Aneurysmal bone cyst on top of fibro-osseous lesion of the ethmoid sinus with orbital and intracranial extension in a child.

Objective: Aneurysmal bone cysts (ABCs) rarely involve the cranium. We report a case arising in the ethmoid sinus with orbital and intracranial invasion. Imaging suggested an associated fibro-osseous lesion. The lesion was completely resected. Histology confirmed the imaging diagnosis of ABC on top of an ossifying fibroma. A multidisciplinary approach is essential for optimal surgical outcomes. Methods: We report a case of an 8-year-old boy with a 5 week history of painless, increasing prominence of the left eye. Clinical examination revealed non-pulsatile left proptosis. Visual acuity and ocular movements were normal. CT and MRI scans of the maxillofacial regions showed a large space-occupying lesion involving the left ethmoid air cells with left orbital and left inferior frontal intracranial extension. Multiple fluid levels with blood products were seen. Areas of the bony component of the lesion showed ground-glass density on CT. Imaging was consistent with an ABC with an underlying fibro-osseous lesion; probably fibrous dysplasia which was confirmed after surgical removal of the lesion. A multidisciplinary team of maxillofacial, neurosusugery and ENT surgeons performed the surgery. Results: ABC arising from an osseous fibroma of the skull is rare. Total resection can be achieved with a multidisciplinary surgical approach. Post-operative histology confirmed by the imaging findings.

A postal survey of hereditary hemorrhagic telangectasia in the northeast of England.

The aim of this study is to identify the demographics and epistaxis burden of hereditary hemorrhagic telangiectasia (HHT). A questionnaire was sent to participants with HHT who were recruited from a prospectively maintained respiratory clinic data base in a tertiary hospital. Details on demographics, HHT symptoms, family history, epistaxis severity, and treatment received were recorded. There were 34 of 60 responses (57%). Two responses were from families of the deceased. Of the 32 evaluable patients (men, 14; women 18), the average age was 51 years (range, 23-78 years). The average age of HHT diagnosis was 31 years (range, 3-61 years). The diagnosis of HHT was made by the respiratory team in 13 patients; neurologist (2); ear, nose, and throat (ENT) specialist (4); general practitioner (5); hematologist (4); gastroenterologist (1); and not mentioned in two patients. Twenty-seven of 32 patients (84%) had a positive family history of HHT. Only 13 patients had formal genetic testing (4 endoglin, 1 activin receptor-like kinase, 8 unknown gene). All patients who presented to the respiratory clinic had a background of epistaxis, which was noted on presentation. The average age at initial epistaxis was 14 years (range, 2-50 years). The frequency of epistaxis was daily 63% (n = 20), weekly 9% (3), monthly 16% (5), and a few times a year 10% (3), and unstated in one patient. Nine of 32 patients (28%) required a transfusion. Six patients thought that they were unable to perform daily activities due to epistaxis. Only 15 of 32 patients (47%) were under the care of an ENT specialist. The treatment plan for epistaxis management was deemed good by 7 patients, adequate in 8, poor in 6, and not stated by 11 patients. In conclusion, this survey is the first to quantify the epistaxis burden within the northeast of England. The management of epistaxis needs specific education and treatment to optimize the quality of life among these patients.

Efficacy of a marine bacterial nuclease against biofilm forming microorganisms isolated from chronic rhinosinusitis.

BACKGROUND: The persistent colonization of paranasal sinus mucosa by microbial biofilms is a major factor in the pathogenesis of chronic rhinosinusitis (CRS). Control of microorganisms within biofilms is hampered by the presence of viscous extracellular polymers of host or microbial origin, including nucleic acids. The aim of this study was to investigate the role of extracellular DNA in biofilm formation by bacteria associated with CRS. METHODS/PRINCIPAL FINDINGS: Obstructive mucin was collected from patients during functional endoscopic sinus surgery. Examination of the mucous by transmission electron microscopy revealed an acellular matrix punctuated occasionally with host cells in varying states of degradation. Bacteria were observed in biofilms on mucosal biopsies, and between two and six different species were isolated from each of 20 different patient samples. In total, 16 different bacterial genera were isolated, of which the most commonly identified organisms were coagulase-negative staphylococci, Staphylococcus aureus and α-haemolytic streptococci. Twenty-four fresh clinical isolates were selected for investigation of biofilm formation in vitro using a microplate model system. Biofilms formed by 14 strains, including all 9 extracellular nuclease-producing bacteria, were significantly disrupted by treatment with a novel bacterial deoxyribonuclease, NucB, isolated from a marine strain of Bacillus licheniformis. Extracellular biofilm matrix was observed in untreated samples but not in those treated with NucB and extracellular DNA was purified from in vitro biofilms. CONCLUSION/SIGNIFICANCE: Our data demonstrate that bacteria associated with CRS form robust biofilms which can be reduced by treatment with matrix-degrading enzymes such as NucB. The dispersal of bacterial biofilms with NucB may offer an additional therapeutic target for CRS sufferers.

Removal of biofilms from tracheoesophageal speech valves using a novel marine microbial deoxyribonuclease.

OBJECTIVE: The growth of biofilms on tracheoesophageal speech valves shortens their life span and produces a reservoir of pathogens that may infect the respiratory tract. The authors have discovered a novel nontoxic deoxyribonuclease, NucB, from a marine isolate of Bacillus licheniformis that is effective at dispersing a variety of mono and mixed-species bacterial biofilms. The aim of this preliminary study was to determine whether NucB could also disrupt and remove mixed-species biofilms from tracheoesophageal speech valves. STUDY DESIGN: Laboratory-based treatment and analysis of discarded tracheoesophageal speech valves. SETTING: University human biology laboratory and the Department of Speech and Language Therapy at a tertiary referral hospital. SUBJECTS AND METHODS: Seventeen ex vivo tracheoesophageal speech valves fouled with natural human biofilms were collected and divided into 2 equal parts. One half was treated with NucB and the other half with a control buffer solution. Biofilm removal was measured by microscopy and by culture of dispersed biofilm organisms on agar plates. RESULTS: Significantly more organisms were released from biofilms using NucB than with buffer solution alone. On nonselective medium, more organisms were cultured in 11 samples (65%, n = 17, P < .05). Using growth media favoring fungi, more organisms were cultured in 14 samples (82%, n = 17, P < .05). CONCLUSION: The nontoxic deoxyribonuclease NucB was effective in releasing more microorganisms from biofilms on tracheoesophageal speech valves. This reflects its potential ability to break up and disperse these biofilms. Future studies will aim to develop NucB as a novel agent to prolong the life span of tracheoesophageal speech valves, thus reducing health care costs.