any one of the following: cognitive abilities falling more than 1 standard deviation below average for corrected age (below the 16th percentile),¹ motor abilities falling more than 2 standard deviations below average for corrected age (below the 2nd percentile), a diagnosis of moderate or severe cerebral palsy, bilateral blindness, or permanent hearing loss (Adams-Chapman et al., 2018; Stephens and Vohr, 2009). Similarly, the majority of infants born preterm or LBW will not experience neonatal conditions traditionally considered major morbidities, such as a grade III or IV intraventricular hemorrhage, hydrocephalus or periventricular leukomalacia, necrotizing enterocolitis, retinopathy of prematurity, sepsis, or bronchopulmonary dysplasia (Stoll et al., 2015). Yet while most children born preterm or LBW will not be severely impacted by the neurodevelopmental impairments and major or several neonatal conditions discussed in this chapter, research dating back to as early as the 1990s clearly indicates that these populations experience elevated rates of mild to moderate chronic conditions—such as cognitive, attention, and learning difficulties; behavioral challenges; altered neuromotor functioning; altered growth; and asthma—and that these conditions have meaningful functional impacts into childhood and throughout adolescence (Hack et al., 1995, 2002).

Infants born at older gestational ages and higher birth weights typically will be less severely affected by the health conditions and impairments discussed here than those born at younger gestational ages and lower birth weights (see Table 4-1). In addition, infants’ medical and developmental profiles will change over time, with some becoming more severe and some improving (Taylor et al., 2021). Annex Tables 4-1 through 4-5 at

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¹ See Chapter 3 for an explanation of “corrected age.”

the end of this chapter list health conditions and impairments that may affect LBW infants in the age groups at which they are most likely to be identified.

Among the health conditions that may affect LBW infants, few (e.g., sepsis, hypoglycemia) are truly acute conditions that resolve completely following appropriate treatment. Many other conditions (e.g., intraventricular hemorrhage [IVH], periventricular leukomalacia [PVL], and diffuse white matter injury [DWMI]) can have an acute onset, followed by chronic sequelae that persist with some level of severity. Other conditions are chronic, but the person’s functioning may resolve with timely and appropriate intervention (e.g., internalizing disorders, externalizing disorders, anxiety disorders, depressive disorders), while still others are chronic and anticipated to be lifelong (e.g., intellectual disability). This range of outcomes blurs the lines between conditions that might be considered acute versus chronic.

The remainder of this section summarizes a variety of health conditions and impairments that can affect children born LBW. The discussion of each includes its prevalence or risk, diagnosis, course and treatment, and functional impact. Although each condition is described independently, comorbidities and co-occurrence of many of these conditions are common among children born preterm or LBW. The occurrence of multiple morbidities may emerge diagnostically over time, consistent with changes in health status or development. The terms comorbidity and co-occurrence (or multimorbidity) overlap and often imply an additive effect on disease burden, including increased severity of associated functional limitations (Valderas et al., 2009).

Neuromuscular Conditions

Preterm birth and LBW are associated with a number of neuromuscular conditions, notably white matter injury and cerebral palsy (CP), but also developmental coordination disorder (DCD), as well as musculoskeletal abnormalities (e.g., plagiocephaly, truncal hypotonia) that are related to positioning and abnormal signaling because of LBW and long-term care in the neonatal intensive care unit (NICU). Some of these conditions result in long-term sequelae that can affect a child’s functioning to varying degrees throughout the lifespan.

Cerebral Palsy

Description

CP is a clinical umbrella syndrome representing the most common chronic or lifelong physical developmental disability of childhood (Sadowska et al., 2020). The etiology of CP is heterogeneous, but all cases have in common a static insult that has occurred to the developing brain

up to age 3 years. In 2006, an international consensus group agreed on the current definition:

Prevalence or Risk

The prevalence of CP in the United States has been estimated at 3–4 per 1,000 children (Van Naarden Braun et al., 2016). One study found the prevalence of severe CP to be 70 percent higher in Black than in White children (odds ratio [OR] 1.7 95%; confidence interval [CI] 1.1–2.4) (Maenner et al., 2012). Although preterm children are at higher risk for developing CP, a large number of children who are born full term also develop the disorder, presenting with delays in meeting developmental milestones or manifesting atypical motor signs, such as tightness in the extremities, changes in muscle tone, or asymmetries of movement. CP is associated with SGA among infants born at term, and SGA appears to be a risk factor for CP in moderate to late preterm births as well (Zhao et al., 2016). SGA is found twice as frequently among infants with congenital heart disease (CHD) compared with those without (Ghanchi et al., 2020; Malik et al., 2007), and CP appears to occur at higher rates among preterm infants with complex CHD than among those without of the same gestational age (Cheung et al., 2021). Data from Europe and Australia indicate that CP is five times more prevalent among children with severe CHD, and a higher proportion acquire it postnatally compared with the general population (Garne et al., 2023).

Diagnosis

With early screening, such as use of the International Early Detection Guidelines, diagnosis can be made as soon as 5 months of age and commonly before 1 year (Maitre et al., 2020a; Novak et al., 2017a). Notably, CP diagnoses historically were not made until age 2 years, and, because the consensus standards for early detection are recent, much of the information presented about the condition in this report is based on literature published prior to their adoption.

Course and Treatment

CP is a lifelong condition with increased costs associated with care (Kruse et al., 2009; Murphy et al., 2006). During the first year, a child with CP will have variable issues with strength, muscle tone, and postural reflexes and will demonstrate a delay in reaching motor milestones. Typical gross motor delays include sitting, crawling, and stance.

Impairments are often seen in fine motor skills, including reaching, grasping, and dexterity; oral skills; and visual motor abilities. CP may affect eating, coordination of breathing, and sleeping (Duncan and Maitre, 2021) and can cause problems with posture and comfort. Early intervention to address these impairments occurs up to age 3. Ideally, children with CP should have a pediatric medical home that manages their medical stability and preventive care, nutrition, vision, hearing, and other medical conditions throughout childhood until the transition to adult care (Noritz et al., 2022). Most often, however, general pediatricians are charged with managing these children, coordinating their care among subspecialists and therapies, and they may lack the same level of knowledge and experience in managing children with CP as well as the resources to provide the same complex care coordination that would be available in a medical home.

During the growth years, including preschool through high school, musculoskeletal deformity is a characteristic feature of almost all children with CP as the result of a cascade of motor impairments, such as spasticity, poor selective motor control, and weakness. Children with CP need hip surveillance via X-rays and may need orthopedic attention to prevent hip dislocation and maximize alignment during the growth years (Shore et al., 2017). Frequently, hypertonia is addressed with medical management. During periods of rapid growth, children with hypertonia ideally are followed by a team of specialized physicians and therapists to reduce deformity and improve function (Brandenburg et al., 2023). Functional prognosis is established (see below), coordinated school and medical therapy maximize activity and participation within the International Classification of Functioning, Disability and Health (ICF) framework, and environmental barriers in the community and home must be addressed with assistive technologies and adaptive equipment to support mobility and activities of daily living. Pediatric rehabilitation medicine physicians may be particularly helpful, employing a biopsychosocial model of care for children with disabilities (Bosques et al., 2023). Unfortunately, adverse social determinants of health too often result in delay of intervention and lack of coordination of care (Ostojic et al., 2023). There are many barriers to obtaining appropriate durable medical equipment for children with CP that reflect difficulties in the system, restricted choices, and the need for professional training (Feldner et al., 2022).

Since most children with CP survive into adulthood, the transition from the pediatric model of care to the adult model is a major concern, as individuals continue to require therapeutic intervention, environmental modification, and adaptive equipment to maintain function throughout the lifespan.

Functional Impact

The severity of the condition and an individual’s functional prognosis depend on the degree of motor impairment and are classified using the Gross Motor Function Classification System (GMFCS)

(Palisano et al., 2000). Since CP can affect all movement, not just gross motor ability, there are separate functional classification systems for hand manipulation (the Manual Ability Classification Scale [MACS]), communication (the Communication Function Classification System [CFCS]), vision (standard vision assessments), and eating (the Eating and Drinking Classification Scale [EADCS]) (Morris and Bartlett, 2004). All of these classification systems use a similar 5-point scale for independence and assistance, 1 being the least involved and 5 being the most involved. Importantly, gross motor severity explains much of an individual’s functional and medical prognosis; however, gross motor impairment does not fully explain deficits in hand function and communication, so it is necessary to assess function in all domains.

A GMFCS score can be reliably determined at 2 years of age and remains fairly stable (Gorter et al., 2009). Depending on the GMFCS level, children will plateau or reach their level of gross motor function at about 6 years of age. In the past, the ability to sit independently by the age of 2 would have been used to predict ambulatory status. However, the ability to ambulate may decline over time because of the increased energy demands, strength and endurance requirements, and changes to postural alignment that occur as the child ages. In adolescence, the GMFCS remains relatively stable. Importantly, children at GMFCS level 3 who use a walker for household and other mobility can decline as they age because of increased weight, changes in biomechanics, and fatigue (Alriksson-Schmidt et al., 2017).

Children with poor hand manipulation skills, or MACS level 3 or 4, may require help with dressing, bathing, grooming, eating, or toileting. They may also need technology to improve writing, communication, and feeding. MACS is usually determined by age 4 and corresponds to WeeFIM outcomes of self-care (Morris et al., 2006). Although improvement in hand function can occur, MACS scores also appear to remain stable over time.

Children with CFCS level 4 or 5 will also benefit from assistive or adaptive technologies. Speech services will enhance and coordinate language skills.

Between infancy and age 3, eating safely may require extra time, and special diets or even a gastrostomy tube may be necessary to eliminate the risk of aspiration. Feeding can remain a challenge with some children unable to keep up with nutritional requirements during phases of rapid growth.

Children with CP are also at increased risk of cognitive impairments, autism, and mental/behavioral health conditions (Christensen et al., 2014; Duncan and Maitre, 2021; Gupta et al., 2020; Stadskleiv, 2020; Whitney et al., 2019). Importantly, treatable mental/behavioral health conditions may worsen morbidities associated with CP.

The symptoms and morbidities associated with CP result in functional impairments in acquiring and using information, particularly at young ages; attending and completing tasks; interacting and relating with others;

moving about and manipulating objects; caring for oneself; and health and physical well-being.²

Developmental Coordination Disorder

Description

DCD is a common and chronic disorder characterized by a significant delay in the acquisition of higher-level gross and fine motor skills. CP and DCD are often viewed as being on a spectrum of motor dysfunction. Unlike CP, however, DCD has neither a neurological etiology nor neurological findings on exam. In the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5; APA, 2013), DCD is defined by the following four criteria:

Dyspraxia, “motor learning difficulty,” “physical awkwardness,” and “movement difficulty” all refer to a significant difficulty with coordinated motor skills, which is the main feature of DCD (WHO, 2019/2021, 6A04).

Prevalence or Risk

DCD is estimated to affect 5–6 percent of school-aged children (Blank et al., 2019; Uusitalo et al., 2020; Van Hoorn et al., 2021). Preterm birth and LBW are the only risk factors consistently associated with diagnosis (Edwards et al., 2011; Liu et al., 2023; Van Hoorn et al., 2021). Evidence from systematic review has shown that children born preterm or LBW are 3–8 times more likely than children born at term or with typical birth weight to be diagnosed with DCD (Edwards, et al., 2011; Van Hoorn et al., 2021; Williams et al., 2010). It is estimated that 8–10 percent of children with DCD are born very preterm, and 12–44 percent are born preterm (Van Hoorn et al., 2021).

The risk of DCD in general population studies is significantly associated with male sex; in preterm populations, however, male sex was found

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² For each of the conditions described in this section, the committee has listed, based on the members’ collective expertise, which of the Social Security Administration’s (SSA’s) six functional domains (see Chapter 3) may be negatively affected by the condition: moving about and manipulating objects, acquiring and using information, attending and completing tasks, interacting and relating with others, caring for [oneself], and health and physical well-being.

to be associated with DCD in only one of seven studies (Van Hoorn et al., 2021). Findings on the association between socioeconomic status and DCD are inconsistent (Van Hoorn et al., 2021).

Diagnosis

The onset of difficulties with coordinated motor skills occurs during the developmental period and is typically apparent from early childhood, although there are several challenges in diagnosing DCD in children less than 5 years of age (Blank et al., 2019). As stated by Blank and colleagues (2019), first, young children may demonstrate delayed motor development followed by a spontaneous “catch-up” (i.e., late bloomers) (Cantell et al., 2003). Second, young children may vary in their cooperation and motivation to complete the motor assessments appropriate to diagnosis of DCD (Blank et al., 2019). Third, there is considerable overlap in the symptoms of DCD and of other developmental disorders, such as autism, intellectual disability, and other nonverbal learning disabilities.

Diagnosis of DCD typically evolves and is made in children as they are assessed for preschool and school readiness or as they begin to execute complex learned motor tasks, such as emerging skills in activities of daily living (e.g., dressing and grooming) and higher-level gross motor skills (e.g., skipping, hopping, climbing, and prehension for writing). The use of standardized criterion-referenced testing to make the diagnosis is recommended. Under the age of 5, testing is unreliable except for the Movement Assessment Battery for Children, Second Edition (MABC-2), which has good test–retest reliability and reasonable construct validity in 3-year-old to 5-year-old children (Henderson et al., 2007).

Course and Treatment

Occupational and physical therapy services are used to support the child’s educational involvement. The few studies that have examined the natural course of DCD have provided evidence that DCD persists into adolescence and that 50–70 percent of these youth continue to have motor difficulties (Blank et al., 2019; Cantell et al., 1994; Visser et al., 1998). Adults with DCD continue to experience difficulties in performing a range of motor skills and in learning new skills, such as driving, as well as difficulties with executive functioning and attention, anxiety and symptoms of depression, and low global self-esteem (Blank et al., 2019). DCD is often associated with other learning or behavioral disorders (Blank et al., 2019).

Functional Impact

The severity of motor impairment affects not only the presentation of DCD but also the child’s participation, which has important implications for treatment. Difficulties with coordinated motor skills “cause significant and persistent limitations in functioning (e.g., in activities of daily living, school work, and vocational and leisure activities)” (WHO, 2019/2021, 6A04). In school-aged children, specific fine motor problems

may be more relevant than gross motor problems for school achievement, while gross motor problems appear to be important for participation in play, sport, and leisure and the development of social contact with peers (Blank et al., 2019). As noted above, many children with DCD have a constellation of other overlapping learning and behavioral conditions and may experience low self-esteem and emotional adjustment issues (Blank et al., 2019), which can be helped with psychological or social support. Adults with DCD continue to have a range of nonmotor problems, including problems with executive functioning, attention, and anxiety, as well as symptoms of depression and low global self-esteem. The deficits associated with DCD result in functional impairments in acquiring and using information, interacting and relating with others, moving about and manipulating objects, and caring for oneself.

Positional Cranial Deformation

Description

Plagiocephaly is deformation of the skull that occurs as a result of prenatal, perinatal, or postnatal factors such as first birth, restricted uterine environment, assisted labor, multiple pregnancies, prematurity, congenital muscular torticollis, or dependent positioning of the head (Jung and Yun, 2020).

Prevalence or Risk

Prolonged exposure to continuous external forces on the posterior portion of the head as a result of supine sleeping leads to cranial deformation, especially in the initial months after birth, as infants have limited ability to reposition or lift their heads independently. Infants, especially those born prematurely, are at risk of developing such deformities because of their soft skulls, unfused sutures, and positioning restrictions of the NICU (Nuysink et al., 2013; Yang et al., 2019). In a study of 195 infants, 73 percent of infants born very preterm and 28 percent of those born late preterm exhibited dolichocephaly compared with 11 percent of infants born at term (Ifflaender et al., 2013; Nuysink et al., 2013; Yang et al., 2019).

Diagnosis

The most common presentation of plagiocephaly is a flattening on the posterolateral side of the head due to the external forces of gravity and the weight-bearing surface acting on the skull (Jung and Yun, 2020). It is diagnosed by clinical examination and can be measured using a caliper tool, a craniometer headband, plagiocephalometry, a flexible ruler, advanced imaging technology, or plaster molding of the infant’s head. Plagiocephaly can be categorized by severity, using the Children’s Healthcare of Atlanta Plagiocephaly Severity Scale, and by type, using the Argenta Scale (Kaplan et al., 2018).

2018). Brain injury may include IVH, PVL, and DWMI (Thompson et al., 2019). Over the long term, these injuries may result in deficits in all neurodevelopmental domains.

Brain injury (IVH, PVL, and DWMI)

Description

Premature infants are at risk of brain injury due to their vulnerability to both impaired maturation and specific injury, as the brain must continue critical developmental processes, such neuronal maturation, outside of the normal, supportive intrauterine environment (Demaster et al., 2019). Brain injury may be caused or worsened by exposure to such factors as maternal infection and chorioamnionitis (inflammation of the placenta and the placental membranes), infant sepsis and meningitis, intrauterine and extrauterine drug exposures, poor nutrition, hemodynamic changes, hypoxia, ischemia, and mechanical ventilation (Demaster et al., 2019; Penn et al., 2016). Brain injuries such as IVH, PVL, and DWMI result from these exposures (Thompson et al., 2019).

IVH, which involves bleeding into a region of the developmentally immature brain, is believed to result from immature vasoregulation and variable blood flow hemodynamics in the germinal matrix (a highly cellular vascular region in the brain from which nerves migrate during brain development), along with increased cerebral arterial pressure after birth (Demaster et al., 2019). IVH is graded from I to IV. Grade I is the most mild and Grade IV the most severe (Burstein et al., 1979). IVH is an acute injury, but may lead to secondary, long-term brain conditions, including posthemorrhagic hydrocephalus (PHH) or PVL, both of which are more common following severe IVH. Proinflammatory cytokines alter normal maturation in vulnerable cell populations in the brain, adversely affecting white matter development in particular (Paton et al., 2017). DWMI, the hallmark of preterm brain injury, is caused by injury to immature oligodendrocytes. PVL is the most common nonhemorrhagic abnormality in the white matter of infants born preterm. It may range from large focal necroses with cystic changes (cystic PVL) on the most severe end to small focal necroses without cystic changes (which predominates).

Prevalence or Risk

More than 70 percent of extremely preterm infants have white matter abnormalities in the brain, 20 percent of which are moderate to severe (Inder et al., 2003; Volpe, 2009). Among those born <30 weeks gestational age, roughly 30 percent will have IVH. About 6 percent of very LBW (<1500 grams at birth) and 15 percent of extremely and very preterm infants have severe IVH (Demaster et al., 2019; Limbrick and de Vries, 2022), which is associated with a greater than 75 percent chance of neurodevelopmental impairment and/or CP (Demaster et al., 2019;

Roberts et al., 2018). PHH may develop in 40–50 percent of infants following severe IVH, and the risk of poor neurodevelopmental outcomes is markedly increased in infants with rapidly progressive PHH that requires neurosurgical intervention (Kuo, 2020).

Diagnosis

Diagnosis of IVH is made using head ultrasound or magnetic resonance imaging (MRI). The majority of IVH occurs during the first week of life, while PVL, PHH, and DWMI may be recognized later in the course of NICU hospitalization. While cystic PVL may be detected on head ultrasound, noncystic PVL is not (Ahya and Suryawanshi, 2018). Following severe IVH, head ultrasounds are monitored more frequently than the standard to monitor for ventricular dilatation consistent with PHH.

Course and Treatment

PHH most commonly develops within 7–14 days following IVH, although this timing may vary with the extent of bleeding and its location (Limbrick and de Vries, 2022). Once PHH has been diagnosed, head ultrasound will be repeated frequently, and with worsening PHH, physical examination findings consistent with increased intracranial pressure may be observed (e.g., decreased level of consciousness, abnormal motor and/or sensory function, abnormal increase in occipital–frontal circumference, bulging anterior fontanelle, or splaying of the midparietal sagittal suture). Significant PHH may require neurosurgical evaluation and intervention, including placement of a ventricular access device or a shunt. Placement of such devices following the development of clinical symptoms has been associated with poorer neurodevelopmental outcomes, but all children requiring shunt placement are at elevated risk for neurodevelopmental deficits. Complications following shunt placement may be significant and can include shunt malfunction, with the need for surgical intervention or shunt revision, and shunt infections (Limbrick and de Vries, 2022). Shunt placement requires long-term follow-up with neurosurgery and very close monitoring for any signs or symptoms of infection.

Functional Impact

Over the long term, cystic PVL is associated with neurodevelopmental impairment in all domains: acquiring and using information, attending and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being. It is particularly associated with the spastic diplegic type of CP; if damage is extensive, quadriplegia may result. Visual impairment may be an important consequence of PVL, as the optic radiations may be involved (Ahya and Suryawanshi, 2018). IVH (especially more severe IVH) is associated with both short- and long-term morbidity (e.g., PHH; PVL; CP; cognitive, language, motor, and behavioral disabilities) and

mortality in preterm children. Consistent with long-term cystic PVL, severe IVH can impact all domains of functioning: acquiring and using information, attending to and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being.

Infection

Description

Preterm infants are at high risk for infection, leading to inflammation, which increases the risk of damage to the developing brain, including IVH and DWMI (Demaster et al., 2019). The risk of invasive bacterial infection is several times higher for preterm infants than for their term counterparts (Strunk et al., 2014). Importantly, this increased risk of serious infection continues into childhood and is not restricted to extremely preterm infants. The risk of infection is also heightened for moderately and late preterm infants (Strunk et al., 2014). Histological and clinical chorioamnionitis is associated with an increased risk of neonatal sepsis, particularly among preterm infants (Beck et al., 2021). Infants born preterm are also at higher risk of infection during their NICU stay.

Prevalence or Risk

Preterm infants are roughly twice as likely as their term counterparts to have an invasive bacterial infection during their hospitalization (Greenhow et al., 2023). Twenty-five percent of extremely preterm infants (<28 weeks gestation at birth) have at least one invasive bacterial infection during their hospitalization (Perez et al., 2023).

Diagnosis

The gold standard for diagnosis is a bacterial or viral culture. However, with clinical and laboratory factors consistent with infection, infants may be diagnosed with and treated for culture-negative sepsis.

Course and Treatment

Treatment type and length are based on the presumptive organism.

Functional Impact

There is an increased risk of PVL in infants exposed to neonatal sepsis or maternal infection, although the most common brain injury associated with inflammation is DWMI. All neonatal infections, including culture-negative and culture-positive sepsis and meningitis (with or without accompanying sepsis), are associated with neurodevelopmental impairment in all domains: acquiring and using information, attending and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being. Repeated infections are associated with the highest risk (Demaster et al., 2019; Strunk et al., 2014).

At 3–5 years of age, children should also be screened for refractive errors (Gudgel, 2021; National Eye Institute, 2020). If any concerns about vision loss arise at any point in childhood, children should be sent for ophthalmological examination immediately. CVI may prove more subtle to diagnose, and may require the involvement of other subspecialties, such as neuro-ophthalmology.

Course and Treatment

Treatment depends on the cause of visual impairment. In the case of ROP, treatment may involve laser or Avastin therapy. Refractive errors may require glasses, and strabismus may require patching or surgery, depending on its severity. There currently is no standardized treatment for CVI (Chang and Borchert, 2020; Jimenez-Gomez et al., 2022), although refractive correction with glasses was associated with the greatest improvement over time on a custom CVI severity grading system compared with Early Childhood Intervention (ECI) visual therapy services, motor development exercises incorporating visuospatial training, and a combination of all the therapies (Jimenez-Gomez et al., 2022).

Functional Impact

Visual impairment that remains uncorrected may lead to difficulties with moving about and manipulating objects and caring for oneself and adversely affect school performance (acquiring and using information) and the ability to attend and complete tasks. Vision impairment may also impact social-emotional development, which may negatively affect interacting and relating with others, as well as overall health and physical well-being.

Hearing Dysfunction

Description

Hearing loss refers to a restricted or complete inability to hear. It can result from problems with the ear (outer, middle, and/or inner ear), the vestibulocochlear nerve (i.e., cranial nerve eight), and/or the auditory system (ASHA, n.d.-b). Hearing loss can be congenital (at birth) or acquired (diseases, ototoxic medications, head injury, loud noises), and conductive (object in outer ear, fluid in middle ear, poor eustachian tube function) or sensorineural (inner ear damage). A large number of genetic syndromes that may lead to preterm birth can also include hearing loss as a part of their sequelae (ASHA, n.d.-b). Untreated hearing loss can lead to detrimental impacts on the development of communication and cognitive skills, education, and well-being (Lieu et al., 2020).

Prevalence or Risk

The Centers for Disease Control and Prevention’s (CDC’s) Early Hearing Detection and Intervention (EHDI) program tracks screening data state by state. In the general population, it is estimated that

2 to 3 out of 1000 children born in the United States have some form of detectable hearing loss (NIDCD, 2021). Reports show a range of hearing loss between 1 percent and 9 percent in extremely LBW infants (Vohr, 2016). Infants and children are also at risk for acquiring hearing loss postbirth for the reasons stated above.

Diagnosis

Universal hearing screening at birth or shortly thereafter is the standard of care in the United States. Audiologists and hearing professionals follow the EHDI benchmarks: screening before 1 month, evaluation and audiologic diagnosis of infants who failed their screen before 3 months, and early intervention for those infants identified with hearing loss before 6 months (Joint Committee on Infant Hearing, 2019). States that currently meet the 1-3-6 benchmarks are encouraged to strive to meet new 1-2-3 benchmarks (Joint Committee on Infant Hearing, 2019). Hearing loss can also occur in infants, toddlers, and children at any time, and all children should be screened at well-child visits following the schedule in the Bright Futures/American Academy of Pediatrics (AAP) Recommendations for Preventive Pediatric Health Care (AAP, 2023; Bower et al., 2023). Any child at risk for or suspected of having hearing loss can be diagnosed through hearing testing by an audiologist and fit with an assistive hearing device (e.g., hearing aid or cochlear implant) if necessary. It should be noted that lack of access to consistent primary care and hence to screening can result in delayed access to an audiologist for the identification and treatment of hearing loss.

Course and Treatment

Conductive hearing loss in children, caused by fluid in the middle ear or the absence or malformation of any part of the ear, is frequently remediated with medications or surgery. If necessary, the infant or child can be fitted with an appropriate hearing aid and enrolled in early intervention to promote communication development (ASHA, n.d.-a). Sensorineural hearing loss cannot be remediated with medication or surgery. The infant or child can be fitted with hearing aids or cochlear implants and enrolled in Early Intervention to improve access to environmental sounds and language and better promote development and communication skills (ASHA, n.d.-a; Lieu et al., 2020).

Functional Impact

Untreated conductive hearing loss and unaided sensorineural hearing loss, whether due to loss to follow-up, lack of primary care, or lack of access to an audiologist, can lead to communication and learning delays (CDC, 2023a). Hearing loss can impact a child’s functional abilities in terms of acquiring and using information, attending to and completing tasks, interacting and relating with others, caring for oneself, and overall health and physical well-being.

<32 weeks gestational age. Research has demonstrated increased resource utilization in preterm children due to respiratory infections because of the need for hospital readmissions and more medical support compared with their full-term counterparts (Greenough, 2012).

Prevalence or Risk

The incidence of RDS is inversely proportional to the gestational age of the infant, with more severe disease being seen in the smaller and more premature neonates. The rate of BPD among surviving infants born is ~44 percent among those born extremely preterm (Lapcharoensap et al., 2015), more than double that of those born very preterm, and 40 percent among those born at <=28 weeks gestation (Davidson and Berkelhamer, 2017). BPD rates increase as gestational age at birth decreases (Lapcharoensap et al., 2015). As survival rates of prematurity have increased, so has the prevalence of BPD among survivors.

Diagnosis

RDS can be diagnosed via findings of ground-glass opacities on chest radiograph and respiratory distress in a newborn infant. Diagnosis of mild, moderate, and severe BPD can be made at 36 weeks adjusted age in the NICU for those born at <32 weeks based on criteria described by Jobe and Bancalari (2001).

Course and Treatment

RDS management includes close monitoring of oxygenation and ventilation; administration of exogenous surfactant into the lungs; provision of assisted ventilation as indicated; supportive management of nutrition, fluids, and thermoregulation; and provision of antibiotics if indicated (Yadav et al., 2022). In developed countries, mortality associated with RDS is less than 10 percent. Surfactant production often begins with the use of appropriate ventilatory support alone, with improvement generally occurring within 4–5 days. Left untreated, RDS can lead to severe hypoxemia, which may result in death.

Acute complications associated with RDS are due largely to assisted ventilation and include air-leak syndromes. Neurodevelopmental delay is a long-term complication of RDS, particularly in infants requiring long-term mechanical ventilation. Further, the development of CP is increased in infants with RDS, although its incidence decreases with increasing gestational age. Increased time on mechanical ventilation increases the risk of CP and neurodevelopmental delay. BPD is also a long-term complication of RDS. In addition to the lack of surfactant, the premature lung has poor compliance and fluid clearance, as well as decreased ability to clear fluid and immature development of the lung vasculature. All of these factors predispose the lung to inflammation and injury and further disrupt lung development and, coupled with oxidative stress and decreased antioxidant capacity, predispose the infant to the development of BPD, which is also a

long-term complication of RDS (Yadav et al., 2022). Infants diagnosed with BPD have higher rates of poor pulmonary function, upper airway abnormalities, asthma-like symptoms, predisposal to infection, rehospitalization, exercise intolerance, pulmonary arterial hypertension, and abnormal ventilatory responses.

The worst respiratory outcomes are among those who need ongoing respiratory support after 36 weeks adjusted age; the hallmarks of severe disease are coexisting pulmonary hypertension and/or frequent airway hyperreactivity. The risk of infection increases the risk of contracting respiratory syncytial virus (RSV), which may result in severe morbidity, hospitalization, or death in infants with lung disease. Some infants with BPD require monthly intramuscular administration of Synagis during RSV season (Narayan et al., 2020; Simpson and Burls, 2001). Those infants born SGA are at higher risk for developing adverse pulmonary outcomes compared with their counterparts of appropriate weight for gestational age (Osuchukwu and Reed, 2022). In addition, growth failure after prematurity is a risk factor for developing BPD/CLD.

Delivery room and NICU strategies, including mode of ventilation, supplemental medication, weaning protocols, and prevention of severe respiratory infection, can improve pulmonary function in premature infants. The mainstays of treatment for BPD are supplemental oxygen delivered by nasal canula or positive pressure via tracheostomy. Apnea, or pauses in breathing, can be caused by immaturity of the brain and obstruction of the airway. Assessment tools, such as a sleep study, as well as medication and treatment modalities, are used at different stages of apnea or obstructive sleep apnea. Although most infants born with BPD can be weaned from supplemental oxygen within 1 year of birth, others with more severe BPD will need ongoing oxygen, tracheostomy, or ventilator support (Anderson and Hillman, 2019). Infants who go home on oxygen will be rehospitalized for intercurrent respiratory infection and insufficiency in the year after birth (DeMauro et al., 2019). It is important to note that prematurity disproportionately impacts minority, inner-city families, and access to regional and timely NICU care and specialized support impact BPD outcomes, especially once the infant is discharged. Black infants, compared with their White or Hispanic counterparts, have higher rates of tracheostomy and comorbidities from BPD (Johnson et al., 2022).

Functional Impact

Infants with BPD are almost always delayed in motor functioning because of the positioning needed for oxygen delivery by ventilation in the NICU. Cognitive functioning can also be determined by the intensity of respiratory interventions needed in the NICU and BPD outcomes: It is thought that BPD explains 65 percent of cognitive outcomes among those born extremely premature (De Mello et al., 2017; Hack et al.,

2000; Twilhaar et al., 2018). Positioning, feeding, and early developmental play are significantly limited by BPD until pulmonary function, the need for supplemental oxygen, and motor conditions improve. Most of the “catch-up” in functional domains occurs once the need for supplemental oxygen has decreased and feeding and mobility have increased. BPD is thus associated with deficits in the functional domains of acquiring and using information, attending and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being.

Cardiovascular Conditions

Hypertension

Description

Hypertension is defined as a sustained blood pressure attained on three separate occasions that is above the 95th percentile for infants of similar birth weight, sex, gestational age, and postnatal age (Giri and Roth, 2020). Identification of elevated blood pressure in neonates and particularly in preterm infants can be difficult because of variations in blood pressure that depend on the measurement technologies used; changes in blood pressure with gestational age and weight; and other factors that affect blood pressure readings, such as level of wakefulness, feeding, crying, and pain.

Prevalence or Risk

Giri and Roth (2020) report the incidence of hypertension in neonates in the NICU to be 0.2–3 percent, while Flynn (2023) reports a narrower range of 1–2.5 percent, compared with 0.2 percent among healthy term infants. Neonatal hypertension is generally a complication of thrombosis associated with umbilical arterial catheterization, renal parenchymal disease, or BPD (Flynn, 2023; Starr and Flynn, 2019). Risk factors include preterm birth and LBW, as well as maternal diabetes, sepsis, dehydration, birth trauma, perinatal asphyxia, patent ductus arteriosus, and cocaine and heroin exposure (Flynn, 2023). In addition, hypertension may present in response to renovascular disease; obstructive uropathy; coarctation of the aorta; seizures; drug withdrawal; pain; exposure to drugs such as corticosteroids, catecholamines (vasopressin), caffeine, and bronchodilators; history of abdominal wall closure; and, rarely, endocrine disorders and tumors (Flynn, 2023; Starr and Flynn, 2019).

Diagnosis

Most often, infants with hypertension are asymptomatic, and the condition is detected upon routine monitoring. If symptomatic, the symptoms are nonspecific, such as poor feeding, irritability, and lethargy, and at times with tachypnea, cyanosis, apnea, poor perfusion, and vasomotor instability. Congestive heart failure and hepatosplenomegaly may present when

hypertension is severe. Complications of hypertension can include acute kidney insufficiency and sodium wasting associated with pressure-related natriuresis. Thus, proper measurement of blood pressure when the infant is calm is essential in establishing the diagnosis. To diagnose the cause of hypertension, a renal ultrasound with Doppler assessment of renal blood flow is essential. At times, further imaging, such as MRI angiography, may be warranted in difficult cases. It is also essential to monitor simultaneously for the presence of nephrolithiasis, particularly in preterm infants exposed to furosemide in the NICU. It is always important to obtain echocardiograms to rule out a cardiac cause and to monitor the impact of hypertension on heart function.

Course and Treatment

The course of hypertension varies. If caused by umbilical arterial catheterization or BPD, the hypertension generally resolves within 1 year of life. However, hypertension due to kidney disease can persist. Risk of hypertension is inversely associated with gestational age at birth across all age groups 0–39 years, with each additional week of gestation conferring a 4 percent lower risk (Crump et al., 2020). Depending on the cause and severity of hypertension, treatment may vary. If an acute episode is related to umbilical arterial catheterization, generally the catheter is removed, and intravenous heparinization or antithrombotic/thrombolytic therapy may be considered. If the hypertension is chronic and moderate to mild, intermittent oral therapy with hydralazine or labetalol may be considered. At times, other antihypertensives, such as calcium channel blockers, nicardipine, or ACE inhibitors (not to be used prior to 44 weeks postconception age), should be considered. Hypertension related to BPD is well treated with chlorothiazides. Nevertheless, regardless of the antihypertensive agent used, these children need close follow-up monitoring of their blood pressure and their kidney, cardiac, and at times cerebral function.

Functional Impact

The functional impact of hypertension can be seen in kidney function and poor postnatal growth. These complications can interfere with the infant’s normal and timely development. This condition and its cardiovascular complications can affect health and physical well-being, attending to and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being, depending on the experienced level of loss of energy.

Endocrine Conditions

Hypoglycemia

Description

Hypoglycemia is defined in an infant as a blood glucose of less than 60 mg/dl at greater than 96 hours of life (Rozance, 2017). Most often,

transient hypoglycemia due to prematurity resolves by the time the baby is discharged from the NICU, exceptions being infants born SGA, who may manifest hypoglycemia for a very long time, at times taking months to resolve. It is important to monitor preprandial blood glucose to ensure that the glucose is within normal limits.

Prevalence or Risk

Almost all premature and SGA babies are at high risk for hypoglycemia, the incidence of which increases progressively with decreasing gestational age and body weight. In fact, all guidelines for routine screening list these two conditions as high risk for hypoglycemia. The incidence of hypoglycemia in preterm infants varies widely from 20 to 73 percent depending on the population studied, the time of study, and the setting (Koolen et al., 2023), as well as whether the infant is extremely premature or late preterm. Similarly, the incidence in SGA infants varies from 25 to 70 percent, depending on whether the condition is found in isolation or along with prematurity (Duvanel et al., 1999; Sharma et al., 2017; Smits et al., 2022). Heterogeneity within this category of the causes of SGA also dictates the condition’s prevalence. In addition, the presence of any metabolic disorder resulting in hypoglycemia or genetic disorder of hyperinsulinism, sometimes encountered in SGA infants, requires long-term monitoring of blood glucose values.

Diagnosis

Hypoglycemia (persistent, and if present >96 hours to a week of life) is generally due to prematurity, being born SGA, metabolic disorders, endocrine disorders, genetic causes of hyperinsulinism, or perinatal asphyxia. Depending on the presentation and associated features, diagnostic testing may vary. Clinical presentation may not be noticed, as most infants may be asymptomatic or present with nonspecific symptoms such as poor feeding, vomiting, irritability, jitteriness, tachycardia, tachypnea, sweating in older infants, somnolence, and lethargy.

Course and Treatment

Most infants are treated initially with intravenous glucose infusions to stabilize the blood glucose, with close point-of-care monitoring. Once the infant has been switched to oral feeding, such monitoring is spaced out and continued only in the presence of any complicating diagnoses, such as metabolic or endocrine disorders, hyperinsulinism, or a history of perinatal asphyxia, that are superimposed on prematurity and SGA. Some infants may continue to require frequent oral feedings even after discharge, while others may require nighttime continuous feedings through a PEG/G-tube. In addition, certain conditions will require ongoing fine tuning of medication dosing based on ongoing blood glucose monitoring.

complications. The condition has a lifelong impact on the functioning of the child/adolescent and may impair psychological well-being. The associated conditions can interfere with interacting and relating with others, health and physical well-being, and at times caring for oneself.

Short Stature

Description

Short stature is defined as a height that is 2 standard deviations below the sex-specific height appropriate for the child’s chronological age (da Silva Boguszewski and Cardoso-Demartini, 2017; Houk and Lee, 2012; Lee et al., 2003).

Prevalence or Risk

Short stature emerges as a baby’s height is followed. It is highly prevalent in extremely preterm infants, with prevalence rising as gestational age at birth declines, and is most prevalent in infants born SGA. The prevalence in SGA infants at 1 year of age is 9 percent (Tamaro et al., 2021).

Diagnosis

Short stature related to familial (genetic) short stature or to delayed (constitutional) delay that also includes delayed puberty is considered normal or benign, not requiring interventions such as growth hormone (Aguilar and Castano, 2023). It is essential to rule out all other causes of short stature, including nutritional deficiencies, infections, and organ dysfunction (cardiac, kidney, and others). Typically, bone age is obtained (via wrist X-ray) to determine whether there is a delay compared with chronological age. Once these conditions have been ruled out, other causes of short stature are explored by assessing various hormone levels (e.g., thyroid, growth hormone) and the possibility of a genetic disorder (e.g., Turner’s syndrome, Noonan’s syndrome, Prader-Willi syndrome, Russel-Silver syndrome, HOX gene disorders).

Course and Treatment

Depending on its cause, the course of short stature varies. Generally, the infant’s weight is within normal limits, but the height lags in development. Once other causes have been ruled out, about 10 percent of SGA infants fail to catch up in growth by 2 years of age, and 15 percent of these infants present with a genetic cause for short stature upon testing (da Silva Boguszewski and Cardoso-Demartini, 2017; Houk and Lee, 2012; Lee et al., 2003). When short stature persists beyond 2 to 3 years of age, and if all other endocrine, metabolic, and infectious causes have been ruled out, growth hormone treatment may be warranted (Lee et al., 2003).

Functional Impact

Generally, short stature has a psychological impact on the child, affecting their self-esteem when they begin comparing themselves

with their peers, thereby affecting health and physical well-being. If associated with SGA at birth, however, short stature can be associated with neurological problems that may result in difficulties in interacting and relating with others, acquiring and using information, attending and completing tasks, and at times moving about and manipulating objects and caring for oneself.

Gastrointestinal Conditions and Nutrition Disorders

Postnatal growth failure

Description

Postnatal growth failure in a preterm infant is defined as body weight or length below the 10th percentile of expected intrauterine growth at the time of hospital discharge or 36–40 weeks postmenstrual age (Fenton et al., 2020). A more recent method of diagnosing postnatal growth failure is based on the definition of a decrease in weight Z-score between birth and discharge of more than −1.28 using the Fenton growth charts. Risk factors are evaluated in relation to birth weight for gestational age—namely, SGA or appropriate for gestational age (AGA) (Fenton et al., 2020).

Prevalence or Risk

Postnatal growth failure is common in very LBW infants; the overall incidence is 45.5 percent, with a rate of 68.9 percent in the SGA group and 36.2 percent in the AGA group (Fenton et al., 2020). Risk most importantly depends on a delay in achieving an enteral intake of 100 ml/kg, which may be related to the severity of such illnesses as respiratory distress and BPD, necrotizing enterocolitis with or without short gut syndrome, cardiac involvement, sepsis, and other conditions that lead to malnutrition because of difficulty in achieving the target enteral intake.

Diagnosis

Diagnosis is made by weekly monitoring of weight gain and the Z-scores from birth until discharge, or 36–40 weeks postmenstrual age (Fenton et al., 2020). The condition is more pronounced in SGA than in preterm infants, requiring proactive management. While premature infants may catch up in growth by 2 years of age, SGA infants may not manifest catch-up growth until just before school age (Fenton et al., 2020).

Course and Treatment

The introduction of total parenteral nutrition with the addition of protein, vitamins, and minerals within 24 hours after birth has become the universal norm. Early introduction of protein has become the mainstay of practice, increasing in steady increments to achieve a maximum of 2.8–3.0 g/kg/day, with the aim of preventing postnatal growth failure. In addition, steady increase in calories is designed to ensure that

the infant achieves the amount necessary to prevent malnutrition. The most important intervention is early and aggressive introduction of enteral feeds and their steady increase to achieve 100 ml/kg/day of enteral intake (Fenton et al., 2020). While breast milk is best at preventing other complications of prematurity, such as necrotizing enterocolitis ([NEC]; see below) and sepsis, supplements are necessary along with an increase in calories to 24 cal/oz to optimize postnatal growth. Any hinderance to achieving these targets results in a delay in growth velocity, leading to postnatal growth failure.

Functional Impact

The functional impact of postnatal growth failure is significant, as it can affect neurodevelopment, including cognitive development. Thus, infants with postnatal growth failure are at high risk for developmental delays and poor functioning even at school age, which can lead to difficulties in acquiring and using information, attending and completing tasks, interacting and relating with others, moving about and manipulating objects, caring for oneself, and health and physical well-being.

Necrotizing Enterocolitis

Description

NEC is a disease of the intestinal tract in which the tissue lining becomes inflamed, dies, and can slough off, leading to systemic or bloodstream infection (Ginglen and Butki, 2022). NEC can result in an infant’s being unable to digest food or move food through the digestive tract (NICHD, 2006).

Prevalence or Risk

NEC is the most common gastrointestinal (GI) disease seen in typically preterm or sick newborns, affecting approximately 5–7 percent of preterm neonates (Alganabi et al., 2019) and usually occurring before the newborn leaves the hospital. In 2017, NEC was the 11th leading cause of death among infants (both sexes combined) of all races and origins, with a mortality rate (MR) of 8.8 (Alganabi et al., 2019; CDC, 2018). It is the 13th leading cause of death among non-Hispanic White infants (MR 5.8); the 10th leading cause among Hispanic (MR 9.2), non-Hispanic Black (MR 20.3), and non-Hispanic American Indian and Alaska Native infants (MR not available); and the 12th leading cause among non-Hispanic Asian and Pacific Islander infants (MR not available) (CDC, 2018).

The main risk factors for NEC are LBW, prematurity, formula feeding, and intestinal dysbiosis (bacteria imbalance in the gut). Maternal risk factors include increased body mass index (BMI), smoking, in utero growth restriction, preeclampsia, and mode of delivery. Breastfeeding has been found to decrease the incidence of NEC (Alganabi et al., 2019).

to ingest adequate nutrition via breast or bottle, and therefore may also require nasogastric tube feeds to supplement or replace oral feeds.

Diagnosis

Infants, toddlers, and children with PFD can be diagnosed at any time at which health care professionals believe they are not ingesting enough calories to grow and thrive. These children are typically seen by a team that may include a primary care provider (PCP), speech-language pathologist, occupational therapist, psychologist, neuropsychologist, and clinical dietitian. Diagnosis of PFD typically is based on developmental norms, criterion-referenced checklists, and observation. Concerns regarding possible aspiration of liquids into the lungs warrant an assessment of swallowing either through a fiberoptic endoscopic evaluation of swallowing test, typically at bedside or close by, or a modified barium swallow study, which is conducted in radiology.

Course and Treatment

While in the NICU, infants receive specialized feeding support by feeding therapists to develop safe suckle–swallow–breathe patterns and transition from tube to bottle or breastfeeding. After discharge, children with feeding disorders and intolerances need close follow-up and specialized formulas and equipment. Some infants born LBW may not acquire the needed developmental skills at 1, 4, 8, and 12 months of age to eat by mouth safely and efficiently (Pridham et al., 2007). Some children who attain the ability to transition to full oral feeds as infants may not exhibit efficient oromotor/sensory skills when transitioning to solids (Kamity, et al., 2021). In these cases, evaluation and management of PFD by a specialized team is indicated.

Functional Impact

The functional impact of PFD is intense for LBW infants and their families. The constant need to be aware of calories, tolerances, and feeding is taxing and limits the ability of the infant to grow and develop normally and of the family to engage in activities outside the home. The caloric and nutritional deficits that often occur can negatively impact all six SSA domains of functioning. Decreased intake of calories and nutrients limits optimal growth, negatively impacting overall health and physical well-being. This can lead in turn to low energy for moving about and manipulating objects, interacting and relating with others, acquiring and using new information, attending and completing tasks, and caring for oneself.

Conditions Affecting Cognitive Functioning

The first years of life are a time of rapid cognitive development. Neural networks underlying various cognitive functions are most vulnerable to

disruption during “sensitive periods” of development (Johnson, 2001, 2003). The perinatal period, including the last trimester of pregnancy and the immediate postnatal period, is particularly important for neural anatomical development. LBW and preterm birth disrupt typical patterns of brain development. The altered cognitive developmental trajectory results in higher rates of various patterns of cognitive dysfunction in children who experienced LBW or preterm birth (Allotey et al., 2018; Taylor and O’Shea, 2022). The impact of this history is exacerbated by the occurrence of brain injury and medical complexity commonly experienced among this group, as well as various socioeconomic factors (Taylor and O’Shea, 2022; Taylor et al., 2004). The sections below describe various patterns of cognitive dysfunction commonly observed in children with a history of LBW or preterm birth.

Attention-Deficit/Hyperactivity Disorder and Executive Functioning

Description

ADHD is defined in the American Psychiatric Association’s (APA’s) Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, Text Revision (DSM-5-TR) and the International Classification of Diseases, 11th Revision (ICD-11) as a marked and consistent pattern of inattention, hyperactivity, and impulsivity of at least 6 months’ duration and of such intensity that it significantly impacts an individual’s functioning. ADHD diagnoses include four different presentations: symptoms consistent with predominantly hyperactive-impulsive presentation, predominantly inattentive subtype, combined hyperactive and inattentive presentation, and not otherwise specified. ADHD’s core symptoms of inattention, hyperactivity, and impulsivity are each considered aspects of a set of higher-order, or advanced, cognitive skills known as executive functioning. More specifically, executive functioning is described as metacognitive skills comprising inhibition, initiation, task shifting, organization and planning, monitoring activities (e.g., checking work), and working memory. For LBW and SGA infants, deficits in executive functioning are thought to be related to a confluence of neurological risk associated with disrupted brain growth and development that occurs in the second and third trimesters of pregnancy, and/or sequelae of disrupted growth and nutrition for brain development (Duncan and Matthews, 2018).

Prevalence or Risk

LBW and preterm birth have been identified as risk factors for ADHD (Banerjee et al., 2007; Carlsson et al., 2021; Halmøy et al., 2012; Hultman et al., 2007; Momany et al., 2018; Serati et al., 2017). Children weighing less than 1000 grams at birth are at least twice as likely as their peers of normal birth weight to demonstrate symptoms consistent with ADHD by the time they are enter kindergarten (Scott et al., 2012).

Whereas the U.S. national prevalence of ADHD is 8.5–13.3 percent (Cénat et al., 2021), as many as 33 percent of children born weighing <1000 grams demonstrate symptoms of ADHD of the combined presentation, 28 percent the predominantly hyperactive-impulsive presentation, and 19 percent the predominantly inattentive presentation (Scott et al., 2012). A recent meta-analysis of psychiatric outcomes from the Adults born Preterm International Collaboration (APIC) found that individuals born very preterm or very LBW had 5 times higher odds of meeting criteria for ADHD compared with a term/normal birth weight control group (Anderson et al., 2021).

The disproportionate diagnosis of ADHD in the preterm/LBW group is not explained by generally low cognitive ability or IQ (Scott et al., 2012). Diagnosis of ADHD is significantly impacted by social determinants of health. In the United States, Black youth are more likely to be diagnosed with ADHD than are their non-Black peers (14.5 percent of Black youth versus the national norm of 8.5–13.3 percent; Cénat et al., 2021). ADHD diagnoses also are 2 to 3 times more common among boys than girls and are more common among youth raised in families of lower socioeconomic status (Cénat et al., 2021; NIMH, n.d.). Structural racism and interpretations of the behavior of Black youth are hypothesized to contribute to high rates of ADHD diagnosis in Black youth; teachers, for example, report more behavioral symptoms in Black youth, especially those of low socioeconomic status, compared with their peers (Cénat et al., 2021). The challenge, of course, is that higher rates of preterm and LBW birth also are observed in Black populations and those of lower socioeconomic status (Burris and Hacker, 2017). And as noted above, both prematurity and LBW (Banerjee et al., 2007) confer increased risk for ADHD regardless of race or socioeconomic status. Thus, clinicians and families must balance a landscape in which Black youth are indeed disproportionately likely to be rated as having significant behavior problems as a result of systemic racism, even as the rates of ADHD in children from Black families and those of lower socioeconomic status may be even higher because of the confounding impact of neurological and social risks. For culturally resonant ADHD diagnoses, then, clinicians will benefit from both recognizing racism in the interpretation of behavior and acknowledging risk of ADHD in Black youth.

Deficits in the broader category of executive functioning are seen in as many as 50 percent of extremely LBW infants (Anderson and Doyle, 2008; Duncan, 2023; Woodward et al., 2011). Executive functioning deficits are not a diagnostic entity independently and are most commonly observed in association with ADHD. Given their inherent connection to higher-order or complex cognition, executive dysfunction is also observed in a variety of other disorders, including learning disabilities and autism spectrum disorder (Anderson and Doyle, 2008; Duncan, 2023; Woodward et al., 2011). In a recent study, 100 percent of a group of youth with ADHD and/

or a specific learning disorder demonstrated executive functioning deficits (Crisci et al., 2021).

Diagnosis

The guidelines of the AAP for diagnosis of ADHD recommend that pediatricians and/or PCPs conduct ADHD screening using standardized behavioral questionnaires for youth aged 4–18 demonstrating inattention, hyperactivity, academic difficulties, or behavioral difficulties. Documentation of behavioral symptoms that impact functioning in two or more settings, typically home and school, is sufficient for diagnosis. Previously, gold standard diagnostic evaluations included a thorough psychological diagnostic interview that assessed child functioning in home and school settings and disentangled symptoms of ADHD from relevant comorbidities, including social and emotional conditions, developmental conditions, and physical conditions (Wolraich et al., 2019). While the AAP recognizes that ADHD can be diagnosed as early as 4 years of age and the DSM does not indicate a lower age limit on the diagnosis, the CDC’s National Survey of Children’s Health indicates that the median age of diagnosis for youth with severe ADHD is 4 years and for youth with moderate ADHD is 6 years (NIMH, n.d.).

As noted above, executive functioning deficits are not a recognized diagnostic category in the DSM-5-TR. However, executive functioning deficits are recognized as a specific diagnostic entity in the ICD-11. While the research literature has yet to produce a definitive consensus, a growing body of research combined with clinicians’ judgment suggests that executive functioning deficits do not occur in isolation, separate and distinct from other cognitive disorders. This body of research and clinical judgment further indicate that executive functioning deficits are better conceptualized as core cognitive problems underlying disorders of higher-order and complex cognitive functioning, such as ADHD, specific learning disorders, and autism spectrum disorder.

Course and Treatment

ADHD is considered a chronic condition throughout childhood with persistence into adulthood, particularly for LBW infants. Its prevalence in adulthood is estimated at 4 percent (Banerjee et al., 2007), while its prevalence in childhood is 8.5–13.3 percent, as stated previously (Cénat et al., 2021). Behavioral symptoms in early childhood are predictive of poorer academic outcomes and underemployment (Breslau et al., 2009). The AAP standards for treatment recommend that training in behavioral management for parents and/or behavioral classroom interventions be used as front-line interventions for preschool-aged children (i.e., aged 4–6), and that medication such as methylphenidate be considered only if behavioral treatment does not improve functioning and symptoms are causing moderate to severe impairments in functioning. Some evidence suggests that while

preschool-aged youth demonstrate symptom improvement with medication for ADHD approved by the Food and Drug Administration (FDA), overall symptom improvement demonstrated by preschoolers is attenuated relative to the improvement seen in school-aged youth (Greenhill et al., 2006). For youth aged 6–18, gold standard treatment includes an FDA-approved medication alongside behavioral intervention and/or educational supports at school. The AAP notes that for youth aged 12–18, FDA-approved medications should be prescribed with the adolescent’s assent.

Given the role of executive functioning in disorders such as ADHD, it has been suggested that the creation and implementation of behavioral interventions designed to directly address cognitive weaknesses in core executive functioning (Duncan, 2023) may have some benefit, particularly in children born preterm/LBW.

Functional Impact

As noted above, ADHD by definition is associated with significant impacts on functioning. Its core symptoms of inattention, hyperactivity, and impulsivity can be associated with functional limitations in acquiring and using information, attending and completing tasks, social skills in the form of interacting and relating with others, moving about and manipulating objects, and caring for oneself. Youth with ADHD also are at greater risk of accidental injury, and thus their health and physical well-being can be impacted by the diagnosis.

Specific Learning Disorders

Description

Specific learning disorders are a series of formal diagnoses in the DSM-5-TR and ICD-11 described as a pattern of academic achievement that, after targeted educational intervention, does not meet expectations given a child’s age, educational level, or cognitive ability. Specific learning disorders exist across the domains of mathematics, reading, and writing.

Prevalence or Risk

Youth born preterm or LBW are known to be at elevated risk of math and reading disorders due to known weaknesses with perceptual motor abilities, language, and executive functioning (Anderson and Doyle, 2008; Aylward, 2014; Downie et al., 2003; Grunau et al., 2002; Kovachy et al., 2015; Litt et al., 2012; Taylor et al., 2009, 2011). Consistent with patterns seen in LBW youth with ADHD, the elevated risk for specific learning disorders in this population is not better explained by low cognitive abilities (Taylor et al., 2009, 2011). Birth weight, gestational age at birth, and the presence of major neonatal morbidities and abnormalities in white brain matter and structure have been associated with the risk of specific learning disorders in this population (Taylor et al., 2009). Preterm infants’ susceptibility to altered development of the prefrontal cortex and

the role of this brain region in executive functioning are also thought to play a role in the development of specific learning disorders for this group (Anderson and Doyle, 2008; Duncan, 2023; Woodward et al., 2011).

Across the United States, 9.7 percent of youth have a specific learning disorder (Altarac and Saroha, 2007). In infants born weighing <750–<1000 grams, as many as 46–60 percent are diagnosed with a math disorder (Litt et al., 2012; Taylor et al., 2009). The data are more inconsistent and limited with regard to the incidence of reading disorders in this population. Across LBW infants and their non-LBW peers, male gender, non-White race, language minority, and low socioeconomic status are significantly associated with an elevated risk of learning disorders (Altarac and Saroha, 2007; Litt et al., 2012; Shifrer et al., 2011; Taylor et al., 2009). Indeed, research indicates that lower socioeconomic status specifically appears to account for higher rates of learning disorders in non-White and language minority youth (Shifrer et al., 2011), hypothesized to result from the confluence of poverty; lower community, school, and family resources; and family stress.

Diagnosis

Previous versions of the DSM used a discrepancy model of learning disorders that required the presence of a significant discrepancy between a child’s cognitive abilities and academic achievement, often defined as 1–2+ standard deviations, on norm-referenced standardized assessments (DSM-IV-TR; APA, 2000). While the DSM-5-TR and ICD-11 have moved away from this discrepancy-based definition, many clinicians still observe this diagnostic practice. In educational settings, a child typically must demonstrate persistent below-average academic performance following adequate exposure to an early learning intervention (DSM-5-TR). While there is no minimum age requirement for diagnosis of a learning disorder, its academic nature makes it likely that most children will be diagnosed during their school years and not before. In general, earlier diagnosis is considered better for academic intervention.

Course and Treatment

Overall, specific learning disorders are considered chronic and impact acquiring and using information and attending and completing tasks. Treatment is typically administered in the form of interventions and accommodations within the school setting, delineated in an Individualized Education Plan (IEP) as required by the Individuals with Disabilities Education Act (IDEA).

Functional Impact

Children with learning disabilities have well-documented functional impairments in acquiring and using information and attending and completing tasks.

associated disorders is limited. It is generally believed that children born preterm and demonstrating delayed acquisition of expressive language will have difficulty with speech sounds because of their lesser language output with which to practice saying sounds. In their statewide case control study, Hillman and colleagues (2019) found that 10-year-old children born LBW were diagnosed with speech and language problems at a higher rate compared with those born at term, but they do not discuss speech sound disorder individually. LBW infants typically experience long-term oral ventilation. Current research does not show the effect of long-term ventilation on an infant’s oral structures that are used during sound production. One study from Serbia found an increase in sound substitution errors and distortion errors in children born under 32 weeks gestation and less than 1500 grams compared with those children born at term (Milankov and Mikov, 2009).

Diagnosis

Children who are difficult to understand when talking to family members and familiar people at 3 years of age, or to many adults at 4 years of age, are typically referred for an evaluation of speech sounds by a speech-language pathologist (ASHA, n.d.-c). An evaluation is completed by using standardized tests and listening to the child in connected speech. An evaluation also rules out the possibility that sound errors are actually differences in dialect. At the time of evaluation, it is determined whether the speech sound disorder is characterized by simple sound distortions, patterns of error (phonological disorder), significant motor planning deficit (childhood apraxia of speech), or dysarthria.

Course and Treatment

If an evaluation shows that a child has a speech sound disorder, initiation of speech therapy is warranted. A review of factors that influence treatment of speech sound disorder is provided in Speech and Language Disorders in Children: Implications for the Social Security Administration’s Supplemental Security Income Program (NASEM, 2016, pp. 82–83). Effective therapeutic techniques may include phonologic approaches utilizing complex sounds (Gierut, 2001; Storkel, 2019) or a cycles approach (ASHA, n.d.-c; Hodson, 2011). If the speech sound disorder is more motor based—for example, childhood apraxia of speech—there are several treatment processes found to be effective (Murray et al., 2014). As yet, there have been no randomized controlled trials to identify the best treatment for dysarthria in children. With effective speech therapy, the child will be more likely to develop the ability to articulate sounds correctly, be understood by others, and develop a strong sound–symbol correspondence to prepare for early literacy skills.

Functional Impact

A child who is not understood by family members or familiar adults as expected by the age of 4 years or older (ASHA, n.d.-c)

or mostly intelligible to all by 5 years of age (ASHA, n.d.-c; Schölderle et al., 2021) will have significant difficulty interacting and relating with family members, other adults, and peers, as well as acquiring and using information and attending and completing tasks. Being poorly understood will impact a child’s health and physical well-being.

Receptive and/or Expressive Language Delay and Disorder

Description

A receptive and/or expressive language delay or disorder is the inability to understand spoken or written language and to use spoken or written language effectively and functionally in daily life. In young children, a delay in or disorder of receptive language includes difficulty responding to one’s name, following directions, pointing to objects or pictures, and knowing how to take turns (ASHA, 2015). A delay in or disorder of expressive language (also characterized as spoken language disorders) includes difficulty with naming objects and people, putting words together into phrases or sentences, asking questions, and learning songs (ASHA, 2015). As the child ages, expectations for understanding and using language increase, as does complexity (reading and writing language).

Prevalence or Risk

It is estimated that 5–12 percent of late toddler and preschool-aged children are diagnosed with a speech and language delay (Othman, 2021). Limited research is available on language deficits in extremely LBW children, but by some estimates, up to 23 percent of those babies have severe receptive language delay and 16 percent have severe expressive language delay at 30 months (Adams-Chapman et al., 2015). A systematic review of the literature found that early school-aged children born very LBW scored significantly worse across receptive and expressive language measures compared with their term-born peers (Zimmerman, 2018).

Diagnosis

Receptive and/or expressive language delays and disorders are diagnosed via standardized testing, criterion-referenced testing, and observation completed by speech-language pathologists, developmental pediatricians, clinical psychologists, and/or neuropsychologists. These communication delays can be identified as early as 9–12 months of age, as 75 percent of most infants will babble different sounds at 9 months and use a name to refer to a parent by 12 months (CDC, 2023b); difficulties in any area of communication can be identified throughout early childhood and into school-age years.

Course and Treatment

A child identified with a receptive and/or expressive language delay or disorder can be enrolled in speech and language

therapy with a speech-language pathologist. Goals may include improving understanding and use of age-appropriate vocabulary and grammar. There is limited to no evidence on specific language therapy techniques to use with children born LBW. Research on statistical learning (Plante and Gómez, 2018) demonstrates that language therapy integrating statistical learning techniques can be effective across children with a wide variety of deficits. A review of effective early intervention approaches is provided in NASEM (2016, pp. 89–94), and of effective intervention for preschoolers in NASEM (2016, pp. 95–102). Evidence-based practices in late-talking toddlers include input-based techniques (Alt et al., 2021). Other evidence-based practices in preschool children with developmental language disorders include auditory bombardment (Plante et al., 2018), enhanced conversational recast (Plante et al., 2019), and other statistical learning techniques. Frequently, children will be discharged from speech-language therapy if they have acquired the skills necessary to communicate with teachers and peers, to read and write effectively while accessing the curriculum, and to engage in social interactions appropriate for their age, although this varies with the child and the underlying diagnoses (ASHA, n.d.-d). Others may require services into middle and high school to achieve the necessary functional skills for life after high school.

Functional Impact

A receptive and/or expressive language delay or disorder significantly impacts a child’s ability to function across five of the six SSA functional domains. Difficulties with receptive or expressive language directly affect a child’s ability to acquire and use information, attend and complete tasks, interact and relate with others, and care for oneself, as well as overall health and physical well-being.

Autism Spectrum Disorder

Description

Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by a pervasive and persistent pattern of significant (1) social communication deficits and (2) repetitive, restricted, or stereotyped behaviors or interests (DSM-5-TR). Although both areas of deficit are required for the diagnosis, the severity and specificity of aspects of each domain of functioning can vary in individuals, resulting in the “spectrum” of presentations. Approximately 40 percent of individuals with ASD also have some degree of intellectual disability, with higher rates in non-White children (Maenner et al., 2023). The most recent prevalence data from the CDC indicate that 1 in 36 children are diagnosed with ASD, with boys approximately 4 times more likely to be diagnosed than girls (Maenner et al., 2023). Symptoms are first evident within the early childhood years (APA, 2023).

is imperative to note that symptom overlap also results in false negatives due to diagnostic overshadowing (Mendez et al., 2023). Diagnoses of ASD are missed in youth born prematurely when evaluators attribute the entirety of their social communication presentation solely to their history of prematurity (Mendez et al., 2023). Diagnostic evaluation by providers with expertise in both autism and prematurity is recommended for diagnostic clarity.

Course and Treatment

ASD is a chronic condition that will impact functioning to varying degrees over the course of the individual’s life. It has no cure. Its symptoms can improve over time with appropriate interventions; typically, however, some difficulties persist regardless of intervention.

Functional Impact

ASD is a lifelong chronic neurobehavioral disorder that can impact an individual’s functioning across multiple domains throughout the life course. Individuals with ASD may have functional limitations in moving about and manipulating objects, acquiring and using information, attending and completing tasks, interacting and relating with others, or caring for oneself.

Emotional and Behavioral Functioning

LBW infants are at elevated risk for problems with emotional and behavioral functioning throughout childhood. These problems are associated with LBW infants’ disproportionate risk for altered neurological functioning (Peralta-Carcelen et al., 2018), socioeconomic challenges (Peralta-Carcelen et al., 2017), and disrupted family functioning in the form of parental psychological distress and altered parental sensitivity to infants’ cues (Wolke et al., 2014; Zelkowitz et al., 2011). Once these problems with emotional and behavioral functioning have been detected, they can be treated successfully with behavioral interventions, including parent–child or family therapy and/or psychiatric medication.

Internalizing and Externalizing Disorders and Behavioral Dysregulation

Description

Internalizing and externalizing behaviors are two broad bands of childhood behavioral disorders. Internalizing disorders are conceptualized as behaviors directed toward the self, and traditionally consist of such disorders as generalized anxiety disorder, separation anxiety disorder, and major depressive disorder. Externalizing disorders are conceptualized as behaviors directed toward others within a social context, and consist of such behaviors as aggression, disruptive behavior, impulsive behavior, and defiance. General difficulties with regulating behavior are considered

externalizing disorders. Common externalizing disorders include disruptive behavior disorder, ADHD, oppositional defiant disorder and conduct disorder.

Prevalence or Risk

While no gold standard exists for the earliest possible or recommended age for the emergence of internalizing or externalizing disorders as broadly defined, LBW infants demonstrate elevated rates of internalizing and externalizing behaviors before 2 years of age (Peralta-Carcelen et al., 2017). In a group of extremely preterm and/or extremely LBW children, as many as 35 percent were found to have externalizing behavior problems and as many as 26 percent to have social and emotional problems as early as 18–22 months corrected age (Peralta-Carcelen et al., 2017).

Some theorists suggest that structural brain differences associated with prematurity result in a neurological predisposition to internalizing and externalizing behaviors in LBW infants (Peralta-Carcelen et al., 2018). Specifically, altered or thinning corpus callosum and differences in the cerebellum have been implicated in externalizing behavior and social-emotional difficulties in children born preterm (Ghassabian et al., 2013; Peralta-Carcelen et al., 2018). Social determinants of health also play a role in internalizing and externalizing behaviors in LBW children. Male biological sex assigned at birth and other social variables, such as lower maternal educational attainment and public insurance, have been significantly associated with a greater likelihood of externalizing behaviors in this population (Peralta-Carcelen et al., 2017; Scott et al., 2012). Finally, parental factors influence behavioral outcome. Maternal psychological distress in the form of anxiety during their infant’s NICU stay has been associated with the child’s internalizing disorders at 2 years of age (Zelkowitz et al., 2011). Authoritative parenting styles, characterized by a combination of warmth and clear, developmentally appropriate expectations and consequences, are associated with a decreased risk of internalizing and externalizing behaviors for preterm infants (Neel et al., 2019). Permissive parenting, described as high levels of parental warmth without clear, developmentally appropriate expectations and consequences, is associated with an elevated risk of externalizing disorders in preterm infants (Neel et al., 2019).

Diagnosis

Diagnoses of internalizing (e.g., anxiety disorders or depressive disorders) and externalizing (e.g., ADHD, oppositional defiant disorder, disruptive behavior disorder) disorders are behavioral and based on caregiver report of behavioral symptoms, clinician’s diagnostic interview with the caregiver(s) and youth, and behavioral observation. Ideally, caregiver report captures the youth’s functioning across environments and settings to aid in

determining whether the reported concerning behaviors are consistent and reliable across settings. If so, the report would indicate the presence of a disorder across settings, as opposed to being limited to interactions with one caregiver or in one setting, which would indicate a problem or challenge with the caregiver–youth dyad or the setting. Specifically, it is important to consider the youth’s functioning in the home and in social environments, such as school or daycare. Many standardized and norm-referenced questionnaires are available for quantifying youth’s internalizing and externalizing functioning and placing it in the context of peer functioning. Yet while use of standardized and norm-referenced questionnaires is considered ideal or best practice, it is not required for diagnosis of internalizing or externalizing disorders. For school-aged and adolescent youth, self-report is also an important tool. Diagnoses of specific internalizing and externalizing disorders are made by a range of pediatric specialists, from pediatricians, child psychiatrists, and developmental-behavioral pediatricians to psychologists and other mental health professionals (e.g., licensed clinical social workers, licensed clinical professional counselors).

Course and Treatment

Without intervention, behavioral problems identified as early as 3 years of age in a population of preterm infants have been found to be stable over time (Gray et al., 2004). For externalizing behaviors, parent training in behavioral management is considered a gold standard and an effective treatment for children as young as 18 months of age who were born prematurely (Bagner et al., 2010). Cognitive-behavioral therapy is considered the gold standard treatment for youth with diagnoses of depression and anxiety. In sum, absent known and effective treatment, internalizing and externalizing disorders can be considered chronic in nature, whereas with appropriate and timely treatment, these behavioral disorders can be considered either acute or relapsing and remitting over time.

Functional Impact

Internalizing and externalizing disorders can impact youths’ functioning at home and at school and impair their ability to acquire and use information, attend and complete tasks, interact and relate with others, move about and manipulate objects, care for oneself, and experience health and physical well-being.

Sleep Disorders

Description

Pediatric sleep disorders are categorized as medically based or behavioral. Medically based sleep disorders include sleep-disordered breathing, obstructive sleep apnea, apnea of infancy, circadian rhythm disorder, restless legs syndrome, periodic limb movement disorder, and narcolepsy.

Behavioral sleep disorders include behavioral insomnia (Meltzer et al., 2010). Signs and symptoms of these pediatric sleep disorders include, but are not limited to, difficulty falling asleep, difficulty staying asleep, snoring, and daytime sleepiness (Trickett et al., 2022).

Prevalence or Risk

Sleep disorders occur in ~4 percent of the general pediatric population and are known to co-occur with disorders of growth, ADHD, and ASD (Meltzer et al., 2010). Youth residing with families in the lowest quartile of household income (household income <25th percentile) also are disproportionately likely to be diagnosed with a sleep disorder (Meltzer et al., 2010). Disorders involving sleep-disordered breathing, including obstructive sleep apnea, are among the more common pediatric sleep disorders, occurring in ~2–4 percent of the pediatric population (Meltzer et al., 2010; Sadras et al., 2019). Preterm infants are 2 to 4 times more likely than their full-term peers to experience sleep-disordered breathing, the risk for which increases with lower gestational age at birth (Crump et al., 2019; Sadras et al., 2019).

LBW and preterm infants have an elevated risk of sleep problems due to their immature respiratory development and unstable breathing, their immature nervous system and altered neurological development, and the impact of the NICU environment. Preterm infants’ immature respiratory and neurological development places them at particular risk for sleep-disordered breathing, including obstructive sleep apnea (Sadras et al., 2019). Additionally, the sights and sounds of the NICU, combined with medical interventions and equipment, combine to create a backdrop for sleep development that is remarkably different from the protective intrauterine environment, and preterm infants’ exposure to this altered environment occurs during a time of sensitivity in neural development and programming (Trickett et al., 2022).

Diagnosis

Assessing sleep quality in preterm infants is difficult because pediatric sleep assessments were created for neonates who were born at, or have reached the equivalent of, full term. Specifically, prior to 30 weeks gestational age, electroencephalogram (EEG) cannot be used reliably to assess sleep (Trickett et al., 2022). As infants progress through the neonatal period and into childhood and adolescence, assessment of sleep and diagnosis of sleep problems are based on both subjective and objective measures (Trickett et al., 2022). Commonly used subjective measures of sleep include standardized questionnaires and sleep diaries, while commonly used objective measures include actigraphy, videosomnography (VSG), polysomnography (PSG), EEG, and functional MRI (fMRI). Total quantity of sleep (i.e., duration) and quality of sleep (i.e., efficiency) can be assessed through actigraphy and/or PSG. PSG is considered the gold standard for diagnostic

assessment of sleep-disordered breathing, including obstructive sleep apnea (Trickett et al., 2022).

Course and Treatment

For preterm infants, sleep disruption in the form of more nocturnal awakenings and lower sleep efficiency persists at least through toddlerhood (Asaka et al., 2022). LBW and preterm infants remain at elevated risk for sleep disorders, especially sleep-disordered breathing, from childhood to middle adulthood (Crump et al., 2019). Sleep disorders, particularly those that are medically based, are characterized as chronic and in need of treatment and intervention for symptom improvement.

Treatment for sleep disorders involves medication, breathing devices, and surgery. A range of medication classes are prescribed for sleep disorders. They include medications traditionally associated with psychiatric and behavioral disorders (i.e., alpha-2 adrenergic receptor agonists, antidepressants, selective serotonin reuptake inhibitors, antihistamines, antipsychotic agents, benzodiazepines, hypnotic agents, melatonin, chloral hydrate), as well as topical nasal steroids for sleep-disordered breathing (Meltzer et al., 2010). Oral appliances such as mouth guards, continuous positive airway pressure (CPAP) devices, and bilevel positive airway pressure (BiPAP) devices also are prescribed for sleep-disordered breathing, including obstructive sleep apnea. Moderate to severe obstructive sleep apnea may warrant surgical intervention in the form of adenotonsillectomy.

Functional Impact

Sleep problems in preterm infants have been shown to be associated with cognitive and behavioral problems throughout the first 2 years of life (Caravale et al., 2017). Specifically, for these infants, earlier rise time is associated with weaker attention and social skills, and shorter sleep duration is associated with higher activity levels and impulsivity (Caravale et al., 2017). Sleep problems in preterm infants also have been associated with maternal psychological distress (Trickett et al., 2022). Given these associations, pediatric sleep problems are known to impact the ability to acquire and use information, attend to and complete tasks, interact and relate with others, move about and manipulate objects, and care for oneself, as well as health and physical well-being.

Other Conditions Impacting Child Functioning

Vulnerable Child Syndrome and Parental Distress

Description Vulnerable child syndrome (VCS), also known as parental perception of child vulnerability (PPCV), is defined as the persistent impression that a child is at greater risk for illness, injury, and/or behavioral or

developmental disorder than is actually likely (Greene et al., 2017). VCS is known to occur in families of children who have faced a medical condition that is potentially life-limiting and/or life-threatening. Relatedly, families of LBW infants are known to experience VCS and PPCV. Anxiety during an infant’s NICU stay predicts VCS/PPCV in parents of very LBW infants (Greene et al., 2017), and families and parents of LBW and preterm infants versus those of infants born at normal birth weight and/or full term demonstrate disproportionately elevated levels of psychological distress, conceptualized as depression, anxiety, and/or perinatal-specific posttraumatic stress disorder (PTSD) (Staver et al., 2021).

Prevalence or Risk

Parents of LBW and preterm infants experience psychological distress at higher rates compared with parents of normal birth weight and full-term infants (Staver et al., 2021). As many as 40 percent of mothers of very LBW infants report elevated symptoms of depression near the beginning of their child’s NICU stay, with this percentage dropping to approximately 20 percent near NICU discharge (Garfield et al., 2015; Greene et al., 2015; Rogers et al., 2013). Similarly, as many as 55 percent of mothers report elevated anxiety near the beginning of the NICU hospitalization, dropping to near 36–43 percent near discharge (Greene et al., 2015; Rogers et al., 2013). Rates of perinatal-specific PTSD are reported in 25–30 percent of mothers of very LBW infants throughout their NICU hospitalization (Garfield et al., 2015; Greene et al., 2015). While there is some inconsistency in the research literature on this subject, sociodemographic status, such as single-relationship status, is associated with higher rates of distress (Greene et al., 2015). A history of previous reproductive trauma and such factors as previous loss during a pregnancy and primipara status (i.e., first-born child) are associated with anxiety and PTSD (Greene et al., 2015). To date, research on parental distress in the NICU has focused on mothers and neglected adequate study of fathers and other caregivers (Greene et al., 2015). Emerging research on fathers of preterm infants reveals elevated rates of distress relative to their peers with term-born infants and higher rates of anger and hostility compared with mothers of preterm infants (Ionio et al., 2016).

Diagnosis

Depression, anxiety, and PTSD occuring in the postpartum period are most often diagnosed by a mental health professional, either embedded in or consulting to a NICU or providing care on an outpatient basis. Diagnoses are based on DSM-5-TR criteria and include the presence of functional limitations. Diagnoses are made based on informed clinical interviews, often supplemented by validated screening questionnaires. The Edinburgh Postnatal Depression Scale (EDPS) is a particuarly well-known and commonly used validated screening instrument used in conjunction with clinical interview to diagnose depression (Greene et al., 2017). VCS and PPCV are not recognized as formal diagnoses.

developmental disorder), and social-emotional/behavioral disorders (e.g., autism spectrum disorder).

4-3. Persistent medical and neurodevelopmental conditions associated with LBW can provide alternative diagnoses or listings by which infants allowed under the LBW listing may continue to qualify for disability benefits following a Continuing Disability Review.

REFERENCES

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