Issue |
J Oral Med Oral Surg
Volume 28, Number 2, 2022
|
|
---|---|---|
Article Number | 17 | |
Number of page(s) | 5 | |
DOI | https://doi.org/10.1051/mbcb/2021049 | |
Published online | 08 April 2022 |
Case Report
Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report
1
Department of Oral & Maxillofacial Surgery, Armed Forces Medical College,
Pune,
Maharashtra,
India
2
Department of Oral & Maxillofacial Surgery, Command Military Dental Centre (SC),
Pune,
Maharashtra,
India
3
Department of Oral & Maxillofacial Surgery, Dr. D.Y. Patil Vidyapeeth Dental College & Hospital,
Pimpri, Pune,
Maharashtra,
India
* Correspondence: praneetkrana@gmail.com
Received:
14
April
2021
Accepted:
18
October
2021
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.
Key words: OLHS / syngnathia / hypoglossia-hypodactylia / hypoplastic syndactyly / case report
© The authors, 2022
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Current usage metrics show cumulative count of Article Views (full-text article views including HTML views, PDF and ePub downloads, according to the available data) and Abstracts Views on Vision4Press platform.
Data correspond to usage on the plateform after 2015. The current usage metrics is available 48-96 hours after online publication and is updated daily on week days.
Initial download of the metrics may take a while.