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Congenital spontaneous otogenic cerebrospinal fluid leak as a cause of recurrent meningitis in a 9-year-old child – case report and literature review

100%

Amernik K., Jaworowska E.

Polski Przegląd Otorynolaryngologiczny

2022

vol. 11

issue 1

41-44

Introduction: Congenital otogenic CSF leak is a rare cause of meningitis. The underlying reason may be a defect of the fundus of internal auditory meatus with pathological communication with the inner ear and CSF leak to middle ear space. This condition requires surgical closure. Case report: A 9-year-old boy with a history of recurrent meningitis – 3 episodes. During the last episode, β-2 transferrin test from ear fluid was positive. The patient complained of leakage of clear fluid through the nose on the right side. Otoscopy revealed a bulging tympanic membrane on the right side. The right ear was deaf with normal hearing on the left side. HRCT revealed congenital deformity of the right inner ear. Right side lateral petrosectomy with blind sac closure of the right external auditory canal was performed. During surgery there was an extensive CSF leak through the oval window niche due to a lack of the stapes footplate in the anterior part. Recovery was uneventful. After 24 months of follow up, no more leakage through the nose was observed. Control magnetic resonance performed one year after surgery showed no CSF in the mastoid cavity. Discussion: Inner ear malformations are a rare cause of meningitis, and the most common sites of congenital otogenic CSF leakage include the oval and round window. Surgical closure is the only solution in such cases. Several methods of sealing the inner ear space were described in the literature. Vestibular packing and stapedectomy were most frequently used. However, recurrences were stated in up to 1/2 of cases. Such a risk can be limited with lateral petrosectomy. For this reason, LP can be considered as the treatment of choice in congenital spontaneous otogenic cerebrospinal fluid leak. Conclusion: Every case of recurrent meningitis in children should be investigated with a suspicion of congenital ear malformation with spontaneous CSF leakage. Lateral petrosectomy is a safe and effective procedure for cases of otogenic CSF leak with a low risk of recurrence.

Second branchial cleft fistula in a child with genetically confirmed branchio-oto-renal (BOR) syndrome

75%

Remjasz A., Clarós P., Clarós P.

Polski Przegląd Otorynolaryngologiczny

2019

vol. 8(1)

63-70

Preface: Branchial cleft anomalies constitute 32% to 45% of all neck pathologies in the pediatric population. These disorders may be a part of a branchio-oto-renal (BOR) syndrome, characterized by branchial arch abnormalities, preauricular pits, hearing impairment, and various types of renal disorders. Usually, the treatment of a branchial fistula does not necessarily require extensive diagnostics before the treatment. However, in some cases, branchial cleft fistulas may occur together with other congenital disorders. A case report: The aim of this study is to present diagnostic and therapeutic difficulties in a 4-year old male patient with a complete second branchial cleft fistula and additional congenital, bilateral hearing loss. The course of the disease, diagnostic difficulties, and its treatment are presented. Genetic counseling finally confirmed the mutation of an EYA1 gene, responsible for the occurrence of BOR syndrome. The child undergone a total fistula resection without any complications during hospitalization or in the postoperative treatment. Summary: Presentation of this clinical case was intended primarily to remind that such defects may occur in the association with other anomalies, for example, deafness or renal disorders. When dealing with such patients, it is worth paying attention to the detailed examination and diagnostics, including genetic counseling, hearing tests or abdominal ultrasound. Special radiological imaging should also be performed in case of the unusual course of the fistula or probable proximity to vital structures, what is essential during qualification for surgery. Finally, we wanted to describe alternative methods of treatment for the standard surgical technique that may be used in a selected group of patients.

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2 Polski Przegląd Otorynolaryngologiczny

1 2022

1 2019

1 Amernik K.

1 Clarós P.

1 Jaworowska E.

1 Remjasz A.

Search results

Congenital spontaneous otogenic cerebrospinal fluid leak as a cause of recurrent meningitis in a 9-year-old child – case report and literature review

Second branchial cleft fistula in a child with genetically confirmed branchio-oto-renal (BOR) syndrome