Elevated Infant Mortality Rate among Dutch Oral Cleft Cases: A Retrospective Analysis from 1997 to 2011

Nunen, Daan P. F. van; Boogaard, Marie José H. Van Den; Griot, J. Peter W. Don; Ruettermann, Mike; Veken, Lars T. van der; Breugem, Corstiaan C.

doi:10.3389/fsurg.2014.00048

Cited by 15 publications

(11 citation statements)

References 18 publications

(14 reference statements)

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“…Our reported mortality rate is in line with the previously described mortality rates in RS infants, which range from 2 to 26% [ 9 , 11 , 12 , 14 , 15 , 19 , 21 , 29 , 32 , 33 , 35 , 37 ], although it was higher than what we expected when the study was initiated. Our group of deceased infants consists of a highly heterogeneous group (Table 2 ).…”

Section: Discussionsupporting

confidence: 92%

“…Clinicians mainly focus on the morbidities of RS, which include respiratory complications due to upper airway obstruction, feeding problems, a related failure to thrive, and the associated cleft palate problems, when present [ 10 , 16 , 33 ]. Reported mortality rates in RS vary from 2 to 26% [ 9 , 11 , 12 , 14 , 15 , 19 , 21 , 29 , 32 , 33 , 35 , 37 ]. Upper airway management plays a central role in the treatment of RS.…”

Section: Introductionmentioning

confidence: 99%

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Mortality in Robin sequence: identification of risk factors

et al. 2018

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Although Robin sequence (RS) is a well-known phenomenon, it is still associated with considerable morbidity and even mortality. The purposes of this study were to gain greater insight into the mortality rate and identify risk factors associated with mortality in RS. We retrospectively reviewed all RS infants followed at the Wilhelmina Children’s Hospital from 1995 to 2016. Outcome measurements were death and causes of death. The authors identified 103 consecutive RS infants with a median follow-up of 8.6 years (range 0.1–21.9 years). Ten of the 103 infants (10%) died at a median age of 0.8 years (range 0.1–5.9 years). Nine of these ten infants (90%) were diagnosed with an associated syndrome. Of these, seven infants died of respiratory insufficiency due to various causes (two related to upper airway obstruction). The other two syndromic RS infants died of arrhythmia due to hypernatremia and of West syndrome with status epilepticus. One isolated RS infant died of brain ischemia after MDO surgery. Cardiac anomalies were observed in 41% and neurological anomalies in 36%. The presence of a neurological anomaly was associated with a mortality rate of 40% versus 7% in infants with no neurological anomaly (p = 0.016), with an odds ratio of 8.3 (95% CI 1.4–49.0) for neurological anomaly versus no neurological anomaly. Mortality was 15% in infants with syndromic RS versus 2% in infants with isolated RS (p = 0.044). Mortality was not significantly associated with the presence of a cardiac anomaly, surgical treatment for severe respiratory distress in the neonatal period, or prematurity.Conclusion: RS represents a heterogeneous patient population and is associated with a high level of underlying syndromes. The present study reports a mortality rate of 10% significantly associated with syndromic RS and the presence of neurological anomalies. A multidisciplinary approach in all infants born with RS, including genetic testing and examination of neurological anomalies in a standardized way, is crucial to identify infants with underlying syndromes potentially associated with increased mortality. What is Known: • Reported mortality rates in Robin sequence vary from 2% to 26%.• Clinicians mainly focus on the morbidity of Robin sequence that includes respiratory complications due to upper airway obstruction in the period after birth.• Robin sequence represents a heterogeneous patient population and is associated with a high level of underlying syndromes. What is New: • The present study reports a mortality rate of 10% significantly associated with syndromic Robin sequence and the presence of neurological anomalies.• A multidisciplinary approach in all infants born with Robin sequence, including genetic evaluation and standardized workup for neurological anomalies, is crucial to identify infants with underlying syndromes potentially associated with increased mortality.

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Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Mortality in Robin sequence: identification of risk factors

et al. 2018

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“…Kang et al found a 15 times greater risk of mortality in CPO patients when compared to the general population, and a 10 times greater risk when compared to other types of clefts (Kang et al, 2012 ). A 14-year study of Dutch patients found an infant mortality rate (IMR) of 2.45% for all CPO, with the most common cause of death for all oral clefts being congenital malformations of the heart (40.6%; van Nunen et al, 2014 ). Congenital heart defects commonly present with oral clefts, and are reported to occur in 1.3 to 27% of affected individuals, although the mechanism is still unknown (Setó-Salvia and Stanier, 2014 ).…”

Section: Morbidity and Mortalitymentioning

confidence: 99%

Epidemiology, Etiology, and Treatment of Isolated Cleft Palate

et al. 2016

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Isolated cleft palate (CPO) is the rarest form of oral clefting. The incidence of CPO varies substantially by geography from 1.3 to 25.3 per 10,000 live births, with the highest rates in British Columbia, Canada and the lowest rates in Nigeria, Africa. Stratified by ethnicity/race, the highest rates of CPO are observed in non-Hispanic Whites and the lowest in Africans; nevertheless, rates of CPO are consistently higher in females compared to males. Approximately fifty percent of cases born with cleft palate occur as part of a known genetic syndrome or with another malformation (e.g., congenital heart defects) and the other half occur as solitary defects, referred to often as non-syndromic clefts. The etiology of CPO is multifactorial involving genetic and environmental risk factors. Several animal models have yielded insight into the molecular pathways responsible for proper closure of the palate, including the BMP, TGF-β, and SHH signaling pathways. In terms of environmental exposures, only maternal tobacco smoke has been found to be strongly associated with CPO. Some studies have suggested that maternal glucocorticoid exposure may also be important. Clearly, there is a need for larger epidemiologic studies to further investigate both genetic and environmental risk factors and gene-environment interactions. In terms of treatment, there is a need for long-term comprehensive care including surgical, dental and speech pathology. Overall, five main themes emerge as critical in advancing research: (1) monitoring of the occurrence of CPO (capacity building); (2) detailed phenotyping of the severity (biology); (3) understanding of the genetic and environmental risk factors (primary prevention); (4) access to early detection and multidisciplinary treatment (clinical services); and (5) understanding predictors of recurrence and possible interventions among families with a child with CPO (secondary prevention).

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“…A national study of Dutch infants with orofacial clefts reported the overall IMR to be elevated at 2.09%, which was signi cantly higher than the Dutch general population IMR of 0.45% [28]. In another report by the East of England Cleft Network of 638 children with clefts, the causes of deaths were reported to be from associated anomalies (61%) and infections (17%), and the IMR was 3.6% for children with clefts [29]. The IMR for children with clefts in all studies was consistently higher than that of the general population.…”

Section: Neonatal and Infant Mortality Ratementioning

confidence: 95%

Epidemiologic Trends of Infants With Orofacial Clefts in a Multiethnic Country: A Retrospective Population-based Study

Yow

Jin

Yeo

2020

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Objectives: To establish the population-based prevalence and trends of cleft live births, evaluate associated malformations and infant mortality rate (IMR), and delineate the orofacial cleft (OFC) topography, ethnic and gender-specific features of the resident-population in Singapore from 2003 to 2012. Cleft-births surveillance and data of affected individuals, families, ethnic groups, locations and environment are essential in upstream healthcare planning. Well-informed decisions supported by data are all-important in health policies.Methods: Resident population-based data were compiled from the National Birth Defects Registry (NBDR). The Registry’s data was established from multiple sources: cytogenetics and histopathology laboratories, neonatal wards and maternity hospitals, medi-claims, birth defects, death certificates with reported congenital anomalies, stillbirths and abortuses (spontaneous and elective). The significance level of trend testing was set at p<0.05.Results: Prevalence per 10 000 for all cleft live births was 16.72; isolated clefts with no associated malformations, cleft lip, cleft palate, and cleft lip with cleft palate were 8.77, 6.85, 3.16, and 6.71, respectively. Prevalence in cleft live births was sexually dimorphic and ethnic-specific: Male, 17.72; Female, 15.78; Chinese group, 17.17; Malay group, 16.92; Indian group, 10.74; and mixed ethnicity group, 21.73. Infants with isolated clefts, non-isolated clefts with other malformations, and syndromic clefts were 52.5%, 42.1%, and 5.4%, respectively. Upward trend in infants with non-isolated clefts was significant (p=0.0287). The mortality rate in infants with clefts was 4.76%.Conclusions: The population-based prevalence for cleft live births was 16.72 per 10 000 with no significant upward trend. Co-occurrence of malformations and syndromes was found in almost half of all cleft live births. The IMR of infants with OFC was double that of the population norm.

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Elevated Infant Mortality Rate among Dutch Oral Cleft Cases: A Retrospective Analysis from 1997 to 2011

Cited by 15 publications

References 18 publications

Mortality in Robin sequence: identification of risk factors

Mortality in Robin sequence: identification of risk factors

Epidemiology, Etiology, and Treatment of Isolated Cleft Palate

Epidemiologic Trends of Infants With Orofacial Clefts in a Multiethnic Country: A Retrospective Population-based Study

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