Invasive Rhinosinusitis Caused by Alternaria infectoria in a Patient with Autosomal Recessive CARD9 Deficiency and a Review of the Literature.

Phaeohyphomycoses comprise a heterogeneous group of fungal infections caused by dematiaceous fungi and have primarily been reported in patients with underlying acquired immunodeficiencies, such as hematological malignancies or solid-organ transplants. Over the past decade, a growing number of patients with phaeohyphomycosis but otherwise healthy were reported with autosomal recessive (AR) CARD9 deficiency. We report a 28-year-old woman who presented with invasive rhinosinusitis caused by Alternaria infectoria. Following a candidate gene sequencing approach, we identified a biallelic loss-of-function mutation of CARD9, thereby further broadening the spectrum of invasive fungal diseases found in patients with inherited CARD9 deficiency. In addition, we reviewed 17 other cases of phaeohyphomycosis associated with AR CARD9 deficiency. Physicians should maintain a high degree of suspicion for inborn errors of immunity, namely CARD9 deficiency, when caring for previously healthy patients with phaeohyphomycosis, regardless of age at first presentation.

Bacterial biofilm in adenoids of children with chronic otitis media. Part II: a case-control study of nasopharyngeal microbiota, virulence, and resistance of biofilms in adenoids.

Background: We previously described that adenoid tissue in children with chronic otitis media (COM) contained more mucosal biofilms than adenoid tissue removed for hypertrophy.Aims/objectives: The aim of the second part was to characterize nasopharyngeal microbiota and explore virulence of the most common middle ear pathogens.Material and methods: Bacteriological analysis was performed following a culture-based approach on the samples recovered from 30 patients of COM group (15 biofilm-positive and 15 biofilm-negative) and from 30 patients of a control group (15 biofilm-positive and 15 biofilm-negative). Virulence factors of Streptococcus pneumoniae, Streptococcus pyogenes, and Haemophilus influenzae were investigated.Results: The most frequent species were Firmicutes followed by Proteobacteria and Actinobacteria. The presence of biofilm was statistically associated with an increase of the number of bacterial species and Firmicutes phylum regardless of the condition (case/control). No virulence factors associated with invasive isolates were found for the most common middle ear pathogens.Conclusions and significance: This case-control study demonstrated that the presence of COM plus biofilm was associated with a given microbiota which contained more Firmicutes. Our study allows a better understanding of physiopathological mechanisms involved in chronic otitis media and paves the way for further investigations.

Bacterial biofilm in adenoids of children with chronic otitis media. Part I: a case control study of prevalence of biofilms in adenoids, risk factors and middle ear biofilms.

BACKGROUND: Biofilms are communities of bacteria embedded in a self-produced glycocalyx matrix. Adenoids have been shown to harbor bacterial biofilms. Aim/objectives: To compare the prevalence of biofilms in adenoid of children with chronic otitis media (COM) (group1) versus a control group without any COM (group 2) having adenoids removed because of hypertrophy. MATERIAL AND METHODS: One hundred and three children were prospectively enrolled in this case-control study, group 1 (n = 52) and group 2 (n = 51). The main outcome measurement was the prevalence of biofilm in adenoidectomy specimens analyzed using confocal laser scanning microscopy. Children in group 1 who had middle ear (ME) effusion and requiring the insertion of a tympanostomy tube underwent biopsy of the ME mucosa and effusion sampling. RESULTS: Biofilms were found in adenoids' specimens of both groups and in the ME biopsy and effusion. The biofilm prevalence in adenoids was 63.5% (33/52) in group 1 and 47.1% (24/51) in group 2. Day nursery and previous antibiotics intake were significantly more frequent in group 1 than in group 2. CONCLUSIONS AND SIGNIFICANCE: This case-control study demonstrates that adenoid tissue in children with COM contains more mucosal biofilms than adenoid tissue removed for hypertrophy. Biofilm was seen in ME biopsies and effusion.

99mTc-HMPAO-leukocyte scintigraphy for diagnosis and therapy monitoring of skull base osteomyelitis.

OBJECTIVE: Skull base osteomyelitis (SBO) is a rare but life-threatening disease observed in elderly diabetic patients, with high risk of recurrence and difficult therapeutic management. The diagnosis is ascertained from a set of clinical, biological, and imaging findings. CT and MRI allow initial diagnosis, but are not accurate to affirm healing at the end of therapy. 99mTc-HMPAO-Leucocyte Scintigraphy (LS) is highly sensitive and specific for the detection of infection. The aim of this study was to evaluate LS i) for initial diagnosis, and ii) to confirm healing at the end of antibiotherapy in SBO. STUDY DESIGN: We retrospectively reviewed from November 2011 to September 2015 all patients with confirmed SBO who underwent LS twice, at diagnosis and at the end of antibiotic therapy in our nuclear medicine department (n = 27). METHODS: Clinical, biological, CT, LS, and follow-up data were recorded in all patients. LS images (planar and tomographic performed 4 hours and 24 hours after intravenous injection of autologous Tc-99m-HMPAO-leucocytes) were visually assessed and quantified. RESULTS: At initial diagnosis, 25 of 27 patients had a positive LS. At the end of antibiotic therapy (3 ± 1 months duration), 26 of 27 patients had a negative LS. During subsequent follow-up (= or >6 months), the disease recurred in four patients including three with a negative postantibiotherapy LS scan. CONCLUSION: In this retrospective study, LS was powerful for initial diagnostic of SBO and for healing assessment at the end of antibiotic therapy. We conclude it is a useful technique for therapeutic monitoring of SBO. LEVEL OF EVIDENCE: 4.

Stent Placement for Disabling Pulsatile Tinnitus Caused by a Lateral Sinus Stenosis: A Retrospective Study.

BACKGROUND: Lateral sinus stenosis is a little-known cause for pulsatile tinnitus (PT). In several small series, stenting has been described as an effective treatment for disabling PT linked with this type of stenosis. OBJECTIVES: To describe the clinical, radiological, and manometric characteristics of patients treated for disabling PT by lateral sinus stenosis. Assessment of the efficacy of stenting for this indication. METHODS: Retrospective study of patients treated for isolated PT by stenting of a lateral sinus stenosis in our institution, between 2009 and 2015. RESULTS: Fourteen patients were included in our study. All of them were women. The median age at the onset of symptoms was 39.0 (21.0) years. The median body mass index was 28.5 (7.0) kg/m 2 . Stenting of the lateral sinus led to the disappearance of PT without recurrence in 13 patients. In one patient, stenting did not modify the noise. In this case, another cause of PT was diagnosed after stent placement. CONCLUSION: Lateral sinus stenosis is a curable cause of venous PT. Other causes of PT must be ruled out before an endovascular treatment is undertaken, due to the frequent asymptomatic nature of Pacchioni granulations in the lateral sinus. Treatment by stenting is effective in all cases, provided that stenosis underlies the PT.

[Sinonasal fungal infections are not exclusively due to mucorales and Aspergillus!]. / Les infections fongiques nasosinusiennes ne sont pas uniquement liées aux mucorales et aux Aspergillus !

Rhino-sinusal infections are serious diseases and possibly lethal. When they are invasive, we easily discuss apergilloses and mucormycoses. The confirmation of the diagnosis of mucormycosis need an extensive surgery for precise histopathological and mycological evaluation. The pathologist may be faced to other rare mycoses such as phaeohyphomycoses, which present different morphological features than mucormycoses and Aspergillus. Once the diagnosis is established, an appropriate antifungal treatment is quickly started. The aim of our work is to report two observations of phaeohyphomycoses, to describe their histopathological features, to discuss complementary diagnostic methods and to present the main differential diagnoses.

Septic cavernous sinus thrombosis secondary to acute bacterial sinusitis: a retrospective study of seven cases.

BACKGROUND: Septic cavernous sinus thrombosis (SCST) is a rare but severe complication of acute bacterial sinusitis. Evaluations of advances in imaging techniques as well as in medical and surgical treatment are hampered by the lack of recent studies. OBJECTIVE: We aim to report our experience in the management of SCST in patients with acute bacterial sphenoid sinusitis over the past 10 years and to discuss the initial work-up and treatment strategies. METHODS: We performed a retrospective study of patients admitted for SCST related to acute sinusitis at a tertiary care center between 2003 and 2013. Clinical charts were reviewed for demographics, clinical presentations, imaging and microbiologic findings, medical and surgical treatments, and outcomes. RESULTS: Seven patients were treated for SCST. Sphenoid sinus was involved in all cases. The most frequent presenting signs included headache (100%), cranial nerve impairment (86%), fever (71%), and orbital symptoms (71%). Diagnosis was confirmed by a cerebral contrast-enhanced CT scan in all cases. Four patients (57%) had an additional intracranial complication. The average time between clinical onset and diagnosis was 13.7 days. All patients were treated by high-dose i.v. antibiotics, anticoagulation therapy, and surgical endoscopic drainage of the infected sinuses. This treatment strategy resulted in a mortality rate of 0%, but four out of the seven patients developed transient or permanent neurologic deficits, including one with permanent unilateral visual loss. CONCLUSION: The combination of high-dose i.v. antibiotics, anticoagulation therapy, and endoscopic drainage of the infected paranasal sinus is an effective strategy for the treatment of SCST, but long-term sequelae remain frequent.

Frontal sinus obliteration with autologous calvarial bone graft: indications and results.

Despite increasing advances in endonasal frontal sinus surgery, frontal sinus obliteration (FSO) is sometimes necessary after failure of other surgical techniques. This procedure has been reported with autologous tissue or synthetic material, but few studies have reported results with autologous calvarial bone graft. The aim of this study was to report our experience with osteoplastic FSO calvarial bone graft. A retrospective review was performed on 11 patients operated upon for FSO with autologous calvarial bone graft from 2005 to 2011. Obliteration was indicated for chronic symptomatic frontal sinusitis with nasofrontal duct stenosis in five cases of nasal polyposis with a history of endoscopic sinus surgery, two cases of frontal trauma, two of surgery for frontal inverted papilloma and two of chronic frontal purulent sinusitis. Ten patients had a history of one or two previous functional endoscopic sinus surgery (FESS) procedures. On outcome assessment, eight patients had no residual complaints after FSO and all patients showed improvement in symptoms. Frontal sinus obliteration with autologous calvarial bone graft showed low donor site morbidity and good aesthetic results. This procedure should be considered in severe frontal sinusitis after repeated FESS procedures have failed.

[Rhino-orbito-cerebral mucormycosis, surgical treatment, state of the art]. / Mucormycoses rhino-orbito-cérébrales - Traitement chirurgical, état de l'art.

Rhino-orbito-cerebral mucormycoses constitute a severe fungal infection. These infections mostly arise in immunosuppressed patients. The surgery aiming at resecting necrosed hurts showed its interest in term of survival for lung and cutaneous mucormycosis. However, treatment of rhino-orbito-cerebral location of mucormycosis is not well defined. Transnasal endoscopic surgery allows local control of the disease, better post-operative outcomes than transfacial approaches and less sequelae. However, transfacial approaches are sometimes necessary to allow cutaneous resection or exenteration, the indications of which still remain controversial. The retrospective study of 22 patients with mucormycosis allowed to show that radical surgical treatment allowed local control of the disease with an improved survival. Further prospective studies (PHRC MICCA, current) are required to standardize the management of this rare but potentially lethal pathology.