Tuberculous mastoiditis - a case series.

SUMMARY: Tuberculous mastoiditis (TBM) is a rare form of extrapulmonary tuberculosis (TB), which may result in catastrophic complications, including mastoid and ossicle destruction, hearing loss and intracranial spread if untreated. Diagnosis is challenging due to the paucibacillary nature of extrapulmonary TB, compounded by limited theatre access for specimen retrieval, resulting in delayed diagnosis and treatment initiation. In this case series, we discuss three cases of TBM (one paediatric and two adults) who presented to the public and private healthcare sectors in the Eastern Cape in 2022, underscoring that TB does not respect socioeconomic status.

Acute mastoiditis in cochlear implanted children: A single-centre experience.

BACKGROUND: Acute mastoiditis (AM) is the most common complication of acute otitis media and primarily affects children under the age of two; current data on its prevalence in paediatric patients with cochlear implant (CI) are still scant. Proper management of AM in CI children is crucial in order to avoid the implications (financial and emotional) of an explant. Aim of this paper is to describe the cases of AM occurred among young patients with CI in follow up at our department, also in order to evaluate its prevalence, potential predisposing factors, clinical course and therapeutic strategies. PATIENTS AND METHODS: Retrospective study. Medical records of all paediatric patients with CI, who had at least one year of follow-up, were searched aiming to identify those who developed AM, from January 1st 2002 to January 31st 2022. The following data were collected and analysed: demographic features, implant type and side, interval between CI surgery and AM, treatment, laboratory tests, clinical course, vaccination history, associated diseases. RESULTS: AM was developed by six (1.3%) of the 439 children with CI (541 implanted ears). In total, 9 episodes (2.05 %) were recorded, as three patients reported two consecutive infections. Average time interval between CI surgery, to the first or only AM diagnosis, was 13.8 months (range 3-30 months). Furthermore, 3/6 of patients had a history of recurrent acute otitis media; 2/6 an autism spectrum disorder, associated to a combined immune deficiency in one case. All patients were hospitalized and promptly treated by intravenous antibiotic therapy; 4/6 also underwent a mastoidectomy. CI was not explanted in any cases of this series. CONCLUSIONS: Over a 20-year period, AM rate in CI children was 1.3%, which is consistent with the current literature rates of 1-4.7%. All cases were successfully treated, preserving the integrity of the device. In our experience, the early parenteral antibiotic therapy and, when necessary, surgical treatment were adequate to eradicate the infection.

X-linked Agammaglobulinemia Diagnosed Following Bezold's Abscess: A Case Report.

Bezold's abscess is an extracranial complication of otitis media, in which a cervical abscess forms from the mastoid process through an ostial fistula, and is a rare condition in recent years. In this study, we experienced a X-linked agammaglobulinemia, which was discovered due to Bezold's abscess. Case: A 12-year-old boy suffering from recurrent right suppurative otitis media for three months was treated with tympanostomy and oral antibacterial therapy at a local otorhinolaryngology clinic. The patient visited the clinic due to a recurrence of symptoms. CT showed bony defects in the cortical bone and mastoid process of the lateral side of the right mastoid cell. The patient was referred to our hospital, admitted the same day and underwent emergency surgery. Intraoperative findings led to the diagnosis of acute mastoiditis and Bezold's abscess c aused b y mastoiditis spreading to the s ternocleidomastoid muscle. After drainage and administration of ABPC/SBT, the abscess disappeared, and the patient's general condition improved. Subsequently, a blood typing test performed on admission suggested the influence of low immunoglobulin levels. A close examination by the pediatric department led to a diagnosis of X-linked agammaglobulinemia. As a result, the patient receives regular immunoglobulin therapy and has been free of infection, including Bezold's abscess. CONCLUSIONS: In the case of recurrent otitis media and rare infections, congenital immune abnormalities should be considered.

Profile of paediatric tuberculosis mastoiditis - a case series.

BACKGROUND: Tuberculosis (TB) otitis media is an uncommon site of extrapulmonary TB and can primarily present as a complicated TB mastoiditis (TBM). This complication is rare in children, even in TB endemic areas but necessitates early identification as delays can lead to severe morbidities. We describe the clinical characteristics as a case series to raise awareness of the condition, and highlight fundamentals related to diagnosis and management. METHODS: A retrospective chart review of clinical and radiological information of five children with TBM seen at the Red Cross War Memorial Children's Hospital in Cape Town, South Africa, over the last 5 years. Variables collected included symptomatology, duration of disease, investigations and management. RESULTS: All were under 5 years of age and presented with typical features of acute bacterial mastoiditis. Mean duration of symptoms was 12 days (range 3-30 days). Two children had known TB contacts. Two children had pulmonary involvement, one with miliary TB. CT of the temporal bone demonstrated extensive bony destruction of the petromastoid and demineralised ossicles in all cases. Three children had intracranial extension. Four children demonstrated hearing loss between 30 dB and 83 dB. Necrotising granulomatous inflammation was present in the mastoid specimens in all cases. Confirmatory diagnosis was made via GeneXpert polymerase chain reaction (PCR) (2), Ziehl-Nielson (ZN) stain (1) or a positive TB culture (2). Postoperatively, one patient had normal hearing, two patients had mild conductive hearing loss (CHL), one had mild-moderate CHL and one had profound hearing loss. CONCLUSION: Delays in identification and management result in marked bony destruction and hearing loss. Radiological and surgical findings typical of TBM, therefore, require tissue sampling from the ear for urgent microscopic, PCR and histologic testing, allowing the avoidance of a mastoidectom. In a TB endemic setting, children with typical findings and necrotising granulomatous inflammation on histology should be considered for prompt commencement of anti-TB therapy while awaiting a definitive diagnosis.

IgG4-related pachymeningitis and mastoiditis, associated with cerebral venous thrombosis: A case report.

IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition; this can be a challenging diagnosis that requires clinico-pathologic correlation. We report a young woman, presenting with cranial nerve palsy. The work-up revealed pachymeningitis, cerebral venous thrombosis (CVT), and a destructive lesion in the mastoid. We diagnosed IgG4-RD through mastoidectomy. Thus, a biopsy of asymptomatic, infrequently affected organs, like the mastoid, can meet all histopathological criteria. In neuro-meningeal presentations, CVT may be secondary to the local inflammatory environment of pachymeningitis. Since our patient had a deep vein thrombosis one year prior, we discuss a possible higher risk of thrombosis in IgG4-RD patients.

Multicorrection Goldenhar syndrome (facio-auriculo-vertebral dysplasia): a rare follow-up case of 12-year-old female.

The MSX homeobox genes cause Goldenhar syndrome (GHS) or facio-auriculo-vertebral dysplasia, a rare developmental defect. Its exact etiology is still unknown. Its incidence lies between 1: 3500 and 1: 5600. In 85% of the cases, the unilateral face is affected. Typical clinical findings in a classic GHS include eye disorders, ear irregularities (with or without hearing loss), facial impairments, dental and oral ailments, cardiac syndromes, central nervous system (CNS) involvement, trachea and lung malformations, kidney and gastrointestinal defects, and skeletal alterations. This case report presents a follow-up case of Goldenhar Syndrome in a 12-year-old female, with no relevant family history, diagnosed with anotia on the left side, cyanosis, and facial asymmetry at birth. She presented with moderate growth failure, bilateral sclerosing mastoiditis and kyphoscoliosis. She underwent posterior scoliosis correction posterior instrumented fusion from D1 to D11.

The importance of imaging in diagnosis of infected otogenic pneumatoceles.

OBJECTIVE: Spontaneous otogenic pneumatocele is a rare entity resulting from a pressure gradient between a dehiscent temporal bone and the intracranial space. Secondary infection can ensue in patients with concurrent otomastoiditis. The current study discusses the clinical presentation and imaging characteristics of two cases of secondarily infected otogenic pneumatoceles. STUDY DESIGN: Case series. RESULTS: Two patients were diagnosed with a temporal lobe abscess in the setting of otogenic pneumatocele. Diagnosis was aided by both CT and MRI demonstrating a diffusion restricting lesion within brain parenchyma in association with free air in close proximity to an underlying tegmen defect. CONCLUSION: Prompt diagnosis of a secondarily infected otogenic pneumatocele with CT and MRI allows for surgical drainage with closure of the tegmen defect to prevent further complications and recurrence.

Pediatric Otogenic Cerebral Sinus Vein Thrombosis and Thrombophilia.

BACKGROUND: Otogenic cerebral sinus vein thrombosis (CSVT) is a rare but severe complication of otitis media in children. To date, the role of prothrombotic evaluation is still controversial. OBJECTIVES: To report the clinical manifestations, prothrombotic evaluation, and current management of CSVT. METHODS: We performed a retrospective study of nine pediatric patients with otogenic CSVT who underwent prothrombotic evaluation between 2008 and 2018. RESULTS: Prominent clinical features included persistent otorrhea (88.8%), signs of mastoiditis (88.8%), high fever ≥ 38.3°C (100%), a classic spiking fever pattern (55.5%), and neurological signs (55.5%). A subperiosteal abscess (66.6%) was the most common otitis media complication associated with mastoiditis and CSVT. No microorganism was identified in 55.5% of patients. Cultures collected from ear secretions had a low yield (6.25%). However, PCR assays had a high detection rate (100%; n=3). The prothrombotic evaluation demonstrated an abnormal LAC-dRVVT ratio (6/9), elevated Factor VIII (5/8) (and a combination of both in four patients), antiphospholipid antibodies (2/8), and high homocysteine levels (1/5).The surgical intervention of choice included one-sided mastoidectomy with myringotomy and ventilation-tube placement on the affected side (77.7%). There were no mortalities and no long-term sequela except chronic otitis media (22.2%). CONCLUSIONS: Our findings demonstrate good outcomes for otogenic CSVT treatment with intravenous antibiotics, anticoagulation, and conservative surgical intervention, which supports the current trend in management. The prothrombotic evaluation revealed transient inflammation-related risk factors but did not alter management. Further prospective multicenter studies are needed to determine its relevance.

A 27-year-old man with recurrent sinopulmonary and cutaneous infections.

The increasing availability of genetic testing for modern immunologists in the evaluation of immune diseases could provide a definite diagnosis in elusive cases. A 27-year-old white male patient presented to the clinic with recurrent sinopulmonary and cutaneous infections since childhood. The patient's mother had seronegative polyarthritis, and one of two sisters of the patient had chronic sinopulmonary infections. Serum immunoglobulins, immunoglobulin G (IgG) subclasses, lymphocyte subset markers, mannose-binding lectin, mitogen and antigen stimulation, bacteriophage study, and Streptococcus pneumoniae titers to 23 serotypes were all normal. B-cell phenotyping revealed a decrease in both nonswitched memory B cells (CD19+CD27+IgD+) and switched memory B-cells (CD19+CD27+IgD-). Genetic testing and the improvement of clinical symptoms after IgG replacement led to the final diagnosis.

Acute mastoiditis in a newborn with 11 days of life: Case report.

Acute mastoiditis is a potentially life-threatening complication and extremely rare in children under six months. We herein report the case of a 11-days-old newborn with acute mastoiditis complicated by subperiosteal abscess, submitted to surgical and medical treatment. A transient hypogammaglobulinemia was finally diagnosed and successfully managed without complications. Early recognition of this rare immunological disorder and prompt intervention are critical to prevent further complications.

Therapeutic approach to pediatric acute mastoiditis - an update / Abordagem terapêutica da mastoidite aguda pediátrica - uma atualização

Abstract Introduction: Acute mastoiditis remains the most common complication of acute otitis media. It may rarely appear also in cochlear implant patients. However, the treatment recommendations for this disease are not precisely defined or employed, and in the current literature the differences regarding both the diagnosis and management are relatively substantial. Objective: The aim of this study was to determine a standard and safe procedure to be applied in case of pediatric acute mastoiditis. Methods: A retrospective chart review of 73 patients with 83 episodes of acute mastoiditis hospitalized at our tertiary-care center between 2001 and 2016 was conducted. Bacteriology, methods of treatment, hospital course, complications, and otologic history were analyzed. Based on our experience and literature data, a protocol was established in order to standardize management of pediatric acute mastoiditis. Results: All the patients treated for acute mastoiditis were submitted to an intravenous antibiotic regimen. In the analyzed group pharmacological treatment only was applied in 11% of children, in 12% myringotomy/tympanostomy was added, and in the vast majority of patients (77%) mastoidectomy was performed. In our study recurrent mastoiditis was noted in 8% of the patients. We also experienced acute mastoiditis in a cochlear implant child, and in this case, a minimal surgical procedure, in order to protect the device, was recommended. Conclusions: The main points of the management protocol are: initiate a broad-spectrum intravenous antibiotic treatment; mastoidectomy should be performed if the infection fails to be controlled after 48 h of administering intravenous antibiotic therapy. We believe that early mastoidectomy prevents serious complications, and our initial observation is that by performing broad mastoidectomy with posterior attic and facial recess exposure, recurrence of acute mastoiditis can be prevented.

Resumo Introdução: A mastoidite aguda continua a ser a complicação mais comum da otite média aguda. Pode ocorrer também, embora raramente, em pacientes com implante coclear. Entretanto, as recomendações de tratamento para essa doença não são bem definidas ou usadas e, na literatura corrente, as diferenças em relação ao diagnóstico e ao manejo são relativamente significativas. Objetivo: O objetivo deste estudo foi determinar um procedimento padrão e seguro a ser aplicado em caso de mastoidite aguda pediátrica. Método: Foi realizada uma revisão retrospectiva de prontuários de 73 pacientes com 83 episó-dios de mastoidite aguda hospitalizados em nosso centro terciário entre os anos de 2001 a 2016. Foram analisados a bacteriologia, métodos de tratamento, evolução hospitalar, complicações e histórico otológico. Com base em nossa experiência e dados da literatura, foi estabelecido um protocolo para padronizar o tratamento da mastoidite aguda pediátrica. Resultados: Todos os pacientes tratados para mastoidite aguda foram submetidos a antibioticoterapia endovenosa. No grupo analisado, o tratamento farmacológico só foi aplicado em 11% das crianças, em 12% a miringotomia/timpanostomia foi adicionada e na maior parte dos pacientes (77%) foi feita a mastoidectomia. Em nosso estudo, mastoidite recorrente foi observada em 8% dos pacientes. Também observamos mastoidite aguda em criança usuária de implante coclear e, nesse caso, foi recomendada a minimização de procedimentos cirúrgicos, a fim de proteger o dispositivo. Conclusões: Os principais pontos do protocolo de conduta são: iniciar um tratamento antibiótico endovenoso de amplo espectro; a mastoidectomia deve ser feita caso a infecção não seja controlada após 48 horas da administração de antibioticoterapia intravenosa. Acreditamos que a mastoidectomia precoce previne complicações graves e nossa observação inicial é que, com uma mastoidectomia ampla com exposição do ático posterior e do recesso facial, a recorrência de mastoidite aguda pode ser evitada.

Granulomatosis with Polyangiitis Manifesting as Refractory Otitis Media and Mastoiditis.

Granulomatosis with polyangiitis (GPA) is a rare rheumatologic disease characterized by small to medium vessel vasculitis and inflammation within multiple organ systems. Majority of cases involve both upper and lower respiratory tracts but other organs including brain, kidneys, joints and skin can also be involved. Patients who have recurrent otitis media and sinusitis often initially present to internists and then to ear nose and throat (ENT) physicians and may be treated with multiple courses of antibiotics without having proper workup for GPA. We present a middle-aged white male who exemplifies this unique presentation with new onset recurrent otitis media and mastoiditis, which did not respond to repeated courses of antibiotics requiring mastoidectomy and myringotomy tube placement. On chest x-ray, he was found to have multiple lung nodules that was followed by a computed tomography (CT) scan of his chest and CT guided biopsy which revealed granulomatous inflammation and necrosis, consistent with GPA. Autoimmune laboratory work-up was also suggestive of GPA. The patient had complete resolution of symptoms with steroid therapy. This case reinforces the concept of high index of suspicion of GPA for patients with recurrent and resistant otitis media or mastoiditis not responding to optimal duration and doses of antibiotics. Appropriate work-up for GPA may allow for earlier diagnosis of this devastating vasculitic disease which can help prevent multi-organ dysfunction.

Mastoiditis caused by intracranial venous sinus stenting in an idiopathic intracranial hypertension patient.

The exact mechanism of idiopathic intracranial hypertension (IIH) is unknown. It needs to be treated because of severe headache and impaired vision. For medically refractory patients, cerebrospinal fluid diversion, optic nerve sheath fenestration and dural venous sinus stenting is applied to relieve the symptoms. As a new therapy, the complication of dural venous stenting was a focus for operators. Here, a woman is reported with IIH who suffered from mastoiditis after stenting in the sigmoid sinus for the first time. The special local anatomy of the sigmoid sinus adjacent to the inner structure made it a noteworthy complication.

Allergic Fungal Otomastoiditis in a Patient without Allergic Fungal Rhinosinusitis: A Case Report.

BACKGROUND Allergic fungal rhinosinusitis is an inflammatory condition involving the paranasal sinuses and linings of the nasal passages that lasts 12 weeks or longer. In 2013 Chen et al. reported the first case of middle ear allergic otomastoiditis in a patient who tested negative for allergic sinus-nasal disease. To the best of our knowledge, this is the second such case report to be published. CASE REPORT Our patient was a 27-year-old immune-competent woman presenting with chronic left-side otorrhea and hearing loss for 2 years. An audiogram showed low- and middle-frequency, mild-to-moderate left-sided conductive hearing loss. A coronal view computed tomography (CT) image of the temporal bone showed coalescent air cells in the left mastoid with opacification of the left middle ear, but no evidence of bony erosion. Diagnosis confirmed by swab culture taken at a clinic showed Aspergillus flavus. The main treatment in such cases is usually a combination of surgical and medical therapy. CONCLUSIONS We present the second case report of allergic fungal otomastoiditis, showing an allergic reaction to fungi in the middle ear and formation of mucin in a symptomatic patient. Otolaryngologists need to be aware of the presence of this sensitivity, both in the clinic and the operating theater.

Intracranial Complications of Chronic Otitis Media: Why Does It Still Occur?

Intracranial complications secondary to chronic otitis media (COM) include otogenic brain abscess and sinus thrombosis. Intravenous antibiotics and imaging have significantly reduced the incidence of intracranial complications secondary to COM. However, the same does not apply to a developing country like Malaysia, which still experiences persisting otogenic complications. This case series describes 3 patients with COM and intracranial complications. All 3 patients had COM with mastoiditis, with 1 of the 3 having a cholesteatoma. Postulated reasons for the continued occurrence include poor access to health care, poor compliance with medication, and the lack of pneumococcal vaccination during childhood. In conclusion, public awareness and a timely specialty referral can reduce the incidence of intracranial complications of COM.

Sudden Diplopia at a Pediatric Emergency Department: A Case of Gradenigo Syndrome in a Child.

OBJECTIVE: Otitis media, facial pain in trigeminal region, and ipsilateral abducens nerve palsy clinically define Gradenigo syndrome, a rare but serious complication of suppurative middle ear infection. Radiological investigation is required to confirm petrous apex involvement and to exclude further consequences as sinus thrombosis, meningitis, and intracranial abscess. METHODS/RESULTS: We report the case of an 8-year-old child referred to our emergency department for recurrent headache and sudden strabismus. Clinical evidence of suppurative otitis media raised the suspicion for Gradenigo syndrome, definitively confirmed at computed tomography and magnetic resonance imaging scans. Conservative treatment alone enabled complete clinical and radiological remission, without long-term sequelae. CONCLUSIONS: Despite being a rare condition, Gradenigo syndrome should be taken into account as potential differential diagnosis in children referred to emergency department for recurrent headache and strabismus. An accurate anamnesis to document recent ear infection is mandatory to orientate the diagnosis and focus radiological investigations. Early recognition and timely intervention may allow conservative management to succeed, avoiding the need for surgery and serious sequelae.