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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-01-01
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| Series: | Turkish Journal of Plastic Surgery |
| Subjects: | |
| Online Access: | http://www.turkjplastsurg.org/article.asp?issn=1300-6878;year=2024;volume=32;issue=1;spage=38;epage=40;aulast=Agrawal |
| _version_ | 1827292685834125312 |
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| author | Kapil S Agrawal Armaan Khosa Vidhi Mehta Vinita Puri |
| author_facet | Kapil S Agrawal Armaan Khosa Vidhi Mehta Vinita Puri |
| author_sort | Kapil S Agrawal |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-24T13:08:59Z |
| format | Article |
| id | doaj.art-d948943d65394db5bc28c949b9780c71 |
| institution | Directory Open Access Journal |
| issn | 2528-8644 |
| language | English |
| last_indexed | 2024-04-24T13:08:59Z |
| publishDate | 2024-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Turkish Journal of Plastic Surgery |
| spelling | doaj.art-d948943d65394db5bc28c949b9780c712024-04-05T05:10:46ZengWolters Kluwer Medknow PublicationsTurkish Journal of Plastic Surgery2528-86442024-01-01321384010.4103/tjps.tjps_53_23Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndromeKapil S AgrawalArmaan KhosaVidhi MehtaVinita PuriBosma 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.http://www.turkjplastsurg.org/article.asp?issn=1300-6878;year=2024;volume=32;issue=1;spage=38;epage=40;aulast=Agrawalarhiniapedicled flapreconstruction |
| spellingShingle | Kapil S Agrawal Armaan Khosa Vidhi Mehta Vinita Puri Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome Turkish Journal of Plastic Surgery arhinia pedicled flap reconstruction |
| title | Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| title_full | Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| title_fullStr | Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| title_full_unstemmed | Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| title_short | Nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| title_sort | nasal reconstruction in a rare case of unilateral arhinia in bosma arhinia microphthalmia syndrome |
| topic | arhinia pedicled flap reconstruction |
| url | http://www.turkjplastsurg.org/article.asp?issn=1300-6878;year=2024;volume=32;issue=1;spage=38;epage=40;aulast=Agrawal |
| work_keys_str_mv | AT kapilsagrawal nasalreconstructioninararecaseofunilateralarhiniainbosmaarhiniamicrophthalmiasyndrome AT armaankhosa nasalreconstructioninararecaseofunilateralarhiniainbosmaarhiniamicrophthalmiasyndrome AT vidhimehta nasalreconstructioninararecaseofunilateralarhiniainbosmaarhiniamicrophthalmiasyndrome AT vinitapuri nasalreconstructioninararecaseofunilateralarhiniainbosmaarhiniamicrophthalmiasyndrome |