Oromandibular Limb Hypogenesis Spectrum (OMLHS) [OMIM 103300] is a rare disease characterized by congenital defects of the face, mandible, tongue and hypoplastic limbs. The exact etiology remains unknown. We present two Filipino children diagnosed with OLMHS. Patient 1 is a 2-year-old female noted to have micrognathia, sygnathia and hypoplasia of distal extremities. Patient 2 is a 6-year-old male with hypoplastic mandible, micrognathia, micromelia of both lower extremities and syndactyly of hands. The early recognition of this disease is important so that early surgical correction of deformities particularly the hypoplastic mandible be addressed to avoid complications such as respiratory distress and feeding difficulties.
Ankyloglossia ; Hanhart syndrome

Ankyloglossia is a congenital condition characterized by a short lingual frenulum, which may result in the restriction of tongue movement and function. Considerable controversy regarding the diagnosis, clinical significance, and management of the condition remains, and great variations in practice have been recorded. Indeed, attitudes toward ankyloglossia differ among professional groups, and opinions may vary remarkably even among those within the same specialty. This article reviews the embryology, genetics, diagnosis, clinical presentation, and treatment of ankyloglossia to help physicians better understand and treat the condition.
Ankyloglossia ; Humans ; Infant ; Infant, Newborn

Objective: To report a case of isolated ankyloglossia superior in a one-month-old boy.

Methods:

Design: Case Report

Setting: Tertiary Government Training Hospital 

Patient: One

Results: A one-month-old boy with failure to thrive, feeding difficulties and a palatoglossal band that limited mouth opening and anterior posturing of the tongue was diagnosed to have non-syndromic ankyloglossia superior and underwent surgical transection of the fibrous ankylosis under intravenous sedation due to difficulty of insinuating an endotracheal tube orally. Direct latch breastfeeding was successfully adapted from the second to tenth postoperative day before they were lost to follow up.

Conclusion: Despite a complicated pediatric airway, coordinated surgical and anesthesia management successfully restored tongue function and mouth opening to allow effective feeding.

Human ; Male ; Infant (a Child Between 1 And 23 Months Of Age) ; Ankyloglossia Superior ; Tongue-palate Fusion ; Palatoglossal Ankylosis ; Oral Synechiae