A rare variant of oromandibular limb hypogenesis syndrome: a case report of glossopalatal ankylosis

Introduction Oromandibular limb hypogenesis syndrome (OLHS) Type IIIA is an exceptionally rare congenital anomaly, with only a few cases documented in the literature. It is characterized by developmental hypoplasia of the tongue, mandible, and limbs, manifesting as hypoglossia, micrognathia, and hypomelia. Case presentation We report the case of a 9-year-old patient presenting with restricted mouth opening and feeding difficulties. Clinical examination revealed fusion of the tongue to the palate with synechia. Surgical intervention involved the release of the dense fibrous band between the tongue and palate, followed by palatoplasty to correct the cleft palate. The cleft palate was successfully repaired using the Von Langenbeck technique, restoring oral functionality. Conclusion This case highlights the clinical manifestations, differential diagnosis, and surgical management of OLHS Type IIIA. It underscores the importance of timely intervention to improve functional outcomes in patients with this rare syndrome.