Nonsyndromic Craniosynostosis Is Associated with Increased Risk for Psychiatric Disorders

Abstract

Background: Craniosynostosis is one of the most common craniofacial malformations demanding surgical treatment in infancy. Data on overall psychiatric morbidity among children with nonsyndromic craniosynostosis remain limited. This study investigated the risk of psychiatric disorders in nonsyndromic craniosynostosis. Methods: The authors reviewed a register-based cohort of all individuals born with nonsyndromic craniosynostosis in Sweden between 1973 to 1986 and 1997 to 2012 (n = 1238). The nonsyndromic craniosynostosis cohort was compared with a matched community cohort (n = 12,380) and with unaffected full siblings (n = 1485). The authors investigated the risk of psychiatric disorders, suicide attempts, and suicides by using Cox regression adjusted for perinatal and somatic factors, season and birth year, sex, parental socioeconomic factors, and parental psychiatric disorders. Results: Children with nonsyndromic craniosynostosis had a higher risk of any psychiatric disorder (adjusted Cox-derived hazard ratio, 1.70; 95 percent CI, 1.43 to 2.02), including intellectual disability (adjusted Cox-derived hazard ratio, 4.96; 95 percent CI, 3.20 to 7.70), language disorders (adjusted Cox-derived hazard ratio, 2.36; 95 percent CI, 1.57 to 3.54), neurodevelopmental disorders (adjusted Cox-derived hazard ratio, 1.30; 95 percent CI, 1.01 to 1.69), and other psychiatric disorders (adjusted Cox-derived hazard ratio, 1.43; 95 percent CI, 1.11 to 1.85). Full siblings with nonsyndromic craniosynostosis were more likely, in the crude analyses, to be diagnosed with any psychiatric disorder, including intellectual disability, language disorders, and neurodevelopmental disorders compared with nonaffected siblings. The higher risk for any psychiatric disorder and intellectual disability remained after adjusting for confounders. Conclusions: Children with nonsyndromic craniosynostosis demonstrated higher risks of any psychiatric disorder compared with children without nonsyndromic craniosynostosis. This risk cannot fully be explained by familial influences (i.e., genetic or environmental factors). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Longitudinal outpatient and school-based service utilization among children with nonsyndromic craniosynostosis

“…9 Similarly, a retrospective registrybased review of children in the nonsyndromic craniosynostosis community in Sweden showed the rate was 14.4 percent, similar to the 11 percent rate of psychiatric assistance reported here. 10 The prevalence of behavioral difficulties (33 percent, teacher-reported) and individualized education plans (29.7 percent, parent-reported) from separate cohorts of children were nearly identical to those reported in the current cohort (34.7 percent and 30.6 percent, respectively). 11,27 Thus, the data from the current study do not appear to differ dramatically from that which has been previously published.…”

“…Nonsyndromic craniosynostosis has been associated with increased risk of psychosocial difficulties, behavioral abnormalities, and learning disabilities. [3][4][5][6][7][8][9][10][11] However, the actual longitudinal impact of these deficits on children and their families remains poorly understood. The data from the current study help build on previous literature by documenting the rate of supportive service use across childhood in a cohort of patients with nonsyndromic craniosynostosis.…”

Sagittal Synostosis and Its Association With Cognitive, Behavioral, and Psychological Functioning

“…25 Moreover, children’s concerns with their appearance may lead to social isolation and anxiety, which can affect their psychological well-being, although research in this area is sparse. 26 , 27 , 28 , 29 …”

“…25 Moreover, children's concerns with their appearance may lead to social isolation and anxiety, which can affect their psychological well-being, although research in this area is sparse. [26][27][28][29] Interpretation of the aforementioned findings is, however, complicated by the use of diverse outcome measures (eg, Bayley Scales of Infant Development [BSID] and Wechsler Preschool and Primary Scale of Intelligence), [30][31][32][33] in addition to differences in the surgical status of samples (eg, conservatively managed vs presurgical vs postsurgical). Moreover, pathological differences in the way that different sutures fuse-with midline sutures (ie, sagittal and metopic) being more vulnerable to some genetic variations (eg, SMAD6) than coronal and lambdoid sutures-could lead to differential outcomes.…”

“…Parental reports indicate that behavioral problems are common, with 26% of children with SS exhibiting externalizing traits (eg, restlessness, temper tantrums) and 14% with internalizing behaviors (eg, fears) . Moreover, children’s concerns with their appearance may lead to social isolation and anxiety, which can affect their psychological well-being, although research in this area is sparse …”