38 Perinatal Disorders

The neonatal period is remarkable for the vast array of physiologic changes that occur as the infant transitions from the intrauterine to extrauterine life. This period is a highly vulnerable time for the infant. In the U.S., about two thirds of all deaths in the first year of life occur among infants less than 28 days old with the highest risk being in the first 24 hours of life (Stoll, 2007a). Because serious health problems can arise for the infant in the hours after the initial transition to extrauterine life, the primary care provider must be prepared to manage these problems while providing psychosocial support and education for the families. An understanding of the physiology of fetal development, risk factors for potential problems, and pertinent physical findings is necessary to effectively assist the newborn’s transition to extrauterine life.

Standards of Care

The Healthy People 2020 objectives (U.S. Department of Health and Human Services [USDHHS], 2010) related to maternal, infant, and child care are available online. The overall goal of these objectives is to improve maternal health and pregnancy outcomes and reduce rates of disability in infants, thereby improving the health and well-being of women, infants, children, and families in the U.S. Since its inception in 1979, the Healthy People program suggests that the health of a population is reflected in the health of its most vulnerable members. A major focus of many public health efforts, therefore, is improving the health of pregnant women and their infants, including reductions in the rate of birth defects, risk factors for infant death, and death of infants and their mothers. Included among these goals are improvements in the rates of breastfeeding, ensuring that all newborns are screened for state-mandated diseases, reducing the proportion of children with a metabolic disorder who experience developmental delay requiring special education services, and increasing the percentage of healthy full-term infants who are put down to sleep on their backs.

The Guide to Clinical Preventive Services (U.S. Preventive Services Task Force [USPSTF], 2009) recommends the following preventive services for neonates:

• Prenatal screening for Rh(D) incompatibility; human immunodeficiency virus (HIV); hepatitis B; syphilis; chlamydia and gonorrhea

• Promotion of breastfeeding

• Neonatal screening for sickle hemoglobinopathies to identify infants who may benefit from antibiotic prophylaxis to prevent sepsis

• Screening for congenital hypothyroidism for all newborns during the first 4 days of life

• Screening for phenylketonuria (PKU) for all newborns before discharge from the nursery. Infants who are tested before 24 hours old should receive a repeat screening test by 2 weeks old.

• Ocular antibiotic prophylaxis of all newborn infants to prevent gonococcal ophthalmia neonatorum

• Routine newborn hearing screening for all infants in the first month of life and an audiologic evaluation for hearing loss in the first 3 months of life

Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents (Hagan et al, 2008) and the American Academy of Pediatrics (AAP) Committee on Practice and Ambulatory Medicine (AAP, 2007) have detailed anticipatory guidelines for the newborn, first-week, and 1-month health supervision visits. Guidelines for Perinatal Care from the AAP and the American College of Obstetricians and Gynecologists (Lemnos and Lockwood, 2008) is another thorough compendium of standards of caring for the newborn.

Anatomy and Physiology

Intrauterine-to-Extrauterine Transition

The infant’s intrauterine-to-extrauterine transition requires an extraordinary number of biochemical and physiologic changes. In utero, the placenta provides metabolic functions for the fetus. Oxygenated blood from the placenta arrives to the fetus through the umbilical vein. Because of high pulmonary vascular pressure, this blood is shunted from the right to the left side of the fetus’s heart through the foramen ovale or to the systemic circulation through the ductus arteriosus. At birth the umbilical cord is severed. Simultaneously, the infant begins to breathe and the high pulmonary vascular pressure drops, allowing blood flow to the lungs for oxygenation. The foramen ovale and ductus arteriosus are no longer necessary and close after birth. The newborn becomes dependent on gastrointestinal tract function to absorb nutrients, renal function to excrete wastes and maintain chemical balance, liver function to metabolize and excrete toxins, and the functions of the immunologic system to protect against infection. Many newborn problems are related to poor transition to extrauterine life as a result of asphyxia, premature birth, congenital anomalies, or adverse effects of delivery.

A predictable series of changes or reactivities in vital signs and clinical appearance take place after the delivery of most normal infants (Fig. 38-1). The first period of reactivity includes sympathetic system changes, such as tachycardia, rapid respirations, transient rales, grunting, flaring and retractions, a falling body temperature, hypertonus, and alerting exploratory behavior. Parasympathetic system changes during the first period of reactivity include the initiation of bowel sounds and the production of oral mucus. After an interval of sleep, the infant enters the second period of reactivity. During this time the oral mucus production again becomes evident, the heart rate becomes labile, the infant becomes more responsive to endogenous and exogenous stimuli, and meconium is often passed.

FIGURE 38-1 Summary of normal transition.

(From Desmond MM, Rudolph AJ, Phitaksphraiwan P: The transitional care nursery, Pediatr Clin North Am 13:651-668, 1966.)

Pathophysiology

High-Risk Pregnancy

High-risk pregnancies are defined as those in which factors exist that increase the chances of abortion, fetal death, premature delivery, intrauterine growth retardation, fetal or neonatal disease, congenital malformations, mental retardation, and other handicaps. Identification of a high-risk pregnancy is the first step toward prevention of neonatal problems (Box 38-1). Comprehensive and frequent prenatal visits for women with high-risk pregnancies are aimed at preventing complications in the newborn.

BOX 38-1 Factors Associated With High-Risk Pregnancies

Adapted from Stoll BJ: High-risk pregnancies. In Kliegman RM, Behrman RE, Jenson HB et al, editors: Nelson textbook of pediatrics, ed 18, Philadelphia, 2007, Saunders, p 684.

Acquired Health Problems

In utero exposure to poor nutrition, alcohol, drugs, viruses or bacteria, and maternal conditions, such as hypertension and diabetes, can result in prematurity and abnormalities at birth. The risk of neonatal problems increases with maternal age younger than 20 years and older than 35 years (see Box 38-1).

Genetic Problems

The presence of chromosomal abnormalities, congenital anomalies, inborn errors of metabolism, mental retardation, and familial diseases increases the risk of the same condition in the infant. Because many conditions are not easily identifiable on physical examination, exploring family histories to identify newborns at risk for any inheritable diseases is important. Anticipation of various inherited conditions leads to their early identification and management of potential problems.

Perinatal Complications and Injuries

Perinatal complications occur immediately before or during birth. Prolonged or dysfunctional labor increases the risk of fetal distress. Prolonged rupture of the membranes and chorioamnionitis increase the risk of infant infection, and ruptured placenta previa increases the risk of infant blood loss. Cesarean deliveries, the use of forceps or vacuum extraction, and the type of maternal anesthesia used also pose risks. The term birth injury includes mechanical and anoxic trauma incurred by an infant during labor and delivery. Predisposing risk factors for birth injury include macrosomia, prematurity, cephalopelvic disproportion, dystocia, prolonged labor, and breech presentation. Birth injuries include caput succedaneum, cephalhematoma, subcutaneous fat necrosis of the face or scalp, fractures of the skull, subconjunctival and retinal hemorrhages, intracranial hemorrhage, peripheral nerve palsies (brachial, phrenic, facial), fractured clavicle or humerus, ruptured liver or spleen, and hypoxic-ischemic insults. Proper steps to monitor and treat an infant with perinatal complications and injuries must be undertaken immediately after birth. The provider must be familiar with perinatal conditions that subject the newborn to a higher risk and be prepared to intervene quickly based on the available perinatal information.

Assessment of the Neonate

History

• Past maternal health history

• Past obstetric history

Number of previous pregnancies; number of infants born alive or stillborn

Number of elective or spontaneous abortions; number of preterm and term deliveries

Cesarean deliveries and indications for them

Health status of living children; if deceased, age and cause of death

• Family history

Genetically acquired conditions, birth defects, mental retardation, or other diseases

Hypertension, hyperlipidemias, heart disease, or familial cancers

Age and health status of living relatives

Causes of death of family members

• Current obstetric history

Present health and medical history including depression or other mental health conditions

Age of mother

Prenatal care—duration of

Medications used during pregnancy including prescription, over-the-counter, and natural health products

Use of pregnancy-enhancing drugs or technology

Infections (including group B streptococcus [GBS] status and results of other screening tests) and illnesses during pregnancy

Alcohol, cigarettes, or other drugs used during pregnancy

Hypertension or glucose intolerance

Duration of labor, duration of ruptured membranes, analgesia, anesthesia, presentation and route of delivery, use of forceps

Polyhydramnios (excessive fluid) or oligohydramnios (little to no fluid)

Infant meconium stained or amniotic fluid foul smelling

Fever

• Social history

Emotional stressors during pregnancy including home-lessness

Unplanned or unwanted pregnancy

Financial and emotional support

Dietary considerations (e.g., strict vegan diet)

Educational background of parents

Father’s anticipated involvement in raising infant

Age of other children in the home

Physical Examination

Immediately After Birth

Apgar Score

Immediate evaluation of the newborn infant at 1 and 5 minutes old can be a valuable routine procedure. An Apgar score is assigned to the baby based on the criteria in Table 38-1.

TABLE 38-1 Apgar Scores

The 5-minute Apgar score is an indication of how well the resuscitation efforts have succeeded. Caution must be exercised when using the Apgar score to predict long-term outcomes of mortality and developmental delay. Only when combined with other factors, such as fetal status, umbilical cord or scalp blood pH, evidence of organ injury, or seizures, can the Apgar score be useful in determining long-term outcome (AAP, 2006). In actual practice, the decision to resuscitate an infant typically is based on a quick assessment of the heart rate, color, and respiratory rate rather than the full 1-minute Apgar score (Fig. 38-2).

FIGURE 38-2 Resuscitation in the delivery room.

(From Niermeyer S, Kattwinkel J, Van Reempts P: International guidelines for neonatal resuscitation: an excerpt from the Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care: International Consensus on Science, Pediatrics 106[3]:29, 2000.)

Gestational Age

Maturational assessment of an infant’s gestational age is based on the physical examination (Fig. 38-3). The assessment is done promptly after birth to confirm maternal estimated dates, and interpreted with information on the mother’s menstrual history, obstetric milestones achieved during pregnancy, and prenatal ultrasonograms. An infant’s length, weight, and fronto-occipital head circumference are measured and plotted on growth curves based on gestational age (Fig. 38-4). Infants whose weights fall above the 90^th percentile for age are classified as large for gestational age (LGA); those whose measurements fall below the 10^th percentile for age are classified as small for gestational age (SGA). Those whose measurements fall between the 10^th and 90^th percentiles are classified as appropriate for gestational age (AGA).

FIGURE 38-3 Classification of newborns by intrauterine growth and gestational age.

(From Ballard JL, Khoury JC, Wedig K et al: New Ballard score, expanded to include extremely premature infants, J Pediatr 119:417-423, 1991.)

FIGURE 38-4 Newborn maturity rating and classification.

(From Ross Hospital Formula System, Ross Products Division, Abbott Laboratories, Columbus, OH; adapted from Battaglia FC, Lubchenco LO: A practical classification of newborn infants by weight and gestational age, J Pediatr 71:159-163, 1967; Lubchenco LO, Hansman C, Boyd E: Intrauterine growth in length and head circumference as estimated from live births at gestational ages from 26 to 42 weeks, Pediatrics 37:403-408, 1966.)

FIGURE 38-5 Fontanelles and sutures.

(From Betz CL, Hunsberger M, Wright S: Family-centered nursing care of children, ed 2, Philadelphia, 1994, Saunders, p 124.)

System	Findings
Vital signs and measurement	Check vital signs frequently in the first hours after birth, then every 6-8 hr when stable. Evaluate ability to maintain temperature (97.7° to 99.3 ° F [36.5° to 37.4° C]) in open crib after transition to extrauterine environment. Failure to maintain temperature requires evaluation for other problems, particularly sepsis. Respirations should remain between 30 and 60 breaths/min. Heart rate should remain between 100 and 160 bpm. Significant molding of the head requires repeated measurements to verify size. Daily weight losses of up to 10% in the first 2-3 days of life are not abnormal because normal infants excrete a large amount of water in the first days of life. Weight loss of greater than 10% is unexpected and is often due to poor intake or excessive losses.
Skin	Lanugo and vernix. Lanugo is fine dark hair, prominent over the trunk and shoulders. It is seen in infants born prematurely, becoming less prominent as the gestation approaches term. Thick, greasy, white vernix is more common on prematurely born infants’ skin. Dry and cracked skin. This is normal over the first several days of life. If associated with thin subcutaneous fat (parchment-like), it is suggestive of a postmature infant, fetal growth retardation, or both. Cyanosis. Acrocyanosis, bluish changes in the color of the hands and feet, and generalized mottling of the skin are frequently noted in the first several days of life when an infant loses body heat. Central cyanosis beyond the first few moments of life is abnormal and can represent a significant problem with oxygenation. Pallor. Many perinatal events can result in pallor, indicating a significant disruption of the infant’s circulatory system. Specific causes include anemia, sepsis, cold stress, hypoglycemia, and seizures. Plethora. An excessively reddish discoloration to the skin can be caused by polycythemia or hyperthermia. Infants born to diabetic mothers can be plethoric. Meconium staining. Antenatal stress can cause the first stool to pass in utero. If this greenish black meconium remains in the amniotic fluid for a prolonged period, staining of the infant’s skin and fingernails results. Jaundice. See the discussion of jaundice in the text under Hematologic Conditions.
Head	Sutures and molding. Vaginally delivered infants demonstrate some degree of molding, usually elongation of the anteroposterior diameter of the skull; if delivered by cesarean method, there are minimal alterations to the shape of the head. Fontanelles. The anterior fontanelle is usually about 2-3 cm in diameter; the posterior fontanelle is about 1 cm in diameter. Both are usually slightly depressed (see Fig. 38-5).
Face	Symmetric structures of the face should be apparent, although unilateral facial edema as a result of delivery conditions can occur normally. Overall view of the face may reveal maxillary or mandibular hypoplasia, distortion, or hemifacial hypoplasia.
Eyes	Too small or large, too widely spaced, or abnormal upward or downward slanting of palpebral fissures should alert the practitioner to potential congenital problems. Uncoordinated eye movements. Intermittent uncoordinated eye movements (disconjugate gaze) during the first weeks after birth are common, improving by 2-4 months old and resolving by 6 months old. Fixed disconjugate gaze is abnormal, even in the neonate. Conjunctivae. Reddening in the first 24-48 hours of life caused by chemical irritation of the eyes from silver nitrate drops or erythromycin ointment is normal. Purulent discharge in the first days or weeks of life can be associated with gonococcus, chlamydia, or herpes. Conjunctival hemorrhages secondary to delivery resolve spontaneously over the first weeks of life. Sclerae. Yellowing is associated with hyperbilirubinemia. Small hemorrhages secondary to delivery resolve spontaneously over the first weeks of life. Thinning of the sclera, common in African-Americans, is manifested by dark blue or black patches. Blue sclerae are associated with osteogenesis imperfecta. Red reflex. Shining an ophthalmoscope’s white light through the pupil reveals the “red reflex,” a disk ranging from pearly gray to orange. Absence of a red reflex may indicate the presence of lens opacities secondary to cataracts, congenital infection (rubella), or calcium metabolism abnormality. A white reflex can indicate retinoblastoma. Absence of the expected red reflex indicates the need for an immediate ophthalmologic evaluation.
Ears	Identify normalcy in the size, rotation, shape, position, and patency of the external auditory canal. Presence of low-set ears should prompt careful examination for other dysmorphic features. Abnormalities in shape require thorough physical examination, especially of the genitourinary system. Assessment of hearing is done by noting a startle response to a loud noise, avoiding any tactile sensations, such as a wind current on the face as a result of clapping near the ear. Auditory brain response testing should be ordered for any infant in whom a question of hearing exists. Screening for universal detection of infants with hearing loss is recommended and is especially important for high-risk infants (e.g., family history, in utero infection, craniofacial anomalies, syndromes associated with hearing loss). Preauricular skin tags or significant pits should be noted (can be a genetic red flag). See text section on skin dimpling.
Nose	Patency of the nasal passages can be tested by closing the mouth and one nostril at a time or by passing a small catheter into the nasopharynx to see if the passage is clear. Nasal flaring is a sign of respiratory distress that can be caused by any number of abnormalities, including mechanical obstruction, parenchymal lung disease, or acidosis.
Mouth	Size and symmetry of the lips • Thin lips with a smooth philtrum (the area between lips and nose) are associated with fetal alcohol syndrome. • Asymmetric movements while crying can be due to nerve palsies or absence of perioral muscles. Cleft lip and palate can be associated with midline CNS abnormalities. Incomplete cleft palates are recognized by digital examination of the mouth for bony defects of the hard palate in the presence of normal palatal mucosa. Excessive salivation can be related to reflux of gastric contents or esophageal atresia. Epstein pearls are small white epithelial inclusion cysts on the palate and gums. An excessively large tongue can be associated with genetic or metabolic abnormalities, such as hypothyroidism or Down syndrome. Natal teeth are sometimes seen at birth (approximately 1 in 3000 live births). If they are extremely loose, aspiration is a concern. Consultation with a pediatric dentist is indicated.
Neck	Short neck indicates the possibility of Klippel-Feil syndrome or other vertebral problems. Webbing. Redundant skin is seen in trisomy 21, Turner syndrome, and Noonan syndrome. Masses • Thyroglossal duct cysts (midline) or branchial cleft cyst (along the edge of the sternocleidomastoid muscles) can be found. • Other masses that can be seen include a hematoma in the sternocleidomastoid muscle, cystic hygromas, and, rarely, goiters. Torticollis. Asymmetric shortening of the sternocleidomastoid muscle results in preferential turning of the head to one side, not to be confused with irritability of neck movement associated with meningitis or subarachnoid hemorrhage. Hematoma of the sternocleidomastoid muscle can result in the development of torticollis and requires early management.
Thorax	Shape, symmetry. Rounded appearance measuring about 2 cm less than the fronto-occipital head circumference (approximately 33 cm): • Minimization of rounding occurs with RDS, atelectasis, and other diseases of decreased expansion of the chest. • Accentuation is seen in meconium aspiration. Wide-spaced nipples and a shieldlike appearance are characteristic of Turner syndrome. Chest movement on inspiration should be symmetric and unlabored. Movement of the abdomen with respirations is normal. Asymmetric movement occurs with unilateral pneumothorax. Intercostal, subcostal, or supracostal retractions indicate respiratory distress. Clavicles. Vaginally delivered LGA babies are especially prone to fractures of the clavicle (see perinatal injury section in text). Breast bones. Pectus excavatum (concave chest) and pectus carinatum (pigeon chest) are occasionally seen. If severe both can lead to restrictive lung disease later in life. Nipples • Fullness and sometimes secretion of a white milky substance are normal and are secondary to maternal hormonal stimulation. • Supernumerary and inverted nipples are not uncommon. • Redness surrounding the nipple, especially with purulent drainage, occurs in neonatal mastitis.
Lung	General. Coughing, retractions, and an intermittently increased respiratory rate occur immediately after birth, resolving by about 12 hours of life to smooth and unlabored respirations at a rate of 30 to 60 breaths/min. Respiratory distress. Tachypnea, apnea (pauses in respiration >15 seconds), grunting (an infant’s attempt to increase functional residual capacity, thereby improving gas exchange), interclavicular, subclavicular, or supraclavicular retractions, nasal flaring, and central cyanosis all indicate distress. Auscultation • Rales or crackles are commonly heard immediately after birth as lung fluid is resorbed. Beyond the immediate postpartum period, rales can indicate pneumonia, delayed resorption of lung fluid, meconium aspiration, or pulmonary edema. • Unilateral absence of breath sounds occurs in pneumothorax, atelectasis, and pleural effusion. • Bowel sounds over the chest, especially with a scaphoid abdomen and significant respiratory distress, indicate a diaphragmatic hernia with displacement of abdominal contents into the chest.
Heart	Inspection. Observe neonate for adequacy of perfusion. Respiratory distress is common with cardiac abnormalities. Edema as a result of cardiac failure is rarely seen in the newborn. Palpation • Point of maximal impulse is displaced from the fourth left intercostal space with pneumothorax, situs inversus, or dextrocardia. • Thrills or heaves are associated with murmurs and cardiac abnormalities. Auscultation. Heart rate is normally 100 to 160 bpm. Detection of skipped beats warrants electrocardiogram. Heart sounds may be muffled or displaced in the infant with a pneumothorax. Murmurs. Common in the newborn period, many murmurs disappear after a few hours or a few days. Significant murmurs should be investigated. Pulses. Brachial or radial pulses are compared with femoral or dorsalis pedis pulses for symmetry of impulse and strength in pulse. Delay or relative weakness of lower extremity pulses occurs in coarctation of the aorta. Blood pressure. By Doppler device using a 2.5-4 cm wide and 5-9 cm long cuff, compare with normals for age and gestation. Systolic blood pressures greater than 96 mm Hg are considered significant hypertension in the newborn, and systolic blood pressures exceeding 106 mm Hg are considered severe hypertension.
Abdomen	General • Normal abdomen is slightly protuberant, is soft, moves smoothly with respirations, and has fine bowel sounds scattered throughout. Absent bowel sounds can indicate ileus. • The liver is usually palpated 1-2 cm below the right costal margin; the spleen tip is sometimes felt at the left costal margin; kidneys, deep within lateral aspects of the abdomen measuring 3-4 cm in size, may be palpated. • Pain is indicated by crying, grimacing, or forceful resistance with palpation. Umbilical hernias. Midline outpouching from the sternum to the umbilicus is seen with weak abdominal musculature (diastasis recti); a large and protuberant umbilicus occurs with an umbilical hernia. Umbilical vessels. The normal cord contains two arteries and a single vein. Absence of the second artery can be associated with congenital abnormalities. Vomiting and abdominal distention. Regurgitation of large volumes of feeding is not expected. • Bilious vomiting is always abnormal and usually a sign of obstruction. • Abdominal distention with enlargement of any of the organs of the abdomen or failure to pass stool is abnormal. • Meconium ileus with failure to pass stool in the first 24-48 hours of life is associated with cystic fibrosis.
Genitalia	Male • The penis should have the urethral opening at the tip of the phallus with completely developed foreskin. Chordee means that the distal end of the penis is bent. • Testes not located in the scrotal sac or inguinal canal but retrievable to the scrotum are normal. Testes not located in or relocated in the scrotal sac from the canal are considered to be undescended. • Hydrocele is identified by transilluminating fluid collection around the testis and is regarded as normal unless it is associated with inguinal hernia or it lasts more than 12 months. • Inguinal hernia with displacement of intestines into the scrotal sac is frequently nontransilluminating and is associated with bowel sounds. Inguinal hernias are sometimes apparent and reduced at other times. A surgical consultation is indicated. Female • Labia majora are large and completely surround the labia minora. • Labia and vagina should be open, often with a white discharge. • Blood-tinged fluid in small amounts by day 2-3 is normal. Ambiguous genitalia are genitalia that do not appear to be completely masculinized or feminized. An endocrine referral is essential. Anus and rectum. Patency of the rectum and placement of the anus should be noted. A small amount of blood streaking in the diaper, especially with a small anal fissure, is common.
Extremities, back, hip	Molding. Intrauterine constraint and resultant molding cause mild curvatures of the forefeet (metatarsus adductus vs. varus [in-toeing or out-toeing]) or the tibia (genu varum [bowleg], genu valgum [knock-knee]), or both. See Chapter 37 for more information. Contractures of the joints and molding of the bones occur if amniotic fluid was decreased and is abnormal. Fractures • Skull fractures can occur as a result of extensive molding of a large head. • Clavicle. Femora and humeri can fracture with difficult deliveries and use of instrumentation. • Multiple fractures can indicate osteogenesis imperfecta. Spine. Dimples, hemangiomas, tufts of hair, or other lesions along the spine may be associated with spinal abnormalities, such as spina bifida occulta. Hips. See Chapter 37 for information about eliciting Ortolani and Barlow signs. Both are indicators of dislocated or dislocatable hips.
Neurologic examination	Muscle tone. Observe tone, movement, and symmetry of the extremities while the infant is awake. Reflexes. Elicit the following: • Rooting • Sucking • Palmar grasp • Moro reflex • Ankle clonus (3 or 4 beats of clonus at ankle is normal) • Stepping and placing response • Galant reflex • Asymmetric tonic neck reflex Cranial nerves. Cranial nerve (CN) I (olfactory) is rarely tested. Vision (CN II) is tested by an infant’s response to a bright light. CNs III, IV, and VI are tested by noting an infant’s ability to gaze in all directions, although intermittent disconjugate gaze is normal through 6 months old. Adequate sucking and swallowing confirm presence of CNs V, IX, X, and XII. Symmetric movement of the face with crying confirms presence of CN VII. Hearing (CN VIII) is assessed by startle to loud noise.

bpm, Beats per minute; CNS, central nervous system; LGA, large for gestational age; RDS, respiratory distress syndrome.

Diagnostic Studies

All states in the U.S. require screening of infants for a variety of congenital abnormalities, although the screening tests performed vary from state to state (see Chapter 40). Infants should be screened before they are discharged and before the seventh day of life; if initial screen was before 24 hours of life, rescreening should be done by 14 days old. Although most infants require no special screening tests, some are at risk for predictable complications in the newborn period. Infants born to mothers with poorly controlled diabetes and LGA or SGA infants are at higher risk for hypoglycemia and usually require serum glucose level screening. Similarly, infants demonstrating Coombs test positivity because of maternal-child blood incompatibility are screened for evidence of hemolysis. Some nurseries screen both mothers and infants for syphilis; mothers should be screened for HIV and hepatitis B unless done prenatally. Universal hearing screening is recommended by 1 month of age (see Chapter 29), and special attention is paid to any newborn at higher risk for hearing loss as a result of low birthweight, rubella or other infection, malformation, trauma, asphyxia, prematurity, intensive care unit stay, or antibiotic use.

Management Strategies

Initial Care

Following birth, newborns require special care and observation as they master the transition to the extrauterine environment. Additional components of care at this period include prophylaxis for eye infection with antibiotic ointment and vitamin K injection for hemorrhagic disease.

Establishing Feeding

Regardless of the route of feeding the family has chosen, the provider must ensure that the infant and parents have well-established feeding patterns before discharge. Follow-up care is scheduled in 2 or 3 days to ensure adequate ongoing nutrition. See Chapters 10 and 11 for more detailed information on breastfeeding and formulas.

Anticipatory Guidance Before Discharge

Physical Care

Umbilical Cord

Applying alcohol to the base of the cord traditionally has been recommended to aid in cord separation, although the utility of this practice has been questioned, especially in industrialized areas; air-drying by tucking the diaper below the cord may be preferable (Lin et al, 2005; Zupan et al, 2004). After cord separation, which usually occurs at 10 to 14 days of life, a slight bloody discharge can be seen for 1 to 2 days. Bellybands or coins to cover the navel are avoided because these increase the chance of infection. If a foul-smelling discharge or erythema appears around the umbilicus, the infant should be evaluated immediately for sepsis. If a granuloma appears after the cord falls off, an application of silver nitrate helps to heal it.

Circumcision

Circumcision, the removal of the foreskin that normally covers the glans penis, is a controversial surgical procedure. The decision to circumcise is the parents’ responsibility, although the provider can supply factual information on the risks and potential benefits of the procedure.

Proponents of circumcision claim that it keeps the glans cleaner; the chance for developing urinary tract infections is reduced (although the chance of urinary tract infections in uncircumcised males is only 1%); it reduces the incidence of penile cancer, phimosis, balanitis, adhesions, and occlusion of the urethral meatus; and the boy may look more like his peers. The opponents of circumcision claim that it does not prevent sexually transmitted infection; that good hygiene prevents penile cancer; that circumcision leaves the glans open to the chance of cautery burns and meatal stenosis; and that because fewer boys are being circumcised, these boys will not be different from many of their peers.

Contraindications to circumcision include epispadias or hypospadias, ambiguous genitalia, exstrophy of the bladder, familial bleeding disorders, and illness. Complications of circumcisions include infections, bleeding, gangrene, scarring, meatal stenosis, cautery burns, urethral fistula, amputation or trauma to the glans, and pain. For infants who undergo circumcision, procedural anesthesia is recommended. A variety of anesthesia techniques are available, including application of topical anesthetics (eutectic mixture of local anesthetics [EMLA] cream), dorsal penile nerve block, and subcutaneous ring block. Postoperative pain relief measures in the form of sucrose on a pacifier, acetaminophen, soft music, and physiologic positioning of the infant in a padded environment are helpful (Brady-Fryer et al, 2004).

Care of the uncircumcised baby includes gentle cleaning around the genital area. The skin normally adheres to the penis and is not retractable at birth, but loosens as the baby grows. The parents are counseled not to force the foreskin back. If the baby is circumcised, the penis should be cleansed daily with cotton balls dipped in tap water followed by the application of a small amount of petroleum jelly to the tip of the penis with each diaper change to prevent discharge from the penis sticking to the diaper. The petroleum jelly is needed only for the first 2 to 3 days after the circumcision.

Bathing, Oils, and Powders

Tradition favors that the infant not be immersed in a tub of water, but rather should be sponge bathed until the umbilical cord separates and the navel appears healed. Mild cleansing agents, such as Dove, Caress, Neutrogena, and Basis, are gentle enough for infants’ skin—oils and powders are not recommended. Oils and greasy substances tend to clog the skin’s pores and can cause acne or rashes. Powders should be avoided because inhaling the talc could lead to respiratory problems. For dry skin, a lotion such as Keri, Eucerin, Aveeno, or Cetaphil is recommended.

Diapers

There is much controversy about whether disposable or cloth diapers are the better choice for infants. The need for frequent changing and proper cleansing is the important message to deliver.

Sleep Position

By 1992 the preponderance of available evidence suggested that the prone sleeping position was associated with an increased incidence of sudden infant death syndrome (SIDS). Recommendations for healthy infants include (AAP, 2005, 2009a):

• Placing infant in a supine position (side-lying is not recommended)

• Avoiding soft surfaces and gas-trapping objects in an infant’s sleeping environment

• Advising that co-sleeping can be hazardous, but sleeping in the same room as the caregiver for the first 6 months of life may be beneficial

• Avoiding overheating

• Offering pacifiers

• “Tummy time” during the awake period—recommended for developmental reasons and to help prevent flat spots on the occiput

Encourage parents to ensure that their childcare center and other caregivers (e.g., grandparents) are following these guidelines. SIDS is discussed later in this chapter.

Injury Prevention

The appropriate use and installation of a crash-tested safety seat are essential. The best child safety seat is the one that fits the child properly, is easy to use, and fits in the parent’s vehicle correctly. The National Highway Traffic Safety Administration rated scores of infant child restraint systems for ease of parental use. Correct installation of the infant safety seat can be checked at a child safety seat inspection station (often located in fire stations) or by a certified child passenger safety technician. Most hospitals have a certified person on-site who can assist parents when the infant leaves the hospital, or one can be located by searching the Internet. At the first visit it is ideal to have a trained staff member check for appropriate positioning and belt use. It is disconcerting that 80% of car seats are used inappropriately (AAP, 2002) (see Chapter 39).

The house should be childproofed before the infant is taken home. Falls and burns are the most common injuries to neonates. Parents should be counseled to avoid shaking their baby for any reason (refer to Chapters 9 and 39 for more information).

Parenting

The parenting role is stressful, even if all goes well. Fatigue and maternal depression resulting from hormonal shifts are common. Encourage parents to identify and make use of supportive people, arrange time for rest and time alone, and keep their expectations reasonable. When a mother seems to be having significant difficulty in adjusting to her new infant, it is imperative that the provider also keep in mind the possibility of severe postpartum depression and be ready to intervene on the behalf of the infant, the mother, and the family. The 10-question Edinburgh Postnatal Depression Scale (EPDS) is an easy-to-administer tool that is a valuable and efficient way of identifying mothers at risk for perinatal depression (Fig. 38-6). Women with postpartum depression need not feel alone; intervention, with possible referral for mothers whose score indicates a depressive illness, should be individualized.

FIGURE 38-6 Edinburgh Postnatal Depression Scale (EPDS).

(From Cox JL, Holden JM, Sagovsky R: Detection of postnatal depression: development of the 10-item Edinburgh Postnatal Depression Scale, Br J Psychiatry 150:782-786, 1987; Wisner KL, Parry BL, Piontek CM: Postpartum depression, N Engl J Med 347[3]:194-199, 2002.)

Early Discharge and Follow-up

Newborns are often discharged after a relatively short period of hospital observation. Although “early discharge” is a common practice, infants can experience difficulty with breastfeeding, poor weight gain, jaundice, and dehydration (Madden et al, 2004). Guidelines for early discharge of normal, healthy newborns are listed in Box 38-2. Plans for follow-up care within 48 to 72 hours and plans for ongoing health maintenance should be confirmed before discharge (Box 38-3). Even newborns who are hospitalized longer may need follow-up care within the first few days of life. All parents leaving the hospital with a newborn should have a confirmed time and place for follow-up, in addition to contacts in case of an emergency or questions.

BOX 38-2 Guidelines for Early Discharge of Normal, Healthy Newborns

• No ongoing medical issues that require continued hospitalization

• Term (37 to 41 completed weeks) baby

• Stable vital signs for at least 12 hours before discharge:

Axillary temperature of 97.7° to 99.3° F (36.5° to 37.4° C ) in open crib

Heart rate 100 to 160 beats per minute

Respiratory rate less than 60 breaths/minute

• Regular passage of urine and at least one stool

• Two successful feedings have been accomplished

• Normal physical examination

• No excessive bleeding at circumcision site

• The clinical significance of jaundice has been determined and appropriate follow-up plans made

• Evaluation and monitoring for sepsis based on maternal risk factors have been accomplished

• Infant laboratory data, including maternal syphilis, hepatitis B, and human immunodeficiency virus (HIV), and infant blood type and Coombs testing (as indicated) completed

• Appropriately timed neonatal metabolic and hearing screenings completed

• Initial hepatitis B administered

• Social support and continuing health care identified

• Social situation adequate: screen for drug abuse, previous child abuse, mental illness, lack of social support, lack of permanent home, history of domestic violence, communicable diseases in the household, teenage mother, inadequate transportation or communication abilities

• Appropriate medical home identified with early follow-up care achievable preferably within 48 hours of discharge but no later than 72 hours in most cases

• Mother knowledgeable in the care of the infant, including the following:

Feeding, with breastfeeding encouraged

Normal stool and urine frequency

Skin, genital, and cord care

Ability to identify illness (especially jaundice)

Proper safety (car seat, sleeping position, smoke-free environment, room sharing)

Smoke alarms in the home

Data from American Academy of Pediatrics (AAP): Policy statement-hospital stay for healthy term newborns, Pediatrics 125:405-409, 2010.

BOX 38-3 Guidelines for 48- to 72-Hour Follow-up Visit of the Normal, Healthy Newborn

• Review delivery and discharge summary for any identified follow-up needs (e.g., hearing screening, specialty referrals)

• Assess the infant’s general health, weight, hydration, and jaundice; identify any new problems; review feeding, stooling, and urination

• Assess quality of bonding

• Reinforce maternal and family education

• Review outstanding laboratory data

• Perform neonatal screen or other tests (such as bilirubin), if indicated

• Develop plan for health care maintenance, including emergency care, preventive care, immunizations, and periodic screenings

• Evaluate mother for post-partum depression

• Refer to Women, Infants, and Children (WIC) program eligibility screening as appropriate.

Premature Infants and Newborns With Special Needs

Premature infants have special needs that must be addressed before discharge (Box 38-4). Newborns with special needs (e.g., anomalies, disease states, social situations) require early assessment, intervention, and referral before discharge to ensure that support, education, and follow-up are in place.

BOX 38-4 Guidelines for Discharge and Follow-up of the High-Risk Neonate

Discharge Planning

• Demonstrate adequate weight gain, temperature control in open crib, adequate feeding without cardiorespiratory compromise, and mature and stable cardiorespiratory function.

• Ensure adequacy of immunizations based on infant’s chronologic age and appropriate metabolic screenings.

• Screen for anemia and nutritional risks; begin therapy, if indicated.

• Conduct funduscopic evaluation if necessary.

• Ensure appropriate hearing screen has been completed.

• Identify all active medical or social problems through a review of the medical record and physical examination of infant; ensure home readiness has been evaluated, especially for the technologically dependent child.

• Complete car seat evaluation.

• Review with family member medications, feeding schedules, well-child care, signs of illness, safety instruction, and appropriate response and follow-up for infants with active medical conditions.

• Identify family and community resources if infant is to be discharged on home oxygen therapy.

• Ensure adequate training of appropriate family members in cardiopulmonary resuscitation (CPR) and, if applicable, home apnea monitor or other equipment use.

• Consider need for visiting nurse, social service, respite care, support groups, early intervention services, or referral to the Women, Infants, and Children (WIC) program.

• Ensure that follow-up care is arranged to include a primary care provider and surgical or other subspecialty providers, if indicated.

Follow-up Planning

• Schedule follow-up hearing screen (if necessary) for infants with:

Craniofacial abnormalities

In utero infections

Birthweight less than 1500 g

Meningitis

Exchange transfusion for hyperbilirubinemia

Use of ototoxic medications

Apgar score of 0 to 4 at 1 minute or 0 to 6 at 5 minutes

Mechanical ventilation for 5 days or longer

Stigmata of syndrome associated with hearing loss

Failed initial screening

Family history of deafness

• Ensure that by about 4 to 6 weeks of chronologic age a dilated binocular indirect ophthalmoscopic examination has occurred for neonates with:

A birthweight of 1500 g or less or with a gestational age of <32 weeks

A birthweight between 1500 and 2000 g or gestational age of more than 32 weeks with an unstable clinical course including cardiorespiratory support, especially if infant is thought to be at high risk for retinopathy of prematurity (see Chapter 28)

• Additional examinations may be recommended based on the results of this first evaluation.

• Follow-up visits every 1 to 2 weeks, especially if infant is on oxygen therapy

• Growth and development should be of prime interest at each routine outpatient visit with referral for formal developmental assessment if any concerns are identified.

Data from American Academy of Pediatrics (AAP): Hospital discharge of the high-risk neonate, Pediatrics 122:1119-1126, 2008; Section on Ophthalmology American Academy of Pediatrics; American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus: Screening examination of premature infants for retinopathy of prematurity, Pediatrics 117:572-576, 2006.

Common Neonatal Conditions

Erythema Toxicum

Description and Epidemiology

Firm, yellow-white 1- to 2-mm papules or pustules with a surrounding erythematous flare characterize erythema toxicum. Lesions are clustered in several sites. These lesions usually develop at 24 to 48 hours old. The cause is unknown, although examination of a Wright-stained smear of the lesion reveals numerous eosinophils. Up to 50% of infants develop erythema toxicum, with a higher incidence in term than in premature infants.

Differential Diagnosis

Pyoderma, candidiasis, herpes simplex, transient neonatal pustular melanosis, and miliaria should be considered (Table 38-3).

TABLE 38-3 Comparison of Erythema Toxicum and Herpes Simplex Virus

Erythema Toxicum	Herpes Simplex Virus
Benign, self-limited	Pathologic, progressive
No specific maternal history	Frequently a history of maternal disease
Usually seen only in term infant	Can occur in infants of any gestational age
Begins on the second or third day of life, lasting as long as 1 week	Often begins late in the first week of life or early in the second week of life
Rash is evanescent, often involving the face, trunk, and extremities	Can be superficial and localized only to the presenting part (vertex or buttocks) or widespread and disseminated with or without cutaneous involvement
1- to 2-mm white papules or pustules on an erythematous base that occasionally may become somewhat vesicular	May manifest similar to sepsis without cutaneous findings or as grouped vesicles on an erythematous base on the presenting part about days 9-11 of life
Wright or Giemsa stain of lesion scraping demonstrates large numbers of eosinophils and no organisms; cultures are sterile	DFA staining or ELISA detection of HSV antigens of vesicle scrapings or growth of the organism from vesicle fluid is diagnostic
No specific therapy necessary	Acyclovir and other antiviral agents