NM 623 Exam 1 part 2

Talk about spina bifida occulta

(failure of the halves of the vertebral arch to fuse) Diagnosed by radiography. Usually no clinical symptoms. Minor anomaly of the spine. Spinal cord and nerves are usually normal. Occasionally indicated by tuft of hair. 5-10% of population

Talk about spina bifida cystica

(includes myeloschisis, meningocele, myelomeningocele - severe types of spina bifida) Myeloschisis - Most severe. No covering over spinal cord. Meningocele - Sac contains meninges and CSF. Spinal cord and roots in normal position. Spinal cord abnormalities may be present. Myelomeningocele - Sac contains spinal cord and/or nerve roots. Spinal cord abnormalities are present. 80% of spina bifida cystica. 1/1000 births

What is the care of a baby with cleft lip and/or palate?

* resuscitation not an issue unless the cleft is associated with pierre robin syndrome, which involves micrognithia and airway obstruction * reassurance to the parents in the delivery room that cleft will not threaten the infants survival and that this is surgically correctable * as long as infant is stable promote normal bonding and attachment * PE includes assessment of all systems to detect or exclude other abnormalities that would indicate the cleft is associated with chromosomes abnormality or syndrome, which might limit prognosis * feeding difficulty is common - although suck is abnormal, swallowing is normal and many can breastfeed successfully. - breastfeeding * isolated cleft lip- breast conforms to the defect and sucking usually generates adequate negative pressure * isolated cleft palate-usually successful with soft palate cleft or narrow hard palate cleft * cleft lip and palate- most likely to have difficulty; breast milk may be pumped and fed through a supplemental nursing system that drips milk through a thin tubing inserted into the mouth along the side of the mothers nipple. supplemental system simulates sucking reflex (promotes continued muscular development) and milk production while promoting adequate feeding - formula feeding * isolated cleft lip-soft wide based nipple, might conform to the defect to allow adequate sucking * isolated cleft palate or cleft lip and palate combination- the based nipple will require enlargement of holes for easy milk delivery because the infant will have an insufficient suck. infants fed by a combination of nipple hole enlargement, suck stimulation and rest following suck, known as ESSR method (table 7.1) showed more wt gain when compared to nipple enlargement alone. * successful feeding by parents MUST occur before dc from hospital setting in order to assure the parents of their ability to care for the infants. * close follow up with craniofacial team is essential Cleft lip - 1/1000, cleft palate - 1/2500

Direct effect of methamphetamines on newborn

*cardiac defects, cleft lip, and biliary atresia *fetal distress and IUGR resulting in SGA *questionable about PTB *withdrawal sx-decreased arousal, increased stress, poor quality movement

Direct effect of marijuana on newborn

*crosses placenta and stored in amniotic fluid *neurobehavioral effects- range from mild visual functioning, heightened tremors, startling, jitteriness, hypotonia, lethargy,t0 difficulties with arousal, regulation, and excitability *some shorter gestations have been reported among heavy users, but NO LBW or PTB has generally been reported

Direct effect of PCP on newborn

*dysmorphologic features that might consist of microcephaly and alterations in facial features *neurobehavioral sx-decreased attention, high pitched cry, poor visual tracking, coarse flapping tremors, lethargy, nystagmus/roving eye movements, poor feeding, and altered newborn reflexes *smaller infants and lower gestational than non-exposed infants, however pcp exposure in not associated with LBW or PTB

Direct effect of benzos on newborn

*most commonly prescribed class of drug during pg *crosses placenta and accumulates in fetus in varying degrees *PTB and LBW *withdrawal sx-hypoventilation, hypertonicity, and floppy infant syndrome (particularly with late in pg use) *sx can appear within a few days to 3wks after birth and last for several months

Direct effect of ETOH on newborn

*passes through placenta and amniotic fluid acts as a reservoir for ETOH, increasing availability *PTB, LBW, & FASD *jittery, irritable, seizures, opisthotonus, abdominal distention, excessive mouthing movement, and reflex abnormalities *FAS-associated with specific facial features, growth deficiency, CNS abnormalities, behavioral abnormalities and intellectual disabilities

Direct effect of nicotine on newborn

*readily crosses placenta *neurobehavioral sx-impairment of arousal, irritability, hyperexcitability, hypertonicity, and tremors *increased risk- SIDS, PTB, IUGR, and link bt development of neonatal and adult diseases * small studies indicate orofacial clefts, neural tube defects and cryptorchidism (lg scale studies found no increase in deformities) *withdrawal syndromes noted in infants of heavy smoking moms

What is the incidence of fetal alcohol syndrome?

1-2 infants in 1,000 births

Exposure to Alcohol has been linked with

Alcohol can have a devastating effect on the fetus causing birth defects,neurodevelopmental disorders, and mental retardation (Kenner & Lott, 2007). There are varying degrees of severity influenced by maternal use. However, it is known that binge drinking may have more profound affects. Fetal Alcohol Syndrome (FAS) has three guidelines for diagnosis: documentation of three facial anomalies, growth deficits (below 10th percentile), and CNS abnormalities (Kenner & Lott). The term fetal alcohol spectrum disorder (FASD) is inclusive of all categories of involvement from prenatal alcohol exposure. Ongoing research is currently striving to define the various diagnoses for FASD. See the CDC guidelines for further definitions (Kenner & Lott, 2007).

Analyze the physiology of the damage caused in FAS and FASD.

Alcohol readily crosses the placenta and the amount of fetal exposure is high resulting in continual damage IUGR resulting in a growth deficiency postnatally despite adequate nutrition. Increased rates of malformations Chronic fetal hypoxia

Talk about anencephaly

Anencephaly (partial absence of brain) May be associated with spina bifida. Often detected by U/S. 0.2/1000 births Incompatible with life. (75% stillborn) Supportive management.

Talk about ambiguous genetalia

Anomalies of external genitalia. Cannot identify sex of newborn. Often associated with internal genital anomalies. Can be caused by Congenital Adrenal Hyperplasia. Sex type determined by chromosome studies.

Exposure to buprenorphine has been linked to

Buprenorphine is a newer opioid substitute introduced to try and reduce maternal and neonatal side effects experienced with Methadone use (Bandstra, et al., 2010). Studies continue to determine if there is less newborn withdrawal with this drug.

Maternal exposure to methadone causes?

CNS dysfunction, smaller head circumference

Talk about bacterial infection

Cause: GBS, E. coli, Klebsiella pneumoniae, Enterococcus (most common) Symptoms: Respiratory distress: tachypnea, labored breathing, poor color, apnea Temperature instability Poor feeding Hypotension Onset: Early onset: Birth to 6 days Late onset: 7 days to 3 months Duration: Therapy for minimum of 10-14 days Treatment: Warm, humidified O2 by hood, NPO, IV fluids, antibiotics

Talk about hypoglycemia

Caused by: LGA, SGA, IDM, <37 or >42 weeks, Hx of birth stress Symptoms: Jitteriness, cyanosis, apnea, weak cry, lethargy, limpness, refusal to feed, respiratory distress, apnea, emesis Onset: First 8-12 hours in high-risk infant, can be any time Duration: Should improve 30-60 min after feeding Treatment: Enteral feeds, IV glucose

Talk about Neonatal Withdrawal Syndrome

Caused: Maternal Drug Use Affects: Neuro: CNS irritability, tremors, increased wakefulness, high-pitched cry, increased muscle tone, hyperactive reflexes, seizures, frequent yawning or sneezing GI: Poor feeding, uncoordinated/constant sucking, vomiting, diarrhea, dehydration, poor wt gain, increased sweating, nasal stuffiness, fever, mottling, temperature instability Time of onset: Birth to 14 days PP depending on drug Duration: 1 day to 18 months PP depending on drug Treatment: Observation with use of abstinence assessment tool, breastfeed if no contraindications, may need pharmacologic treatment for moderate to severe signs

Maternal exposure to methylmercury causes?

Cerebral atrophy, spasticity, seizures, mental retardation

Maternal race, advanced maternal age, chromosomal abnormalities, syndromic development and environmental teratogens (alcohol and thalidomide) have been linked to what?

Cleft lip and palate. Incidence ranges from 0.8-2.7 in 1000 live births. Isolated cleft palate found in 1 in 2000 live births

Summarize at least 4 long-term health and social adaptation problems of children affected by FAS or FASD.

Cognitive deficits (e.g., low IQ) or developmental delays. Examples include specific learning disabilities (especially math), poor grades in school, performance differences between verbal and nonverbal skills, and slowed movements or reactions. Executive functioning deficits: These deficits involve the thinking processes that help a person manage life tasks. Such deficits include poor organization and planning, lack of inhibition, difficulty grasping cause and effect, difficulty following multistep directions, difficulty doing things in a new way or thinking of things in a new way, poor judgment, and inability to apply knowledge to new situations. Motor functioning delays: These delays affect how a person controls his or her muscles. Examples include delay in walking (gross motor skills), difficulty writing or drawing (fine motor skills), clumsiness, balance problems, tremors, difficulty coordinating hands and fingers (dexterity), and poor sucking in babies. Attention problems or hyperactivity: A child with these problems might be described as "busy," overly active, inattentive, easily distracted, or having difficulty calming down, completing tasks, or moving from one activity to the next. Parents might report that their child's attention changes from day to day (e.g., "on" and "off" days). Problems with social skills: A child with social skills problems might lack a fear of strangers, be easily taken advantage of, prefer younger friends, be immature, show inappropriate sexual behaviors, and have trouble understanding how others feel. Other problems can include sensitivity to taste or touch, difficulty reading facial expression, and difficulty responding appropriately to common parenting practices (e.g., not understanding cause-and-effect discipline)

Maternal iodine deficiency causes what

Cretinism (arrested physical and mental development, dystrophy of bones and soft tissue)

Talk about undescended testicles

Cryptorchidism (may be unilateral or bilateral) 30% premature male infants, 3-4% full-term male infants In most cases, testes descend into the scrotum by the end of the first year.

Review the probable and actual incidence of FASD in the US given the difficulty in diagnosis.

Data & Statistics In the United States We do not know exactly how many people have an FASD. CDC studies have shown that 0.2 to 1.5 cases of fetal alcohol syndrome (FAS) occur for every 1,000 live births in certain areas of the United States.1, 2, 3, 4 Other studies using different methods have estimated the rate of FAS at 0.5 to 2.0 cases per 1,000 live births. Scientists believe that there are at least three times as many cases of FASDs as FAS. [Read summary] Prevalence estimates of alcohol use among women of childbearing age vary from state to state.

Talk about renal agenesis

Failure of kidney formation. Cause probably multifactoral. Unilateral (usually left kidney) - 1/1000. Males more than females. Suspected in infants with single umbilical artery. Bilateral - 1/3000. Associated with oligohydramnios. Bilateral - Most die shortly after birth or 1-3 months.

Maternal exposure to lead causes what?

Fetal anomalies, IUGR, functional deficits

Maternal exposure to warfarin during 6-12 weeks after fertilization or 8-14 weeks after LMP causes what?

Fetal hydantoin syndrome. Also caused by exposure to anticonvulsants. Incidence is 5-10% of children born to mothers treated with phenytoins or hydantoin anticonvulsants

Exposure to heroin/oxycontin has been linked to

Heroin and OxyContin have been associated with low birth weight, preterm birth, and withdrawal after birth. In recent years, maternal OxyContin use has increased in popularity and is currently seen more often than maternal heroin use. Long term effects are unknown (Kenner & Lott, 2007).

Why do IDMs have a higher incidence of congenital anomalies?

IDMs have higher incidence of congenital anomalies due to the teratogenic effects of an abnormal maternal metabolic state early in embryogenesis. Incidence of major anomalies in IDMs is about 6-9% overall but increases with poor metabolic control. Most common are CV. Caudal regression syndrome almost uniquely associated with DM. (Thureen, pg. 269).

Maternal exposure to PCBs cause what?

IUGR, skin discoloration

Initial supportive care for meningocele and meningomyelocele

Immediate call for peds assistance, stabilize, transport to tertiary pediatric center. Apply sterile warm saline dressing with a dry sterile overwrap, thermoregulation, fluid maintenance, position infant prone and avoid fecal contamination.

Initial supportive care for gastroschisis and omphalocele

Immediate call for peds assistance, stabilize, transport to tertiary pediatric center. Place newborn in a radiant warmer in as sterile environment as possible. Sterile, warmed saline is applied to the eviscerated abdominal contents via sterile gauze pads. Wrap infants torso in sterile gauze to keep saline in place. No feedings are given; a feeding tube is inserted and any stomach contents are aspirated. The neonate has an acute need for IVF.

Initial supportive care for tracheoesophageal fistula and esophageal atresia

Immediate call for peds assistance, stabilize, transport to tertiary pediatric center.Position infant in prone position with the head elevated . No oral feedings are given. Aspiration of the esophageal contents by feeding tube attached to syringe helps to minimize the chance of aspiration into the lungs.

Talk about hydrocephaly

Impaired circulation and absorption of CSF. Possible increased production of CSF. Enlargement of head may or may not be present. When present: increased head circumference, separation of sutures, bulging tense fontanelles, high pitched cry, downward deviation of the eyes.

What are the leading causes of infant death?

Infant mortality rates for the five leading causes of infant death are presented in Table 7 by race and Hispanic origin of mother. The leading cause of infant death in the United States in 2007 was Congenital malformations, deformations and chromosomal abnormalities (congenital malformations), accounting for 20 percent of all infant deaths. Disorders relating to short gestation and low birth-weight, not elsewhere classified (low birthweight) was second, accounting for 17 percent of all infant deaths, followed by SIDS, accounting for 8 percent of infant deaths. The fourth and fifth leading causes in 2007 were Newborn affected by maternal complications of pregnancy (maternal complications) (6 percent), and Accidents (unintentional injuries) (4 percent). Together, the five leading causes accounted for 56 percent of all infant deaths in the United States in 2007.

Exposure to cocaine has been linked to

Maternal cocaine use has been significantly associated with low birth weight, preterm birth and small for gestational age infants (Gouin, Murphy, Shah, and the Knowledge Synthesis Group on determinants of low birth weight and preterm birth, 2011). It is difficult to determine a causal relationship as there are often concomitant risk factors such as low socioeconomic status, smoking, or other drug use (Kenner & Lott, 2007).

CNM management of diaphragmatic hernia

emergent refer. Needs ET tube immediately (Varney, 1036). Decompress bowel with orogastric tube (Thureen, p58)

Maternal exposure to Thalidmoide causes?

Meromelia-phocomelia "seal limbs"

Exposure to methadone has been linked to

Methadone is now considered the standard of care for pregnant opioid-addicted women per the National Institutes of Health Consensus Panel, but dosing regimens still continue to be debated (Bandstra, Morrow, Mansoor, Accornero, 2010). It is very important that the Methadone treatment be carefully prescribed and monitored (Kenner & Lott). Improved outcomes have been noted with Methadone by increasing the likelihood of the pregnancy going to term and increasing infant birth weights (Bandstra, et al., 2010).

Talk about microcephaly

Microcephaly - Environmental causes - alcohol/drugs, CMV, radiation

CNM management of omphalocele

Needs immediate management. Sterile, warm saline dressing. Not too tight. Cover with plastic. NPO. refer

Maternal exposure to Retinoic acid (vitamin A) - critical period for exposure 5-7 weeks after LMP causes what?

Neuropsychological impairement

Maternal exposure to ACE inhibitors causes what?

Oligohydramnios, fetal death, long-lasting hypoplasia of the bones of the calvarioa, IUGR, renal dysfunction

Maternal exposure to SSRIs causes?

PPHN, neurobehavioral disturbances

Talk about horseshoe kidney

Poles of kidney are fused 1/500

Talk about microcephaly

Small brain and calvaria (face is normal size). Gross mental retardation.

What are the four leading causes of infant death

The four leading causes of infant death are low birth weight, congenital anomalies, and maternal complications, and SIDS. Nearly half of all infant deaths in the US in 2003 occurred to infants with birth weights less than 1000 gm (CDC, 2008).

Exposure to tobacco has been linked with

Tobacco has been associated with spontaneous abortion, preterm birth, stillbirth, and intrauterine growth restriction. Cigarette smoking is the most common substance abused during pregnancy (Kenner & Lott, 2007). Maternal smoking has been determined to be a risk factor for SIDS

Maternal exposure to tetracycline causes what?

Tooth defects, hearing deficits

True/False: The majority of infants born to women who have used street drugs during pregnancy do not have long-term health problems that can be directly attributed to this chemical exposure

True

cyanosis without respiratory distress is almost always a cardiac problem. True or false??

True

Talk about hypospadias

Urethral opening on ventral aspect of penis. Often penis is underdeveloped and curved ventrally (chordee). 1/300 male births. Rare in females with urethral opening in vagina. Circumcision is contraindicated.

What is VACTERL?

Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal and Limb abnormalities. Caused by exposure to androgens and progestogens

Evaluate the difficulties in studying effects of in utero drug exposure on IQ and long-term behavior.

animal studies have typically been used to define effects of in utero substance exposure generalizing results from animal studies to human studies is hampered by differences in timing of brain maturation teratologic effects are dependent on the intersection of exposure, the temporal and regional emergence of critical developmental processes and the sensitivity of the developing specific brain structure or neural circuit to the drug alterations of these events have the capacity to modify brain development as well as the ability to recover from injury

Direct effect of cocaine on newborn

cocaine-stimulant PTB and LBW *Withdrawal s/s common: irritability, *tremors, muscle rigidity, poor suck/poor feeding, excessive suck, hyperactivity , hyperalertness, autonomic instability or hypertonicity, .

CNM management of gastroschisis

cover with warm moist sterile gauze, NPO, and refer

Maternal exposure to benzos causes?

craniofacial anomalies

Maternal exposure to lithium causes?

defects of heart and great vessels

Initial supportive care for diaphragmatic hernia

management is emergent and includes endotracheal intubation in the birth room. Use of bag and mask ventilation will only worsen the situation. A feeding tube, preferably attached to low suction, should be passed and taped in place to vent the stomach of air. The pediatric team should be requested immediately. REFER

Maternal exposure to Organic mercury causes?

minamata disease - resembles cerebral palsy

Direct effect of opioids on newborn

opiods- narcotics, pain killers, heroin/methadone, oxyContin *NAS effects- may include tonal problems, fever, and seizures *PTB, LBW & neurobehavioral sx *recommend eval of infant q 3-4 hours using the Finnegan scale while in the hospital

CNM management of cleft lip and palate

refer. If infant is stable, support bonding and attachment.

CNM management of esophageal atresia

refer. Position newborn in prone position with head elevated. No oral feedings. Aspiration of esophageal contents by feeding tube attached to a syringe (Varney, 1036).

what is dextrocardia?

when the heart is on the opposite side it's severe unless all the organs are reversed