[Children with encephalopathy : the ear, nose and throat perspective]. / Enfants avec encéphalopathie : le point de vue de l'ORL.

Children with encephalopathy are at increased risk of swallowing disorders with aspirations, food blockages, difficulties, and stress during meals. Serious consequences may result, such as recurrent lung infections and failure to thrive. The ENT or phoniatric examination includes the evaluation of food bolus management, possible drooling, and swallowing safety. This article explains the role of the ENT in the management of these children, with a description of the examinations to be carried out for a global evaluation of swallowing and aspirations and the treatments proposed for a better management of food intake by mouth and chronic drooling.

Les enfants avec encéphalopathie sont davantage à risque de présenter des troubles de la déglutition avec fausses routes, blocages alimentaires, difficultés et stress pendant les repas. Des conséquences graves peuvent en résulter, telles que des infections pulmonaires répétitives et un défaut de croissance staturo-pondérale. L'examen ORL ou phoniatrique comporte l'évaluation de la gestion du bol alimentaire, d'un éventuel bavage et de la sécurité de la déglutition. Cet article explique le rôle de l'ORL dans la prise en charge de ces enfants, avec une description des examens à effectuer pour une évaluation globale de la déglutition et des fausses routes ainsi que les traitements proposés pour une meilleure gestion de la prise alimentaire par la bouche et du bavage chronique.

Oral function in amyotrophic lateral sclerosis patients: A matched case-control study.

BACKGROUND & AIMS: Patients with amyotrophic lateral sclerosis (ALS) develop swallowing difficulties with the progression of the disease. The present study aimed at comparing oral function and body composition between ALS patients and healthy controls, and at evaluating which parameters are the most discriminant between both groups. METHODS: We included ALS patients at the start of their multidisciplinary follow-up at the Geneva University Hospitals and healthy age-, gender-, and dental status-matched adults. We assessed the severity of the disease through the ALS Functional Rating Scale and the swallowing difficulties through the EAT-10 score. We performed an intraoral examination of the dental status, and measured chewing performance, bite, lip and tongue force, saliva weight, and body composition. Group comparisons were performed with t-tests or Mann-Whitney tests as appropriate. Linear discriminant analysis was used to determine the most discriminant parameters between groups. RESULTS: Twenty-six ALS patients (bulbar onset: n = 7, median (IQR) ALS Functional Rating Scale: 37 (11)) were included. The ALS patients had a significantly lower chewing performance (p < 0.001), lip force (p < 0.001), tongue force (p = 0.002), saliva weight (p < 0.004) and fat-free mass index (p < 0.001) as compared to the healthy individuals, and a higher EAT-10 score (p < 0.001). In ALS patients, a low chewing performance was correlated with a low bite (r = -0.45, p < 0.05)) and tongue force (r = -0.59, p < 0.05). The most discriminant parameters between both groups, by order of importance, were chewing performance, fat-free mass index and saliva weight and allowed the calculation of a discriminant function. CONCLUSION: Compared to healthy controls, ALS patients have significant alterations of oral function and body composition. The most discriminant parameters between both groups were chewing performance, fat-free mass index and saliva volume. It remains to be demonstrated whether oral parameters predict outcome. CLINICAL TRIAL REGISTRY: clinicaltrials.gov, identifier: NCT01772888.

Early advance care planning in amyotrophic lateral sclerosis patients: results of a systematic intervention by a palliative care team in a multidisciplinary management programme - a 4-year cohort study.

INTRODUCTION: Although recommended, the implementation of early advance care planning is suboptimal in amyotrophic lateral sclerosis (ALS) patients. Barriers to advance care planning include healthcare professionals’ and patients’ reluctance, and uncertainty about the right time to initiate a discussion. AIM OF THE STUDY: To determine how often advance care planning was initiated, and the content of the discussion in a first routine palliative care consultation integrated within a multidisciplinary management programme. METHODS: Between June 2012 and September 2016, a prospective cohort study was conducted in Geneva University Hospitals. Sixty-eight patients were seen every 3 months for a 1-day clinical evaluation in a day care centre. RESULTS: The patients’ mean ± standard deviation age was 68.6 ± 11.9 years, 50% were women. Four patients were excluded because of dementia. Advance care planning was initiated with 49 (77%) patients in the first palliative care consultation. Interventions most often addressed were cardiopulmonary resuscitation (49%), intubation and tracheostomy (47%) and palliative sedation (36.7%). Assisted suicide was discussed with 16 patients (36.6%). Functional disability was the only factor associated with initiation of advance care planning. Nearly half of the patients wrote advance directives (45%) or designated a healthcare surrogate (41%). Bulbar onset, functional disability and noninvasive ventilation were not associated with the completion of advance directives. CONCLUSION: Early initiation of advance care planning is feasible in most ALS patients during a routine consultation, and relevant treatment issues can be discussed. All ALS patients should be offered the opportunity to write advance directives as completion was not associated with disease severity. .

Multidisciplinary care in amyotrophic lateral sclerosis: a 4-year longitudinal observational study.

Over a four-year period, ALS patients complied with the modalities of the multidisciplinary management follow-up without any drop-outs. The multidisciplinary management structure also contributes to increasing the experience and knowledge of the clinicians involved in managing patients suffering from this rare disease.

Assessing Inspiratory Muscle Strength for Early Detection of Respiratory Failure in Motor Neuron Disease: Should We Use MIP, SNIP, or Both?

BACKGROUND: Motor neuron disease (MND) invariably impacts on inspiratory muscle strength leading to respiratory failure. Regular assessment of sniff nasal inspiratory pressure (SNIP) and/or maximal mouth inspiratory pressure (MIP) contributes to early detection of a requirement for ventilatory support. OBJECTIVES: The aim of this study was to compare the feasibility, agreement, and performance of both tests in MND. METHODS: Patients with MND followed by a multidisciplinary consultation were prospectively included. Pulmonary follow-up included forced expiratory volumes, vital capacity (VC) seated and supine, MIP, SNIP, pulse oximetry, and daytime arterial blood gases. RESULTS: A total of 61 patients were included. SNIP and MIP could not be performed in 14 (21%) subjects; 74% of the subjects showed a decrease in MIP or SNIP at inclusion versus 31% for VC. Correlation between MIP and SNIP (Pearson's rho: 0.68, p < 0.001) was moderate, with a non-significant bias in favor of SNIP (3.6 cm H2O) and wide limits of agreement (-34 to 41 cm H2O). Results were similar in "bulbar" versus "non-bulbar" patients. At different proposed cut-off values for identifying patients at risk of respiratory failure, the agreement between MIP and SNIP (64-79%) and kappa values (0.29-0.53) was moderate. CONCLUSIONS: MIP and SNIP were equally feasible. There was no significant bias in favor of either test, but a considerable disparity in results between tests, suggesting that use of both tests is warranted to screen for early detection of patients at risk of respiratory failure and avoid over diagnoses. SNIP, MIP, and VC all follow a relatively linear downhill course with a steeper slope for "bulbar" versus "non-bulbar" patients.

Prevalence of bifid uvula in primary school children.

BACKGROUND: A bifid uvula is an anatomic variation that can be predictive of sub-mucous cleft palate, which may cause velopharyngeal insufficiency (VPI). Bifid uvula prevalence in the literature ranges from 0.18% to 10.3%, depending on the population studied. The aim of this study is to determine the prevalence of bifid uvula in the Geneva's school children population. METHODS: A cross-sectional study was conducted in Geneva's primary school children, from September 2014 to June 2015. An examination of the uvula was performed by dentists working for the Scholastic Dental Service, after a specific training in diagnosing bifid uvulas. The dentists recorded their findings on a standardized form. RESULTS: The total number of school children in Geneva in the school year 2014-2015 was 30,375. 23,961 children had their uvula examined, representing 79% of the total population of school children. Among them, a hundred school children had a cleft uvula. One schoolgirl had no uvula. The prevalence of bifid uvula is 0.42%. Sex ratio (M/F) is 0.96. DISCUSSION: This large study, the second in literature for number of patients examined, identified a prevalence of bifid uvula of 0.42%. This result is in agreement with previous studies.

Bamboo Nodes of Vocal Folds: A Description of 10 Cases and Review of the Literature.

OBJECTIVE: Bamboo nodes are vocal fold lesions, mostly associated with autoimmune diseases. PATIENTS AND METHODS: This is a retrospective clinical study including 10 patients with bamboo nodes. Data were collected regarding associated autoimmune disorder and type of treatment. A systematic review of the literature was conducted. RESULTS: All patients were women, with hoarseness as the most frequent symptom. There was in most cases an associated autoimmune disease: 3 patients with systemic lupus erythematosus; 3 with rheumatoid arthritis; 1 with Sjögren syndrome; 1 with Hashimoto disease; and 1 with mixed connective tissue disease. Four patients were treated with speech therapy, 3 with oral steroids, 1 with speech therapy and oral steroids combined, 1 with oral steroids and laryngeal steroid injections, and 1 had oral steroids, surgery, and speech therapy. Speech therapy was the first-line treatment. CONCLUSION: Bamboo nodes should be looked for in every patient with a diagnosis of autoimmune disease complaining of dysphonia.

Modification of the Surgical Strategy for the Dissection of the Recurrent Laryngeal Nerve Using Continuous Intraoperative Nerve Monitoring.

BACKGROUND: The aim of this study was to describe first experiences and changes in management using continuous intraoperative neuromonitoring (C-IONM) in thyroid and parathyroid surgery. METHOD: Retrospective analysis of patients who underwent surgery with C-IONM since 2012. Surgical maneuvers were modified when electrophysiologic events occurred. Patients with persistent loss of signal (LOS) underwent postoperative laryngoscopy. RESULTS: One hundred and one patients (of 1586 neck surgeries) were included and 19 had events: In 13 these were temporary (resolved before end of surgery) and led to intraoperative modifications in surgical approach; in all cases traction was released, and in 8, recurrent laryngeal nerve (RLN) approach was changed [superior approach (2), inferior approach (2), both (4)]. Six patients had persistent LOS (5.9%, present at end of procedure), with RLN palsy (RLNP) on postoperative day 1: In three, LOS occurred at electrode placement on the vagus nerve, leading to distal placement of the electrode allowing ipsilateral dissection under continuous monitoring; all three had complete recovery at 6 months. In the three other patients, LOS occurred on the RLN: one probable thermal, one traction lesion and one accidental section of the anterior RLN branch. The RLN recovered within 6 months in two patients, and in the third, RLNP persisted after 6 months (1/101 = 1%). CONCLUSION: C-IONM provides real-time evaluation of the RLN function, allowing for adaptation of surgical maneuvers to prevent RLNP. It seems particularly useful in difficult cases like redo neck surgery, invasive thyroid cancer and intrathoracic or large goiter. Care should be given at electrode placement on the vagus nerve.

Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement.

A 73-year-old woman was treated 8 years previously for synchronous breast and uterine neoplasms. She presented with a severe sore throat, odynophagia, dysphonia, dyspnoea, ocular irritation and weight loss over the last 3 months. Physical examination revealed ulcerations in the oral cavity, posterior pharyngeal wall and supraglottic larynx, nasal crusting, bilateral conjunctivitis and three cutaneous blisters. A diagnosis of anti-laminin 5 mucous membrane pemphigoid was retained, based on skin biopsy, direct immunofluorescence and immunoprecipitation. A positron emission tomography (PET)-CT detected multiple adenopathies. Cytology revealed adenocarcinoma with an immunocytology compatible with a breast origin and this was considered as a late metastatic recurrence of her previous breast cancer. A treatment of prednisone, dapsone and hormonotherapy was introduced, but intravenous immunoglobulin and rituximab were added due to new mucosal lesions. Despite treatment, a posterior laryngeal scar and bilateral symblepharon were developed. After 3 years, the patient is still alive and reports a satisfactory quality of life.

[Velopharyngeal insufficiency in children]. / Insuffisance vélo-pharyngée chez l'enfant.

Velopharyngeal insufficiency (VPI) represents an incomplete closure between the soft palate and the posterior pharyngeal wall. Its etiology can be anatomical (cleft palate), neurologic, or iatrogenic (after adenoidectomy). The evaluation of a VPI begins with a through speech and language assessment and can be complemented by instrumental investigations. VPI treatment relies on its early identification, followed by a specific speech therapy management. Surgery is performed in case of no improvement with speech therapy or in case of an anatomical defect not allowing the child to improve. IVP management requires a multidisciplinary team.

L'insuffisance vélo-pharyngée (IVP) désigne un défaut d'occlusion entre le voile du palais et la paroi postérieure du pharynx. Son étiologie peut être d'ordres anatomique (fente palatine), neurologique ou iatrogène (adénoïdectomie). L'évaluation de l'IVP débute par un bilan phoniatrique et orthophonique détaillé et peut être complétée par des explorations instrumentales. Son traitement repose sur une identification précoce puis une prise en charge et un suivi orthophonique spécifiques. Une chirurgie est réalisée en cas d'absence d'amélioration ou de défaut anatomique ne permettant pas à l'enfant de progresser. L'IVP est une pathologie intéressant plus particulièrement l'enfant et sa prise en charge se fait par une équipe multidisciplinaire.

Rhinopharyngeal autologous fat injection for treatment of velopharyngeal insufficiency in patients with cleft palate.

Patients with cleft palate are prone to velopharyngeal insufficiency. In minor cases or when hypernasal speech does not resolve after velopharyngoplasty, an augmentation pharyngoplasty with autologous fat can be proposed. The aim of the present study is to evaluate the short-term (within 2 months) and long-term efficiency (during the 24 months following the procedure) of our procedure in the setting of velopharyngeal insufficiency related to a cleft palate. Twenty-two patients with cleft palate related velopharyngeal insufficiency were included in this retrospective study. All patients were operated following the same technique, in the same institution. The pre- and postoperative evaluations included a nasometry, a subjective evaluation using the Borel-Maisonny score, and a nasofibroscopy to assess the degree of velopharyngeal closure. Scores of Borel-Maisonny and nasometry were compared before, shortly after the procedure (within 2 months) and long term after the procedure (within 24 months). Forty-one procedures in 22 patients with a cleft palate performed in our institution between October 2004 and January 2012 were included in the study. Nine patients had a previous velopharyngoplasty with persistent rhinolalia despite intensive speech therapy. In 14 patients the procedure was repeated because of recurrent hypernasal speech after the first injection. The average number of procedures per patient was 1.8. Postoperative nasometry and Borel-Maisonny scores were statistically significantly improved and remained stable until the end of the follow-up (median 42 months postoperative) in most patients. Complications were rare and minor. Autologous fat injection is a simple procedure for treatment of minor velopharyngeal insufficiencies in patients with cleft palate, with good long-term results and few complications.

Imaging of laryngeal trauma.

External laryngeal trauma is a rare but potentially life-threatening situation in the acutely injured patient. Trauma mechanism and magnitude, maximum focus of the applied force, and patient related factors, such as age and ossification of the laryngeal cartilages influence the spectrum of observed injuries. Their correct diagnosis and prompt management are paramount in order to avoid patient death or long-term impairment of breathing, swallowing and speaking. The current review provides a comprehensive approach to the radiologic interpretation of imaging studies performed in patients with suspected laryngeal injury. It describes the key anatomic structures that are relevant in laryngeal trauma and discusses the clinical role of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the acute emergency situation. The added value of two-dimensional multiplanar reconstructions (2D MPR), three-dimensional volume rendering (3D VR) and virtual endoscopy (VE) for the non-invasive evaluation of laryngeal injuries and for treatment planning is discussed. The clinical presentation, biomechanics of injury, diagnostic pitfalls and pearls, common and uncommon findings are reviewed with emphasis of fracture patterns, involvement of laryngeal joints, intra- and extralaryngeal soft tissue injuries, and complications seen in the acute emergency situation. The radiologic appearance of common and less common long-term sequelae, as well as treatment options are equally addressed.

Is tracheostomy still an option in amyotrophic lateral sclerosis? Reflections of a multidisciplinary work group.

QUESTION UNDER STUDY: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with a poor prognosis. Survival and quality of life of ALS patients have improved through the implementation of multidisciplinary approaches, the use of percutaneous gastrostomy and of noninvasive (NIV) or invasive ventilation. The question of whether or not to propose invasive ventilation (by tracheostomy: TPPV) to ALS patients remains a matter of debate. METHODS: The study reviews the medical literature, the practice in three Swiss and two large French ALS expert centres and reports the results of a workgroup on invasive ventilation in ALS. RESULTS: Improved management of secretions and use of different interfaces allows NIV to be used 24-hours-a-day for prolonged periods, thus avoiding TPPV in many cases. TPPV is frequently initiated in emergency situations with lack of prior informed consent. TPPV appears associated with a lesser quality of life and a higher risk of institutionalisation than NIV. The high burden placed on caregivers who manage ALS patients is a major problem with a clear impact on their quality of life. CONCLUSIONS: Current practice in Switzerland and France tends to discourage the use of TPPV in ALS. Fear of a "locked-in syndrome", the high burden placed on caregivers, and unmasking cognitive disorders occurring in the evolution of ALS are some of the caveats when considering TPPV. Most decisions about TPPV are taken in emergency situations in the absence of advance directives. One exception is that of young motivated patients with predominantly bulbar disease who "fail" NIV.

Pharyngolaryngeal involvement by varicella-zoster virus.

INTRODUCTION: Involvement of cranial nerves V, VII, and VIII by varicella-zoster virus (VZV) is widely reported in the literature, whereas involvement of cranial nerves IX and X is rarer and therefore poorly characterized. MATERIAL AND METHODS: We performed a systematic review of the literature through MEDLINE (up to January 2012). We selected cases reporting pharyngolaryngeal involvement by VZV and extracted clinical features, complementary studies, treatments, and outcome. We added three cases to the existing literature. RESULTS: Of the 65 screened articles, 38 were included reporting 54 cases. The main clinical features were odynodysphagia and dysphonia reflecting underlying hemipharyngolaryngeal palsy. Vesicles were seen in 66% of the patients. Besides the involvement of cranial nerves IX and X, concomitant involvement of other cranial nerves was seen in 48% of the cases. The most concerned nerves were cranial nerves VII and VIII. Virological tests (63%) and imaging (28%) were performed, with the latter being systematically normal. Seventy-two percent of patients were treated with antiviral agents and/or corticosteroids. Twenty-six percent of patients made a full recovery while the remaining had some persistent deficits. We did not find statistically significant differences in outcomes according to age or treatments received. CONCLUSIONS: Pharyngolaryngeal involvement by VZV is rare and seldom restricted to the ninth and tenth cranial nerves. It occurs mostly within the context of cranial polyneuropathy. Regardless of the treatment, full recovery is rare and long-term sequelae persist in many cases, especially with speech and swallowing impairment. Close monitoring and follow-up are therefore essential.

Acute isolated velopharyngeal insufficiency in children: case report and systematic review of the literature.

Acute isolated velopharyngeal insufficiency (VPI) is a clinical entity mainly reported in children. We undertook a systematic review in order to better characterize its features. Following a Medline search (1960-2012), the authors reviewed and analyzed the cases of acute VPI in children; 36 cases were found. The most common presenting features were hypernasal speech (97 %), nasal reflux (73 %), and dysphagia (49 %). 73 % of the children were males and 27 % females, of 8.9 ± 2.5 years. In all the cases the VPI was unilateral. One quarter of the children had a recent episode of febrile illness and 11 % of the children had an identified infection at the time of presentation (HAV, parvovirus B19, measles, and Coxsackie virus). No associated cause was found in the other cases. All cases resolved completely (67 %) or partially (33 %) without any treatment (89 %) or with prednisolone (11 %). Acute VPI represents a separate entity within the spectrum of VPI and it is a benign self-limiting disorder. The cause remains undetermined but an infectious disorder may play a role at least in some cases. Follow-up is mandatory in order to eliminate progressive conditions such as brainstem neoplasms or inflammatory diseases.