Case Reports in Dentistry

Case Report

Craniodentofacial Manifestations in a Rare Syndrome: Orofaciodigital Type IV (Mohr-Majewski Syndrome)

Table 1

Clinical features of current family and patients reported in the literature.
AnomalyIV-3IV-71
[11]		2
[11]		3
[12]		4
[15]		5
[16]		6
[16]		7
[17]		8
[18]		9
[2]		10
[19]		11
[20]		12
[21]		13
[22]		14
[22]
Orofacial                
 Frenula−+++++++−−+−−?++
 Lobulated tongue+++?−−++++++++++
 (Pseudo) cleft lip−−++−−−−++−−−+++
 Cleft palate−+−−+−−+++++++++
 Micrognathia−++++++++++++?++
 Hypertelorism−++++++++++−??++
 Broad nasal tip−+?++++++++?????
 Dental anomalies??????+?+???????
Hand                
 Preaxial polydactyly+−−−−−−+++−−−−++
 Postaxial polydactyly++−−++++++++++++
 Syndactyly++−−++++−+++++++
Foot                
 Preaxial polydactyly++++++++++++−−++
 Postaxial polydactyly+++−++++++++−−++
 Clubfoot+−−−++++++−+−−−−
Skeletal                
 Short ribs−−+−−−−−−−−+−+−−
 Short tibia++++++−−++++++++
 Short radius/ulna−−??−−−−−−++++++
Visceral                
 Heart defects−++???−−+−+−−+++
 Renal defects++????−?−−++++−−
 Larynx hypoplasia−+??++++−−++−−++
 Epiglottis hypoplasia−++?+++++−+++−++
 Larynx hamartoma−−????+?−−−−−−++
CNS                
 Brain malformation−+??+?????++++++
 Mental retardation?+??+++?????????
Other                
 Ambiguous or hypoplastic genitalia−−?+−−−−−−++−−++
 Deafness?+?+++??+???+???
 Early deathYesNoYesNoNoNoNo?YesYesYesYesYesYesYesYes