Overview

Premature Infant with various monitors attached

Babies are surviving increasingly premature births due to the dramatic improvements in neonatal intensive care, advances in neurodevelopmental care techniques, and use of prenatal steroids, surfactant, and continuous positive airway pressure (CPAP). This module focuses on the care of infants born at extremely low gestational ages and weights - typically ≤26 weeks and/or 1500 grams (about 3 pounds) - although much of this information also pertains to preterm infants born later in gestation.

Premature infants, particularly those born extremely early, often have or are at risk of developing bronchopulmonary dysplasia, retinopathy of prematurity, intraventricular hemorrhage, hypoxic-ischemic encephalopathy (HIE), necrotizing enterocolitis, and other complications that require follow-up in the neonatal period and beyond. The medical home will often need to manage supplemental oxygen, feedings through gastrostomy or jejunostomy tubes, specialized immunizations, and specialized formulas, as well as coordinate the care provided by multiple subspecialists and/or developmental therapists.

Specialized preterm infant follow-up clinics are becoming more available and can help detect and address complications. International guidelines emphasize the importance of early diagnosis so that targeted early interventions can begin. [Novak: 2017] The goal is to maximize the outcomes for babies at high risk for motor impairment and cerebral palsy (10-15% of infants born ≤26 weeks develop CP). [Ferré: 2016]

Extreme prematurity
Preterm infants
Very low birth weight

Premature Infant and Low Birthweight provides frequently used definitions for some of the many terms used to describe prematurity.

ICD-10 coding

P07.0x, Extremely low birth weight newborn (up to 999 grams)

P07.1x, Other low birth weight newborn (1000-2499 grams)

P07.2x, Extreme immaturity of newborn (through 27 completed weeks)

P07.3x, Preterm (premature) newborn (28-36 6/7 completed weeks)

The last digit, represented above as an "x," signifies the need for further coding details about weight or gestational age. Coding for Disorders of Newborn Related to Short Gestation and Low Birth Weight (icd10data.com) provides these coding details.

Prevalence

The preterm birth rate (<37 weeks) in the United States in 2014 was 9.45% - the rate of infants born at <27 weeks was 1%. [Ferré: 2016] [McCormick: 2011] [Purisch: 2017] [National: 2020]

Genetics

Women who have had 1 infant born prematurely are at higher risk for a second premature infant. This risk increases with decreasing gestational age of the first premature child. While the overall rate of prematurity is 9.45% in the United States, the rate for African-American women is 13.4%. African American women are 5 times more likely than Caucasian women to experience a recurrent preterm birth. [Purisch: 2017] This risk factor is found in studies that have controlled for economic status, maternal education, and coexistence of other maternal medical risk factors. Additionally, women born prematurely have an increased risk of delivering their infant prematurely.

Prognosis

Prematurity is the leading cause of infant mortality in the US. [Klebanoff: 2011] Of all infant deaths in the US in 2006, 54% occurred in the 2% of infants born at <32 weeks of gestation. Despite this, survival and outcomes for children born very prematurely have improved dramatically over the past 4 decades. Associated morbidities can occur following delivery or may unfold as chronic disabilities, such as CP, cognitive impairment, vision and hearing impairment, feeding disorders, cardiac or respiratory conditions, and/or behavioral disabilities. In a multi-centered, longitudinal study, 12% of children born at <26 weeks had CP and 28 had cognitive delay (IQ <85). In this study, the severity of CP decreased over time. [Adams-Chapman: 2018] Primary care clinicians have unique opportunities to prevent or limit secondary disabilities in preterm infants and to help prevent subsequent preterm deliveries in the family. [Novak: 2017] Primary care physicians have an important role in detecting early risk factors for CP beginning with knowledge of the initial history and NICU course. Prompt referral to diagnostic-specific early intervention to optimize infant motor and cognitive plasticity, prevent secondary complications, and enhance caregiver well-being is essential. [Novak: 2017] Using tools with the highest evidence, the age of diagnosis of CP can be decreased from 12-24 months to a corrected age of 6 months or earlier. [Novak: 2018]

No data yet exists on what percentage of infants with extreme prematurity end up with no significant complications.

Practice Guidelines

Phillips RM, Goldstein M, Hougland K, Nandyal R, Pizzica A, Santa-Donato A, Staebler S, Stark AR, Treiger TM, Yost E.
Multidisciplinary guidelines for the care of late preterm infants.
J Perinatol. 2013;33 Suppl 2:S5-22. PubMed abstract / Full Text

Roles of the Medical Home

Often, during prolonged NICU hospitalizations, families become attached to and dependent upon the NICU staff, making the transition to primary care difficult. To help with the transition, the medical home clinician ideally would communicate with parents and the NICU staff, or visit with the infant, prior to NICU discharge. The initial visit, whether at discharge or within 1 to 2 days of leaving the hospital, also enables the primary care clinician to:

Establish a baseline weight, which helps guide feeding adjustments over time.
Provide additional training and reassurance about infant care outside of the hospital setting.
Educate families about additional medications and supplies that may be needed at home.
Update vaccinations as needed (family members can be encouraged to obtain vaccines as well). Guidance on immunizing former premature infants can be found in a Clinical Report from the American Academy of Pediatrics’ Committee on Infectious Diseases. [Saari: 2003] In general, vaccinations should be given based on the actual age of the child and not their corrected age.

Ongoing medical home care includes coordination of care specialists, studies, and programs such as a Neonatal Follow-Up to avoid service duplications. Ideally, the NICU discharge summary would indicate needed subspecialty follow-up (e.g., cardiology, pulmonology, ophthalmology).

Pearls & Alerts for Assessment

Infant feedings

Advance infant feedings according to the patient’s adjusted age, not their chronologic age.

Abnormal tone in infants is often misinterpreted

It is critical to closely monitor muscle tone and pay attention to parental reports of “early standing,” “strong legs,” or early handedness. Though these things make parents feel proud, they are red flags for increased tone. The Hammersmith Neurologic Exam (HINE) is a useful tool for detecting atypical tone.

Terms describing age and birth weight are not always standard

A list of commonly used words and definitions can be found at Premature Infant and Low Birthweight.

Confirm that the following were performed or indicated as outpatient:

Newborn screening: For information about the conditions tested, see the Portal’s Newborn Disorders.
Hearing screening: For more information on how hearing is tested and normal milestones for hearing and language, see the Portal's Hearing Screening.
Car seat testing: Oximetric evaluation of ability to safely transport in car seat vs. car bed should be performed prior to hospital discharge for infants born <37 weeks gestation. It may need to be repeated in the outpatient clinic to wean an infant discharged on oxygen for use in the car. Guidelines include oxygen saturation not falling <90% for >10 seconds or heart rate < 80 bpm for >10 seconds on room air, for at least 90 minutes or the duration of the car ride (whichever is longer).
Congenital heart disease screening: Pre- and post-ductal oximetry may suggest congenital heart disease; an echocardiogram would help confirm anatomy.
Screening for developmental dysplasia of the hip (DDH): Pelvic (hip) ultrasonography screens for DDH for infants born breech, with multiple gestations or with concerning findings on routine hip exam. For premature breech infants, timing of the hip ultrasound may be adjusted to 4-6 weeks for corrected post-gestational age. Screening for Developmental Hip Dysplasia—Clinical Algorithm (AAP) [Committee: 2000] provides recommendations for timing of screens and follow-up actions.
Vision screening: Infants less than 32 weeks get ROP screening in the hospital and should have ophthalmologic follow-up after discharge from the NICU within 4 to 6 months after discharge. For premature infants who were not identified with ROP, routinely perform vision screening with increased vigilance for strabismus, which occurs more frequently in premature infants.

Of Family Members

Postpartum depression is 3 times more likely to occur in mothers of preterm infants than in mothers of full-term infants. [Phillips: 2013] Examples of Standardized Screening Tools are:

Edinburgh Postnatal Depression Scale (English) ( 120 KB) or the Edinburgh Postnatal Depression Scale (Spanish) ( 54 KB) - free, 10-question screening tool
PHQ-2 and PHQ-9 - free and available in many languages from Patient Health Questionnaire (PHQ) Screeners (select screen from drop-down menu on the right)

Given the peak times for postpartum depression, screening should be integrated at the 1-, 2-, 4-, and 6-month visits. [Earls: 2019] Management of positive screens and additional information can be found at Postpartum Depression Screening.

For Complications

According to the AAP’s recommended schedule for developmental screening (see the Portal’s Developmental Screening page), the primary care clinician should perform developmental screening at the child’s adjusted age rather than chronologic age until 30 months (or 24 months if there will be no 30-month evaluation). Children enrolled in Neonatal Follow-Up Programs will undergo formal developmental testing through the program. Other screens to consider include:

Serial ophthalmologic evaluations to screen and monitor for retinopathy of prematurity. Typically infants with a history of ROP that have resolved ROP should be examined again by a Pediatric Ophthalmologist at 6 months of age. Routine well-child checks should include an eye exam that screens for strabismus, difficulty with visual fixation/following and acuity and atypical eye movements.
Head ultrasonography to screen for intraventricular hemorrhage, hydrocephalus, structural anomalies, etc. Infants born at 30 [Lahood: 2007] to 32 weeks [Sauve: 2001] gestation or earlier may benefit from routine cranial ultrasound examinations at 7 to 10 days of age and at 36 to 40 weeks' postmenstrual age. [Sauve: 2001] [Nwafor-Anene: 2003]
Hemoglobin/hematocrit to screen for anemia. Be aware of timing of blood transfusions and if the infant is receiving iron supplementation. The AAP advises anemia screening for all infants at ages 9-12 months and repeated screening between the ages of 1 and 5 years for patients at risk. For premature infants and infants small for gestational age, earlier screening around 4 months of life is advised. (See the Bright Futures/AAP Periodicity Schedule ( 119 KB).)

Consider additional screening for autism or for disorders of hyperactivity and the ability to focus. (See the Portal's Infant & Early Childhood Social-Emotional Screening and Developmental Screening for information about response to positive screens.)

Comorbid & Secondary Conditions

Numerous problems related to prematurity or complications related to care needed in the NICU may persist and require attention and care in the outpatient setting:

Developmental delays: According to the AAP’s recommended schedule for developmental screening (see Developmental Screening), the primary care clinician should perform developmental surveillance and screening at the child’s adjusted age rather than chronologic age until reaching 30 months. Use formal developmental screening tools at the 9-, 18-, and 30-month health supervision visits. [Hagan: 2008] Children enrolled in Neonatal Follow-Up Programs will undergo formal developmental testing.
Sensory disorders (hearing or vision impairment): Serial ophthalmologic evaluations screen and monitor for retinopathy of prematurity. This condition primarily affects premature infants weighing about 2¾ pounds (1250 grams) or less who are born before 31 weeks of gestation. [National: 2019] Hearing screening is typically performed prior to discharge in the NICU, but diagnostic audiology testing is recommended by 24-30 months for all infants who stayed in the NICU 5 days or more, were exposed to ototoxic medications or who have other risk factors, congenital infections or parental concerns. [Harlor: 2009] See Hearing Screening for more information.
Neuro-behavioral difficulties, including autism and ADHD [Wong: 2014] [Lindström: 2011]: Autism screening should be performed at the 18-month visit and again at either the 24- or 30-month visits. [Johnson: 2007] At any time if caregivers are concerned about the child’s emotional and/or behavioral development, consider further evaluation for autism, attention, or mood disorders. (See Infant & Early Childhood Social-Emotional Screening for information about response to positive screens.)
Neurological disorders, including cerebral palsy, attention deficits, and seizures: Head ultrasonography is typically used to screen premature infants for intraventricular hemorrhage, hydrocephalus, structural anomalies, etc. Infants born at 30 [Lahood: 2007] to 32 weeks [Sauve: 2001] of gestation, or earlier, may benefit from routine cranial ultrasound examinations at 7 to 10 days of age and 36 to 40 weeks postmenstrual age. [Sauve: 2001] [Nwafor-Anene: 2003]
Head shape deformities (from limited independent head mobility in very young infants or congenital muscular torticollis; less frequently, from craniosynostosis. See Cranial Deformation and Craniosynostosis.
Musculoskeletal problems related to cerebral palsy or developmental dysplasia of the hip
Cardiovascular disorders, including patent ductus arteriosus and persistent pulmonary hypertension. Implementation of universal screening for congenital heart disease is helping identify some but not all cardiovascular lesions.
Hypertension: Due to an increased risk of developing hypertension, consider blood pressure monitoring of premature infants at all routine visits, and obtain blood pressures for all children during well-child checks starting at age 3. (See the Bright Futures/AAP Periodicity Schedule ( 119 KB).) [Norman: 2010] Ambulatory blood pressure monitoring may be useful. [Flynn: 2014] Persistently hypertensive infants or children may require medications or specialist consultations. Adolescents with a history of prematurity remain at increased risk of hypertension. [de: 2012]
Respiratory disorders, including bronchopulmonary dysplasia (aka chronic lung disease of prematurity), prematurity-related respiratory disease, sleep-disordered breathing (obstructive and/or central sleep apnea), and chronic hypoxemia
Gastrointestinal disorders, including dysphagia, reflux, and surgically related bowel disorders
Anemia: Be aware of timing of prior blood transfusions and if the infant receives iron supplementation through formula and/or supplements. Hemoglobin/hematocrit is often used to screen for anemia; however, testing reticulocyte hemoglobin, iron studies, reticulocyte counts, or ferritin will increase sensitivity.
Endocrine disorders, including hypoglycemia and cortisol deficiency
Urological problems, such as undescended testes, hernias, or stones
Infections such as RSV that can lead to increased risk of subsequent hospitalization

Current & Past Medical History

The NICU discharge summary is the primary source of medical details; however, the caregiver’s story also is vital in determining family priorities.

The following items from the discharge summary warrant special attention in primary care follow-up:

Cardiac and/or respiratory arrest
Complex congenital heart disease
Heart murmur that is still present at discharge and cause, if known
Necrotizing enterocolitis (NEC) with perforation
Bronchopulmonary dysplasia / Chronic lung disease of prematurity
Pulmonary hypertension
Prolonged period on ventilator for respiratory failure (beyond the time of initial stabilization of the transitioning infant)
Chronic hypoxemia and discharged on home supplemental oxygen
Evaluation for and, if present, the degree of retinopathy of prematurity
Failed hearing screens and follow-up instructions
Intraventricular hemorrhage grade. (While all level bleeds I-IV represent an adverse event in the brain, grades III and IV are associated with a significantly increased risk for neurodevelopmental impairment)
Periventricular leukomalacia
Hydrocephalus
Evidence of cerebellar injury
Neonatal seizures
Hypoxic ischemic encephalopathy
Current feeding plan, dietary supplements, and need for feeding therapy and/or tube feedings
Surgical scars

Premature infants may have episodes of apnea and bradycardia. For families monitoring at home, inquire about desaturations, apneas, and bradycardia frequency, duration, and interventions.

Family History

A maternal history, including medication and substance use, history of prior pregnancy complications, and chronic medical conditions, helps determine risk for having another premature infant. If subsequent children are desired, referral to appropriate obstetric experts (maternal-fetal medicine) will help the mother plan for a safe delivery. Mothers who were themselves born prematurely also are at increased risk of giving birth prematurely. [Institute: 2007] Premature birth prevention programs may be available in certain areas. See the Portal’s page Prevention of Recurrent Preterm Birth.

Developmental & Educational Progress

Even those with normal intelligence and cognitive scores can have subtle learning disabilities that affect functioning in the regular classroom and require additional educational support. Following records of developmental screenings and evaluations, progress with early intervention or developmental therapies, and educational assessments and performance enables early recognition of problems and the need for further intervention.

Social & Family Functioning

Assess family functioning, postpartum depression, and confidence in performing the infant’s care at home.

Physical Exam

One important tool for identifying those at risk has emerged and is the Hammersmith Neurologic Exam (HINE), which can be incorporated into a routine physical examination.

General

In the first 2 years, make judgments about typical or atypical findings based on the adjusted age (not the chronological age), particularly for the neurologic exam.

Vital Signs

Assess blood pressure periodically, monitoring for hypertension. Use blood pressure norms for premature infants up to 44 weeks post-conception age. [Dionne: 2012] Use blood pressure norms for premature infants up to 1 year of age. Pediatric Hypertension (Medscape) (log-in required) provides Normal Blood Pressure Percentile Curves for Older Infants from [Lowry: 1971]; to access click “Tables” on left menu. Blood pressure norms for these ages are still under study. See [Nickavar: 2014] for more discussion. [Flynn: 2017]

Oxygen saturation is measured for infants requiring supplemental oxygen. Saturation measurements should indicate whether supplemental oxygen was used during the oximetry. For infants whose eyes have not fully vascularized, oxygen saturation may be maintained in a lower range as recommended by their NICU team or ophthalmologist. [Hayes: 2019]

Respiratory rate generally follows corrected gestational age norms. [Trachtenbarg: 1998]

Heart rate generally follows chronological age norms. [Trachtenbarg: 1998]

Growth Parameters

Plot measurements for preterm infants weighing 1500 grams or more by chronological age on a standardized growth chart for term infants, and then correct back for adjusted age. This can be continued until the child reaches 2 years of age when plotting solely by chronological age becomes appropriate.

Skin-fold measurements can help identify older children who may be “overfat” despite “normal weight,” due to low muscle mass and bone density, such as in non-ambulatory children. See Premature Infant Growth Charts.

Skin

Surgical scars and traumatic scars from intravenous (IV) infiltrations and other mishaps related to very fragile skin may be seen. If the infant has an ostomy, periodically visualize the skin around the tube by removing the dressing. See Feeding Tubes & Gastrostomies in Children and Tracheostomy.

Surgical scars and traumatic scars from intravenous (IV) infiltrations and other mishaps related to very fragile skin may be seen.

PDA ligation results in a relatively large scar on the left posterior and lateral chest wall.
Inguinal hernia repair scars are often hard to detect as they are made along the skin fold lines in the groin.
Incision scars related to IV catheters may be found on the wrists and ankles.
Exploratory laparotomy and resection of bowel due to necrotizing enterocolitis (NEC) may leave abdominal scars.

HEENT/Oral

Visually inspect for plagiocephaly or asymmetry
Palpate for premature fusion of sutures (craniosynostosis, or early closure of 1 or more cranial sutures, is far less common for both preterm and term infants but can look similar to some types of deformational abnormalities.) See Cranial Deformation and Craniosynostosis.
Inspect palate, tongue, uvula, buccal mucosa, gums, as well as emerging dentition.

Chest

Assess respiratory rate and work of breathing, including subcostal and supraclavicular retractions, head bobbing, or nasal flaring. Listen for crackles, rhonchi, wheezing, or stridor.

Heart

Assess rate, rhythm, and pulses. Note the grade (1-6) of any murmurs. The most common heart murmurs heard in premature infants include the patent ductus arteriosus and physiologic peripheral pulmonary stenosis, but murmurs associated with congenital heart disease are also common. Murmurs often come and go within the first 18 months of life and often are benign. Seek further evaluation in murmurs that sound ominous, that worsen over time, or are associated with feeding difficulties, respiratory distress, poor growth, sweating while feeding, tachycardia, and tachypnea. Demonstrations: Heart Sounds & Murmurs (University of Washington) has audio demonstrations of various murmurs.

Abdomen

Assess for bowel sounds, abdominal distension or masses, hepatosplenomegaly. Monitor for gradual resolution of umbilical hernias and diastasis recti.

Genitalia

In females: Monitor inguinal hernias (more common in premature infants).

In males: Monitor for inguinal hernias and hydroceles (both are more common in premature infants) and undescended testes. Cryptorchidism is more common in premature versus term baby boys, but also has a higher rate of spontaneous descent (80-90%) within the first year of life.

Extremities/Musculoskeletal

Evaluate for dislocation or subluxation of hips. Assess general appearance of extremities and spine, noting any congenital malformations.

Neurologic Exam

The neurologic exam is very sensitive to gestational age and to the state of the child. The basic neurologic exam, including cranial nerves, muscle strength, tone, and deep tendon reflexes, should be performed routinely and documented to allow ready tracking of progress. Motor delays, before 1 year adjusted, are not uncommon and warrant further evaluation. [Wilson: 2004] [D'Agostino: 2010]

The Hammersmith Neurologic Exam (HINE) is a scorable neurological exam for those 2-24 months of age. It was developed first as a clinical tool and has subsequently been validated and standardized for use in different populations, including the preterm population. Novak’s article emphasizes its importance as a tool to help with early detection of CP and those at high risk for CP. The Early Detection/Early Identification/Early Intervention initiative (Cerebral Palsy Foundation) includes training providers to perform the HINE so it can be used to identify those children at risk for CP and enroll them in targeted early intervention services to maximize their outcomes.The HINE can be done in 5-10 minutes. [Byrne: 2017] demonstrates how the international guidelines for early diagnosis and intervention can be implemented in US high risk follow-up clinics.

Sensory Testing

Hearing testing
Recommended ages for follow-up testing for high-risk premature infants are at 9 months, 2 years, and 4 years (adjusted ages). Premature infants are considered high risk for any of the following:

Hypoxic-ischemic encephalopathy (HIE)
Extracorporeal membrane oxygenation (ECMO) use
Meningitis
Congenital cytomegalovirus (CMV)
Hyperbilirubinemia (specifically for infants with history of total serum bilirubin >=20, a brainstem auditory evoked response (BAER) test is recommended within 3 months of birth) [Phillips: 2013]
Craniofacial abnormalities
Family history of childhood hearing loss
Parental concerns
Chronic otitis media

Refer sooner if there are language or hearing concerns.
Infants who did not pass an initial hearing test in the NICU should have repeat testing completed within 2-4 weeks. See Hearing Loss & Deafness in Newborn Disorders and Hearing Loss and Deafness in Diagnoses & Conditions for more information on these topics.

Vision testing
Follow-up assessments for retinopathy of prematurity (ROP) are based on initial findings. Initial ROP screening typically takes place in the NICU and should be completed at 32 weeks postmenstrual age or 5 weeks postnatal age, whichever comes last. See the Portal's Premature Infant and Retinopathy of Prematurity issue page for information about risk factors and treatment considerations.

All infants born weighing 1500 grams or less, as well as those affected by intraventricular hemorrhage (IVH), should be considered for ophthalmology referral. Even in infants whose ROP exams reveal the resolution of ROP, follow-up with ophthalmology is recommended due to the high prevalence of problems of visual acuity and strabismus in this population.

Other Testing

Cognitive and motor testing
Children enrolled in NICU or Neonatal Follow-Up Programs will access testing at specific periods, although there is no national standard for which tests to use. Evaluations may include:

Infant motor testing such as the General Movements Assessment, Test of Infant Motor Performance, and Peabody Developmental Motor Scales
Bayley Scales of Infant Development (BSID-IV)
IQ tests (such as the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) or the Differential Ability Scale (DAS-2))
School Readiness evaluations such as the Bracken

More testing may be performed through Early Intervention, the public school district, and other developmental specialists.
Communication testing
Children enrolled in a Neonatal Follow-Up Program may be evaluated at set intervals by a speech-language pathologist for expressive, receptive, and pragmatic language skills. If difficulties are identified, children are referred for treatment provided through Early Intervention, developmental preschools, or the private sector.

Specialty Collaborations & Other Services

Neonatal Follow-up Programs (see ID providers [0])

The NICU typically does referral based on inclusion criteria, such as extreme prematurity. Primary care clinicians can also refer eligible premature infants for additional assessment and care coordination. The services/disciplines offered may include medical evaluation, physical therapy, occupational therapy, developmental psychology, speech pathology, and neurology.

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Refer when an infant or toddler under 36 months of age needs further assessment for possible developmental delays in cognitive, social, communication, and/or motor skills. Premature infants may qualify for Early Intervention based solely on their risk of developmental delays.

Developmental - Behavioral Pediatrics (see ID providers [2])

Refer for assistance in evaluating children with more complex developmental and behavioral problems. They assist with an overall diagnosis and help with specific recommendations for therapy, but do not typically provide ongoing primary care.

Pediatric Physical Medicine & Rehabilitation (see ID providers [3])

Refer to pediatric rehabilitation (“rehab") doctors, or physiatrists, for more in-depth assessment of musculoskeletal, neurologic, genetic, or conditions resulting in abnormal muscle tone and spasticity, such as cerebral palsy.

Audiology (see ID providers [21])

Consult for infants with hearing impairments and for those who have failed hearing screens. Parental concern for atypical hearing in their child is also a valid reason to obtain a formal Audiology consult. In clinics with limited or no hearing screening available, refer the premature infant to an audiologist for routine testing.

Pediatric Ophthalmology (see ID providers [9])

Premature infants with retinopathy of prematurity should be referred even if cleared of the ROP in the NICU.

Physical Therapy (see ID providers [34])

Refer for formal assessment and management of suspected motor skill delays.

Speech - Language Pathologists (see ID providers [68])

Refer for assessment and help evaluating feeding and early language and communication development. SLPs and OTs often work with radiologists to perform swallowing evaluations.

Pearls & Alerts for Treatment & Management

Ensure eligible families are connected with a Neonatal Follow-Up Clinic

Connecting families with a neonatal clinic is sometimes challenging, or overlooked, since families may be local only for a short time while their child is in the NICU. In addition, different states have different criteria for eligibility. Neonatal Follow-up Programs are appropriate and needed for all children meeting criteria, not just the children who have known developmental delays or complex medical conditions. Many children who appear to be developing typically have issues that can be addressed by early recognition and intervention.

Monitor for executive function difficulties in school-age children

All preterm infants carry some risk for executive function difficulties at school age. This may first be identified when the child is not performing at a peer grade level and having difficulties with reading and mathematics skills. Evaluation of learning difficulties may be performed at the school district.

Respiratory disease

Monitor for respiratory disease related to prematurity, such as symptoms of shortness of breath with activities, or hypoxemia with viral infections.

Growth or Weight Gain

During the first few months, term infants typically gain 20-30 g/day, after which their gain gradually slows down. For premature infants with lower birth weights, continued post-discharge growth of at least 10 g/kg/d is more likely. [Lapillonne: 2013] For infants with IUGR, a slow, steady weight gain is preferred as rapid weight gain can be related to adult chronic disease and obesity. Up to 85% of infants born SGA will “catch up” by age 2, but such rapid growth is uncommon after 2-3 years of age. For those with short stature after 2 years of age, consider endocrinology referral for evaluation of growth and growth hormone treatment, which has been found to be efficacious and safe in children with a history of SGA. [Claas: 2011] [Houk: 2012] Children with Short Stature Born Small for Gestational Age provides additional information.

Other

In addition to routine vaccines, infants and children with BPD should be considered for immunization with palivizumab (Synagis) and 23-valent pneumococcal (Pneumovax-23 or PPSV23). [Pickering: 2012]

Palivizumab is given in monthly intramuscular injections during the RSV season, which generally occurs during fall, winter, and spring in most locations in the United States. For the latest palivizumab guidance, please consult the Updated Guidance for Palivizumab Prophylaxis Among Infants and Young Children at Increased Risk of Hospitalization for Respiratory Syncytial Virus Infection.
See also Pneumococcal Disease (CDC), and Immunization Schedules (CDC).

Systems

Development (general)

Children who are born prematurely, particularly those born extremely early, often experience developmental delays. For some children, these delays will resolve over time; however, others will have lifelong disabilities. Early detection and intervention is the primary care provider's responsibility.

When a delay is recognized, further evaluation may be warranted (e.g., hearing testing for the child with speech/language delay or neurologic evaluation for abnormalities of movement, muscle tone, or limb asymmetry). In some situations, the child just needs time to “catch up.” Typical and Atypical Motor Development Videos (Pathways.org) has 3, 12-minute videos comparing typically developing and atypically developing children at 2 months, 4 months, and 6 months old. 0-3 Months Milestones (pathways.org) lists milestones for 0-3 years. The Milestone Tracker App (CDC) app is a helpful tool. Caution must be given to the parents to use the child’s adjusted age when viewing the app.

Special Education Services may include:

Developmental preschool: Developmental preschool is provided by the public school system for children over age 3 with developmental delays. Developmental preschool provides a structured learning environment with peer interaction and modeling by children who are “typically” developing. Therapies such as physical therapy, occupational therapy, and speech-language therapy are provided within the school setting when needed. Parental support is provided to families as well. These programs are operated by the local school district.
Specialized kindergarten: Available through the public school system to help kindergarten-age children who need special services. These programs go by various names, such as “Diagnostic Kindergarten."
Elementary and secondary school resources: Resources for learning disabilities are usually established around 7 years of age when IQ scores have become more reliable (barring extreme environmental challenges) and IQ subtests and academic achievement tests can distinguish learning strengths and weaknesses. Sometimes early indications of a learning disability can be seen at a Neonatal Follow-Up Clinic's 4-year-old cognitive testing, and the parents can be informed that they need to follow-up with academic achievement testing and cognitive testing in the early elementary school years.

Specialty Collaborations & Other Services

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Refer premature children ages 0-35 months who are at risk for (or who are demonstrating) developmental delays. Services may include visits by therapists (physical, occupational, speech, vision, etc.) and specific programming for a disability.

Head Start/Early Head Start (see ID providers [71])

Refer children ages 0-5 from low-income families for this federally funded school readiness program. Head Start can accommodate delays that are not severe, such as speech articulation, or a need to develop play skills, decrease aggression, etc.

Preschools (see ID providers [11])

Contact the local school district for eligibility information and refer children with developmental delays who over the age of 3.

School Districts (see ID providers [116])

Refer children ages 3 and up to their public school district for evaluation and services, including occupational, physical, and speech therapies, as needed to participate in educational activities.

Occupational Therapy (see ID providers [28])

Refer babies and children who have feeding or sensory difficulties, or motor delays that impact the child’s ability to participate in basic care activities. Refer older children to occupational therapists who specialize in hand function and activities of daily living.

Physical Therapy (see ID providers [34])

Referral can help evaluate delays in gross motor function, improving mobility, and customizing devices that enhance mobility.

Mental Health Evaluation/Assessment (see ID providers [32])

Referral can be useful for evaluation of delayed social skills.

Mental Health/Behavior

Infant and toddler behavioral problems, including ADHD, anxiety, and autism, are common among preterm infants and should be evaluated and treated in the same manner as for any other child. Some medical concerns are more common in children born prematurely and may cause or contribute to behavioral problems:

Inadequate sleep: Sleep Medications and Sleep Issues provide further diagnosis and management information.
Constipation: Constipation provides further diagnosis and management information.
Seizures: Seizures/Epilepsy provides further diagnosis and management information.
Side effects of medications
Gastroesophageal reflux:: Gastroesophageal Reflux Disease provides further diagnosis and management information.
Skin conditions causing chronic irritation: Skin and Wound Care for CYSHCN provides further diagnosis and management information.
Chronic ear infections and other sources of pain
Physical neglect or sexual or other physical abuse: Foster Care provides further diagnosis and management information for these toxic stress issues.
Oral aversion: feeding refusal. See Nutrition/Growth/Bone, below.

If primary care management of these issues and behavior problems proves unsuccessful, referral should be considered. The Medical Home Portal has diagnosis and management modules for Attention-Deficit/Hyperactivity Disorder (ADHD), Autism Spectrum Disorder, and a special topic page about Missing link with id: b46586ee.xml.

Specialty Collaborations & Other Services

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Some programs offer treatment for social problems (e.g., aggression or lack of social engagement, which can occur with autism) and classes to improve parenting skills. Many children who have been in the NICU qualify based on their diagnosis. See Baby Watch Early Intervention Program Eligibility Criteria (UDHHS) ( PDF Document 407 KB).

Head Start/Early Head Start (see ID providers [71])

Refer children ages 0-5 from low-income families for this federally funded school readiness program that can help with areas of non-severe delay, such as speech articulation, developing play skills, or decreasing aggression. This is generally not a placement for children with autism.

School Districts (see ID providers [116])

Refer children ages 3 and up with significant behavioral issues or social skills deficits to their public school district for evaluation and school-based behavioral services. Although variable, publicly supported services may include counselors and psychologists, special education services, social skills groups, and specialized behavioral classrooms.

Behavioral Therapies (see ID providers [31])

Refer children who need assistance with specific behaviors, mood disorders, or problem-solving skills. Children with autism spectrum disorder benefit from Applied Behavioral Analysis.

General Counseling Services (see ID providers [196])

Refer children who need private therapy for specific behaviors, mood disorders, or problem-solving skills. For delayed social skills, evaluation may be useful. Access varies with family’s income and insurance.

Psychiatry/Medication Management (see ID providers [20])

Refer children with complex mental health issues, particularly if they require psychiatric medication management beyond the primary care provider's expertise. Psychiatrists may be willing to help the medical home clinician manage medications without referral.

Communication

Graduates from the NICU are at high risk for communicative disorders such as:

Delayed early language development
Auditory processing deficits, generally diagnosed starting at age 7
Difficulties with articulation or production of speech sounds
Motor speech disorders of apraxia and dysarthria
Voice problems due to vocal cord paralysis or velopharyngeal incompetence

Parents should be educated about speech and language milestones and guided in modeling and language stimulation activities. When appropriate, home programs to work on specific deficit skills can be developed. Occasionally, significant motor impairment necessitates the use of augmentative communication devices.

Specialty Collaborations & Other Services

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Refer infants or children under the age of 3 with a suspected diagnosis of speech or language delay for developmental assessment and relevant services.

Speech - Language Pathologists (see ID providers [68])

Refer children with speech disorders or language delays whose needs are not adequately met through Early Intervention and the public school system as part of Special Education services. Speech and language therapists may be accessed through referrals to private therapists or through community-based and not-for-profit programs.

School Districts (see ID providers [116])

Refer children ages 3 and up with speech and language delays to their public school district for evaluation and services through Special Education. If a child has only a speech or language delay, it will need to be fairly severe to qualify for speech and language therapy in the schools. Private or community-based speech therapy is an option for children who do not qualify for school-based services.

Head Start/Early Head Start (see ID providers [71])

Refer children ages 0-5 from low-income families for this federally funded school readiness program. Head Start can help with speech articulation when the delay is not severe enough to qualify the child for developmental services such as Early Intervention or Special Education.

Eyes/Vision

Preterm infants are at risk for many problems that affect vision including retinopathy of prematurity (ROP), strabismus, cortical visual impairment, and problems with visual acuity.

Retinopathy of prematurity (ROP)
Each year in the United States, 14,000-16,000 infants develop retinopathy of prematurity (ROP), characterized by abnormal retinal vascularization in preterm infants; of these infants, approximately 400-650 become legally blind (>20/200). Infants less than 30 weeks or below 1500 grams should be screened for ROP. [Fierson: 2018] Even infants cleared of ROP in the NICU should be periodically followed by an ophthalmologist. Premature Infant and Retinopathy of Prematurity provides further information.

Strabismus
Strabismus is the improper alignment of the eyes. It can lead to diminished visual acuity and depth perception.

Cortical visual impairment (CVI)
CVI is a decreased visual response due to a neurological problem affecting the visual part of the brain. With CVI, either the eye exam is normal, or the child has an eye condition that cannot account for the abnormal visual behavior. [American: 2019] This condition used to be called cortical blindness, but this is misleading since vision can improve with time. Several conditions in preterm infants place them at increased risk of complications, such as:

<1500 grams
<32 weeks
High-grade IVH
Hydrocephalus
Severe illness (the need for ECMO)
Hypoxia
Infection, particularly CNS infection
PVL
Stroke

Many premature infants will receive their initial ophthalmologic exam in the NICU. Primary care providers can work with families to establish an ophthalmologic follow-up plan and to monitor for changes through routine vision screening and eye exams.

Specialty Collaborations & Other Services

Pediatric Ophthalmology (see ID providers [9])

Refer for expertise in evaluating and managing ophthalmologic complications of prematurity, especially retinopathy of prematurity and strabismus.

Special Education/Schools (see ID providers [7])

Refer children with significant vision and/or hearing sensory disorders.

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Refer infants or children under the age of 3 with visual impairment.

Ears/Hearing

Premature infants are at increased risk of hearing impairment, including later-onset and progressive hearing loss and auditory neuropathy. Contributing factors that can increase risk include:

Extreme prematurity as well as prolonged oxygen use
Infants with a history of in-utero infections, such as cytomegalovirus, rubella, syphilis, herpes, or toxoplasmosis
Infants who have received certain antibiotics, have received extracorporeal membrane oxygenation (ECMO), or have suffered other neurological complications

Infants should receive their first audiology screening prior to discharge from the NICU. Ensure follow-up for those children having abnormal screens. If hearing loss is identified, the first steps in assisting the child and family include:

Educating the family regarding the need for:
- Speech and language therapy
- Hearing amplification to overcome hearing loss
- Evaluation by an Ear, Nose and Throat Specialist (ENT)
- Early intervention services
Providing information about communication options and hearing technologies
Evaluating for associated medical conditions, such as heart arrhythmias, vision problems, and kidney problems

Hearing Loss and Deafness provides further diagnosis and management information.

Specialty Collaborations & Other Services

Audiology (see ID providers [21])

Refer for testing and evaluation of hearing at all ages; assistance in selecting, fitting, and counseling related to the use of augmentative hearing devices; and for mapping for cochlear implants.

Special Education/Schools (see ID providers [7])

Refer children with significant vision and/or hearing sensory disorders to services offered.

Nutrition/Growth/Bone

Premature infants’ nutritional needs and feedings are not the same as for full-term infants; they often require concerted effort to manage them effectively in the medical home. Feeding difficulties or gastrointestinal complications can affect the preterm infant’s nutrition. Despite the NICU feeding summary, unanticipated changes may occur after discharge, or the infant may no longer tolerate the schedule or the formula used in the NICU. Therefore, monitoring the infant’s growth rates, feeding volumes and skills, and adjusting nutrition are important roles of the medical home team. Consideration of caregiver’s skills and understanding is key with regards to feeding and should be assessed and monitored. Adaptations may have to be made to the NICU discharge feeding plans. Specialists in lactation, nutrition, or gastroenterology can be helpful resources. Preterm infants have increased demands for protein, calcium, and energy. For information about energy/protein/vitamin needs of the premature infant, human milk fortifier use, and specialized preterm formulas components, see the Portal’s issue page about the Missing issue with id: f109787f.xml.

If infants experience complications that interfere with adequate nutrition, osteopenia may result and fractures could occur. See Osteoporosis and Pathologic Fractures. Preterm infants are at risk for developing oral motor dysfunction and oral aversion, as well as gastroesophageal reflux and dysmotility, all of which can negatively affect feeding.

Feeding tubes
Motility problems, feeding difficulties, reflux, and/or aspiration may contribute to the need for gastric or jejunal feeding tubes. If such a device is in use, the family and primary care provider need to understand how the device works and how to access information should it stop working or need to be replaced. The primary care clinician should review this care plan with the family. See the Portal's page about Feeding Tubes & Gastrostomies in Children for further information.

Oral aversion
Feeding an infant can be an easy, natural process that gives the parent and child tremendous satisfaction and pride. However, when infants refuse food, parents can struggle to understand why and can have difficulty coping with the behavior and its reasons. They may even falsely believe that it is their fault. Oral aversion in the preterm infant may result from a complex interplay of both medical and behavioral factors. Oral aversions may be short-lived but can go on for years. When an oral aversion occurs, it is important to get specialists involved as soon as possible to begin teasing out the reasons for the problems and begin the therapeutic intervention. This can be as important for the parent as for the child. Speech and language pathologists, occupational therapists, and psychologists with experience in infant feeding difficulties can be very helpful and supportive to families. Often Early Intervention specialists also have a special interest in, and experience with, these difficulties.

Specialty Collaborations & Other Services

Nutrition Assessment Services (see ID providers [1])

Consultation can be helpful to determine nutritional needs and special dietary requirements for more complicated patients.

Gastro-Intestinal & Bowel Function

This section provides management information for an array of issues that infants and children with a history of prematurity can develop with the digestive tract, such as gastroesophageal reflux/disease(GER/GERD), cholestasis, oral motor dysfunction, and dysmotility. Infants who have undergone abdominal surgery for abdominal wall defects, obstructions, spontaneous intestinal perforation, necrotizing enterocolitis, or anorectal anomalies may be at increased risk for adhesions or strictures and dysmotility (either constipation or diarrhea). Hernias may also occur in premature infants. Related information on oral aversion and tube feeding can be found in Nutrition, above.

Gastroesophageal reflux (GER)
Premature infants may have difficulties with GER due to the immaturity of, and insult to, the gastrointestinal tract. In most babies, GER is a benign occurrence that does not cause pain or impair feeding or growth. It peaks around 4 months of age and gradually diminishes over the first year. Sometimes, though, it can cause severe pain and poor growth. Symptoms of GERD include feeding refusal, recurrent vomiting, poor weight gain, irritability, sleep disturbances, and respiratory symptoms such as coughing, wheezing, or recurrent pneumonia. GER is usually managed with avoidance of overfeeding, positioning, removal of dairy from the diet, thickening of formula, or medications. [Lightdale: 2013] Thickening of breast milk usually is not recommended because the live enzymes in breast milk start to digest the thickeners and can make the breast milk thinner. The Portal's page about Gastroesophageal Reflux Disease provides the details of these treatments.

Cholestasis
For those preterm infants with cholestasis (decreased bile flow) either from total parenteral nutrition (TPN) in the NICU or from other underlying medical causes, it is important to monitor laboratory values, such as a direct and indirect bilirubin, CMP, GGT, PT/INR, and PTT, and closely monitor the infant’s growth. Cholestasis can result in pruritus (itching), steatorrhea (fatty stools), and growth difficulties; additional caloric supplementation may be required. Medium-chain triglyceride oil, additional protein, and fat-soluble vitamins may be needed; supportive medications, such as ursodiol, may need to be weaned as an outpatient in consultation with a liver/gastroenterology specialist. [Suchy: 2004]

Oral motor dysfunction
Preterm infants may miss the staged approach to developing a coordinated suck and swallow when they have had a prolonged period of endotracheal intubation and/or illness. In addition, neurologic impairments may be present as with hydrocephalus or intraventricular hemorrhage, and these may contribute to the development of oral motor dysfunction. Oral pharyngeal dysphagia is when the child’s swallowing mechanism is abnormal and can result in frank or silent aspiration of fluid into the lungs, which may also contribute to oral aversion. A video swallow study (aka modified barium swallow study) performed under fluoroscopy can be quite helpful to the family and to the therapist working with the child to determine what happens with food or various thicknesses of liquid boluses and may provide guidance for intervention. When aspiration occurs, there may be degrees of thickness that may be recommended for the child using a variety of thickeners based on the child’s age and diagnosis. It is sometimes necessary to avoid oral feedings altogether and have a gastrostomy tube placed for hydration and nutrition until the aspiration stops. In this setting, continued non-nutritive oral motor stimulation through active therapy is critical to maintain and/or develop coordination of suck and swallow.

Gastrointestinal dysmotility and gastroparesis
Gastrointestinal dysmotility refers to difficulties passing fluid and nutrition along the gastrointestinal tract, most commonly manifesting with distention, vomiting, constipation, or diarrhea. Preterm infants are at risk for functional gastrointestinal dysmotility, possibly related to delayed introduction of enteral nutrition or immaturity of the GI system. Preterm infants are also at risk for gastrointestinal dysmotility due to the higher prevalence of necrotizing enterocolitis (NEC) in the neonatal period. Although the cause of NEC remains unknown despite years of study, around 5% percent of infants born before 33 weeks develop stage 2 or higher NEC, and approximately 20-40% of infants with NEC require surgical intervention with removal of small to large portions of the intestines. [Yee: 2012] Promotility agents, such as erythromycin, have been used as rescue medication for premature infants suffering from functional gastrointestinal dysmotility [Lam: 2011]; however, due to safety concerns including cardiac arrhythmias from erythromycin, use of prokinetic agents for preterm infants with dysmotility is undergoing continual reassessment and scrutiny, so guidance from a pediatric gastroenterologist may be warranted. [Shakir: 2018] Use of probiotics to aid in motility and prevent NEC in the preterm infant is still under study. [Indrio: 2011] Massage therapy to assist premature infants with dysmotility is also under investigation. [Field: 2010]

Gastroparesis, a specific type of dysmotility, is delayed gastric emptying without evidence of gastric outlet obstruction. Its prevalence in children is unknown. “Therapeutic approaches include dietary modifications, medical treatment (prokinetics, antiemetics, intrapyloric injection of botulinum toxin, enteral feeds via jejunostomy, total parenteral nutrition) and surgical interventions (laparoscopic placement of gastric pacemaker) aiming at alleviating symptoms and maintaining optimal nutritional status.” [Saliakellis: 2013]

Umbilical hernias
Umbilical hernias, caused by incomplete closure of the abdominal muscles around the site where the umbilical cord was present in utero, occur more frequently in premature infants. When a loop of intestine protrudes into this area, it can stick out. Crying and bearing down can make these hernias more noticeable. If the hernia remains reducible, surgery may not be required. Typically, umbilical hernias self-resolve over the first few years of life.

Specialty Collaborations & Other Services

Pediatric Gastroenterology (see ID providers [3])

Refer for evaluation and the collaborative management of necrotizing enterocolitis, short bowel syndrome, congenital gastrointestinal abnormalities with perforation, or failure to thrive.

Early Intervention for Children with Disabilities/Delays (see ID providers [149])

Refer for developmental delays affecting the infant’s ability to take oral nutrition.

Occupational Therapy (see ID providers [28])

Refer for feeding therapy. The training backgrounds of feeding therapists vary, and may include occupational therapists, speech-language pathologists, or developmental therapists; additional training in feeding therapy is optimal.

Pediatric Otolaryngology (ENT) (see ID providers [4])

Consult with ENT to evaluate and manage airway issues that impair swallowing and feeding, such as symptomatic laryngomalacia that may need surgical intervention.

Pediatric Otolaryngology (ENT) (see ID providers [4])

Consult with ENT to evaluate and manage airway issues that impair swallowing and feeding, such as symptomatic laryngomalacia that may need surgical intervention.

Speech - Language Pathologists (see ID providers [68])

Refer for feeding therapy. The training backgrounds of feeding therapists vary, and may include occupational therapists, speech-language pathologists, or developmental therapists; additional training in feeding therapy is optimal.

Respiratory

Bronchopulmonary dysplasia (BPD)
Bronchopulmonary dysplasia (BPD) is a form of chronic lung disease that primarily affects children born very preterm. Premature infants have BPD if they receive treatment with supplemental O2 with FiO2 > 21% for 28 days or more. Infants are categorized as having no, mild, moderate, or severe BPD. For infants born at less than 32 wks gestational age, BPD diagnosis and severity grading are made at corrected for gestational age 36 wks or at discharge (whichever occurs first) based on need for supplementation oxygen and/or positive pressure support. The relative incidence of mild, moderate, and severe BPD in infants born prior to corrected for gestational age < 32 are 30%, 30%, and 16%. [Abman: 2017] See Bronchopulmonary Dysplasia for management information.

Apnea of prematurity
Preterm infants display a variety of distinctive breathing patterns. Like most newborns, these infants can vary between normal breathing, periodic breathing (harmless, recurring sequences of several seconds of paused breathing followed by a slightly longer interval of rapid breathing), and more worrisome hypoventilatory and apneic episodes. Apnea is defined as no breathing for at least 20 seconds, or a shorter respiratory pause associated with bradycardia, and may include color changes such as pallor or cyanosis. [Berry: 2012] The infant may appear to be swallowing during these times. Episodes often are mixed central and obstructive apnea. Infections and metabolic conditions can worsen apnea. Infants typically must demonstrate several days of no apnea before leaving the NICU. If monitoring is continued after discharge, it usually may be safely discontinued by 43-44 weeks post-conception age. This apnea of prematurity resolves before the peak incidence of Sudden Infant Death Syndrome (SIDS) and is not known to directly increase the risk of SIDS. However, premature infants are at 3-4 times increased risk of SIDS than term infants. Peak incidence of SIDS extreme preterm infants (e.g., less than 25 weeks) starts at 40 weeks post-conception age, and at 44 weeks post-conception age for term infants - both last for 3-4 months. [Bright: 2017]

Vocal cord paralysis
Paralysis of the vocal cords may be unilateral or bilateral, and they can be secondary to anatomical problems or due to trauma during surgery or intubation procedures. In unilateral vocal cord paralysis, a weak cry is often noted as well as stridor when stressed. Unilateral paralysis often resolves within the first year. Bilateral paralysis has more severe symptoms and may require tracheostomy. When caring for infants with vocal cord paralysis, the clinician must be vigilant for aspiration. A pediatric ENT specialist should follow children with vocal cord paralysis. [Martin: 2006]

Laryngomalacia
Relatively common, this congenitally flaccid larynx can result in upper airway noises, including stridor, a coarse inspiratory noise that may develop typically within a month after birth. The intermittent airway obstruction is worse with agitation and crying, tends to progress during the first 8-12 months, then resolve by age 2. Positioning and calming are conservative management tools; very severe cases may require corrective surgery (supraglottoplasty) or tracheostomy. Control of gastroesophageal reflux and minimizing aspiration is also valuable. [Martin: 2006]

Tracheomalacia
Tracheomalacia occurs when there is weakness of the cartilages that support the trachea, either related to incomplete development or to trauma from prolonged intubation, GERD, or abnormal surrounding structures. Tracheomalacia may arise during the first year of life and is associated with abnormal breathing such as wheezing or stridor, dyspnea or apnea, or recurrent respiratory infections. Children with congenital abnormalities such as Trisomy 21, congenital heart disease, or tracheoesophageal fistula have higher rates of tracheomalacia or bronchomalacia. An ENT specialist or pulmonologist can diagnose tracheomalacia with endoscopy. Interventions may include supportive care, treatment of reflux, CPAP, and/or surgery. Most cases of tracheomalacia resolve spontaneously by age 2. [Graham: 2007]

Aspiration
Aspiration (paradoxical movement of feeds, refluxed gastric contents, or saliva into the airway and lungs) occurs more frequently in preterm infants. History may include choking, gagging, coughing, or brief cyanotic spells typically associated with feeds (including with gavage feeds). Aspiration should be considered in an infant with recurring pneumonitis or pneumonia. A swallow study typically consists of radiologic assessment of the swallow, such as a modified barium swallow study, as well as an evaluation by a feeding therapist (often a speech therapist). This swallow study provides the family, the primary care provider, and the outpatient feeding therapist with information on how to safely feed the infant, and may be repeated as needed to assess for changes. For infants with aspiration, suggested adaptations to routine feeding may range from thickened consistencies of liquids to tube feedings.

Specialty Collaborations & Other Services

Pediatric Pulmonology (see ID providers [2])

Consult for evaluation and management of persistent pulmonary complications, such as BPD, pulmonary hypertension, prolonged supplemental oxygen need for chronic hypoxemia, reactive airways/asthma, noisy breathing, or sleep-disordered breathing.

Pediatric Otolaryngology (ENT) (see ID providers [4])

Refer for evaluation and management of airway complications such as anomalies, laryngo- or tracheomalacia, vocal cord paralysis, stridor and noisy breathing, tracheostomy, or other obstructive breathing concerns.

Occupational Therapy (see ID providers [28])

Refer for assistance with evaluation and management of infants with suspected or known aspiration. The training backgrounds of feeding therapists vary, and may include occupational therapists, speech-language pathologists, or developmental therapists; additional training in feeding therapy is optimal.

Respiratory Therapy (see ID providers [0])

Consult to help guide management of more complex respiratory support needs such as tracheostomies and home ventilation.

Cardiology

Patent ductus arteriosus (PDA)
The ductus arteriosus, an essential structure for fetal circulation, typically closes spontaneously after birth within the first day of life. PDA in full-term infants usually is monitored until spontaneous closure occurs; however, surgery may be recommended if not closed by 6 months of age. [Martin: 2006] PDA occurs more frequently in premature and small babies; 30% of infants weighing <1.5 kg at birth have a PDA. [Martin: 2006] While spontaneous closure may occur in preterm infants, a PDA resulting in congestive heart failure and respiratory distress may require therapy to close the ductus. There are both medical and surgical options, including ductal ligation, and these are typically managed in the hospital setting.

Ventricular septal defect (VSD)
Infants with VSD (a persistent opening between the right and left ventricles of the heart) often are asymptomatic and spontaneous closure frequently occurs. The murmur may be appreciated clinically as a holosystolic or pansystolic murmur; however, large defects may not create an audible murmur. Electrocardiogram and chest radiograph can be reassuring, but echocardiogram is diagnostic. Large defects that do not spontaneously close can lead to congestive heart failure and other complications. Consultation with a pediatric cardiologist is helpful for family reassurance and to manage symptomatic defects.

Atrial septal defect (ASD)
Typically an incidental finding and may resolve in first year.

Hypertension
Preterm infants are at increased risk of hypertension. Approximately 2% of NICU graduates have hypertension, defined as >95th percentile for the infant’s size, gestational age, and postnatal age [Nickavar: 2014], often from renovascular issues. This risk increases if the infant has undergone umbilical artery catheterization, has received extracorporeal membrane oxygenation (ECMO), or has comorbid conditions such as renal, endocrine, cardiac, or pulmonary problems (including bronchopulmonary dysplasia), or has a history of intraventricular hemorrhage (IVH). Up to 9% of infants with a history of umbilical artery catheterization may develop hypertension, underlining the importance of blood pressure monitoring in the outpatient setting particularly for preterm infants. [Flynn: 2016] Some infants with hypertension will require fluid restriction, dietary changes, or medications. Management in consultation with a pediatric nephrologist and/or cardiovascular specialist may be required. Use blood pressure charts with age-appropriate norms; daily home blood pressure monitoring can also help with babies discharged on antihypertensive medication. [Flynn: 2016] Prognosis is usually good.

Neonatal Hypertension (Medscape) (log-in required) provides a table of Neonatal Blood Pressures and Potential Treatment Parameters; to access click “Tables” on left menu.
Pediatric Hypertension (Medscape) provides a table of Normal Blood Pressure Percentile Curves for Older Infants from [Lowry: 1971]; to access click “Tables” on left menu.

Persistent pulmonary hypertension of the newborn (PPHN)
When pulmonary vessels do not relax sufficiently to effectively adapt to the extrauterine environment, the high pulmonary vascular resistance can lead to pulmonary hypertension and right ventricular dilation and/or hypertrophy. In response, the heart may shunt blood across the ductus arteriosus and the foramen ovale, which may lead to hypoxemia. Treatments are focused on improving any underlying lung disease as well as reducing or eliminating pulmonary hypertension. Interventions may include supplemental oxygen (which acts as a pulmonary vasodilator), surfactant, mechanical ventilation, inhaled nitric oxide, ECMO, correction of metabolic abnormalities, thermoregulation, and anti-pulmonary hypertensive medications. This condition tends to happen more frequently in near-term or term infants. These infants are at increased risk of adverse neurodevelopmental outcomes and sensorineural hearing loss; therefor, these conditions that should be monitored for in the outpatient setting. [Tauber: 2019]

Thromboembolism
Premature infants often undergo invasive catheterization at birth or during the NICU stay. Invasive catheterization of arteries and veins increases the risk of thromboembolism such as in the aorta or renal blood vessels. When the renal vessels are affected, the infant may develop hypertension, impaired urine excretion and kidney function, congestive heart failure, and impaired blood flow to the lower extremities. Babies may require intervention for thrombolysis and medications to control blood pressure. Thrombosed kidneys may atrophy over time, leading to sustained renal insufficiency and hypertension. Ongoing management may include monitoring blood pressure and kidney function with serum creatinine clearance and urine studies, and ultrasonography.

Specialty Collaborations & Other Services

Pediatric Cardiology (see ID providers [2])

Consult for assistance in diagnosing and managing persistent cardiovascular problems, such as pulmonary hypertension or persistent ductal or septal defects, and for determining timing of surgery.

Pediatric Cardiothoracic Surgery (see ID providers [0])

Refer for surgical interventions for severe or persistent cardiovascular problems such as congenital heart disease and defects, pulmonary hypertension, or persistent ductal or septal defects.

Neurology

Infants born preterm are at an increased risk for neurological damage, which can lead to cerebral palsy, including tone abnormalities, motor delays, and other neurodevelopmental problems. At particular risk are those infants with a history of intraventricular hemorrhage (IVH), post-hemorrhagic hydrocephalus, neonatal seizure(s), neonatal stroke, periventricular leukomalacia (PVL), cerebellar injury, and porencephaly. Infants with brain injury either from before or during birth, or subsequent to complications of their neonatal care, may also develop seizures.

Cerebral palsy (CP)
CP occurs in approximately 12-15% of extremely premature infants. Although the injury is not progressive, it may take time, serial examinations for developmental delays, and recognition of abnormal movement patterns to recognize CP. Early identification and initiation of intervention are essential to the optimal outcome of the child. Tools for identification may be used in the NICU, such as the General Movements Assessment (GMA). Classification of the infant’s movement as “cramped synchronized” places the infant at high risk for a movement disorder. Further evaluation by the Neonatal Follow-up Clinic may include a specific neurologic exam, the Hammersmith Neurologic Exam (HINE), which evaluates for tone, posture, cranial nerve findings, reflexes, and movements.

HINE scores less than 57 at 3 months of age, less than 63 at 6-9 months are a concern and place the child at risk for CP. Taken together, the GMA and the HINE exams are highly sensitive and specific for cerebral palsy. [Novak: 2017] Delays in diagnosis may delay interventions at a critical time in brain maturation. Therapy for motor abnormalities can and should be initiated within the NICU and at discharge. Waiting for a diagnosis unnecessarily delays therapeutic intervention. Many rehabilitative services are available to help children with CP and their families. Growth should be monitored with the future in mind. Growth charts for children with CP begin at age 2 years. Prior to that age an infant suspected for CP may demonstrate a slower weight growth pattern which may actually be acceptable. Rapid weight gain in an infant suspected of having CP may impede development because the infant is heavy. Please see the Cerebral Palsy module for evaluation, management, and services information. For videos of typically developing infants and scoring by Dr. Frances Cowan, register at Hammersmith Neurologic Exam (HINE).

Seizures
Seizures may occur at any age. Neonatal seizures are those with onset between 0-2 months. Infantile spasms are a severe type of early seizure and are often related to perinatal asphyxia, prenatal infections, or other conditions such as tuberous sclerosis or metabolic disorders. Some seizure types do not require antiepileptic medication, but they do require special precautions during bathing and other activities. The Portal modules Seizures/Epilepsy and Infantile Spasms contain diagnosis and management details.

Specialty Collaborations & Other Services

Pediatric Neurology (see ID providers [1])

Consult for assistance with initial diagnosis of cerebral palsy, evaluation of unusual or atypical tone and movement patterns, and management of seizures.

Pediatric Neurosurgery (see ID providers [2])

Refer for shunt management and ongoing evaluation of hydrocephalus as well as evaluation of atypical head shape.

Medical Genetics (see ID providers [3])

Offer a consultation with geneticist or genetic counselor when there is concern of a genetic abnormality that may be contributing to the infant’s preterm condition and/or complications.

Genito-Urinary

Nephrocalcinosis/kidney stones
Calcium deposits in the renal interstitium, known as nephrocalcinosis, occur in many premature infants. There is increased risk for infants who receive loop diuretics, such as for chronic heart or lung disease, which can cause hypercalciuria. While these kidney stones typically resolve within the first few months after discontinuing diuretics, there is concern that persistent stones may inhibit kidney development and impair function. Ultrasonography is useful for identifying stones, and measurement of spot urine calcium and creatinine ratio can help to monitor for adequate excretion.

Hydrocele
Premature and low birth-weight infants are at risk of developing unilateral or bilateral hydroceles, or excess fluid in the scrotum due to incomplete closure of the processus vaginalis. These typically transilluminate and often resolve spontaneously, but some will require surgery to close. Refer communicating hydroceles upon discovery to prevent incarceration; refer other hydroceles if problematic and not resolved by 1 year. [McInerny: 2008]

Inguinal hernias
Inguinal hernias occur in 11% of infants born under 1500 grams and are more commonly found on the right side and in male infants. [Kumar: 2002] During fetal development, testicles pass from the abdomen into the scrotum through the inguinal canal. If there is incomplete closure of this canal after birth, an inguinal hernia may occur. This can allow a loop of intestine to sag through the inguinal canal and into the scrotum, which presents risks for strangulated bowel. Since girls have inguinal canals, they are also at some risk for hernias. Refer inguinal hernias for surgical repair. [McInerny: 2008]

Undescended testes
In premature males, undescended testes are common. If not self-resolving in the first 6 months, or if worsening, then a surgical referral should be made.

Circumcision
Male infants born prematurely may not be offered a circumcision procedure while in the NICU. Information can be provided to interested families about safely obtaining circumcision based on the age, size, and health of their child. Some families may elect to have a circumcision performed if the child is undergoing sedation for another procedure.

Specialty Collaborations & Other Services

Pediatric Urology (see ID providers [1])

Refer for evaluation and surgical management of persistent urologic problems such as urinary reflux, posterior urethral valves and hydronephrosis, inguinal hernias, or persistent hydroceles.

Pediatric Nephrology (see ID providers [1])

Consultation is helpful in evaluating and managing kidney and urinary tract issues such as nephrocalcinosis, hypertension, reflux, and abnormal kidney function.

Musculoskeletal

Head shape
Head deformation occurs in just under 1 out of 5 term infants; in infants born prematurely, that rate is higher. [Rogers: 2011] Plagiocephaly, or asymmetric flattening of the posterior skull, is common due to lying in a supine position. Dolichocephaly (narrow biparietal or side-to-side measurement with an elongated skull) is now less common with developmental care in the NICU. Often, head shape variation is a cosmetic issue and requires no treatment. However, there are circumstances when further evaluation should be undertaken and treatment with a helmet is considered. [Rogers: 2011] Craniosynostosis, or early closure of 1 or more cranial sutures, can look similar to some types of deformational abnormalities. For more details about management, see Cranial Deformation and Craniosynostosis.

Hips, extremities, and spine
Formation of the bones and muscles is generally unaffected by premature birth. As with term infants, parents may need reassurance about the relative bow-legged appearance of their infant. Hip exams should be performed at routine intervals for the first 1-2 years, and those with suspected hip dysplasia or abnormal hip exams should be referred for imaging and specialist consultation. When performing screening imaging for hip dysplasia based on risk factors such as breech positioning or sibling with hip dysplasia, imaging should be completed at intervals adjusted for prematurity. For children with cerebral palsy or congenital malformations, the medical home provider should work with a team of specialists to evaluate and manage the condition.

Specialty Collaborations & Other Services

Pediatric Orthopedics (see ID providers [6])

Refer for help managing congenital malformations, suspected hip or spine problems, and for routine care and management of children with cerebral palsy.

Physical Therapy (see ID providers [34])

Refer for help managing persistent torticollis and to prevent associated cranial deformity.

Pediatric Plastic Surgery (see ID providers [4])

Refer to a plastic surgeon, neurosurgeon, or craniofacial specialist to evaluate and manage cranial deformities.

Pediatric Neurosurgery (see ID providers [2])

Refer to a neurosurgeon, plastic surgeon, or craniofacial specialist to evaluate and manage cranial deformities.

Dental

Because infants born preterm are at increased risk for enamel hypoplasia and dental defects, good dental hygiene and dental health-promoting practices within a pediatric dental home are especially important. Infants born prematurely should see a dentist by 1 year of age or after eruption of the first tooth. [Schaaf: 2011] A narrow palate is common and can cause difficulties moving food in the mouth and contribute to short-term feeding difficulties. It is presumed to be a result of orotracheal intubation. [Hohoff: 2005] [Paulsson: 2004] Teething may occur following corrected gestational age, but the age ranges vary. It is not known what the long-term consequences of prematurity are on the palate or its morphology and the need for treatment such as orthodontia.

Specialty Collaborations & Other Services

Pediatric Dentistry (see ID providers [60])

Referral is helpful for infants and children with abnormal dentition or sensory issues affecting their ability to cooperate with dental care and examinations.

Skin & Appearance

Remind families to protect scars with sunblock during periods of sun exposure.

The following Portal topics contain diagnosis and management information for conditions often related to premature infant follow-up care:

On the Web

Follow-Up of the NICU Patient (Medscape)
Includes information about areas of assessment, long-term monitoring, testing, and outcomes.

Neonatal Hypertension (Medscape)
Provides presentation, diagnosis, and treatment information.

The American Academy of Cerebral Palsy and Developmental Medicine (AACPDM)
Multidisciplinary, scientific education for health professionals and promote excellence in research and services for the benefit of people with and at risk for cerebral palsy and other childhood-onset disabilities.

Helpful Articles

TeKolste T, Bragg J, Wendel S.
Extremely Low Birth Weight NICU Graduate.
2004; Washington State Department of Health, Children with Special Health Care Needs Program; https://depts.washington.edu/dbpeds/ELBW-NICU-Graduate.pdf
Supplement to: Low Birth Weight Neonatal Intensive Care Graduate. Specifically addresses post-NICU care of ELBW infants who: 1) experienced the usual complications associated with extreme prematurity and/or extreme low birth weight, and 2) were discharged home in a relatively healthy condition.

Villar J, Giuliani F, Barros F, Roggero P, Coronado Zarco IA, Rego MAS, Ochieng R, Gianni ML, Rao S, Lambert A, Ryumina I, Britto C, Chawla D, Cheikh Ismail L, Ali SR, Hirst J, Teji JS, Abawi K, Asibey J, Agyeman-Duah J, McCormick K, Bertino E, Papageorghiou AT, Figueras-Aloy J, Bhutta Z, Kennedy S.
Monitoring the Postnatal Growth of Preterm Infants: A Paradigm Change.
Pediatrics. 2018;141(2). PubMed abstract

Assessment Tools/Scales

Edinburgh Postnatal Depression Scale (English) ( PDF Document 120 KB)
A self-administered, 10-question, 5-minute screen for maternal depression with scoring instructions. Free, may be printed without permission.

Edinburgh Postnatal Depression Scale (Spanish) ( PDF Document 54 KB)
A Spanish, self-administered, 10-question, 5-minute screen for maternal depression with scoring instructions. Free, may be printed without permission.

Patient Health Questionnaire (PHQ) Screeners
Free screening tools in many languages with scoring instructions to be used by clinicians to help detect mental health disorders. Select from right menu: PHQ, PHQ-9, GAD-7, PHQ-15, PHQ-SADS, Brief PHQ, PHQ-4, PHQ-8.

Screening for Developmental Hip Dysplasia—Clinical Algorithm (AAP)
Algorithm with screening recommendations and recommended actions; American Academy of Pediatrics.

Hammersmith Neurologic Exam (HINE)
This exam consists of 26 items that assess different aspects of neurological function. The HINE is aimed to be used for infants between 3 and 24 months of age.

Growth/BMI Charts

Growth Charts - 22 Weeks - 92 Weeks Postconceptional Age
Evaluates the growth adequacy during the first year of life in preterm infants according to sex (based on intrauterine growth measurements). Charts are from a "Supplement: Nutritional Recommendations for the Late-Preterm Infant and the Preterm Infant after Hospital Discharge;" Journal of Pediatrics - subscription required for access.

Other

Training Module for CDC Growth Charts for Children with Special Health Care Needs (MCHB)
Hour-long, online module that teaches how the CDC Growth Charts can be used with children who have special health care needs; Maternal and Child Health Bureau.

Frequently Asked Questions About the 2000 CDC Growth Charts (CDC)
Answers to 8 questions about CDC growth charts and their use with special populations; Centers for Disease Control and Prevention.

Demonstrations: Heart Sounds & Murmurs (University of Washington)
Audio files of 16 different heart sounds and murmurs.

Immunization of Preterm and Low Birth Weight Infants (AAP) ( PDF Document 256 KB)
Guidance for immunizing preterm and low birth weight infants; American Academy of Pediatrics.

Typical and Atypical Motor Development Videos (Pathways.org)
Three, 12-minute videos that compare typically developing with atypically developing children at 2 months, 4 months, and 6 months old; supported by American Academy of Pediatrics.

Learn the Signs. Act Early. Autism (CDC)
Fact sheets, growth charts, and posters for early identification and diagnosis of autism and other developmental disabilities; National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention.

Milestone Tracker App (CDC)
Track your child’s milestones from age 2 months to 5 years with easy-to-use illustrated checklists; get tips from CDC for encouraging your child’s development; and find out what to do if you are ever concerned about how your child is developing; Centers for Disease Control and Prevention.

Patient Education & Instructions

Tips for Encouraging Speech and Language Development (Pathways.org) ( PDF Document 466 KB)
Two-page brochure with speech and hearing milestones and tips for assisting with their development.

Cerebral Palsy Channel; developed by the Cerebral Palsy Foundation
This free app offers information from the world's leading experts on intervention and therapy, communication, building independence, and more.

Premature Infant Follow-Up (FAQ)

Information on the Web

Care Notebook
The care notebook helps keep track of appointments, resources, labs, medications, tests, care providers, and more. Download the complete notebook, compile in your own binder, or download separate forms.

Taking Your Preemie Home (KidsHealth)
Information to help parents transition from NICU to home care.

Preemie Milestones (AAP) ( PDF Document 824 KB)
Helps parents understand important developmental milestones to watch for at each age and how to determine which milestones to use for preemies; American Academy of Pediatrics, March of Dimes, and National Association of Neonatal Nurses.

Cerebral Palsy Foundation
Nonprofit organization that helps people with cerebral palsy get the interventions and supports they need when they need them.

National & Local Support

Find Your Parent Center
Parent Centers provide education and referrals for families with a child who has a disability, as well as the professionals who work with them. There are almost 100 Parent Training and Information Centers (PTIs) and Community Parent Resource Centers (CPRCs) in the US states and Territories; Center for Parent Information & Resources.

National Center for Hearing Assessment and Management (NCHAM)
Extensive compilation of resources and support for families with a child who is deaf or hard of hearing.

Studies/Registries

Clinical Trials for Preterm Infants (clinicaltrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Services for Patients & Families in Idaho (ID)

The following lists resources that may be particularly helpful for finding services related to premature infant follow-up:

Service Categories	NW		NM	NV	OH	RI
Audiology	3	19	8	4	25	28
Behavioral Therapies	1	8	20	1	31	34
Developmental - Behavioral Pediatrics	1	2	2	2	12	9
Developmental Assessments	1	104	6	1	35	56
Early Intervention for Children with Disabilities/Delays	3	35	32	3	14	55
Family Support Services	13	23	68	15	44	37
General Counseling Services	1	3	209	1	30	362
Head Start/Early Head Start	1	7	58	2	19	32
Medical Genetics	1	2	5	1	4	8
Mental Health Evaluation/Assessment		4	10		24	146
Neonatal Follow-up Programs		1			1	3
Nutrition Assessment Services			3	1	2	6
Occupational Therapy	1	17	27	2	19	38
Pediatric Cardiology		2	4		17	5
Pediatric Cardiothoracic Surgery			1		6	2
Pediatric Dentistry	2	6	31	3	41	50
Pediatric Gastroenterology	1	3	6	1	19	4
Pediatric Nephrology		2	2		10	1
Pediatric Neurology		5	5		17	6
Pediatric Neurosurgery	1	2	4	1	3	2
Pediatric Ophthalmology	1	6	6	1	8	4
Pediatric Orthopedics	4	6	8	4	16	21
Pediatric Otolaryngology (ENT)	1	8	5	1	7	10
Pediatric Physical Medicine & Rehabilitation	3	3	3	3	6	14
Pediatric Plastic Surgery	3	5	4	3	4	5
Pediatric Pulmonology		4	4		8	5
Pediatric Urology			13		1	3
Physical Therapy		12	11	1	5	48
Preschools		4	30		11	79
Psychiatry/Medication Management		2	49		79	56
Respiratory Therapy						1
School Districts		90	22	1	63	47
Special Education/Schools	3	82	9	4	40	44
Speech - Language Pathologists	4	22	13	4	31	69

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: July 2015; last update/revision: August 2020

Current Authors and Reviewers:

Authors:	Jennifer Goldman, MD, MRP, FAAP
	Sarah Winter, MD
	Mary Ann Nelin, MD
Contributing Author:	Annette Haban Bartz, MS, RD, LD, CLC
Reviewers:	Molly O'Gorman, MD
	Khanh Lai, MD, FAAP
	Erin Clark, MD

Authoring history

2015: first version: Jennifer Goldman, MD, MRP, FAAP^A; Sarah Winter, MD^A; Mary Ann Nelin, MD^R

^AAuthor; ^CAContributing Author; ^SASenior Author; ^RReviewer

Abman SH, Collaco JM, Shepherd EG, Keszler M, Cuevas-Guaman M, Welty SE, Truog WE, McGrath-Morrow SA, Moore PE, Rhein LM, Kirpalani H, Zhang H, Gratny LL, Lynch SK, Curtiss J, Stonestreet BS, McKinney RL, Dysart KC, Gien J, Baker CD, Donohue PK, Austin E, Fike C, Nelin LD.
Interdisciplinary Care of Children with Severe Bronchopulmonary Dysplasia.
J Pediatr. 2017;181:12-28.e1. PubMed abstract / Full Text
Recommendations from the “BPD Collaborative, a group of clinicians from interdisciplinary care programs for infants with sBPD at several major medical centers, including neonatologists, pulmonologists, critical care physicians, gastroenterologists, nurse specialists, and others, to address controversies and promote research to enhance the care of children with sBPD. The review emphasizes an interdisciplinary approach throughout the NICU and outpatient courses.

Adams-Chapman I, Heyne RJ, DeMauro SB, Duncan AF, Hintz SR, Pappas A, Vohr BR, McDonald SA, Das A, Newman JE, Higgins RD.
Neurodevelopmental Impairment Among Extremely Preterm Infants in the Neonatal Research Network.
Pediatrics. 2018;141(5). PubMed abstract / Full Text

American Association for Pediatric Ophthalmology and Strabismus.
Cortical Visual Impairment.
(2019) https://aapos.org/glossary/cortical-visual-impairment. Accessed on 07/08/2020.

Berry RB, Budhiraja R, Gottlieb DJ, Gozal D, Iber C, Kapur VK, Marcus CL, Mehra R, Parthasarathy S, Quan SF, Redline S, Strohl KP, Davidson Ward SL, Tangredi MM.
Rules for scoring respiratory events in sleep: update of the 2007 AASM Manual for the Scoring of Sleep and Associated Events. Deliberations of the Sleep Apnea Definitions Task Force of the American Academy of Sleep Medicine.
J Clin Sleep Med. 2012;8(5):597-619. PubMed abstract / Full Text

Bright FM, Vink R, Byard RW, Duncan JR, Krous HF, Paterson DS.
Abnormalities in substance P neurokinin-1 receptor binding in key brainstem nuclei in sudden infant death syndrome related to prematurity and sex.
PLoS One. 2017;12(9):e0184958. PubMed abstract / Full Text

Byrne R, Noritz G, Maitre NL.
Implementation of Early Diagnosis and Intervention Guidelines for Cerebral Palsy in a High-Risk Infant Follow-Up Clinic.
Pediatr Neurol. 2017;76:66-71. PubMed abstract

Claas M.J., de vries LS, Koopman C, Uniken Venema MM, Eijsermans MJ, Bruinse HW, Verrin Stuart AA.
Postnatal growth of preterm born children ≤750g at birth.
Early Human Development. 2011;87(7):495-507. PubMed abstract

Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip.
Clinical practice guideline: early detection of developmental dysplasia of the hip. Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip. American Academy of Pediatrics.
Pediatrics. 2000;105(4 Pt 1):896-905. PubMed abstract

D'Agostino JA.
An evidentiary review regarding the use of chronological and adjusted age in the assessment of preterm infants.
J Spec Pediatr Nurs. 2010;15(1):26-32. PubMed abstract / Full Text

Dionne JM, Abitbol CL, Flynn JT.
Hypertension in infancy: diagnosis, management and outcome.
Pediatr Nephrol. 2012;27(1):17-32. PubMed abstract

Earls MF, Yogman MW, Mattson G, Rafferty J.
Incorporating recognition and management of perinatal and postpartum depression into pediatric practice.
Pediatrics. 2019. PubMed abstract / Full Text

Ferré C, Callaghan W, Olson C, Sharma A, Barfield W.
Effects of Maternal Age and Age-Specific Preterm Birth Rates on Overall Preterm Birth Rates - United States, 2007 and 2014.
MMWR Morb Mortal Wkly Rep. 2016;65(43):1181-1184. PubMed abstract

Field T, Diego M, Hernandez-Reif M.
Preterm infant massage therapy research: a review.
Infant Behav Dev. 2010;33(2):115-24. PubMed abstract / Full Text

Fierson WM.
Screening Examination of Premature Infants for Retinopathy of Prematurity.
Pediatrics. 2018;142(6). PubMed abstract

Flynn J.
Neonatal hypertension.
Medscape; (2016) http://emedicine.medscape.com/article/979588-overview. Editor: Rosenkrantz T; Contributors: Pramanik A, Windle ML. Accessed on 7/8/2020.

Flynn JT, Daniels SR, Hayman LL, Maahs DM, McCrindle BW, Mitsnefes M, Zachariah JP, Urbina EM.
Update: ambulatory blood pressure monitoring in children and adolescents: a scientific statement from the American Heart Association.
Hypertension. 2014;63(5):1116-35. PubMed abstract / Full Text

Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, de Ferranti SD, Dionne JM, Falkner B, Flinn SK, Gidding SS, Goodwin C, Leu MG, Powers ME, Rea C, Samuels J, Simasek M, Thaker VV, Urbina EM.
Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents.
Pediatrics. 2017;140(3). PubMed abstract / Full Text

Graham JM, Scadding GK, Bull PD ed.
Pediatric ENT.
New York: Springer; 2007. 978-3-540-33038-7 http://www.springer.com/medicine/otorhinolaryngology/book/978-3-540-69...

Hagan JF, Shaw JS, Duncan PM, eds. .
Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents.
3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2008. 978-1-58110-223-9 http://brightfutures.aap.org/
Authoritative compilation of guidelines and evidence for health supervision, including developmental surveillance and screening, physical exam, laboratory and other testing, and anticipatory guidance. The Affordable Care Act of 2010 cites Bright Futures as the standard for well child and adolescent care.

Harlor AD Jr, Bower C.
Hearing assessment in infants and children: recommendations beyond neonatal screening.
Pediatrics. 2009;124(4):1252-63. PubMed abstract / Full Text

Hayes D Jr, Wilson KC, Krivchenia K, Hawkins SMM, Balfour-Lynn IM, Gozal D, Panitch HB, Splaingard ML, Rhein LM, Kurland G, Abman SH, Hoffman TM, Carroll CL, Cataletto ME, Tumin D, Oren E, Martin RJ, Baker J, Porta GR, Kaley D, Gettys A, Deterding RR.
Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.
Am J Respir Crit Care Med. 2019;199(3):e5-e23. PubMed abstract / Full Text

Hohoff A, Rabe H, Ehmer U, Harms E.
Palatal development of preterm and low birthweight infants compared to term infants - What do we know? Part 2: The palate of the preterm/low birthweight infant.
Head Face Med. 2005;1:9. PubMed abstract / Full Text

Houk CP, Lee PA.
Early diagnosis and treatment referral of children born small for gestational age without catch-up growth are critical for optimal growth outcomes.
International Journal of Pediatric Endocrinology. 2012;1:11. PubMed abstract / Full Text

Indrio F, Neu J.
The intestinal microbiome of infants and the use of probiotics.
Curr Opin Pediatr. 2011;23(2):145-50. PubMed abstract / Full Text

Institute of Medicine (US) Committee on Understanding Premature Birth and Assuring Healthy Outcomes.
Preterm Birth: Causes, Consequences, and Prevention.
Washington DC: National Academies Press; 2007. 978-0-309-10159-2 http://www.ncbi.nlm.nih.gov/books/NBK11362/
The extensive report provides information on risk factors and suggested strategies to reduce preterm births.

Johnson CP, Myers SM.
Identification and evaluation of children with autism spectrum disorders.
Pediatrics. 2007;120(5):1183-215. PubMed abstract
Comprehensive clinical report addressing the definition, history, epidemiology, diagnostic criteria, early signs, neuropathologic aspects, and etiologic possibilities in autism spectrum disorders. This report also provides the primary care provider with an algorithm for assistance in the early identification of children with autism spectrum disorder.

Klebanoff MA, Keim SA.
Epidemiology: the changing face of preterm birth.
Clin Perinatol. 2011;38(3):339-50. PubMed abstract

Kumar VH, Clive J, Rosenkrantz TS, Bourque MD, Hussain N.
Inguinal hernia in preterm infants (< or = 32-week gestation).
Pediatr Surg Int. 2002;18(2-3):147-52. PubMed abstract

Lahood A., Bryant, C.
Outpatient Care of the Premature Infant.
American Family Physician. 2007;76(8). / Full Text

Lam HS, Ng PC.
Use of prokinetics in the preterm infant.
Curr Opin Pediatr. 2011;23(2):156-60. PubMed abstract

Lapillonne A, O'Connor DL, Wang D, Rigo J.
Nutritional recommendations for the late-preterm infant and the preterm infant after hospital discharge.
J Pediatr. 2013;162(3 Suppl):S90-100. PubMed abstract

Lightdale JR, Gremse DA.
Gastroesophageal reflux: management guidance for the pediatrician.
Pediatrics. 2013;131(5):e1684-95. PubMed abstract

Lindström K, Lindblad F, Hjern A.
Preterm birth and attention-deficit/hyperactivity disorder in schoolchildren.
Pediatrics. 2011;127(5):858-65. PubMed abstract

Lowry RB, Renwick DH.
Relative frequency of the Hurler and Hunter syndromes.
N Engl J Med. 1971;284(4):221-2. PubMed abstract

Martin RJ, Fanaroff AA, Walsh MC ed.
Fanaroff and Martin's neonatal‐perinatal medicine diseases of the fetus and infant, Vols I and II.
8th ed. Philadelphia, PA: Elsevier Mosby; 2006. 0-323-02966-3 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2672777/

McCormick MC, Litt JS, Smith VC, Zupancic JA.
Prematurity: an overview and public health implications.
Annu Rev Public Health. 2011;32:367-79. PubMed abstract

McInerny T, Adam H, Campbell D, Kamat D, Kelleher K.
American Academy of Pediatrics Textbook of Pediatric Care.
1st Chapter 216 ed. American Academy of Pediatrics; 2008. 1581102682 http://www.amazon.com/American-Academy-Pediatrics-Textbook-Pediatric/d...

National Center for Health Statistics.
Births and Natality.
Centers for Disease Control and Prevention; (2020) https://www.cdc.gov/nchs/fastats/births.htm. Accessed on July 2020.

National Eye Institute.
Retinopathy of Prematurity (ROP).
(2019) https://www.nei.nih.gov/health/rop/rop. Accessed on 7/8/2020.

Nickavar A, Assadi F.
Managing hypertension in the newborn infants.
Int J Prev Med. 2014;5(Suppl 1):S39-43. PubMed abstract / Full Text

Norman M.
Preterm birth--an emerging risk factor for adult hypertension?.
Semin Perinatol. 2010;34(3):183-7. PubMed abstract

Novak CM, Ozen M, Burd I.
Perinatal Brain Injury: Mechanisms, Prevention, and Outcomes.
Clin Perinatol. 2018;45(2):357-375. PubMed abstract

Novak I, Morgan C, Adde L, Blackman J, Boyd RN, Brunstrom-Hernandez J, Cioni G, Damiano D, Darrah J, Eliasson AC, de Vries LS, Einspieler C, Fahey M, Fehlings D, Ferriero DM, Fetters L, Fiori S, Forssberg H, Gordon AM, Greaves S, Guzzetta A, Hadders-Algra M, Harbourne R, Kakooza-Mwesige A, Karlsson P, Krumlinde-Sundholm L, Latal B, Loughran-Fowlds A, Maitre N, McIntyre S, Noritz G, Pennington L, Romeo DM, Shepherd R, Spittle AJ, Thornton M, Valentine J, Walker K, White R, Badawi N.
Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment.
JAMA Pediatr. 2017;171(9):897-907. PubMed abstract

Nwafor-Anene VN, DeCristofaro JD, Baumgart S.
Serial head ultrasound studies in preterm infants: how many normal studies does one infant need to exclude significant abnormalities?.
J Perinatol. 2003;23(2):104-10. PubMed abstract

Paulsson L, Bondemark L, Söderfeldt B.
A systematic review of the consequences of premature birth on palatal morphology, dental occlusion, tooth-crown dimensions, and tooth maturity and eruption.
Angle Orthod. 2004;74(2):269-79. PubMed abstract

Phillips RM, Goldstein M, Hougland K, Nandyal R, Pizzica A, Santa-Donato A, Staebler S, Stark AR, Treiger TM, Yost E.
Multidisciplinary guidelines for the care of late preterm infants.
J Perinatol. 2013;33 Suppl 2:S5-22. PubMed abstract / Full Text
Gives guidelines for caring for late preterm infants in the hospital and after discharge. Includes short- and long-term follow up; each care recommendation is associated with counseling points to share with the family.

Pickering LK, Baker CJ, Kimberlin DW, Long SS ed.
Red Book: 2012 Report of the Committee on Infectious Diseases.
29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012. 158110703X

Purisch SE, Gyamfi-Bannerman C.
Epidemiology of preterm birth.
Semin Perinatol. 2017;41(7):387-391. PubMed abstract

Rogers GF.
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part I: terminology, diagnosis, and etiopathogenesis.
J Craniofac Surg. 2011;22(1):9-16. PubMed abstract

Rogers GF.
Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part II: prevention and treatment.
J Craniofac Surg. 2011;22(1):17-23. PubMed abstract

Saari TN.
Immunization of preterm and low birth weight infants. American Academy of Pediatrics Committee on Infectious Diseases.
Pediatrics. 2003;112(1 Pt 1):193-8. PubMed abstract / Full Text

Saliakellis E, Fotoulaki M.
Gastroparesis in children.
Ann Gastroenterol. 2013;26(3):204-211. PubMed abstract / Full Text

Sauve R.
Routine screening cranial ultrasound examinations for the prediction of long term neurodevelopmental outcomes in preterm infants.
Paediatr Child Health. 2001;6(1):39-52. PubMed abstract / Full Text

Schaaf CP, Scott DA, Wiszniewska J, Beaudet AL.
Identification of incestuous parental relationships by SNP-based DNA microarrays.
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Shakir AK, Altaf MA.
Azithromycin Induces Migrating Motor Complexes in Pediatric Patients Undergoing Antroduodenal Motility Studies.
J Pediatr Pharmacol Ther. 2018;23(5):390-394. PubMed abstract / Full Text

Suchy FJ.
Neonatal cholestasis.
Pediatr Rev. 2004;25(11):388-96. PubMed abstract

Tauber KA.
Persistent newborn pulmonary hypertension.
Medscape; (2019) http://emedicine.medscape.com/article/898437-overview. Editors: Windle ML, Pramanik AK; Chief Editor: Berger S; Contributors: Steinhorn RH. Accessed on 7/8/2020.

TeKolste T, Bragg J, Wendel S.
Extremely Low Birth Weight NICU Graduate.
2004; Washington State Department of Health, Children with Special Health Care Needs Program; https://depts.washington.edu/dbpeds/ELBW-NICU-Graduate.pdf
Supplement to: Low Birth Weight Neonatal Intensive Care Graduate. Specifically addresses post-NICU care of ELBW infants who: 1) experienced the usual complications associated with extreme prematurity and/or extreme low birth weight, and 2) were discharged home in a relatively healthy condition.

Trachtenbarg DE, Golemon TB.
Care of the premature infant: Part I. Monitoring growth and development.
Am Fam Physician. 1998;57(9):2123-30. PubMed abstract / Full Text

Villar J, Giuliani F, Barros F, Roggero P, Coronado Zarco IA, Rego MAS, Ochieng R, Gianni ML, Rao S, Lambert A, Ryumina I, Britto C, Chawla D, Cheikh Ismail L, Ali SR, Hirst J, Teji JS, Abawi K, Asibey J, Agyeman-Duah J, McCormick K, Bertino E, Papageorghiou AT, Figueras-Aloy J, Bhutta Z, Kennedy S.
Monitoring the Postnatal Growth of Preterm Infants: A Paradigm Change.
Pediatrics. 2018;141(2). PubMed abstract

Wilson SL, Cradock MM.
Review: Accounting for prematurity in developmental assessment and the use of age-adjusted scores.
J Pediatr Psychol. 2004;29(8):641-9. PubMed abstract / Full Text

Wong HS, Huertas-Ceballos A, Cowan FM, Modi N.
Evaluation of early childhood social-communication difficulties in children born preterm using the Quantitative Checklist for Autism in Toddlers.
J Pediatr. 2014;164(1):26-33.e1. PubMed abstract

Yee WH, Soraisham AS, Shah VS, Aziz K, Yoon W, Lee SK.
Incidence and timing of presentation of necrotizing enterocolitis in preterm infants.
Pediatrics. 2012;129(2):e298-304. PubMed abstract / Full Text

de Jong F, Monuteaux MC, van Elburg RM, Gillman MW, Belfort MB.
Systematic review and meta-analysis of preterm birth and later systolic blood pressure.
Hypertension. 2012;59(2):226-34. PubMed abstract / Full Text

Overview

Other Names & Coding

Prevalence

Genetics

Prognosis

Practice Guidelines

Roles of the Medical Home

Clinical Assessment

Pearls & Alerts for Assessment

Screening

Of Family Members

For Complications

Comorbid & Secondary Conditions

History & Examination

Current & Past Medical History

Family History

Developmental & Educational Progress

Social & Family Functioning

Physical Exam

General

Vital Signs

Growth Parameters

Skin

HEENT/Oral

Chest

Heart

Abdomen

Genitalia

Extremities/Musculoskeletal

Neurologic Exam

Testing

Sensory Testing

Other Testing

Specialty Collaborations & Other Services

Treatment & Management

Pearls & Alerts for Treatment & Management

How should common problems be managed differently in children with Premature Infant Follow-Up?

Growth or Weight Gain

Other

Systems

Development (general)

Specialty Collaborations & Other Services

Mental Health/Behavior

Specialty Collaborations & Other Services

Communication

Specialty Collaborations & Other Services

Eyes/Vision

Specialty Collaborations & Other Services

Ears/Hearing

Specialty Collaborations & Other Services

Nutrition/Growth/Bone

Specialty Collaborations & Other Services

Gastro-Intestinal & Bowel Function

Specialty Collaborations & Other Services

Respiratory

Specialty Collaborations & Other Services

Cardiology

Specialty Collaborations & Other Services

Neurology

Specialty Collaborations & Other Services

Genito-Urinary

Specialty Collaborations & Other Services

Musculoskeletal

Specialty Collaborations & Other Services

Dental

Specialty Collaborations & Other Services

Skin & Appearance

Issues Related to Premature Infant Follow-Up

Ask the Specialist

Resources for Clinicians

On the Web

Helpful Articles

Clinical Tools

Assessment Tools/Scales

Growth/BMI Charts

Other

Patient Education & Instructions

Resources for Patients & Families

Information on the Web

National & Local Support