[Persistent Postural-Perceptual Dizziness (PPPD) - A clinical case and essentials for the primary care medicine]. / Vertiges posturaux-perceptuels persistants (VPPP) - Cas clinique et notions essentielles pour le généraliste.

Persistent Postural-Perceptual Dizziness (PPPD) is a chronic functional disorder that manifests with symptoms of dizziness, unsteadiness or non-spinning vertigo that lasts for at least three months. These symptoms are exacerbated by upright posture, active or passive motion, and exposure to complex or moving visual stimuli. This -pathology has been known for a long time ago. Still, after an expert's consensus in 2017, this term was born, and some precise diagnostic criteria have been defined based on identifying key symptoms in the patient's clinical history. Treatment is multimodal, incorporating -vestibular rehabilitation, cognitive-behavior therapy, and serotonergic medication.

Les VPPP ou vertiges posturaux-perceptuels persistants (Persistent Postural-Perceptual Dizziness (PPPD)) sont une pathologie fonctionnelle chronique, caractérisée par une sensation de déséquilibre, d'instabilité ou de vertige non rotatoire. Les symptômes peuvent être exacerbés par la position debout, la mobilisation active ou passive et l'exposition à des stimuli visuels complexes ou en mouvement. Si la pathologie est connue de longue date, c'est suite à un consensus d'experts qu'est né ce terme en 2017, et que des critères diagnostiques clairs ont été définis, qui se basent sur l'identification de symptômes clés dans l'histoire ­clinique du patient. Le traitement est multimodal, ­comprenant une réhabilitation vestibulaire, des thérapies cognitivo-comportementales et une médication sérotoninergique.

Ciliary beating recovery in deficient human airway epithelial cells after lentivirus ex vivo gene therapy.

Primary Ciliary Dyskinesia is a heterogeneous genetic disease that is characterized by cilia dysfunction of the epithelial cells lining the respiratory tracts, resulting in recurrent respiratory tract infections. Despite lifelong physiological therapy and antibiotics, the lungs of affected patients are progressively destroyed, leading to respiratory insufficiency. Recessive mutations in Dynein Axonemal Intermediate chain type 1 (DNAI1) gene have been described in 10% of cases of Primary Ciliary Dyskinesia. Our goal was to restore normal ciliary beating in DNAI1-deficient human airway epithelial cells. A lentiviral vector based on Simian Immunodeficiency Virus pseudotyped with Vesicular Stomatitis Virus Glycoprotein was used to transduce cultured human airway epithelial cells with a cDNA of DNAI1 driven by the Elongation Factor 1 promoter. Transcription and translation of the transduced gene were tested by RT-PCR and western blot, respectively. Human airway epithelial cells that were DNAI1-deficient due to compound heterozygous mutations, and consequently had immotile cilia and no outer dynein arm, were transduced by the lentivirus. Cilia beating was recorded and electron microscopy of the cilia was performed. Transcription and translation of the transduced DNAI1 gene were detected in human cells treated with the lentivirus. In addition, immotile cilia recovered a normal beat and outer dynein arms reappeared. We demonstrated that it is possible to obtain a normalization of ciliary beat frequency of deficient human airway epithelial cells by using a lentivirus to transduce cells with the therapeutic gene. This preliminary step constitutes a conceptual proof that is indispensable in the perspective of Primary Ciliary Dyskinesia's in vivo gene therapy. This is the first time that recovery of cilia beating is demonstrated in this disease.

Management of tracheobronchial stenoses with endoprostheses: experience with 103 patients and 11 models.

OBJECTIVE: To describe in a retrospective study our experience in endoscopic management of tracheobronchial stenosis with 145 stents (11 different models). METHODS: We analyzed the medical records of 103 patients (67 males and 36 females) who underwent the placement of prosthesis for any causes of tracheobronchial stenoses between 1990 and 2005. RESULTS: A total of 145 prostheses were placed. Of these, 96.1% of the patients had a relief of dyspnea, and 73.8% had only one prosthesis. Stent removal and replacement were significantly linked with etiologies (more frequently in patients with an inflammatory component), but not with the type of stent. CONCLUSION: Endostenting is a safe and effective treatment for tracheobronchial stenoses. Removal and replacements were due to etiologies of the strictures, but not to the type of stent. Stenoses with an inflammatory component were prone to iterative stenting. Only benign diseases that are a contra-indication to open surgery should be treated by endoscopic stenting.

Hemorrhagic hereditary telangiectasia (Rendu-Osler disease) and infectious diseases: an underestimated association.

Among 353 patients with hereditary hemorrhagic telangiectasia retrospectively analyzed during the period 1985-2005, we identified 67 cases of severe infection that affected 48 patients (13.6%). Extracerebral infections accounted for 67% of all infections, and most involved Staphylococcus aureus and were associated with prolonged epistaxis. Cerebral infections accounted for 33% of all infections, were mainly due to multiple and anaerobic bacteria, and were associated with the presence of pulmonary arteriovenous malformations and a short duration of epistaxis.

Histologic structure and development of the laryngeal macula flava.

The laryngeal maculae flavae (MF) have been previously described as cellular condensations at the extremities of the vocal cords, but the exact nature and role of the MF are unknown. The purpose of this study is a histomorphological analysis of the nature and development of the MF in human vocal cords, from the beginning of fetal life to adulthood. Eighty-six larynges, from the fetus at 12 weeks of amenorrhea (WA) to adulthood, were collected from autopsies and studied by light microscopy with hematoxylin and eosin, orcein, and Alcian blue staining and pS 100 immunostaining. Our observations allowed us to separate the fetal development of the MF into 4 periods. The first corresponds to the appearance of the posterior MF between 13 and 15 WA. In the second period, the anterior MF appeared between 16 and 18 WA, allowing definition of the intermacular space. Fibrous structures, composed of collagen and elastic fibers, of the MF and intermacular space developed between 20 and 33 WA. The last period, from 33 WA to birth, corresponded to a maturation of these structures. In adults, the histologic structure of the MF presented a concentric peripheral fibrous layer associated with a central cellular component. Structural analysis of the MF concludes in favor of the chondroid nature of the macular cell. These observations bring into question the current knowledge on the MF. From these observations, the role of the MF in the developing vocal cord is discussed.

Thyroid alar cartilage graft in laryngoplasty: anatomical study in premature and newborn babies.

The aim of this prospective anatomical study was to determine the place of the thyroid alar cartilage graft (TAC) in the laryngotracheoplasty in a population of premature and newborn babies. Ninety-eight fresh larynges, collected from autopsies, were included in the study. After measurement of cartilage dimensions and thickness, the potential dimensions of TAC and its widening capacity were calculated for each specimen. The results showed that TAC thickness was similar to the anterior cricoid arch but was significantly less than the posterior cricoid arch. The maximal degree of stenosis which could be normalized by TAC ranged between 47.8 and 74.3%. The height of the TAC allowed a widening, from the level of the glottis to the inferior part of the second tracheal ring in 100% of cases and to the inferior part of the third tracheal ring in more than 70% of cases. In conclusion, the TAC could be used for anterior grafting in laryngotracheoplasty. Its indications should be limited to grade II and a few grade III of the Myer subglottic stenosis classification without glottic or tracheal extension. Moreover, variability of TAC dimensions leads to peroperatively reassess its indication.

Diagnosis of a multicentric extranodal nasal-type natural killer T-cell lymphoma made with an anterior chamber tap.

PURPOSE: To report the case of an unusual multicentric Epstein-Barr virus (EBV)-associated extranodal nasal-type natural killer T-cell lymphoma masquerading as unilateral panuveitis, diagnosed with an anterior chamber tap. METHODS: The clinical history and the morphological, immunohistochemical, and molecular features of a 51-year-old white man with left severe panuveitis were retrospectively evaluated. RESULTS: The patient initially presented with a 3-month history of recalcitrant sinusitis and new onset of unilateral loss of vision. Clinical examination revealed right peripheral facial nerve palsy, severe panuveitis of the left eye, and nasopharyngeal obstruction. Anterior chamber aspirates were examined. The combination of the presence of small- to intermediate-size lymphocyte proliferation, moderate elevation of the interleukin-10 level on cytokine profiling, and slightly positive polymerase chain reaction for EBV in the aqueous humor indicated an EBV-induced nasal-type natural killer T-cell lymphoma. Transnasal biopsy revealed the presence of numerous irregular lymphoma cells with positive staining for CD3, CD56, EBV-encoded RNA in situ hybridization, and negative staining for CD4, CD8, and CD1a. Lumbar puncture, cerebral magnetic resonance imaging, thoracoabdominal computed tomography, and upper digestive tract endoscopy revealed meningeal, renal, adrenal, and digestive involvement. Massive hemorrhage of the upper digestive tract caused rapid death. CONCLUSION: Extranodal nasal-type natural killer T-cell lymphoma is a very uncommon disease that may present acutely, sometimes as pseudouveitis. Simple investigations such as anterior chamber aspirates for cytological examination, reinforced by cytokine profiling and viral polymerase chain reaction looking for EBV, may provide a rapid diagnosis, necessary given the poor prognosis of the disease. To our knowledge, and after extensive review of the literature, we did not find another case report diagnosing this entity by anterior chamber paracentesis.

Cartilaginous tumors of the larynx: endoscopic laser management using YAG/KTP.

BACKGROUND: The objective of this study was to analyze results of endoscopic YAG laser management as a treatment of chondroma and/or low-grade laryngeal chondrosarcoma. METHODS: Between 1996 and 2006, 13 patients were included, 11 men and 2 women with a median age of 69.7 years at diagnosis. Primary treatment included endoscopic laser resection in all cases. All tumors were diagnosed as cricoid cartilaginous tumor by histopathologic examination: 6 chondromas and 7 chondrosarcomas grade 1. RESULTS: The global actuarial survival rate was 95% for 5 years. The 5-year Kaplan-Meier disease-free survival and overall recurrence rate estimates were 72% and 54%, respectively. With a median follow-up time of 5 years, 12 of 13 patients were without recurrence. Nine were alive and 4 died during follow-up, and only 1 patient died from disease. Three patients required definitive tracheotomy. None had required it for endoscopic laser complications. CONCLUSION: Laser endoscopic surgery is 1 of the conservative surgical ways to manage laryngeal chondroma especially in cases of elderly patients with poor health status. It permits adequate removal of the tumor and still maintains structural and functional integrity and preserves possibilities of subtotal or total salvage surgery.

Primary cancer of the sphenoid sinus--a GETTEC study.

BACKGROUND: Primary involvement of the sphenoid sinus occurs in 2% of all paranasal sinus tumors and is associated with dismal prognosis. Optimal management remains debatable. METHODS: A total of 23 patients were treated for a primary cancer of the sphenoid sinus from 1988 to 2004. Charts were reviewed for patient-, tumor-, and treatment-related parameters. Univariate and multivariate analyses were conducted to identify prognostic factors for locoregional control and survival. RESULTS: Cranial neuropathies were present in 12 patients. Pathologic findings included adenoid cystic carcinoma, adenocarcinoma, lymphoma, squamous cell carcinoma, sarcoma, neuroendocrine carcinoma, melanoma, and malignant hemangiopericytoma. All but 2 patients had stages III to IV cancer. Radiotherapy was performed in 18 patients and chemotherapy in 12. Of 10 patients undergoing surgery, total excision with grossly negative margins was achieved in 4 patients and subtotal resection in 6. Median locoregional control and overall survival were 12 and 41 months, respectively. On multivariate analysis, cranial neuropathy was associated with worse locoregional control and survival. Surgery was rarely complete because of advanced stages at presentation, but it yielded better outcomes than other treatments without surgery in non lymphoma-cases. CONCLUSION: Early CT and MRI should be performed when facing aspecific, rhinological, or neuro-ophtalmological symptoms. Cranial neuropathies indicate a worse prognosis. Surgery, including debulking surgery, may be preferred to combined modality treatments without surgery. Its apparently favorable impact on prognosis would need to be tested in homogenous histological groups of patients, which is impossible because of the rarity of the disease. Highly conformal radiotherapy (adjuvant or definitive) should be encouraged and optimized with concurrent chemotherapy in advanced stages. Aggressive multidisciplinary management including surgery, chemotherapy, and radiotherapy should be encouraged and adapted on histology and tumor extensions. Progress is still warranted to improve outcomes.

Determination of endotracheal tube size in a perinatal population: an anatomical and experimental study.

BACKGROUND: This study aimed at correlating anatomical dimensions of the larynx and trachea to age and weight in a prenatal population. Endotracheal tube size determination was then proposed according to these considerations. METHODS: Anatomical measurements were obtained during 150 fetal and infant postmortem examinations. The optimal endotracheal tube size was determined by three methods: clinically, by a pressure method using calibrated inextensible balloons, and anatomically by comparing the laryngotracheal perimeter to the tube perimeters. Based on these results, recommended tube sizes were calculated. RESULTS: In premature babies before 37 weeks gestation, the optimal tube size according to pressure estimation was significantly greater than that determined by anatomical measurement alone. This difference was no longer valid after 40 weeks gestation. CONCLUSIONS: This study identified the elasticity of laryngeal structures in premature babies, allowing intubation with tube sizes greater than predicted by anatomical measurements with an increasing injury risk located in the posterior part of the glottic plane. This elasticity disappears near 40 weeks gestation, and the injury risk then predominates in the subglottic region. These results lead the authors to recommend that the size of the endotracheal tube used in the perinatal population should be based on anatomical and experimental data to limit the injury risks.