The embryonic head development, including the formation of dental structures, is a complex and delicate process guided by specific genetic programs. Genetic changes and environmental factors can disturb the execution of these programs and result in abnormalities in orofacial and dental structures. Orofacial clefts and hypodontia/ oligodontia are examples of such abnormalities frequently seen in...

During craniofacial development, the Hedgehog (HH) signaling pathway is essential for mesodermal tissue patterning and differentiation. The HH family consists of three protein ligands: Sonic Hedgehog (SHH), Indian Hedgehog (IHH), and Desert Hedgehog (DHH), of which two are expressed in the craniofacial complex (IHH and SHH). Dysregulations in HH signaling are well documented to result in a wide...

Strabismus may result from abnormal innervation, structure, or function of the extraocular muscles. Abnormalities of the orbital bones or masses within the orbit may also cause strabismus due to indirect effects on the extraocular muscles. This paper reviews some disorders of the orbit that are associated with strabismus, including craniofacial malformations, orbital masses, trauma, and anomalo...

We describe a two generation family with variable ulnar and radial ray reduction and midline craniofacial abnormalities. The features suggest a diagnosis of Weyers' ulnar ray/oligodactyly syndrome originally described in two isolated cases. Syndromes of ulnar ray reduction are briefly reviewed and the relationship between limb bud and midline development discussed.

Ablepharon syndrome is an extremely rare genetic problem that causes severe craniofacial deformities and numerous other abnormalities of the face, genitalia, and skin. The literature regarding this condition is scarce. We present a case of this syndrome with dental manifestations, not reported before, and discuss its characteristics in order to increase the knowledge of this condition among the...

The craniofacial abnormalities of popliteal pterygium syndrome present unique challenges to the pediatric anesthetist. Congenital fibrous bands between the maxilla and mandible limit mouth opening and the tongue may be fused to the palate. We describe the use of the Shikani Optical Stylet, a novel fiberoptic endoscope, in the airway management of a neonate with popliteal pterygium syndrome and ...

The Dubowitz syndrome is a rare, autosomal recessive disorder including intrauterine growth retardation, craniofacial abnormalities, mental retardation and eczematous skin eruption. Ocular problems occur in about 20%: strabismus, blefarophimosis, ptosis, telecanthus and epicanthal folds being the most frequent ones. We present a three years old girl, diagnosed with Dubowitz syndrome, with sudde...

BACKGROUND The RSK2 gene is responsible for Coffin-Lowry syndrome, an X-linked dominant genetic disorder causing mental retardation, skeletal growth delays, with craniofacial and digital abnormalities typically associated with this syndrome. Craniofacial and dental anomalies encountered in this rare disease have been poorly characterized. METHODOLOGY/PRINCIPAL FINDINGS We examined, using X-Ra...

PURPOSE: Hard palate trauma is a relatively infrequent occurrence compared with other craniofacial injuries. Several techniques of hard palate fracture repair have been described. To date, there is no consensus on the optimal management of this type of fracture. The purpose of this study was to compile and analyze studies describing hard palate fracture repair techniques with

OBJECTIVE The aim of the present study was to investigate the incidence of occipitalization of the atlas among Thai dried skulls, in order to contribute to baseline awareness of this condition. MATERIALS AND METHODS The skulls of 633 adult Thais from the collection maintained in the Department of Anatomy, Faculty of Medicine, Khon Kaen University, Thailand, were examined for evidence of occip...