Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report
Background: A positive prenatal history of maternal fever has been found to express as a range of defects in infants including limb reduction, central nervous system defects, facial dysmorphogenesis and foetal death. Oromandibular limb hypogenesis syndrome is a rare spectrum of congenital disorders...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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EDP Sciences
2022-01-01
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| Series: | Journal of Oral Medicine and Oral Surgery |
| Subjects: | |
| Online Access: | https://www.jomos.org/articles/mbcb/full_html/2022/02/mbcb210103/mbcb210103.html |
| _version_ | 1818024408050040832 |
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| author | Sahoo Nandakishore Rana Praneet K. Semi Ravinder S. Shetty Lakshmi |
| author_facet | Sahoo Nandakishore Rana Praneet K. Semi Ravinder S. Shetty Lakshmi |
| author_sort | Sahoo Nandakishore |
| collection | DOAJ |
| description | Background: A positive prenatal history of maternal fever has been found to express as a range of defects in infants including limb reduction, central nervous system defects, facial dysmorphogenesis and foetal death. Oromandibular limb hypogenesis syndrome is a rare spectrum of congenital disorders characterised by malformations of the tongue, mandible, maxilla and limbs. They present as sporadic cases with extremely low incidence and seldom occur with associated syngnathia. Syngnathia can manifest as fibrous, bony or in combination. Observation: Here we represent a rare case of Oromandibular limb hypogenesis syndrome type IV E with combination of syngnathia, cleft palate, retrognathia and hypoglossia-hypodactylomelia. An early surgical release of syngnathia was undertaken on having features of failure to thrive, high risk of aspiration and related complications due to enteral feeding. Conclusion: Delaying the surgical procedure could result in growth restriction and progressive ankylosis of the Temporomandibular joint (TMJ). It is essential to document the singular variant of Oromandibular limb hypogenesis syndrome (OLHS) to the medical literature. |
| first_indexed | 2024-12-10T03:59:44Z |
| format | Article |
| id | doaj.art-38219c196f764963a594c5abc1897566 |
| institution | Directory Open Access Journal |
| issn | 2608-1326 |
| language | English |
| last_indexed | 2024-12-10T03:59:44Z |
| publishDate | 2022-01-01 |
| publisher | EDP Sciences |
| record_format | Article |
| series | Journal of Oral Medicine and Oral Surgery |
| spelling | doaj.art-38219c196f764963a594c5abc18975662022-12-22T02:03:00ZengEDP SciencesJournal of Oral Medicine and Oral Surgery2608-13262022-01-012821710.1051/mbcb/2021049mbcb210103Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case reportSahoo Nandakishore0Rana Praneet K.1Semi Ravinder S.2Shetty Lakshmi3Department of Oral & Maxillofacial Surgery, Armed Forces Medical CollegeDepartment of Oral & Maxillofacial Surgery, Armed Forces Medical CollegeDepartment of Oral & Maxillofacial Surgery, Command Military Dental Centre (SC)Department of Oral & Maxillofacial Surgery, Dr. D.Y. Patil Vidyapeeth Dental College & HospitalBackground: A positive prenatal history of maternal fever has been found to express as a range of defects in infants including limb reduction, central nervous system defects, facial dysmorphogenesis and foetal death. Oromandibular limb hypogenesis syndrome is a rare spectrum of congenital disorders characterised by malformations of the tongue, mandible, maxilla and limbs. They present as sporadic cases with extremely low incidence and seldom occur with associated syngnathia. Syngnathia can manifest as fibrous, bony or in combination. Observation: Here we represent a rare case of Oromandibular limb hypogenesis syndrome type IV E with combination of syngnathia, cleft palate, retrognathia and hypoglossia-hypodactylomelia. An early surgical release of syngnathia was undertaken on having features of failure to thrive, high risk of aspiration and related complications due to enteral feeding. Conclusion: Delaying the surgical procedure could result in growth restriction and progressive ankylosis of the Temporomandibular joint (TMJ). It is essential to document the singular variant of Oromandibular limb hypogenesis syndrome (OLHS) to the medical literature.https://www.jomos.org/articles/mbcb/full_html/2022/02/mbcb210103/mbcb210103.htmlolhssyngnathiahypoglossia-hypodactyliahypoplastic syndactylycase report |
| spellingShingle | Sahoo Nandakishore Rana Praneet K. Semi Ravinder S. Shetty Lakshmi Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report Journal of Oral Medicine and Oral Surgery olhs syngnathia hypoglossia-hypodactylia hypoplastic syndactyly case report |
| title | Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report |
| title_full | Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report |
| title_fullStr | Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report |
| title_full_unstemmed | Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report |
| title_short | Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report |
| title_sort | oromandibular limb hypogenesis syndrome a singular variation of a rare syndrome a case report |
| topic | olhs syngnathia hypoglossia-hypodactylia hypoplastic syndactyly case report |
| url | https://www.jomos.org/articles/mbcb/full_html/2022/02/mbcb210103/mbcb210103.html |
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