[Clinical Management of Choanal Atresia]. / Klinisches Management der Choanalatresie.

Choanal atresia (CA) is a rare congenital anomaly of the nasal airway with an incidence of 1/5000 to 1/9000, which may occur unilateral (uCA) or bilateral (bCA). bCA manifests as an acute emergency immediately after birth by airway obstruction and paradoxical cyanosis, whereas uCA may present with a heterogeneous clinical picture in addition to unilateral nasal airway obstruction. Fiber endoscopic examination and cranial computed tomography are the gold standard in the diagnosis of CA. CA often occurs in association with congenital malformation syndromes, among which CHARGE syndrome stands out. Due to cardiopulmonary instability and difficult intubation conditions, syndromic CA patients should be considered as a separate risk group. After securing the airway, bCA must be treated surgically without delay, whereas correction of uCA should not be performed until after six months of age. Endoscopic techniques are the surgical standard in the treatment of CA. Different approaches can be distinguished: transnasal puncture of the atresia plate with subsequent extension medially and laterally, creation of a septal window with subsequent resection of the posterior vomer and atresia plate, and elevation of mucoperiosteal flaps with subsequent opening of the atresia plate. The transpalatal approach should only be employed in anatomically complex cases. The use of conventional choanal stents in the primary treatment of CA is increasingly rejected and should be reserved for high-risk constellations. Similarly, local application of mitomycin C should be avoided.

[Sarcoidosis in head and neck area - a diagnostic challenge using the example of skin manifestations]. / Sarkoidose im Kopf-Hals-Bereich ­ eine diagnostische Herausforderung am Beispiel lokaler Hautmanifestationen.

BACKGROUND: Sarcoidosis is a granulomatous multisystem disease of unknown etiology and relatively rare. The heterogeneous clinical picture is a diagnostic challenge. We are investigating whether the superficially visible cutaneous lesions can lead to the differential diagnosis of sarcoidosis and what systemic manifestations are present. MATERIAL AND METHODS: As part of our exploratory retrospective investigation (eight years) a total of 32 patients with cutaneous sarcoidosis were identified and analyzed. RESULTS AND CONCLUSION: In many cases the dermatologists considered the differential diagnosis of sarcoidosis even before biopsy (71.8%); in our previous study with ENT-patients the diagnosis wasn't considered in a single case by the attending doctors at this time and without any prevoius suspicion. Sarcoidosis of the skin in the head and neck area is the second most common cutaneous manifestation. After biopsy (Gold standard) the search for further possible organ manifestations is essential (e.g. lungs, heart) to treat them in an early stage and to prevent complications of a possible chronic course (including cardiac arrhythmias, pulmonary fibrosis).

Alveolar Echinococcosis of the Parotid Gland-An Ultra Rare Location Reported from Western Europe.

(1) Background: Alveolar echinococcosis (AE) is restricted to the northern hemisphere with high endemic regions in Central Europe, North and Central Asia as well as Western China. The larval stage of Echinococcus multilocularis (E. multilocularis) causes AE with tumor-like growth. Humans are accidental hosts. This report is on the first case of AE becoming clinically manifested in the parotic gland. (2) Case presentation: A 52-year-old male patient presented with progressive and painful swelling of the right parotid gland persisting for one year. We performed a partial parotidectomy. The histological examination and immunohistological staining revealed larval stage of E. multilocularis. (3) Conclusion: E. multilocularis is known to infect animals and humans coincidentally, and leads to AE. It is one of the most life-threatening zoonoses in Europe. It typically manifests in the liver (50-77%), with further spreading to other organs being a rare phenomenon. Echinococcosis should be considered in the differential diagnosis of lesions of the parotid gland in endemic areas, but AE has not been described so far in the parotid gland as the sole manifestation and, therefore, impedes the correct diagnosis. A complete resection should be the aim, however, preservation of the facial nerve and adjuvant albendazole therapy is mandatory.

Etiology, Management, and Outcome of Pediatric Epistaxis.

OBJECTIVE: Epistaxis in children is one of the most common causes for seeking professional medical help. Patients may be treated by several disciplines with various approaches to pediatric epistaxis. We reviewed cases of pediatric epistaxis from an otorhinolaryngologist's point of view. METHODS: A retrospective chart review was performed on all patients younger than 18 years presenting with epistaxis to the Department of Otorhinolaryngology at the University of Bonn, Germany. RESULTS: Sixty episodes of epistaxis in 58 patients were included in the study. Mean age was 10.1 ± 4.5 years. In terms of risk factors, 3 patients had a hemorrhagic diathesis, 3 had taken medication that interfered with hemostasis, and 8 had a history of previous trauma, most of which was digital manipulation. Twenty-six patients did not need invasive therapy. Twenty-six patients received cauterization to control the bleeding, and 4 patients needed surgery. The necessity for surgery was mainly noncooperation. CONCLUSIONS: Epistaxis in children is seldom serious. However, hemorrhagic diathesis needs to be kept in mind as a potential cause of epistaxis. In most cases, careful instruction of the patients and the relatives concerning nasal mucosal care is sufficient. If cauterization is necessary, silver nitrate coagulation should be preferred over electrocoagulation.

Hyaluronic Acid-based Filler Injection: Late-onset Thrombosis of the Frontal Vein.

Thrombosis is a serious complication of a hyaluronic acid-based filler injection. Little is known about the late-onset complications of fillers; therefore, an optimal complication management is necessary. In this case report, we describe a rare complication of thrombosis after a filler injection. A 35-year old woman was admitted to the emergency department, with swelling on her forehead in association with recurrent pain and light flashes in her right eye. Sonographic examination showed a thrombosis of the right frontal vein. The patient reported that a hyaluronic acid filler injection had been administered on the forehead 3 months ago. After several weeks of anticoagulation with heparin and apixaban, the symptoms persisted. The vein with thrombosis was ultimately resected under local anesthesia. Histological findings showed a chronic inflammatory reaction of the tissue to hyaluronic acid. Vascular complications may appear as late-onset complications even several months after the filler injection. Subcutaneous application of low molecular weight heparins is the therapy of first choice. If this treatment is not effective, resection of the thrombosis may be performed.

Higher number of multidisciplinary tumor board meetings per case leads to improved clinical outcome.

BACKGROUND: This analysis aims at evaluating the impact of multidisciplinary tumor boards on clinical outcome of multiple tumor entities, the effect of the specific number of multidisciplinary tumor boards and potential differences between the tumor entities. METHODS: By a matched-pair analysis we compared the response to treatment, overall survival, relapse or disease free survival and progression free survival of patients whose cases were discussed in a tumor board meeting with patients whose cases were not. It was performed with patients registered in the cancer registry of the University of Bonn and diagnosed between 2010 and 2016. After the matching process with a pool of 7262 patients a total of 454 patients with 66 different tumor types were included in this study. RESULTS: First, patients with three or more multidisciplinary tumor board meetings in their history show a significantly better overall survival than patients with no tumor board meeting. Second, response to treatment, relapse free survival and time to progression were not found to be significantly different. Third, there was no significant difference for a specific tumor entity. CONCLUSION: This study revealed a positive impact of a higher number of multidisciplinary tumor boards on the clinical outcome. Also, our analysis hints towards a positive effect of multidisciplinary tumor boards on overall survival.

Clinical features of sarcoidosis patients presenting with head and neck manifestations - a two-center retrospective study and proposal of a diagnostic algorithm for the otorhinolaryngologist.

Background and Aims: We aimed to investigate the clinical characteristics of patients presenting with sarcoidosis of the head and neck as the initial manifestation and to provide recommendations for the diagnostic work-up for the practicing otorhinolaryngologist.Material and Methods: We performed a retrospective cohort study at two university medical centers in Germany. Patients with a histopathologically confirmed diagnosis of sarcoidosis treated in the otorhinolaryngology departments were analyzed.Results: We identified 62 patients (2003-2016). In total, 85.4% (n = 53) of patients received the initial diagnosis of sarcoidosis during their ENT treatment. Sarcoidosis was detected in the lymph nodes in 42.3% (n = 30) of the patients; 57.7% had extra-lymphatic manifestations. Fifteen patients (24.2%) showed pulmonary involvement. 30.6% (n = 19) were treated with oral glucocorticoids (GC) alone, three patients with GC and methotrexate, one patient initially received a combination of GC and azathioprine, one patient rejected the recommended treatment.Conclusions: Sarcoidosis should be considered as a differential diagnosis in patients presenting with head and neck symptoms. The most frequent presenting symptoms were cervical lymphadenopathy and affection of the paranasal sinuses. Therefore, otorhinolaryngologists should be aware of sarcoidosis and help guide referral strategies as they may be the first physicians treating these patients.

Neurosarcoidosis As a Rare Differential Diagnosis for Single Or Multiple Lesions of the Nervous System.

Objective: Sarcoidosis is a multisystemic granulomatous disease of unknown cause which affects the lung or bilateral hilar lymphadenopathy in over 90% of the cases. Neurosarcoidosis (NS) is rare and accounts for approximately 5 - 15% of the cases. Involvement of all parts of the central and peripheral nervous system is possible with various clinical symptoms, e. g. seizures, hydrocephalus, optic/facial nerve palsy or hearing loss.Methods: We screened the neuropathological data bases and the medical records of two neurosurgical university hospitals for cases of NS. All these cases had been verified by surgical biopsy. We retrospectively evaluated the patient's records with special regard to the histopathology reports and specific clinical symptoms.Results: We identified 9 cases of NS between 1994 and 2014 (3 female, 6 male patients). The average age at the time of diagnosis of NS was 41,4 years. Various clinical symptoms like hydrocephalus (n = 3), seizures (n = 1), meningitis (n = 1), optical nerve involvment with vision disorder (n = 1), myelitis with paraplegia (n = 1), mastoiditis with hearing loss (n = 1), back pain syndrome (n = 2) were present. 7 patients were treated with corticosteroids, 1 patient with cyclophosphamide and 1 with a combination of corticosteroids and methotrexate.Conclusion: NS is a rare but potentially life-threatening disease. It is difficult to distinguish sarcoidosis from other granulomatous diseases, infectious diseases like tuberculosis, multiple sclerosis or neoplasm. For a definite diagnosis, a neurosurgical biopsy with histological evidence of noncaseating epithelioid cell granulomas is required, followed by multidisciplinary treatment.

Reconstruction of the skull base in spontaneous rhinoliquorrhea.

Objective/Hypothesis: Spontaneous rhinoliquorrhea often occurs due to defects of the skull base. It is often misinterpreted as rhinitis and is surgically the most difficult rhinoliquorrhea entity to close. Methods: We conducted a retrospective chart analysis of patients that were diagnosed with spontaneous rhinoliquorrhea at the University Hospital Bonn between 2001 and 2017. Results: Overall, twelve patients were included in this study. On average, the time between occurrence of nasal discharge and diagnoses of rhinoliquorrhea was 123 days. In ten patients, the localization of the skull base defect could be localized by computed tomography or MRI cisternography. Ten patients underwent surgery, of which 9 remained recurrence free. One patient underwent revision surgery and from thereon was recurrence free. Conclusion: Spontaneous rhinoliquorrhea still remains a diagnostic and therapeutic challenge. Whenever persistent watery nasal discharge appears in a patient, rhinoliquorrhea must be considered. Endoscopic surgical reconstruction of the skull base is the therapeutic gold standard and should be attempted as soon as the diagnosis is secured.

Sarcoidosis of the paranasal sinuses.

BACKGROUND: Sarcoidosis is a chronic disease, which predominantly affects the lung. Since sinonasal sarcoidosis is rare, little is known about the sarcoidosis manifestation at this site. Therefore, the aim of our study was to detect the prevalence of sinonasal sarcoidosis, its clinical occurrence, diagnosis, and therapy. METHODS: The database of all patients having visited the otorhinolaryngology departments of the universities in Göttingen and in Bonn between 2003 and 2016 was searched for the diagnosis of sinonasal sarcoidosis. RESULTS: Thirteen patients with a biopsy-proven sinonasal sarcoidosis were identified. Most patients presented non-specific clinical symptoms, which are also found in acute and chronic sinusitis. None of the patients was suspected to have sinonasal sarcoidosis by the ENT doctor before histological validation. The mean diagnostic delay was 262 (± 195) days. An additional pulmonary involvement was detected in four of six patients. CONCLUSIONS: Sinonasal sarcoidosis is presenting with heterogeneous clinical presentations. An early biopsy of granulomatous lesions is mandatory. A multidisciplinary approach is needed to exclude serious lung or heart manifestations, because even asymptomatic organ involvement is possible. A CT-scan may be useful even if unspecific. Local or systemic therapy has to be prepared individually using local and systemic corticosteroids, antimetabolites, or anti-TNF-alpha.

Management and outcome of epistaxis under direct oral anticoagulants: a comparison with warfarin.

BACKGROUND: Epistaxis is one of the more common reasons for emergency room visits. The main risk factor for epistaxis is anticoagulant therapy. Until recently, the main culprit was oral intake of a vitamin K antagonist, such as warfarin, which has a number of side effects. Even more recently, several direct oral anticoagulants, rivaroxaban and dabigatran, have been approved for use. We investigated the possible differences between treatment of epistaxis with direct oral anticoagulants and vitamin K antagonists. METHODS: We conducted a retrospective cohort study at a tertiary referral center in Germany. All patients who were admitted within a 1-year period were included. Patient files were used to obtain the information. RESULTS: Overall, 677 patients were included in our study. Of these, 159 had been treated with vitamin K antagonists and 49 with direct oral anticoagulants. There were no significant differences in terms of age (p = 0.592), sex (p = 0.372), vital signs, bloodwork, or location of bleeding (p = 0.372). Management of epistaxis between the groups was also comparable (p = 0.399), with similar hospital admission rates (37.1% vs 24.5%; p = 0.145) and duration of stay (3.5 ± 2.1 days vs 3.8 ± 3.3 days; p = 0.650). CONCLUSION: We found no evidence to suggest epistaxis is more severe or requires more invasive therapy in patients given direct oral anticoagulants. A significant proportion of patients on vitamin K antagonists were not within the target range for international normalized ratio, highlighting one of the main issues with oral anticoagulation by vitamin K antagonists.

Management and Follow-up Results of Salivary Fistulas Treated With Botulinum Toxin.

OBJECTIVES/HYPOTHESIS: Salivary fistulas are a common minor complication of parotid surgery. botulinum toxin has repeatedly been reported to be an adequate treatment of this entity. To date, there is little scientific evidence for clinical decision making after appearance of a salivary fistula. STUDY DESIGN: Retrospective chart analysis of 16 patients who had been treated with botulinum toxin for salivary fistula. METHODS: All patients who had been treated for salivary fistula at a tertiary referral hospital from 2010 to 2016 were included. Patient files were used to obtain characteristics of patients, treatment, and follow-up. RESULTS: Sixteen patients with salivary fistula received 27 injections of botulinum toxin. Nine patients required one injection for the fistula to heal, five patients needed two injections, and one patient needed three and four injections, respectively. No patient underwent additional surgery or radiotherapy. We observed no adverse effects in any patient treated with botulinum toxin. CONCLUSIONS: In most cases of salivary fistula, injections of botulinum toxin are a valid treatment. If the initial injection is not successful, injections may be repeated once. Otherwise, revision surgery should be considered. In general, treatment with botulinum toxin should be commenced in an earlier stage and with higher dosages. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:403-408, 2019.

Amyloidosis of the Upper Aerodigestive Tract: Management of a Rare Disease and Review of the Literature.

Amyloidosis in the upper aerodigestive tract is a very rare disease with mainly case reports documented so far. In the pathogenesis, amyloid protein fibers are deposited in organs and tissue. In the upper aerodigestive tract, mostly localized amyloidosis occurs with unspecific symptoms, e.g., dysphagia. We conducted a retrospective multicenter study with two study centers in Germany (tertiary referral hospitals), the University Hospital of Bonn and the University Hospital of Goettingen. For a period of the last 7 years, data were analyzed and patients were recruited consecutively. 14 cases were included to the study. The most common manifestation was in the larynx (n = 11); in one case each localized amyloidosis was found in the tongue, trachea and in the pharynx. Since the majority of our cases (n = 13; 92.6%) presented with unspecific symptoms, biopsy results confirmed the diagnosis of localized amyloidosis. Resection of the lesion was only performed in patients reporting of symptoms, in asymptomatic patients only a non-invasive biopsy was done. In two patients (14.2%), in addition to the focal lesion a systemic amyloidosis was found. Amyloidosis in the upper aerodigestive tract is a rare disease. Nonetheless, every otorhinolaryngologist should be aware of this disease. When detected it is a straight forward to treat illness. The appearance of a systemic amyloidosis needs to be ruled out; thus, there is a chance to develop a multiple myeloma. Given the slow progressive character of amyloidosis, a long-term follow-up up to 10 years is inevitable.

Spot light on skeletal muscles: optogenetic stimulation to understand and restore skeletal muscle function.

Damage of peripheral nerves results in paralysis of skeletal muscle. Currently, the only treatment option to restore proper function is electrical stimulation of the innervating nerve or of the skeletal muscles directly. However this approach has low spatial and temporal precision leading to co-activation of antagonistic muscles and lacks cell-type selectivity resulting in pain or discomfort by stimulation of sensible nerves. In contrast to electrical stimulation, optogenetic methods enable spatially confined and cell-type selective stimulation of cells expressing the light sensitive channel Channelrhodopsin-2 with precise temporal control over the membrane potential. Herein we summarize the current knowledge about the use of this technology to control skeletal muscle function with the focus on the direct, non-neuronal stimulation of muscle fibers. The high temporal flexibility of using light pulses allows new stimulation patterns to investigate skeletal muscle physiology. Furthermore, the high spatial precision of focused illumination was shown to be beneficial for selective stimulation of distinct nearby muscle groups. Finally, the cell-type specific expression of the light-sensitive effector proteins in muscle fibers will allow pain-free stimulation and open new options for clinical treatments. Therefore, we believe that direct optogenetic stimulation of skeletal muscles is a very potent method for basic scientists that also harbors several distinct advantages over electrical stimulation to be considered for clinical use in the future.

Robot-assisted endoscope guidance versus manual endoscope guidance in functional endonasal sinus surgery (FESS).

BACKGROUND: Having one hand occupied with the endoscope is the major disadvantage for the surgeon when it comes to functional endoscopic sinus surgery (FESS). Only the other hand is free to use the surgical instruments. Tiredness or frequent instrument changes can thus lead to shaky endoscopic images. METHODS: We collected the pose data (position and orientation) of the rigid 0° endoscope and all the instruments used in 16 FESS procedures with manual endoscope guidance as well as robot-assisted endoscope guidance. In combination with the DICOM CT data, we tracked the endoscope poses and workspaces using self-developed tracking markers. RESULTS: All surgeries were performed once with the robot and once with the surgeon holding the endoscope. Looking at the durations required, we observed a decrease in the operating time because one surgeon doing all the procedures and so a learning curve occurred what we expected. The visual inspection of the specimens showed no damages to any of the structures outside the paranasal sinuses. CONCLUSION: Robot-assisted endoscope guidance in sinus surgery is possible. Further CT data, however, are desirable for the surgical analysis of a tracker-based navigation within the anatomic borders. Our marker-based tracking of the endoscope as well as the instruments makes an automated endoscope guidance feasible. On the subjective side, we see that RASS brings a relief for the surgeon.

Optogenetic control of contractile function in skeletal muscle.

Optogenetic stimulation allows activation of cells with high spatial and temporal precision. Here we show direct optogenetic stimulation of skeletal muscle from transgenic mice expressing the light-sensitive channel Channelrhodopsin-2 (ChR2). Largest tetanic contractions are observed with 5-ms light pulses at 30 Hz, resulting in 84% of the maximal force induced by electrical stimulation. We demonstrate the utility of this approach by selectively stimulating with a light guide individual intralaryngeal muscles in explanted larynges from ChR2-transgenic mice, which enables selective opening and closing of the vocal cords. Furthermore, systemic injection of adeno-associated virus into wild-type mice provides sufficient ChR2 expression for optogenetic opening of the vocal cords. Thus, direct optogenetic stimulation of skeletal muscle generates large force and provides the distinct advantage of localized and cell-type-specific activation. This technology could be useful for therapeutic purposes, such as restoring the mobility of the vocal cords in patients suffering from laryngeal paralysis.