Stepwise approach towards adoption of allergen immunotherapy for allergic rhinitis and asthma patients in daily practice in Belgium: a BelSACI-Abeforcal-EUFOREA statement.

Allergic rhinitis (AR) affects 23-30% of the European population with equal prevalence reported in Belgium. Despite guidelines on the correct use of effective treatment, up to 40% of AR patients remain uncontrolled. Allergen immunotherapy (AIT) has been shown to improve the level of control up to 84% of patients being controlled by AIT. Recently, new guidelines for AIT have been published, supporting the clinical evidence for effectiveness of various subcutaneous and sublingual products for AIT in patients who are allergic to airborne allergens. AIT in AR patients not only reduces nasal and/or ocular symptoms but also induces tolerance and has preventive potential. Adoption of AIT into daily clinical practice in Belgium and other European countries is hampered primarily by reimbursement issues of each of the single products but also by several patient- and physician-related factors. Patients need to be better informed about the effectiveness of AIT and the different routes of administration of AIT. Physicians dealing with AR patients should inform patients on tolerance-inducing effects of AIT and are in the need of a harmonized and practical guide that supports them in selecting eligible patients for AIT, in choosing evidence-based AIT products and in following treatment protocols with proven efficacy. Therefore, a stepwise and holistic approach is needed for better adoption of AIT in the real-life setting in Belgium.

mySinusitisCoach: patient empowerment in chronic rhinosinusitis using mobile technology.

Mobile health technology is emerging to take a prominent position in the management of chronic diseases. These technologies aim at enhancing patient empowerment via education and self-management. To date, of all the different apps available for patients with sinus disease, none were developed by medical experts dealing with chronic rhinosinusitis (CRS). The European Forum for Research and Education in Allergy and Airway diseases (EUFOREA) has undertaken a multi-stakeholder approach for designing, developing and implementing a tool to support CRS patients in monitoring their symptoms and to provide patients with a digital support platform containing reliable medical information about their disease and treatment options. mySinusitisCoach has been developed by medical experts dealing with CRS in close collaboration with patients, primary care physicians and community pharmacists, meeting the needs of both patients and health care providers. From a research perspective, the generation of real life data will help to validate clinical studies, patient stratification and improve understanding of the socio-economic impact of CRS, thereby paving the way for better treatment strategies.

Transoral robotic surgery for parapharyngeal lesions: a case series of four benign tumours.

OBJECTIVES: The parapharyngeal space (PPS) is an anatomically complex space in the vicinity of vital structures. With the introduction of the daVinci robot in head and neck surgery, the surgical robotic system is now being used to gain direct access to the parapharyngeal space and to excise the tumors endoscopically. This study evaluates the outcomes of four patients with benign PPS tumors treated with a transoral robotic surgery approach in a single centre. MATERIAL AND METHODS: All patients with benign tumors of the PPS who underwent transoral resection (between January 2012 and June 2014) using the robot were included in this retrospective study. RESULTS: The study population comprised of two males and two females with a mean age of 52 (range 34-66 years). The parapharyngeal mass was successfully transorally removed in all cases. Overall, mean length of stay was 3.25 days with mean time to oral diet of one day. No intraoperative, perioperative or postoperative complications were encountered. The histological diagnosis was pleomorphic adenoma in two cases (50%). The other two cases were: schwannoma and angioma. There were no recurrences on radiological investigations during a mean follow-up of 14.5 months. MRI scan showed a stable residual fibrotic lesion in case of the angioma. The preoperative complaints of mucus in the throat, painless swelling of the soft palate or throat burden of all patients resolved after surgery. CONCLUSION: With the assistance of the surgical robotic system, benign tumors within the PPS can be excised safely without neck incisions. Further long-term evaluation is needed to define patient selection and the role of TORS for PPS neoplasms.

Type III frontal sinusotomy: surgical technique, indications, outcomes, a multi-university retrospective study of 120 cases.

Draf in 1991. The procedure--which is also known as the modified endoscopic Lothrop procedure--aims to create the largest possible anteroposterior and lateral to lateral opening between both frontal sinuses and the nasal cavities. This requires the resection of the medial floor of both frontal sinuses, the intersinus septum and the superior nasal septum. The authors present a retrospective study including a cohort of 120 patients who underwent surgery in six Belgian university ENT departments. Mean follow-up was 24.6 months (range: 5-36 months). This paper describes the surgical procedure and reviews the indications, comorbidities, outcomes and complications of the type III frontal sinusotomy. Some correlations are also established with the data published in the worldwide literature. The authors conclude that the Draf III is a demanding procedure requiring considerable expertise in endoscopic sinus surgery. The procedure is effective with a success rate of 87.5%. Indeed, 12.5% of patients only experienced closure of the neoostium while 20% of all the patients had unchanged or worse symptomatology. The percentage of post-operative complications is 7.5%. All complications were managed successfully.

Why do I continue to perform vestibular osteotomies?

The rhinoplasty surgeon can choose from different osteotomy techniques. Historically, when changing the bony contour of the nasal pyramid, more attention has been devoted to refining approach to osteotomy techniques and adapting the instruments used. The importance of less damage to the soft tissues led rhinology surgeons to develop more refined osteotomy instruments. The author gives an overview of historical developments leading to our current array of techniques for addressing specific demands about aesthetic and functional outcome. A brief outline is given of the advantages, drawbacks and pitfalls of different osteotomy techniques.

Genetic evidence for a role of IL33 in nasal polyposis.

BACKGROUND: Little is known about the genetic factors that contribute to nasal polyposis (NP). A genome-wide association study identified 10 single nucleotide polymorphisms (SNPs) associated with eosinophilia. As eosinophils play a key role in the pathogenesis of NP, we assessed if any of these SNPs contribute to genetic susceptibility of NP. METHODS: We recruited 284 patients with NP in four participating hospitals in Belgium and 427 healthy controls, and genotyped 10 SNPs affecting eosinophilia (rs1420101 in IL1RL1, rs12619285 in IKZF2, rs4431128 in GATA2, rs4143832 in IL5, rs3184504 in SH2B3, rs2416257 in WDR36, rs2269426 in MHC, rs9494145 in MYB, rs748065 in GFRA2, and rs3939286 in IL33) using MALDI-TOF. A two-stage design was used while correcting for multiple testing. RESULTS: First stage analysis, involving 150 NP patients and 250 controls, identified rs3939286 nearby IL33 as a susceptibility factor for NP. Per at-risk A-allele, rs3939286 increased the risk for NP with an odds ratio (OR) of 1.60 (95% CI = 1.16-2.22; P = 0.0041). Second stage replication analysis in another 123 NP patients and 165 controls confirmed this association (OR = 1.43; CI = 1.00-2.06; P = 0.046). The combined analysis of both stages revealed an OR of 1.53 (CI = 1.21-1.96; P = 0.00041). Given the association of IL33 with NP, we also investigated rs1420101 in IL1RL1, which is the receptor for IL33. Although rs1420101 itself failed to associate with NP, a combined risk assessment of rs3939286 and rs1420101 further increased the risk for NP. CONCLUSION: We provide unprecedented genetic evidence suggesting a role for the IL33 pathway in the pathogenesis of NP.

Reparative granuloma related to perilymphatic fistula.

Reparative granuloma is defined as an 'exaggeration of the normal reparative process' (Schuknecht) after stapes surgery, often resulting in a destruction of the labyrinth. It is ascribed to a foreign body reaction, yet there are insufficient histological data to prove this and although rare, the problem is still with us. We encountered two patients in whom the history contains elements evoking a perilymphatic fistula. Cerebrospinal fluid causes irritation of the middle ear mucosa. This is demonstrated in an exemplary way in case of a spontaneously developed cerebrospinal fluid leak from a defect in the tegmen tympani. The leak itself may be very small, but is almost always surrounded by a large mass of granulation tissue. Since the chemical composition of perilymph and cerebrospinal fluid is known to be identical, a persistent perilymphatic leak might likewise be responsible for the formation of a granuloma in the middle ear, eventually invading and destroying the labyrinth. Laboratory experiments to investigate this hypothesis are recommended.

Eosinophilic fungal rhinosinusitis (EFRS): a distinct CT/MRI-entity? A European experience.

OBJECTIVE: To determine the value of radiological features in the diagnosis of Eosinophilic Fungal Rhinosinusitis (EFRS). STUDY DESIGN: Retrospective review of the radiological materials of 65 patients with documented Eosinophilic Fungal Rhinosinusitis treated at the same institution. METHODS: Evaluation by the ENT surgeon and the head and neck radiologist. RESULTS: EFRS was more common in female patients in this series. Fifty-four (83%) patients were above 30 years of age, with a peak of 18 patients (27.7%) in the seventh decade. All the patients except one (98.5%) showed bilateral mucosal thickening on unenhanced CT scans. Thirty-eight patients (58%) showed increased intrasinus attenuation on unenhanced CT scans. Thirty-seven patients (57%) showed opacification of at least one sinus; 25 (38%) showed osteitis; 11 (17%) had erosion of the sinus wall and only one patient showed minor expansion of an involved sinus. In 6 patients, typical hyperattenuation patterns on CT scans, together with distinctive MRI images, were highly suggestive of EFRS. CONCLUSION: Our data show that hyperattenuation on CT images with bone window settings suggests the presence of EFRS. This hyperattenuation is more clearly seen with soft-tissue window settings. When necessary, adjunctive MRI can provide information which might be highly predictive for the diagnosis of EFRS. However, non-specific imaging findings of chronic rhinosinusitis (CRS) should also be seen as possible EFRS pathology.

Varicella zoster virus: beyond facial paralysis.

J. Ramsay Hunt's hypothesis that herpes zoster oticus results from a reactivation of the herpes zoster virus in the geniculate ganglion, has been supported by the demonstration of varicella zoster viral DNA in the geniculate ganglion of the side with facial paralysis in patients with Ramsay Hunt syndrome, with the use of the polymerase chain reaction. Similarly, DNA of the varicella zoster virus has been identified in the spiral and vestibular ganglion as well. We report on three patients with cochleovestibular symptoms as the first manifestations of Ramsay Hunt syndrome. A 64-year old woman and a 72-year old man presented with vertigo and an auricular herpetiform eruption. Only the woman developed later on a mild facial paralysis. A 58-year old man presented with an acute cochleovestibular syndrome, serologically proven to be a varicella zoster viral reactivation, which was followed three weeks later by the typical cutaneous recrudescence. We believe that these cases result from reactivation of latent varicella zoster virus in the spiral and/or vestibular ganglion. As the varicella zoster virus is dormant in the non-neuronal satellite cells, the facial symptoms in our patients as well as the high incidence of cochleovestibular symptoms in classical Ramsay Hunt syndrome can be explained by viral transmission across the nerves inside the internal auditory canal. Therefore, we think there are grounds to recommend a prompt treatment with an antiviral and a corticosteroid agent, not only in case of an acute facial paralysis but also when confronted with an acute cochleovestibular syndrome.

Ramsay Hunt syndrome: pathophysiology of cochleovestibular symptoms.

Ramsay Hunt's hypothesis that herpes zoster oticus results from reactivation of the varicella zoster virus (VZV) in the geniculate ganglion is supported by the detection of viral genome in archival temporal bones of normals and Ramsay Hunt patients by the polymerase chain reaction. Ramsay Hunt syndrome is characterized by the presence of cochleovestibular symptoms in association with facial paralysis. VZV has also been demonstrated in the spiral and/or vestibular ganglion. Two cases are reported in which cochleovestibular symptoms outweighed the facial nerve symptoms, presumably representing VZV reactivation in the spiral and/or vestibular ganglion. From these observations and the known dormancy of VZV in non-neuronal satellite cells, it is argued that the cochleovestibular symptoms in Ramsay Hunt syndrome may result from VZV transmission across the nerves inside the internal auditory canal and that prompt treatment with an antiviral-corticosteroid combination might be justified in the management of any acute non-hydropic cochleovestibular syndrome.

Symmetrical ethmoidal metastases from ductal carcinoma of the breast, suggesting transcribrosal spread.

Symmetrical ethmoidal metastases from ductal carcinoma of the breast, suggesting transcribrosal spread. While half of breast cancers develop metastases, the appearance of metastatic disease in paranasal sinuses from this origin is very rare. Eighteen other cases were found in the literature, dating from 1939 till now. A case of metastatic breastcancer presenting as a subacute therapy-resistant pansinusitis is described. The perfect symmetry was misleading. Bilateral ethmoidal biopsies were compatible with metastases from a ductal adenocarcinoma. Further investigation revealed meningeal carcinomatosis in the supra-orbital region and locoregional recurrence in the mastectomy scar and axilla. Comparing these 19 cases in chronological order, it was noticed that symptoms at time of diagnosis shift from those of space occupying lesions to those suggestive for sinusitis. This shift could be explained by earlier diagnosis. High index of suspicion is the key to diagnosis. Earlier diagnosis does not result in longer survival since in most cases patients have already widespread disease and die within one year. Most authors mention the role of the vertebral venous plexus in hematogeneous spreading of tumor cells. Another pathway of hematogenous spread is via (occult) lung metastases. This case prompts the hypothesis of transcribrosal spread from meningeal involvement.

Orbital edema resulting from Haller's cell pathology: 3 case reports and review of literature.

Sinuspathology must be considered when rapid onset of unilateral orbital edema is found in the absence of ophtalmologic signs. Urgent medical treatment is necessary in these patients when headache, fever, facial pain and vision problems are present. However, symptoms may be more subtle. Three female patients with unilateral orbital edema and facial pain are presented. Inflammation of an ipsilateral Haller's cell should be considered as one of the potential causes of unilateral orbital edema and it can be the only cause. To our knowledge this is the first report of Haller's cell disease resulting in an orbital complication.

[The Noonan syndrome]. / Het Noonan-syndroom.

Two cases of Noonan syndrome are observed. The Noonan syndrome is a Turner-like syndrome with normal chromosomal constitution. Essential criteria in diagnosing the syndrome are the facial expression (hyper-telorisme, low-set and malformed ears, micrognathie, fish-like mouth). The short webbed neck and the cubitus valgus. These children are rather small and mental retardation can be present, especially in comparison with their siblings. Associated cardiac malformations occur in approximately 50% of the cases. We insist on audiologic findings. One case has a hearing impairment due to a malformation of the middle ear, recognised radiologically. The other has a deafness of perception type. Yet, the familial occurrence of last patient makes it doubtful whether this hearing loss can be linked to the Noonan-syndrome.