AI Article Synopsis
- - Congenital arhinia-microphthalmos syndrome, or BOSMA syndrome, is a rare condition marked by the complete lack of one or both nasal cavities, along with various facial and eye-related abnormalities.
- - The absence of the nasolacrimal duct leads to issues with tear drainage, manifesting as dilated lacrimal sacs or mucoceles.
- - A team of specialists from several fields, including genetics and surgery, should collaborate early in treatment to ensure comprehensive care for affected individuals.
Article Abstract
Congenital arhinia-microphthalmos syndrome or BOSMA syndrome is an exceptionally rare clinical syndrome characterized by unilateral or bilateral complete absence of the nasal cavity associated with several craniofacial, ocular, and systemic anomalies. Lacrimal drainage anomalies are secondary to absent nasolacrimal duct and usually present as dilated lacrimal sac or mucoceles. While navigation-guided dacryocystorhinostomies into the contralateral nasal cavity are described for unilateral arhinia, the way forward for the complete absence of the nose and nasal cavity is still unclear. A multidisciplinary team from the specialties of genetics, plastic surgery, ophthalmic plastics and reconstructive surgery, otorhinolaryngology, and endocrinology should get involved very early on for better continuity of care.
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| http://dx.doi.org/10.1080/01676830.2024.2346568 | DOI Listing |
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