Background: Trigonocephaly results from premature closure of the metopic suture. Recently, there has been increasing interest in cognitive, behavioral and speech problems associated with trigonocephaly. To date, there have been no prospective multidisciplinary evaluations to assess these issues in this patient population. This study was performed in order to establish the incidence of associated developmental, behavioral and speech anomalies in patients presenting with trigonocephaly.

Methods: Between 1990 and 2006, all patients presenting with trigonocephaly at St Justine University Hospital - Craniofacial Center, were prospectively assessed by a qualified specialized psychologist and speech therapist at two years of age, five to six years of age and eight to ten years of age. Medical charts of all these patients were retrospectively reviewed.

Results: Ninety two children with trigonocephaly were treated and followed between 1990 and 2006. Of these, twenty seven were excluded from the study because of incomplete assessments. Among the sixty five patients included (49 M, 16 F), 68% (44/65) were operated on and 32% (21/65) were observed. 83% (54/65) of the cases were isolated deformities, 6% (4/65) were syndromic, and 11% (7/65) had associated extra-cranial anomalies. The average age of patients at the time of last assessment was 6.7 years (ranging from 2 – 17 years). 29% (19/65) of patients had normal assessments, whilst 71 % (46/65) had documented developmental, behavioral, language or speech anomalies at some point in their course of follow-up. This incidence is significantly above that seen in the general population. Among the 44 operated children, normal assessments were seen in 25% (11/44), and abnormal in 75% (33/44). Among the 21 observed children, 38% (8/21) had normal assessments and 62% (13/21) had abnormal assessments. These results were not statistically significant (p > 0.05).

Conclusions: Trigonocephaly is associated with a higher incidence of developmental, behavioral, language and speech anomalies. Surgery does not seem to have an impact on this outcome. Early recognition, follow up, and appropriate specialized treatment is warranted in order to optimize long term development.