapy-resistant otitis and/or peripheral facial paralysis malignancy should be excluded by computer tomography of the mastoid. For histopathological diagnosis unfixed specimens are preferable. Uniform treatment of lymphomas of the ear has not been established.

Gastropharyngeal and gastroesophageal reflux in globus and hoarseness.

BACKGROUND: The role of gastropharyngeal reflux in patients with globus pharyngeus and hoarseness remains unclear. OBJECTIVE: To evaluate patients with complaints of globus, hoarseness, or globus and hoarseness combined for the presence of gastropharyngeal and gastroesophageal reflux. DESIGN: Prospective clinical cohort study of 3 groups of patients undergoing ambulatory 24-hour double-probe pH monitoring. In patients with pathologic gastroesophageal reflux, an upper gastrointestinal endoscopy was also performed. SETTING: Tertiary care, outpatient clinic. PATIENTS: Twenty-seven patients with globus alone, 20 patients with hoarseness alone, and 25 patients with globus and hoarseness combined. RESULTS: Patients with well-defined pathologic reflux (ie, gastroesophageal reflux with or without gastropharyngeal reflux) were present mainly in the group of patients with globus combined with hoarseness: 18 (72%) of 25 patients, compared with 7 (35%) of 20 patients with hoarseness alone and 8 (30%) of 27 with globus alone. Seven (10%) of all 72 patients had prolonged acid exposure at the laryngopharyngeal junction in the presence of a normal gastroesophageal pH registration. Abnormal findings in the esophagus were found at endoscopy in 17 (65%) of 26 patients with pathologic gastroesophageal reflux (with or without gastropharyngeal reflux). CONCLUSIONS: We found a high prevalence of pathologic reflux in patients with both globus and hoarseness. Based on these findings, we strongly advise upper gastrointestinal endoscopy for symptomatic otolaryngological patients with pathologic gastroesophageal reflux.

High incidence of laryngopharyngeal reflux in patients with head and neck cancer.

OBJECTIVES: Laryngopharyngeal reflux may play a role in the etiology of squamous cell cancer of the head and neck and contribute to complications in head and neck cancer patients after surgery or during radiotherapy. STUDY DESIGN: Prospective study. METHODS: To investigate the incidence of laryngopharyngeal and gastroesophageal reflux in patients with head and neck cancer, ambulatory 24-hour double-probe pH monitoring was performed in 24 untreated patients with laryngeal or pharyngeal squamous cell carcinoma. In addition, 10 patients who had been irradiated in the head and neck area were analyzed for reflux to study the effect of radiotherapy on reflux. RESULTS: Only 4 of the 24 head and neck cancer patients (17%) had neither pathological laryngopharyngeal nor gastroesophageal reflux. Esophageal acid exposure was abnormal in five patients and acid exposure at the level of the upper esophageal sphincter was abnormal in four patients. Eleven patients had pathological reflux in both areas. Irradiated patients did not differ from the untreated patients considering the incidence of pathological laryngopharyngeal or gastroesophageal reflux. CONCLUSIONS: The data obtained in this study indicate that reflux is a common event in head and neck cancer patients.

Microbial colonization of silicone voice prostheses used in laryngectomized patients.

The aim of this study was to identify the microbial colonization of dysfunctioning voice prostheses in laryngectomized patients and determine the influence of patient radiation therapy on prosthesis life span. In a 40-month period, 257 outpatient voice prosthesis replacements were carried out in a laryngectomized group of 31 patients. The voice prostheses were all removed from the tracheo-oesophageal fistula after dysfunctioning of the prosthesis. Of the replaced prostheses 183 were cultured. The microbial cultures showed a predominant colonization with Candida albicans and commensal oral microflora. Radiation therapy induced xerostomia shortened the lifetime of the first inserted prosthesis in particular.

Evidence for recurrent laryngeal nerve contribution in motor innervation of the human cricopharyngeal muscle.

OBJECTIVE: To study the functional motor nerve supply of the upper esophageal sphincter in humans. STUDY DESIGN: Intraoperative electromyographic study. METHODS: The contribution of the recurrent laryngeal nerve and the pharyngeal plexus in the motor nerve innervation of the cricopharyngeal muscle and the inferior pharyngeal constrictor muscle was examined intraoperatively. RESULTS: Electromyography showed that there is a considerable overlap in the innervation of the cricopharyngeal muscle and the inferior pharyngeal constrictor muscle. The recurrent laryngeal nerve functionally contributes to the motor innervation of the cricopharyngeal muscle in all patients and contributes to the motor innervation of the inferior pharyngeal constrictor muscle in most patients. The pharyngeal plexus functionally contributes to the motor innervation of the inferior pharyngeal constrictor muscle but does not always contribute to the motor innervation of the cricopharyngeal muscle. CONCLUSIONS: This is the first report which provides evidence that the recurrent laryngeal nerve functionally contributes to the motor innervation of the cricopharyngeal and inferior pharyngeal constrictor muscle. Furthermore, this study shows that intraoperative electromyography in humans is a feasible method to analyze the physiology of the motor innervation of the upper esophageal sphincter.

High incidence of gastropharyngeal and gastroesophageal reflux after total laryngectomy.

BACKGROUND: Gastroesophageal reflux (GER) appears to be related to laryngeal carcinoma. Little is known about GER and gastropharyngeal reflux (GPR) in the laryngectomized patient. Therefore, GER and GPR were studied in laryngectomized patients. METHODS: In 11 patients, 24-hour double-probe pH monitoring was performed in an ambulant setting. An optic fiberscope was used for the accurate positioning of the proximal probe in the upper esophageal sphincter. RESULTS: In 9 of 11 patients pathologic GPR was found. Four of these 9 patients had reflux in upright and supine position, 5 patients had reflux only in upright position. CONCLUSIONS: A high incidence of GPR in laryngectomized patients was found. These results raise the question whether all laryngectomized patients should be investigated for reflux and in the presence of pathologic reflux findings should be treated with reflux prophylaxis.

Bilateral stapedotomy in patients with otosclerosis: a disability-orientated evaluation of the benefit of second ear surgery.

This study reports the evaluation of the results of 80 stapedotomies in patients with bilateral otosclerosis. All pre- and postoperative audiological data, together with all relevant information of the operations, were stored in a database and analysed retrospectively. A new approach has been developed in order to evaluate the benefit of second ear stapes surgery in a more disability-orientated way using the AMA-criteria in the Guides to the Evaluation of Permanent Impairment. In all patients the percentage of Binaural Hearing Impairment (BHI) and the percentage of Impairment of the Whole Person (IWP) were determined according to the AMA-criteria. In patients who had both operations at the Academic Medical Centre it was found after the first operation that there was an important decrease in the BHI-percentage (from 26% to 10%) as well as for the IWP-percentage (from 9% to 4%). In addition, the percentages dropped significantly after the second operation (from 11% to 7% and from 4% to 2%, respectively). During follow-up there were no serious complications. It is concluded that bilateral stapedotomy is a safe procedure with good results.

New combined treatment of surgery, radiotherapy, and reconstruction in head and neck rhabdomyosarcoma in children: the AMORE protocol.

BACKGROUND: If no complete remission on chemotherapy is reached in head and neck rhabdomyosarcoma (RMS) in children, adjuvant radiotherapy and/or surgery are indicated. This often causes severe mutilation, and the prognosis is poor. Ablative surgery, moulage technique with afterloading brachytherapy, and reconstructive surgery, called the AMORE protocol, is developed for residual or recurrent disease after chemotherapy. METHODS: Ablative surgery with preservation of important structures is followed by the embedding of a gutta-percha mold in the created tissue defect. The wound bed is irradiated with Iridium192. Within 1 week, the defect is reconstructed with a muscle transplant. Fifteen children (mean age, 5.7 years) were treated accordingly. RESULTS: All patients were discharged within 4 weeks. Eleven patients are tumor-free, with a mean follow-up period of 2.8 years. No signs of radiation sequelae were observed. CONCLUSION: The preliminary results of this new, combined treatment are encouraging, if the advanced stage of disease is considered.

Airflow and pressure characteristics of three different tracheostoma valves.

Tracheoesophageal speakers can achieve speech without digital occlusion by using a tracheostoma valve. Laryngectomized patients who are successful with this device can regain considerable freedom. However, little is known about which valve suits the patient best. Valve aerodynamics may give a guideline for its use. Three major tracheostoma valves, each divided into four subtypes, were repeatedly measured in this study. Dynamic pressure and airflow rate signals were sampled through an analog-digital interface into a computer. Considerable aerodynamic differences were observed between the tested valves. The maximum airflow rates, closing pressures, and resistances at low velocities were compared. The presented data may help increase the successful use of tracheostoma valves in tracheoesophageal speakers. Patient factors and additional valve factors should always be taken into account. Further clinical study to validate the clinical relevance of the data is needed.

The VoiceMaster voice prosthesis for the laryngectomized patient.

Since the introduction and performance of total laryngectomy, voice restoration has become an important issue. Voice prostheses are widely used for this purpose. Based on experience with previous and current prostheses, the important characteristics for any voice prosthesis can be defined. These characteristics are: indwelling fixation, low resistance to airflow, less bothersome and easier insertion into the tracheoesophageal (TE) fistula. Also, predictable long device life span (resistance to Candida deterioration) and easy removal of the prosthesis to allow inspection or cleaning and consequent re-insertion are desirable. The VoiceMaster voice prosthesis was developed with these characteristics in mind. The VoiceMaster prosthesis is described in this paper. The first results from the preliminary study in which the currently available VoiceMaster was tested are very encouraging.

DNA-analysis is mandatory in case of an uncommon malignancy.

In a child a diagnosis of sweat gland carcinoma was made on basis of a surgical specimen presumed to be taken from an occipital lymph node. DNA analysis confirmed mixing of specimens in the referring hospital.

An efficiency comparison of four heat and moisture exchangers used in the laryngectomized patient.

Bypassing the upper airway places the burden of humidification on the lower airway. For this reason passive heat and moisture exchangers (HMEs) are used in the laryngectomized patient in an attempt to minimize the effect of lost upper airway function. We measured efficiency and airflow resistance and calculated the costs of four HMEs used in the laryngectomized patient. The HMEs were measured according a modified International Standards Organization (ISO) 9360 standard. The airflow resistance was measured at flow rates of 15, 30, and 60 L/min. The measurements were repeated three times. Costs were calculated with two realistic scenarios. The study found that there are significant differences in moisture output and airflow resistance between the HMEs tested. There are major daily cost differences between these devices. This study shows that filter material and size influence the HME's moisture output efficiency and airflow resistance considerably. The construction differences and filter and housing type have great influence on the HME's daily costs. We believe that knowledge of the efficiency in combination with the average daily costs of the HMEs allows the clinician to make a balanced choice of which filter to use.

Perineural tumor extension along the trigeminal nerve: magnetic resonance imaging findings.

OBJECTIVE: To evaluate the magnetic resonance imaging (MRI) findings of 15 patients with perineural tumor extension along the trigeminal nerve in correlation with clinical data. METHODS: The clinical records and MRI studies of 15 patients with perineural tumor extension along the trigeminal nerve were retrospectively reviewed. Imaging studies included plain and contrast-enhanced thin section T1-weighted spin echo (T1-WSE) MRI with and without fat-suppression. The studies were compared to determine which sequence provided greatest tumor conspicuity and best depiction of tumor extent. The conspicuity of these tumors was assessed on the available sequences by two observers by consensus. RESULTS: The contrast-enhanced T1-weighted spin echo fat-suppressed images (T1-WSECEFS) demonstrated greatest tumor conspicuity and best depiction of tumor extent in the extracranial head and neck and skull base region. The conventional T1-weighted spin echo pre- and postcontrast images were, however, diagnostic of perineural tumor extension in 11 patients due to the presence of considerable tumor bulk and extension well above the skull base. In the other four patients the perineural tumor was poorly visualized on the conventional T1-WSE images and well visualized on the fat-suppressed images. The mandibular division of the trigeminal nerve (V3) was most commonly involved (n = 10), followed by the maxillary (V2; n = 5) and ophthalmic (V1; n = 2) division. Two patients had both mandibular as well as maxillary nerve involvement. The finding of perineural tumor extension had significant impact on patient management: based on the MR imaging study, the primary tumor was considered inoperable (n = 13), the extent of surgery was expanded (n = 2) and radiation therapy (RT) ports were extended (n = 12). CONCLUSION: Complete trigeminal nerve imaging is recommended when evaluating (suspected) head and neck malignancies with a high risk for perineural extension. In these cases thin section axial and coronal precontrast T1-WSE MR images and postcontrast T1-WSE MR images with fat-suppression should be obtained. In the rare event that artifacts degrade the quality of the fat-suppressed images, contrast-enhanced T1-WSE sequences without fat-suppression can additionally be used.