Abstract
Objectives: First, to determine the infant mortality rate (IMR) for Dutch patients with isolated oral clefts (OC) as well as for patients with clefts seen in association with other malformations. Second, to conduct a similar analysis per cleft type: cleft lip with or without cleft palate (CP), CP (including Robin sequence). Third, to examine the underlying causes of death.Material and Methods: A retrospective review of the charts of patients with OC born in the period 1997–2011 and treated in three regional cleft centers in the Netherlands.Results: One thousand five hundred thirty patients with OC were born during the study period and treated in the cleft centers. The overall IMR for all clefts was 2.09%, significantly higher than the general Dutch IMR of 0.45%. In a subanalysis per cleft type, the IMRs were 1.22, 1.38, 2.45, and 3.62% for cleft lip, cleft lip with CP, CP, and Robin sequence, respectively. The mortality rates for isolated OC did not differ significantly from the general Dutch rate. Causes of death were congenital malformations of the heart in 40.6%, airway/lungs in 15.6%, nervous system in 15.6%, infectious disease in 12.5%, and other or unknown in 15.6%.Conclusion: The elevated IMR observed in Dutch patients with OC is almost exclusively caused by associated congenital malformations. After diagnosis of an oral cleft an in-depth medical examination and a consult by the pediatrician and clinical geneticist is imperative to instigate the appropriate medical management.
Highlights
Oral clefts (OC) in the form of cleft palate (CP) and cleft lip with or without CP (CLP, CL) are among the most common congenital malformations with a prevalence of 2.7 per 1000 live births in the Netherlands [1]
The elevated infant mortality rate (IMR) observed in Dutch patients with OC is almost exclusively caused by associated congenital malformations
INFANT MORTALITY From 1997 to 2011 5401 infants with OCs were registered with the NVSCA by all regional OC teams in the Netherlands, of which 1420 (26.3%) had CL, 2144 (39.7%) CLP, and 1837 (34.0%) CP
Summary
Oral clefts (OC) in the form of cleft palate (CP) and cleft lip with or without CP (CLP, CL) are among the most common congenital malformations with a prevalence of 2.7 per 1000 live births in the Netherlands [1]. In addition to isolated occurrence, OCs are frequently associated with other congenital malformations and recognized syndromes [2]. Historical studies demonstrated an increased mortality rate amidst infants with OCs in conjunction with further malformations [3,4,5]. After excluding patients with additional malformations in a subanalysis, the odds ratio remained a significant 2.07 (95% CI 1.39–3.09) [6], which implies that even infants with an isolated OC are at an increased risk of early death. No figures have been published on the mortality rates of infants with OCs in the Netherlands. The objective of the present study was to obtain the Dutch infant mortality rate (IMR) for patients with OCs, including those with additional congenital malformations or syndromes. Special attention is given to the IMR in patients with CP in the context of Robin sequence
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