Long-term Effect of Oral Cleft Depends on Type

Susan London

September 28, 2016

The prognosis for infants born with oral clefts in the absence of any other congenital anomalies largely depends on the type of cleft, according to a population-based cohort study conducted among nearly 1.5 million individuals in Norway.

At approximately 30 years of age, individuals born with a cleft lip, with or without cleft palate, did not have an elevated risk for death and had fairly small increases, if any, in morbidity compared with unaffected counterparts, according to results reported in an article published online September 26 in JAMA Pediatrics.

However, those born with cleft palate alone had a much rockier course, with about three times the risk for death from medical causes relative to that seen in unaffected peers. They also had sharply higher risks for conditions such as intellectual disability, cerebral palsy, and epilepsy.

"The present results are good news for parents of children with isolated cleft lip," write Erik Berg, MD, from the Department of Global Public Health and Primary Care, University of Bergen, Norway, and colleagues. In contrast, "[t]horough screening for other underlying conditions in [infants with cleft palate alone] is highly recommended from a young age to ensure necessary interventions and treatment as early as possible."

The authors continue, "This information should be provided to genetic counselors, parents of children with clefts, and health care workers involved in the treatment or follow-up of these children," they further recommend.

"The current population-based study provides an important contribution, highlights the potential for linking large data sets, identifies relevant end points, and motivates the continued work to conduct quality research with an ultimate goal to improve cleft-related health care," write Carrie L. Heike, MD, and Kelly N. Evans, MD, both from the Craniofacial Center at Seattle Children's Hospital, the Center of Clinical and Translational Research at Seattle Children's Research Institute, and the Department of Pediatrics at the University of Washington, in an accompanying editorial.

"Further investigations to explore the association between treatments and holistic outcomes and to incorporate information from patient perspectives will augment such large scale, population-based quantitative approaches and allow for a more holistic understanding of adult outcomes," they maintain.

For the current study, the investigators compared survival and health outcomes between 2337 individuals with an isolated oral cleft and 1,413,819 patients without an oral cleft born between 1967 and 1992 and followed through 2010, when they were between 18 and 43 years old. In the cleft group, 695 individuals had cleft lip only, 918 had both cleft lip and palate, and 724 had cleft palate only.

Results showed that relative to the unaffected individuals, the group with cleft lip only did not have any significant increase in morbidity or mortality by young adulthood, Dr Berg and colleagues report. Individuals born with both cleft lip and cleft palate had moderately elevated risks for intellectual disability (relative risk [RR], 2.2; 95% confidence interval [CI], 1.2 - 4.1) and cerebral palsy (RR, 2.6; 95% CI, 1.1 - 6.2), and no increase in the risk for death.

However, individuals having cleft palate only were much more likely to have died from medical causes (hazard ratio, 3.4; 95% CI, 2.1 - 5.7). They also had elevated risks for intellectual disability (RR, 11.5; 95% CI, 8.5 - 15.6), anxiety disorders (RR, 2.9; 95% CI, 1.3 - 6.5), autism spectrum disorders (RR, 6.6; 95% CI, 2.8 - 15.7), severe learning disabilities (RR, 10.6; 95% CI, 5.5 - 20.2), cerebral palsy (RR, 4.8; 95% CI, 2.3 - 10.0), epilepsy (RR, 4.9; 95% CI, 2.2 - 10.8), and muscular or skeletal disorders (RR, 2.7; 95% CI, 1.4 - 5.4).

The investigators and editorialists have disclosed no relevant financial relationships.

JAMA Pediatrics. Published online September 26, 2016. Article full text, Editorial extract

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