Bilateral congenital cholesteatomas are rare entities. Nine cases have been previously described in the literature. Many different etiologies for the development of congenital cholesteatoma have been proposed. The case of a five-year-old boy with bilateral congenital cholesteatoma is discussed. A lesion of the left ear was apparent clinically. However, the right-sided lesion was silent and was demonstrated only by radiologic exam. Theories of pathogenesis are reviewed.
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Department of Otolaryngology, Head and Neck Surgery, Kobe University Faculty of Medicine, Hyogo, Japan.
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Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul, Korea.
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- First branchial cleft anomalies (FBCAs) are rare congenital disorders that can cause variable symptoms and complex anatomical issues, often misdiagnosed as other conditions like cholesteatoma.
- A 4-year-old girl presented with a painless mass in her left ear, leading to a thorough diagnostic process involving multiple imaging techniques that confirmed an FBCA.
- Surgical intervention to resect the fistula was successful, with no postoperative complications or recurrence, emphasizing the need for precise diagnosis and treatment in such cases.
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