A Nationwide Analysis of the Impact of Cardiopulmonary Anomalies on Cleft Palate Surgical Outcomes.

OBJECTIVE: To increase awareness and improve perioperative care of patients with cleft palate (CP) and coexisting cardiopulmonary anomalies. DESIGN: Retrospective cohort. SETTING: Multi-center. PATIENTS/PARTICIPANTS: Patients who underwent surgical repair of CP between 2012-2020 identified in the American College of Surgeons National Surgical Quality Improvement Program Pediatric Data File. Chi-squared analysis and Student's t-test were implemented to make associations between congenital heart disease (CHD) and congenital pulmonary disease (CPD) and postoperative complications. Multiple logistic regression was performed to identify associations between CP and CHD/CPD while controlling for age, gender, and ASA class. C2 values were used to assess the logistic regressions, with a significance level of 0.05 indicating statistical significance. MAIN OUTCOMES MEASURES: Length of stay (LOS), perioperative complications (readmission, reoperation, reintubation, wound dehiscence, cerebrovascular accidents, and mortality). RESULTS: 9 96 181 patients were identified in the database, 17 786 of whom were determined to have CP, of whom 16.0% had congenital heart defects (CHD) and 13.2% had congenital pulmonary defects (CPD). Patients with CHD and CPD were at a significantly greater risk of increased LOS and all but one operative complication rate (wound dehiscence) relative to patients with CP without a history of CHD and CPD. CONCLUSION: This study suggests that congenital cardiopulmonary disease is associated with increased adverse outcomes in the setting of CP repair. Thus, heightened clinical suspicion for coexisting congenital anomalies in the presence of CP should prompt referring providers to perform a comprehensive and multidisciplinary evaluation to ensure cardiopulmonary optimization prior to surgical intervention.

Evaluation of Obstructive Sleep Apnea in Prone Versus Nonprone Body Positioning With Polysomnography in Infants With Robin Sequence.

OBJECTIVE: Management of obstructive sleep apnea in infants with Robin sequence (RS) includes prone positioning during sleep, which conflicts with safe infant sleep data. We examined changes in polysomnography (PSG) parameters for prone versus nonprone body positions in these infants. DESIGN: Pre-post interventional, nonblinded study. PARTICIPANTS: Infants with RS referred for PSG were recruited from craniofacial clinic and inpatient units at Children's Hospital Los Angeles, a tertiary pediatric center. Fourteen infants were recruited, and 12 studies were completed on both body positions; 11 studies were used in the analysis. INTERVENTIONS: The PSG was divided into nonprone and prone sleep, moving from their usual sleep position to the other position midway in the study. MAIN OUTCOME MEASURES: Data was collected in each position for obstructive apnea-hypopnea index (oAHI), central apnea index (CAI), sleep efficiency (SE), and arousal index (AI). Signed rank test was used to evaluate the change in body position. RESULTS: All infants were term except 1, age 7 to 218 days (mean: 55 days; standard deviation: 58 days), and 8 (57%) of 14 were female. From nonprone to prone sleep position, the median oAHI (16.0-14.0), CAI (2.9-1.0), and AI (28.0-19.9) decreased (P = .065); SE increased (67.4-85.2; P = .227). CONCLUSIONS: Prone positioning may benefit some infants with RS. However, even those with significant improvement in obstructive sleep apnea did not completely resolve their obstruction. The decision to use prone positioning as a therapy should be objectively evaluated in individual infants.

Postpartum Depression in Mothers of Infants With Cleft Lip and/or Palate.

This study describes postpartum depression rates and risk factors for mothers with infants with cleft lip and/or palate as postpartum depression has been associated with a range of negative maternal and child outcomes. A retrospective chart review from August 2009 to May 2015 included medical diagnoses, demographics, receipt of prenatal diagnosis, and the Edinburgh Postnatal Depression Scale (EPDS). Mothers (N = 206) had infants (59.2% male; mean age in weeks 5.1 ±â€Š6.9) with isolated cleft lip (18%), cleft palate (22.8%), or cleft lip and palate (59.2%). Mothers ranged from 16 to 45 years old (mean age 29 ±â€Š6.2) and half had received a prenatal diagnosis. Patients mostly had public insurance (57.8%) and represented diverse ethnicities. Based on the EPDS, 11.7% of mothers met the depression cutoff of 10 or higher. The majority endorsed self-blame (68.9%), difficulty coping (59.2%), and feeling anxious (57.3%). Mothers of infants with cleft lip or cleft lip and palate who did not receive a prenatal diagnosis had higher total EPDS scores, anxiety, and incidence of feeling scared. Higher EPDS scores were predicted by not having a prenatal diagnosis and by older maternal age. Mothers of infants with a cleft had similar rates of postpartum depression as the general population; however, those who were older and who did not receive a prenatal diagnosis endorsed more symptoms. Prenatal diagnosis may contribute to positive maternal postpartum adjustment. Providers should incorporate screening for risk factors into their evaluation and treatment planning.

Familial Recurrence of 3MC Syndrome in Consanguineous Families: A Clinical and Molecular Diagnostic Approach With Review of the Literature.

We report four individuals from two unrelated consanguineous families with 3MC syndrome. In the first family, chromosome microarray data revealed that the two affected sisters, born to first-cousin parents, shared a unique homozygous C-terminal deletion in the COLEC11 gene. Two affected brothers from a second family, also born to first-cousin parents, shared a region of homozygosity that included the second gene known to cause the 3MC syndrome, MASP1. We discuss the diagnostic approach of craniofacial disorders born to consanguineous parents and highlight a literature search and reference a helpful dysmorphology solution powered by FDNA (Facial Dysmorphology Novel Analysis) technology.

An update on emergency contraception use in adolescents.

Changing circumstances regarding access to emergency contraception (EC) warrant nurses' review of its current status. Nurses have the potential to improve adolescents' awareness of and access to EC so that adolescents may prevent pregnancy. Research suggests that adolescents lack awareness of EC methods that prevent pregnancy following an unprotected act of intercourse. Many pregnancies occurring in adolescence could be prevented with appropriate EC use. Studies show that adolescents are capable of using EC correctly and safely, and that access to EC is not associated with increased rates of unprotected intercourse or with higher rates of pregnancy or sexually transmitted infections.

Applying cost analyses to drive policy that protects children: mercury as a case study.

Exposure in prenatal life to methylmercury (MeHg) has become the topic of intense debate in the United States after the Environmental Protection Agency (EPA) announced a proposal in 2004 to reverse strict controls on emissions of mercury from coal-fired power plants that had been in effect for the preceding 15 years. This proposal failed to incorporate any consideration of the health impacts on children that would result from increased mercury emissions. We assessed the impact on children's health of industrial mercury emissions and found that between 316,588 and 637,233 babies are born with mercury-related losses of cognitive function ranging from 0.2 to 5.13 points. We calculated that decreased economic productivity resulting from diminished intelligence over a lifetime results in an aggregate economic cost in each annual birth cohort of $8.7 billion annually (range: $0.7-$13.9 billion, 2000 dollars). $1.3 billion (range: $51 million-$2.0 billion) of this cost is attributable to mercury emitted from American coal-fired power plants. Downward shifts in intellectual quotient (IQ) are also associated with 1566 (range: 115-2675) excess cases of mental retardation (MR defined as IQ < 70) annually. This number accounts for 3.2% (range: 0.2-5.4%) of MR cases in the United States. If the lifetime excess cost of a case of MR (excluding individual productivity losses) is $1,248,648 in 2000 dollars, then the cost of these excess cases of MR is $2.0 billion annually (range: $143 million-$3.3 billion). Preliminary data suggest that more stringent mercury policy options would prevent thousands of cases of MR and billions of dollars over the next 25 years.

Pediatrician attitudes, clinical activities, and knowledge of environmental health in Wisconsin.

Pediatricians can reduce exposures to environmental hazards but most have little training in environmental health. To assess whether Wisconsin pediatricians perceive a relative lack of self-efficacy for common environmental exposures and diseases of environmental origin, we assessed their attitudes and beliefs about the role of the environment in children's health. A 4-page survey was sent to the membership of the Wisconsin Chapter of the American Academy of Pediatrics. We obtained a 35.4% response rate after 1 follow-up mailing. Respondents agreed that the role of the environment in children's health is significant (mean 4.28 +/- .78 on 1-5 Likert scale). They expressed high confidence in dealing with lead exposure (means 4.22-4.27 +/- 1.01-1.09), but confidence in their skills for pesticide, mercury, and mold was much lower (means 2.49-3.09 +/- 1.06-1.26; P<.001). Of those surveyed, 88.6% would refer patients to a clinic "where pediatricians could refer patients for clinical evaluation and treatment of their environmental health concerns." These findings indicate that Wisconsin pediatricians agree that children are suffering preventable illnesses of environmental origin, but feel ill equipped to educate families about many common exposures. Significant demand exists for centers that can evaluate environmental health concerns, as well as for educational opportunities.

Mental retardation and prenatal methylmercury toxicity.

BACKGROUND: Methylmercury (MeHg) is a developmental neurotoxicant; exposure results principally from consumption of seafood contaminated by mercury (Hg). In this analysis, the burden of mental retardation (MR) associated with methylmercury exposure in the 2000 U.S. birth cohort is estimated, and the portion of this burden attributable to mercury (Hg) emissions from coal-fired power plants is identified. METHODS: The aggregate loss in cognition associated with MeHg exposure in the 2000 U.S. birth cohort was estimated using two previously published dose-response models that relate increases in cord blood Hg concentrations with decrements in IQ. MeHg exposure was assumed not to be correlated with native cognitive ability. Previously published estimates were used to estimate economic costs of MR caused by MeHg. RESULTS: Downward shifts in IQ resulting from prenatal exposure to MeHg of anthropogenic origin are associated with 1,566 excess cases of MR annually (range: 376-14,293). This represents 3.2% of MR cases in the US (range: 0.8%-29.2%). The MR costs associated with decreases in IQ in these children amount to $2.0 billion/year (range: $0.5-17.9 billion). Hg from American power plants accounts for 231 of the excess MR cases/year (range: 28-2,109), or 0.5% (range: 0.06%-4.3%) of all MR. These cases cost $289 million (range: $35 million-2.6 billion). CONCLUSIONS: Toxic injury to the fetal brain caused by Hg emitted from coal-fired power plants exacts a significant human and economic toll on American children.