Epidemiology of orofacial clefts in the East of ireland in the 25-year period 1984-2008.

Objective : To describe the epidemiology of orofacial clefts in the east of Ireland. Design and Setting : A descriptive epidemiologic study on 851 cases of orofacial cleft identified over a 25-year period from 1984 to 2008 from more than 500,000 births. Results : There were 438 (51.5%) cases of cleft lip with or without cleft palate and 413 (48.5%) cases of cleft palate. The total birth prevalence was 16.0 per 10,000 births for all orofacial clefts, 8.2 for cleft lip with or without cleft palate, and 7.8 for cleft palate. Of all cases, 63.7% (542/851) occurred as isolated anomalies, 21.5% (183/851) were associated with multiple anomalies, and 14.8% (126/851) were associated with a syndrome or chromosomal anomaly. A significantly increasing trend over the 25-year period was observed for cleft lip with or without cleft palate associated with syndromes or chromosomal anomalies among mothers younger than 35 years but not in those older than 35 years. Conclusion : A slightly higher rate of orofacial clefts was observed in the east of Ireland than was observed in European and multinational studies during the study period, and there were higher rates of cleft palate. The rising trend in the proportion of mothers aged 35 years or older in Ireland is not contributing significantly to orofacial clefts associated with chromosomal syndromes.

Behavioral, developmental, and educational problems in children with nonsyndromic trigonocephaly.

OBJECT: The neurobehavioral morbidity of nonsyndromic trigonocephaly is incompletely understood. The purpose of this study was twofold: first, to assess the degree of developmental, educational, and behavioral problems in patients with nonsyndromic trigonocephaly and second, to establish whether patients with mild degrees of trigonocephaly had a lower frequency of such problems. METHODS: The authors performed an observational study of the frequency of developmental, educational, and behavioral problems in 63 children with trigonocephaly at the National Craniofacial Centre in the Republic of Ireland between 1989 and 2004. The parents of the children completed a follow-up questionnaire. Thirty percent of patients had a mild form of trigonocephaly and were treated conservatively. The remainder underwent surgical correction. Speech and/or language delay was reported in 34% of the children. Thirty-three percent of the children needed to be assessed by a school psychologist, and 47% were receiving remedial or resource hours within the school system. Twenty percent of children required a special needs classroom assistant because of behavioral issues, and 37% of parents expressed concerns about their child's behavior. There were no statistically significant differences between children treated with surgery and those who had a mild deformity and were treated conservatively. CONCLUSIONS: Nonsyndromic trigonocephaly is associated with a high frequency of developmental, educational, and behavioral problems. The frequency of these problems is not related to the severity of the trigonocephaly.

Non-syndromic trigonocephaly: surgical decision making and long-term cosmetic results.

BACKGROUND: Surgical correction is often required for cosmetic correction of trigonocephaly. The purpose of this paper was to report the long-term aesthetic outcome as appreciated by the parents/patients themselves. The self-evaluation of cosmetic outcome after trigonocephaly correction has not previously been reported. The management and different surgical techniques utilized over a 16-year period are discussed. MATERIALS AND METHODS: An observational study was undertaken of the clinical outcome, operative data, complications and cosmetic satisfaction of these trigonocephaly patients. The parents/child were asked to rate their satisfaction with the cosmetic outcome both in terms of head shape and scar appearance, on a five-point scale (excellent-5, very good-4, good-3, fair-2 and poor-1). RESULTS: Sixty-three patients presented with non-syndromic trigonocephaly over the 16 years. Nineteen of 63 had a mild form of trigonocephaly and were managed conservatively. The remainder underwent surgical correction. Forty-two of 44 (95%) underwent fronto-orbital advancement with either barrel staving (26/44) or frontal bone rotation/re-modelling (16/44), with 2 of 44 having burring of the metopic ridge. Head shape rating was regarded as excellent in 25 of 63 (40%), very good in 18 of 63 (28%), good in 18 of 63 (28%) and fair in 2 of 63 (4%). Of those that underwent surgery, the scar was zigzag in 32 of 44 and straight in 12 of 44. Scar was rated as being excellent in 21 of 44 (48%), very good in 12 of 44 (28%) good in 11 of 44 (24%). CONCLUSIONS: Metopic synostosis can result in varying degrees of severity. Milder forms can be treated conservatively, with more severe forms requiring both frontal bone re-modelling and fronto-orbital advancement. Surgical correction results in a high level of patient/parent satisfaction.