Phoniatric provision and training: current European perspectives.

Communication disorders represent a major and growing problem worldwide. In Europe, the specialty of phoniatrics has developed partly in response to this important issue. This article reviews training and workforce issues in phoniatrics and raises key questions and issues that need resolution in the future.

Keys to a successful cleft lip and palate team.

The severity and complexity of the defects in patients with cleft lip and/or palate (CLP) require a complex and long-term programme of care, aiming primarily at the best aesthetic and functional outcome, with a minimum of procedures and optimal cost-effectiveness. This delivery of comprehensive and coordinated health care to patients with CLP is optimally provided through collaboration in a specialised CLP team. There is international consensus about the fundamental elements of CLP treatment: multidisciplinary teamwork, centralisation, high-volume care, team continuity, long-term treatment planning from birth to adulthood, standardised protocols, documentation, evaluation, follow-up studies, research, training and quality assurance.

The Leuven staged supraperiosteal retropositioning repair: long-term velopharyngeal function in non-syndromic cleft palate.

BACKGROUND: From 1989 to 1993, 113 previously untreated patients were admitted to the Multi-disciplinary Cleft Lip and Palate Team of the University Hospitals Leuven. Palate repair was performed in our centre by one surgeon (FO) in 88 patients. Our current surgical technique consists of a single-stage supraperiosteal retropositioning (modified Veau-Wardill-Kilner) for patients with a soft cleft palate only (SCP) or a soft cleft palate with up to 1 cm of the hard palate (HSCPpa). Patients with a larger or complete cleft of the secondary hard palate (HSCP) and patients with unilateral (UCLP) or bilateral (BCLP) cleft lip and palate undergo two surgical stages for palate closure: a supra-periosteal retropositioning is performed around 12 months of age, and a modified Langenbeck closure of the hard palate around 60 months of age. AIM: To assess velopharyngeal function with speech as outcome measure. PATIENTS AND METHODS: Velopharyngeal function was assessed in two ways. In one assessment, a "hard" outcome measure was the number of patients undergoing pharyngoplasty following palate repair in our centre (n = 88). In the other assessment, velopharyngeal function was evaluated in a homogeneous sub-population of 44 non-syndromic cleft patients with normal to slight impairment of the following functions: mental development, language development, and hearing. In this group, prospectively collected data about hypernasality and nasal emission were analysed retrospectively using a semi-objective nasality index (NI). Articulation was evaluated using a subjective articulation index (AI) representing articulation errors (retro-articulation, glottal stops and facial grimacing) associated with velopharyngeal insufficiency (VPI). Mean follow-up was 114 months. RESULTS: Despite rigid assessment by a phoniatrician and speech pathologist, only 1 patient out of 88 patients with soft palate surgery in our institution was thought to need pharyngoplasty. In the sub-cohort of 44 non-syndromic patients, nobody needed a pharyngoplasty. In the latter cohort, at the age of about eight years, 27 patients (61.5%) had undetectable nasality, 13 patients (29.5%) had an NI of 1 or "mild" nasality, and 4 patients (9%) had moderate nasality. At this point in time, articulation errors associated with VPI were noted in 14% of patients. CONCLUSION: In this subgroup of cleft palate patients treated following the Leuven protocol, there was no need for secondary pharyngoplasty. Ninety-one per cent of patients had no, or only mild, rhinolalia aperta by the age of eight years, and 84% did not display VPI-related articulation disorders. This suggests that velopharyngeal function in patients treated by this protocol is excellent compared to results in the literature.

Gastroesophageal reflux in patients with laryngeal disorders.

INTRODUCTION AND AIM: The aim of this study is to use anamnesis and laryngeal examination to investigate the occurrence of the gastroesophageal reflux disease GERD (with symptoms of regurgitation and heartburn, signs of oesophagitis, and pathological pH monitoring) among patients with suspected laryngopharyngeal reflux. MATERIALS AND METHODS: One hundred and sixty-nine patients with laryngopharyngeal reflux indicated by anamnesis and laryngeal examination were evaluated retrospectively with endoscopy and 24-hour double-probe pH monitoring. RESULTS: Combining endoscopy and pH monitoring made possible a diagnosis of GERD in 130 patients (77%). Of these patients, 58% had erosive oesophagitis and 84% had pathological oesophageal acid exposure. Of the patients with suspected laryngopharyngeal reflux and with established GERD, 49% reported gastrointestinal symptoms like heartburn or acid regurgitation. Erosive oesophagitis was confirmed in 45% of patients: grade 1 in 60 (35%), grade 2 in 10 patients (6%), and grades 3 and 4 in 3 patients each (2%). Distal oesophageal pH monitoring was pathological in 65% of patients. Of these, 93% had upright pathological acid exposure. Proximal oesophageal acid exposure was pathological in 39% of patients. CONCLUSIONS: Reflux can be suspected when a patient presents typical laryngeal symptoms and signs. The majority of these patients will not have typical GERD symptoms like heartburn, and endoscopic oesophagitis, mostly grade 1, is present in only a subset of patients with LPR. Oesophageal pH monitoring is the most appropriate test to demonstrate pathological oesophageal acid exposure.

Systemic cidofovir in papillomatosis.

An 8-year-old patient with severe recurrent respiratory papillomatosis and pulmonary spread was treated by systemic administration of cidofovir in association with laser treatment for tracheal lesions. Complete disappearance of the lesions in the pharynx and larynx and a significant yet incomplete regression in the bronchi and lung parenchyma were observed without deleterious side effects. This is the first case report of systemic use of cidofovir to treat recurrent respiratory papillomatosis.

Treatment of severe laryngeal papillomatosis with intralesional injections of cidofovir [(S)-1-(3-hydroxy-2-phosphonylmethoxypropyl)cytosine].

Respiratory papillomatosis is a rare and often severe disease, usually localized in the larynx. It may cause respiratory distress and even life-threatening obstruction of the airways. Treatment is generally based on the evaporation of the lesions with a CO2 laser, but microsurgery, cytotoxic and/or cytostatic drugs, interferons, and vaccines are also used. Cidofovir [(S)-1-(3-hydroxy-2-phosphonylmethoxypropyl)cytosine] (HPMPC) was shown to suppress the growth of tumors induced by rabbit papillomavirus as well as human papillomavirus (HPV). The efficacy of cidofovir was assessed in 17 patients with severe respiratory papillomatosis. Cidofovir at a concentration of 2.5 mg/ml was injected directly in the different laryngeal papillomatous lesions during microlaryngoscopy under general anesthesia. Biopsies were taken before the treatment was started both for anatomopathology and viral typing. HPMPC kinetics in serum was monitored in three patients, the drug levels being determined by high-performance liquid chromatography. Complete disappearance of the papillomatosis was observed in 14 patients. Four patients relapsed and were successfully treated again with cidofovir. Of the three remaining patients, one progressed while under treatment with cidofovir, after an initial marked response. One patient had a partial remission and remained stable for more than 1 year after the last injection. He had a very aggressive and extensive disease originally. Finally, one patient was lost to follow-up after four injections. Intratumoral injections of cidofovir for the treatment of severe laryngeal papillomatosis is a powerful new therapeutic approach for this disease. Treatment was well tolerated, and no significant side effects were noted.

Temporary voice changes after uncomplicated thyroidectomy.

Voice characteristics were studied before and after thyroidectomy in patients with intact vocal fold motility. The speaking voice was acoustically analysed in 47 patients and phonetograms were made in 17 patients. Eight parameters were measured and the pre- and postoperative values compared. The results show that the most affected parameter was the pitch of the speaking voice. The fourth postoperative day there was, on average, a lower SFo and a smaller Fo range during speaking. Postoperatively a progressive normalisation took place. After three months there were no more statistical differences and, looking at the individual measures, the SF0 of all patients fell within 2 semitones from their preoperative level. Vocal quality was also altered in the first postoperative examination, as shown by the higher jitter and smaller harmonics. These measures normalised after two weeks. In the same way, the evaluation of the limits of the voice by means of the phonetogram, showed that the maximal performances in the intensity and pitch domain were decreased in the earliest postoperative period. Information about temporary voice change is useful in patients undergoing thyroidectomy.

Acoustic analysis of the speaking voice after thyroidectomy.

Voices of 47 female patients were analyzed before and after thyroidectomy, with preservation of the recurrent and superior laryngeal nerves and normal vocal fold motility during the observation period. A mean decrease of the speaking fundamental frequency (SFF) of 12 Hz was found on day 4; in 8 patients the postoperative vocal pitch was more than 2 semitones lower. The distance between the highest and lowest F0 during speaking was diminished (speech was more monotone) and the vocal jitter was elevated. In the frequency spectrum, there was a diminished prominence of the harmonics. The other spectral parameters (as the slope of the spectrum and the H1/H2 ratio) were unchanged. All changes had disappeared the fifteenth day, except for a lower SFF (> 2 semitones) in 2 cases. It is concluded that after normal dissection of the laryngeal nerves, and in the absence of vocal fold paresis, other reasons for voice changes immediately after thyroidectomy remain: alterations in the neck muscles, in the laryngeal mucosa, and in the patient's general condition. Although the effects seem limited and of short duration, knowledge of them is helpful when informing the patient before thyroid surgery.

CHOP-VP16 chemotherapy and involved field irradiation for high grade non-Hodgkin's lymphomas: a phase II multicentre study.

Sixty previously untreated patients with high grade non-Hodgkin's lymphomas stages II-IV received cyclophosphamide 750 mg m2 i.v., doxorubicin 50 mg m2 i.v., and vincristine 2 mg i.v. on day 1, prednisolone 100 mg p.o. on days 1-5 and etoposide 100 mg m2 i.v. on days 3-5 (CHOP-VP16). After four courses an involved field irradiation with a total dose of 25 Gy was employed and followed by two additional courses of CHOP-VP16. The overall response rate was 93%, with 49 patients (82%) achieving a complete remission (CR). Seven patients had a partial response and four patients showed no response. During a median follow-up period of 55 months, 22 of the 49 patients with CR relapsed, seven of them achieving a second complete remission with the same drug regimen. A maintained complete remission of up to 68 months was seen in 55% of all patients initially achieving CR. The median survival is 43 months. Mean side-effects of this drug regimen were alopecia (89%), nausea/vomiting (76%) and leukopenia (61%). No therapy-related deaths were seen. The results of this study demonstrate that this combined modality treatment produces high complete remission rates and that more than half of these patients achieve long-term disease-free survival.

[Velopharyngeal insufficiency. Anatomo-physiological aspects]. / Insuffisance vélo-pharyngienne. Aspects anatomo-physiologiques.

Palatopharyngeal insufficiency is an important syndrome in Phoniatrics and speech pathology. The anatomy, physiology and pathophysiology of the velopharyngeal mechanism, as well as the nature of the speech and associated problems are described. The chemical diagnostic and instrumental evaluations are briefly enumerated. Major emphasis is placed on the radiographic assessment. A scheme with the etiological categories in both congenital and acquired palatopharyngeal insufficiency is presented.

Total respiratory resistance and reactance in patients with upper airway obstruction.

In 18 patients with upper airway obstruction, we measured dynamic lung volumes, maximal flows, airway resistance (Raw), and total respiratory resistance (Rrs) and reactance (Xrs) at various frequencies by means of a forced oscillation method. Patients were classified according to the site and the type of the obstruction. The values of Raw, Rrs and Xrs were tightly correlated and reflected the overall importance of the obstruction. In comparison with patients with chronic obstructive pulmonary disease the values of Rrs tend to be higher and to be influenced more by support of the cheeks during the measurement. These differences are not diagnostic, however.

[Differential diagnosis of mediastinal tumor: malignant histiocytosis]. / Zur Differentialdiagnose des Mediastinaltumors: Die maligne Histiocytose.

On chest X-ray picture a mediastinal mass may be an indication for manifestation of malignant histiocytosis. The history of two own cases is detailed. More common radiological features are reticular or nodular opacities or other interstitial infiltrations, pleural effusions and circumscribed lymphnode enlargements.

[Phase II study of the water-soluble nitrosourea compound ACNU in advanced colorectal carcinomas]. / Phase-II-Studie des wasserlöslichen Nitrosoharnstoffes ACNU bei fortgeschrittenen kolorektalen Karzinomen.

In a phase-II study 46 patients with advanced colorectal cancer were treated with the new nitrosourea ACNU [1-(2-chloroethyl)-1-nitroso-3 (4-amino-2-methyl-5-pyrimidinyl)methyl-3-nitrosourea]. From 43 evaluable patients, 86% presented distant metastases and 14% an unresectable primary tumour or a recurrent tumour. 24 patients presented a colon and 19 a rectal cancer. Prior anticancer drug treatment was given to 34 patients (79%), 11 (26%) were pretreated with a nitrosourea. ACNU was administered every 4-6 weeks as a single intravenous push injection of 100 mg/m2. Most patients received 2-3 courses. From 43 evaluable patients, one patient achieved a complete and 3 a partial remission (CR + PR 9%). 5 patients reached a minimal regression (tumour regression of less than 50%) and 5 a no change for at least 2 months. The median duration (time from beginning of ACNU therapy until tumour progression) of the 14 responders was 132 days. The median survival time was significantly longer for responders in comparison to patients with progressive disease (9.8 versus 4.1 months). The dose limiting toxicity was delayed bone marrow suppression predominantly in the form of thrombocytopenia. 22/42 patients (52%) presented a thrombocytopenia of under 50.000/mm3 with a nadir after 27 days. Leucocytopenia under 2.000/mm3 were observed in 22/40 patients (30%). A fall of haemoglobin of more than 2 g/dl was seen in 71%. Nausea or vomiting over 1-2 days were found in 59% of the treatment courses. Other drug related side effects were not encountered. ACNU has a similar activity in colorectal cancer as BCNU and CCNU.(ABSTRACT TRUNCATED AT 250 WORDS)

[3 multicenter phase II studies of the treatment of small cell bronchogenic carcinoma (incl. AIO studies BI + BII)]. / Drei multizentrische Phase-II-Studien in der Behandlung des kleinzelligen Bronchialkarzinoms (incl. AIO-Studien BI + BII).

Between March 1979 und January 1983 122 patients with small-cell carcinoma of the lung were treated with different polychemotherapy regimen. 33 patients received VP-16-213 (etoposid), ifosfamide and vindesine (VPIV). 37 patients were treated with adriamycin, cisplatin and vincristine (APO). A third 3-drug combination, cyclophosphamide, methotrexate and CCNU (CMCC), was given to the remaining 52 patients alternating with the two other regimen. Response rates varied between 61% for the APO regimen and 85% for the VPIV regimen. The median survival was 10 months for patients treated with VPIV or APO and 12 months for patients treated with alternating drug combinations. This difference was not statistically significant. The rate of long-term survivors (longer than 2 years after diagnosis) was 9.9%. Side effects were seen more frequently in the group treated with APO.