Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome

Bosma arhinia microphthalmia (BAM) is a very rare illness, with fewer than 100 cases documented worldwide. Criteria for diagnosis of BAM syndrome include congenital absence of nose (arhinia) or hypoplasia, hypogonadotropic hypogonadism, and eye defects (microphthalmia) in males. Our patient was a ma...

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Bibliographic Details
Published inTurkish Journal of Plastic Surgery Vol. 32; no. 1; pp. 38 - 40
Main Authors Kapil S Agrawal, Armaan Khosa, Vidhi Mehta, Vinita Puri
Format Journal Article
LanguageEnglish
Published Wolters Kluwer Medknow Publications 01.01.2024
Subjects
arhinia
pedicled flap
reconstruction
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Summary:Bosma arhinia microphthalmia (BAM) is a very rare illness, with fewer than 100 cases documented worldwide. Criteria for diagnosis of BAM syndrome include congenital absence of nose (arhinia) or hypoplasia, hypogonadotropic hypogonadism, and eye defects (microphthalmia) in males. Our patient was a male child of 4 years of age who was brought by parents with left nasal arhinia for reconstruction. We devised three-layered nasal reconstruction for the child. Flip-over flap from the other half of the nose was used for the nasal lining, conchal cartilage grafts for the nasal framework, and pedicled forehead flap for skin cover.
ISSN:2528-8644
DOI:10.4103/tjps.tjps_53_23