Risk factors associated with the occurrence of frontoethmoidal encephalomeningocele

ObjectivesTo determine factors associated with the occurrence of frontoethmoidal encephalomeningocele (FEEM), a congenital defect with unique geographical distribution.MethodsThe subjects of this study were 160 unrelated cases of FEEM. Subjects were recruited between 1999 and 2006 from 15 medical centers throughout Thailand. Data obtained from FEEM cases were analyzed and compared with data from 349 cases of oral clefts studied in the same centers and during the same time and those from the general population (GP) taken in 2003.ResultsAbout 52% of FEEM cases had brain anomalies which were not different among types of FEEM. We found familial aggregation reflected by an increased risk to siblings. All of the FEEM cases were of Thai nationality and came from low socioeconomic status. Seven FEEM cases had amniotic rupture sequences. Compared with oral clefts, advanced maternal age (OR: 1.08, 95% CI: 1.02–1.15) was found to be associated with FEEM. In addition, the interpregnancy interval between the FEEM cases and their previous siblings was significantly longer than that of the oral cleft patients and unaffected sibs (OR: 1.17, 95% CI: 1.06–1.28).ConclusionsLow socioeconomic status, advanced maternal age, and a long interpregnancy interval may lead to an unfavorable intrauterine environment which, with a certain genetic background such as Thai ethnicity, could contribute to the occurrence of FEEM.