two month old infant tetralogy of fallot post ecmo- nicu_ curran

Running Head: TWO MONTH OLD INFANT BORN 32 WEEKS GESTATION PRESENTING WITH TETRALOGY OF FALLOT POST ECMO AND DIAPHRAGMATIC HERNIA

REPAIR IN THE NICU SETTING: A CASE REPORT.

Evan Foster (Case 12)

Two Month Old Infant Born 32 Weeks Gestation Presenting with Tetralogy of Fallot Post

ECMO and Diaphragmatic Hernia Repair in the NICU: A Case Report

Sophia Andrews, Cara Curran, Ashley Hansen, Megan Hubert

Grand Rounds I PT 743

Sacred Heart University

Doctorate of Physical Therapy

TWO MONTH OLD INFANT BORN 32 WEEKS GESTATION PRESENTING WITH TETRALOGY OF FALLOT POST ECMO AND DIAPHRAGMATIC HERNIA REPAIR IN

THE NICU SETTING: A CASE REPORT.

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Abstract

Background and Purpose

The purpose of this case report is to provide information on the physical therapist's role in

the Neonatal Intensive Care Unit (NICU) setting. This report also entails a limited review of the

examination procedures and implementation for preterm infants, specifically focusing on the

Neonatal Behavioral Assessment Scale (NBAS), Neonatal Behavioral Observation (NBO), and

the Test of Infant Motor Performance (TIMP). This case report outlines the NICU setting, review

of systems, ICF model, and prognosis of the preterm infant patient with Tetralogy of Fallot

(TOF) following diaphragmatic hernia repair post ECMO (extracorporeal mechanical oxygen).

Case Description

Evan Foster is a two-month old infant born prematurely at 32 weeks post conception age.

He was diagnosed with Tetralogy of Fallot and a herniated diaphragm in utero. After birth, Evan

underwent repair of his herniated diaphragm and was placed on ECMO. He presents in the NICU

setting, awaiting repair of the TOF.

Outcomes

The physical therapist performed the NBAS, NBO, and TIMP with Evan. Evan was

found to have cramped synchronized movements that were consistent with a preterm infant with

the NBAS. The NBO observed that hypotonicity is present in Evan. The TIMP showed that Evan

was below average when compared to other infants at 40 weeks post-conceptual age.

Discussion

Information obtained from the outcomes performed will assist in the development of

interventions for Evan. In addition, family centered care (FCC) will aide family involvement in



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the care for the infant. This case report provides awareness to the complex role of the physical

therapist in the NICU setting.

Case Description

Evan Foster is two-month old little baby boy currently in the NICU at Stamford Hospital

awaiting repair for Tetralogy of Fallot (TOF). He was born at 32 weeks of gestation (40 weeks

post conception age) and weighing 1300 grams. Evan was diagnosed with TOF and a

diaphragmatic hernia through ultrasound before his birth, leading to early delivery via Caesarian

section. This is the second pregnancy for Evan’s mother, who is 30 years old. There were no

complications with her first pregnancy. Her pregnancy with Evan was considered high risk due

to his mother’s gestational diabetes. However, genetic testing was conducted with this pregnancy

due to abnormalities seen on the ultrasound.

Immediately following delivery, Evan’s diaphragmatic hernia was repaired. Following

surgery, Evan was placed on nasal Continuous Positive Airway Pressure (CPAP). After 12 hours

on nasal CPAP, it was determined that a more aggressive measure needed to be taken in order to

sustain Evan’s life. Therefore, Evan was placed on ECMO for seven days. After weaning off the

ECMO, Evan was placed back onto nasal CPAP. Evan is still not considered medically stable

enough to return home.

Evan’s mother, Serena, and father, Nathaniel, come to visit every day while his two-year-

old older brother, Luke, stays at home with his grandparents. Luke is eagerly waiting to meet his

new baby brother. The Fosters live in a colonial-styled home in Westport. Serena is on maternity

leave from her part time job as a self-employed interior designer. Nathaniel works in New York

City as a stockbroker in the financial district. Evan's parents met as undergraduate students at



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Yale University ten years ago. The family just relocated out of the city last year to be closer to

Serena’s parents.

Serena is having trouble accepting how sick Evan is. She has become withdrawn and

apathetic towards connecting with him. On the other hand, Nathaniel has taken an interest in

Evan’s care, insisting that his son receive, “the best that this hospital has to offer.” Both parents

are concerned about Evan’s prognosis and continually ask the physical therapist if their son “will

ever be normal.”

Introduction

Special Care Nursery

The special care nursery houses some of the most fragile infants. Serious detrimental

effects can occur as a result of routine caregiving procedures with these patients. Therefore, there

needs to be advanced education in areas in the neonatal intensive care unit (NICU). This includes

physiologic assessment and monitoring, newborn pathologies, treatments and outcomes, optimal

discharge planning, and collaboration with members of a health care team. In addition, it is

imperative that the health care provider has knowledge of neonatal physiology, development, and

health complications (Campbell, 2012).

NICU History

The first text on premature infants was published in 1900 and focused on the main

principles of neonatal care, such as body temperature, control of nosocomial infection, minimal

handling, and provision of special care nursing. In the 1950s, there was an increase in the

development of care for premature infants and a focus on preventing maternal death. In the

1960s, there were major advances in technology and pharmacology for the neonate, such as

ventilators and phototherapy (Campbell, 2012). Subspecialties of neonatology and perinatology



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were implemented in 1975 (Campbell, 2012). All of these advances lead to improved survival for

very low birth weight and extremely low birth weight infants.

There are three main categorizations of low birth weight. These include low birth weight

(LBW: 1501-2500 g), very low birth weight (VLBW: 1000-1500 g), and extremely low birth

weight (ELBW: <1000 g). Evan would be classified as a low birth weight. The causes of low

birth weight and prematurity are not clear, but involve multiple factors (Campbell, 2012). More

than 500,000 infants are born premature in the United States each year. Around 1% of those

infants are born prior to 32 weeks and placed in the NICU.

Perinatal services aid in the monitoring of infants at risk of serious infections or those

receiving treatment for acute illnesses. As outlined in Table 1, the number of NICUs in the

United States has increased to 880 level I units, 120 level II units , and 760 level III units

(Campbell, 2012).



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Table 1: Hospital Perinatal Care Levels (Campbell, 2012)

Level I: BASIC

CARE

Evaluate and provide care to infants 35-37 weeks of gestation, stabilize infants <35 weeks gestation until transfer.

Level II:

SPECIALTY CARE

IIA Provide care for moderately ill infants >32 weeks gestation

IIB Provides mechanical ventilation for brief periods

Level III:

SUBSPECIALTY

CARE

IIIA Provides care for infants >28 weeks, performs minor surgical procedures

IIIB Provides care for infants <28 weeks gestation, provides advanced respiratory support (high-frequency ventilation);

advanced imaging, pediatric surgical specialists, access to pediatric medical subspecialists

IIIC Provides extracorporeal membrane oxygenation (ECMO) and complex cardiac surgery with cardiopulmonary bypass

NICU Environment

Many patients that are admitted into the NICU are born prematurely. Due to this, an

important aspect of the social history is determining the gestational age of the infant. The New

Ballard Score (NBS) is the most widely used assessment of gestational age. It assesses

neuromuscular maturity, physical maturity, and external genitalia to determine gestational age

from 20-44 weeks (Campbell, 2012). It is accurate within 1 week. It uses a 0 to 5 scale.

Gestational Age can also be determined through ultrasound, measurements such as weight,

length, head circumference, wrist, hip, and shoulder ranges of motion, and amniotic fluid

analysis (McManus, 2013). This is determined by physicians and nurses, but physical therapists

should be aware and familiar with how it is assessed (Campbell, 2012).

The caregivers of the infant have an integral role in controlling aspects of the

environment and intensity of medical procedures. Better practices have recently been identified

to support neonatal development. Recommendations of these practices include: implementation



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of guidelines for tactile stimulation, providing early exposure to mother’s scent, minimizing

exposure to noxious odors, developing a system for noise assessment of the NICU, minimizing

ambient noise near the isolette (an incubator for premature infants to control humidity, oxygen,

and temperature), and preservation of sleep. Equipment commonly found in the NICU

environment includes radiant warmers, self-contained isolettes, oxygen hoods, mechanical

ventilators, nasal and nasopharyngeal prongs, resuscitation bags, electrocardiograms,

transcutaneous oxygen monitors, intravenous infusion pumps, neonatal vital sign monitors, and

pulse oximeters (Umphred, 2013).

Deep sleep is vital for development. Therefore, noise and light is bothersome for

neonates (Umphred, 2013). The American Academy of Pediatrics recommends the noise level

not exceed 45 db with an absolute max of 65 db (Campbell, 2012). Equipment such as alarms,

lights, or conversation (60 db), closing drawers, or dragging chairs (80 db), can increase

autonomic responses in infants. This exposes the neonate to bradycardic and hypoxemic events.

Some special care nurseries have implemented “quiet periods,” where hospital staff and

caregivers are quiet. A blanket may be placed over the isolette or a sound absorbing panel can be

placed inside. These periods help decrease diastolic blood pressure and mean arterial pressure.

It is important to also implement “dark periods” as well in order to preserve REM sleep

in infants that are younger than 32 weeks gestational age. These infants have thin eyelids, which

do not limit the amount of light entering their eyes. Private rooms are ideal for such

environmental concerns, however, it is very difficult to provide adequate staff (Umphred, 2013).

Being in the NICU is highly stressful and traumatic for the baby, but also for the family.

The first concern of the infant is survival. Once this has been determined, then the focus is on

developmental outcomes. Parents typically struggle with the loss of parental role and become



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overwhelmed, worried, and panicked. It is common that immediately after discharge, mother’s

will experience a lack of confidence and insecurity in caring for their own child. Post-partum

depression in a mother of a preterm infant (45%) is much higher compared with that of a first-

time mother of a healthy infant (15%). The main challenge is to have the parents interact and

engage with the infant in a way that will aid in the baby’s development (Campbell, 2012).

Preterm infants are typically irritable and show facial grimaces that parents have a hard

time reading and interpreting. Physical therapists play an important role in the support of the

parents by reading the infant’s cues and providing feedback to them (Umphred, 2013). They also

promote neonate’s movement, postural control, and adaptation to extra uterine life. This is done

through collaboration with other professionals and the family in order to prepare for discharge.

Sweeney et al. (2009) describes, in a two-part article series, the Clinical Practice Guidelines for

physical therapists in the NICU. Part I describes the path to professional competence and the

clinical competencies needed for physical therapy. It also includes NICU clinical training models

and a clinical decision-making algorithm. Part II includes the evidence-based practice guidelines,

recommendations, and theoretic frameworks that support neonatal physical therapy practice

(Campbell, 2012).

Role of a Physical Therapist in the NICU

The NICU is a specialized setting where physical therapists work interprofessionally

towards the goals of the neonate. The NICU is different than any other physical therapy setting

in that it treats patients who cannot communicate with their health care professionals. It is up to

the physical therapist to decide for the child when they are in pain, over stimulated, and what

goals the child should accomplish. Physical therapists need to be highly trained to deal with



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stressful situations and be able to properly monitor signs and symptoms of infant distress

(Umphred, 2013).

Neonatal physical therapy requires training in many areas, since this is an advanced

practice. In Neonatal Physical Therapy Part I by Jane Sweeney et al. (2009), the extensive

clinical training a physical therapist needs in order to work in the NICU is discussed. Physical

therapists that work in the NICU must first gain experience in a pediatric intensive care unit

(PICU), pediatric ward, newborn nursery, or immediate care nursery, before going to the NICU.

Fellowship programs are available through a pediatric residency that is accredited by the APTA.

This requires a minimum of ten months of clinical practice. Sweeney et al. (2009) discusses the

importance in understanding the normal trajectory and normal neuromotor development of the

infant. This is critical in assisting new NICU therapists understand the process of discharge. With

this knowledge, therapists can recognize asymmetry, tone abnormalities and jittery movement,

which can help in the understanding of growth and development. The therapist can better

determine prognosis and family education needed. Thus, physical therapists must gain

knowledge in family systems, NICU environment, collaborative teamwork in a critical care unit,

infant development, brain development, physiological evaluation and monitoring, and infant

neurobehavioral functioning.

Physical therapists in the NICU carry on several roles including screening,

examination/evaluation, intervention, consultation, scientific inquiry, clinical

education/professional development, and administration. Screening is highly observational and

allows the therapist to see what other disciplines should be involved in the infant’s wellbeing.

While screening, a physical therapist is watching for signs of neurobehavioral disorders or how

the baby responds to the environment and the stress imposed on them through different stimuli.



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The therapist will likely assess movement characteristics, sensory and perceptual development,

behavioral repertoire, and oral motor development. Planning and implementing physical therapy

interventions allows the therapist to apply stressors to the infant, such as range of motion

exercises. Along with intervention for the child comes collaboration with other disciplines and

discharge planning. Lastly, the therapist consults and collaborates with all of the health

professionals tending to the baby in addition to the parents. This results in the patient receiving

the highest level of care (Sweeney et al., 2009).

A clinical decision-making algorithm is implemented in pediatrics, which provides a

means for using evidence in clinical judgment. This model has been molded after the

Hypothesis-Oriented Algorithm for Clinicians I and II and focuses on family-centered care. As a

physical therapist examines a child, the family and health care professionals develop a “strengths

and challenges” list which leads to intervention strategies. Interventions are implemented

regarding a multitude of systems: cardiovascular and pulmonary, integumentary, autonomic,

musculoskeletal, neuromuscular, behavioral, and responsibility (Sweeney et al., 2009).

Teamwork and collaboration is extremely important in the NICU setting. The

organization and number of professionals in the NICU vary depending on the institution. The

team of professionals that work together include neonatologists, neonatal nurse practitioners,

registered nurses, respiratory therapists, registered dieticians, and the developmental team. More

specifically, the developmental team of the NICU includes physical therapists, occupational

therapists, speech language pathologists, and developmental specialists (Blanchard, 2015). The

developmental team will typically receive orders from the physician or neonatal nurse

practitioner to evaluate and treat the infant. The orders can be standing in which all infants less

than 32 weeks receive therapy. It may also be infant-specific depending on the impairments of



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the neonate. Other professionals involved in care in the NICU include certified lactation

consultants, social workers, and discharge planners. Discharge planners can be social workers or

nurses and assist with helping families transition out of the NICU (Blanchard, 2015). Another

key role in the NICU is the developmental specialist that works hand in hand with physicians to

advocate for the patient and the family (Blanchard, 2015).

A developmental specialist can be a physical therapist, occupational therapist, or speech

therapist, registered nurse, or psychologist. The developmental specialist focuses on locating the

infant’s strengths and weaknesses. In addition, the specialist will promote parent-infant

interaction, adapt the care-giving environment in order to enhance the strengths of the infant, and

work with parents to respond and identify certain infant behaviors (Blanchard, 2015). Therapists

and developmental staff work so closely together that sometimes professional roles and

responsibilities become hard to delineate. This is dependent on the type of hospital, NICU staff

resources, and organizational culture. Advanced training and education in clinical practice and

NICU interventions are can aid in this partnership. Therefore, collaboration between professions

is essential in providing the infant and family with the best possible care (Blanchard, 2015).

There are many methods in which the care giving team can interact in order to provide

optimal care for the infant and family. First, the therapy and developmental intervention can be

represented by only one discipline, such as physical therapy. Second, the multidisciplinary model

allows for each discipline to function separately, but complementary in their roles. For example,

the speech therapists may focus on oral feeding, while the physical therapist may focus on

handling of the infant. Using this approach, the greatest needs of the infant will dictate the

treatment. However, one aspect of the multidisciplinary model is that the family is working

alongside multiple different individuals in different professions. This can easily become



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overwhelming for the family, leading to fragmentation and communication barriers. Third, the

transdisciplinary model allows for professionals in all disciplines to share their roles. This allows

for less individuals to be handling the infant and allows for greatest focus on the needs of the

infant. However, sometimes areas are beyond the scope of practice for one individual of the

interdisciplinary team (Blanchard, 2015).

With all models of delivery of service, it is important to be flexible with the demands of

the institution and work schedule. Physical therapists and other disciplinary team members in the

NICU are limited in visitation due to parental visiting schedules, feeding schedules, medical

procedures, and infection precautions. These are important aspects of the infant’s care to take

into consideration when determining the best approach (Blanchard, 2015).

Background and Purpose

Diaphragmatic Hernia and Extracorporeal Membrane Oxygenation

Congenital diaphragmatic hernia is a rare disorder that affects 3,500 live birth infants a

year. This can be detected prenatally (Wessel et al., 2015). It occurs most commonly on the left

side of the diaphragm when the abdominal contents protrude into the thoracic space. Prognostic

factors include early detection, intrathoracic parts of the liver, small lung volume, poor

ventricular function, and low birth weight (Wessel et al., 2015).

Diaphragmatic hernias occur when there is a hole in the diaphragm and the abdominal

contents bulge through the hole (See Figure 1). Lung development abnormalities may be seen

depending on the size of the hole due to the abdominal contents occupying lung space. Defects

are graded into four categories by the Boston scale. These categories help determine the severity

of the malformations and thus the survival rate of the infant (Wessel et al., 2015). Once born, the



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baby cannot breathe on its own outside of the womb. This is considered a life threatening

medical emergency and needs to be surgically corrected immediately post-partum. Prognosis of

the infant is dependent on how severe the hernia is in addition to how well the lungs develop

postoperatively. Other pathologies that involve the brain, joints, or muscle will take longer to

heal. The further the lung development, the better the prognosis is for the infant. Due to this

developmental complication, some infants will be developmentally delayed in their milestones.

These infants might possibly need an interdisciplinary healthcare team, such as physical

therapists, occupational therapists, and speech language pathologists to help them progress

normally (Tovar, 2012).

Figure 1. Infant with left sided diaphragmatic hernia.

It is thought that deformities of the diaphragm occur from a genetic mutation during

development in the first 4-8 weeks of gestation. One reason for why the herniation occurs on the

left side in 80% of cases is due to the fact that the left side of the diaphragm taking slightly

longer to develop (Wessel et al., 2015). This can be detected by a right to left lung ratio, small or

absent gastric sac, distended gastric and duodenal sac, or small bowel dilation. If needed, an MRI

can provide more information. Another factor that may lead to hernia occurrence is maternal



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vitamin A deficiency, however, the exact pathway of this is still unknown. Ideally, if knowledge

of a hernia is known before delivery, the infant should be delivered in a specialty hospital, where

the repair can be managed promptly (Wessel et al., 2015).

The most common sign of respiratory distress occurs during inhalation. As air is inhaled,

it travels into the gastrointestinal tract rather than into the lungs, thus causing the lungs to not

expand. This can result in cyanosis, signs of pulmonary hypertension, mediastinal displacement,

and pulmonary hypoplasia. In the NICU, an infant can be placed on extracorporeal mechanical

oxygenation (ECMO), once all other ventilatory options have been exhausted. ECMO is a

breathing device used to improve oxygen saturation. This requires a surgery in which the infant

is under general anesthesia (See Figure 2). Surgeons can perform the diaphragmatic hernia repair

once the infant is hemodynamically stable (Wessel et al., 2015). Due to the severity of this

condition, an infant is often delivered preterm. This is done in order to ensure lung development

postnatally after diaphragmatic hernia repair. A normal surgery would involve a minimally

invasive procedure through either thoracoscopic or laparoscopic route (Ferreira, 2009).

Figure 2. Extracorporeal mechanical oxygenation showing the route of blood filtration

through an infant



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Ferreia et al. (2009) is a study that examined neonatal minimally invasive surgery (MIS)

for congenital diaphragmatic hernias. Surgeons used two different approaches, thoracoscopic and

laparoscopic, without any severe complications. In cases where the thoracoscopy became

complicated, the technique was converted to the laparoscopy. Criteria deeming a difficult

procedure involves the inability to reduce the hole, requiring a patch, narrow working space,

bowel malrotation, and anesthetic problems. Surgeons closed the hole with sutures, but used a

patch for the larger holes. It was concluded that those who had no other associated anomalies and

hemodynamically stable would benefit from the procedure. According to Ferreia et al. (2009), a

contraindication of MIS is severe pulmonary hypertension, pulmonary hypoplasia resulting in

respiratory distress, and hemodynamic instability. Therefore, this procedure would not be

suitable for Evan, since his diagnosis of Tetralogy of Fallot is a common form of congestive

heart failure. This makes him a complicated patient.

The surgical approach depends on the size of the defect. If ECMO or another ventilatory

technique was used, the abdominal approach may be preferred, since this approach is better for

cardiopulmonary unstable infants. Thus, the abdominal approach would be a better choice for

Evan. During the procedure, the surgeon repositions the abdominal organs in the abdominal

cavity and then repairs the hernia in the diaphragm. Ideally, the surgeons would try to stitch up

and close the hole with sutures. However, depending on how large the hole is, a patch may be

utilized.

Results of the surgery depend on the size of the hernia that need to be fixed. Jawaid et al.

(2013) explained how the procedure is done in order to fix the herniated diaphragm. It also

reviewed the types of prosthetic patches that are used to cover the hole. One type of patch is a

biological patch (Surgisis) that is designed to grow with the infant. A polytetreafluoroethylene



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patch is used where it is unable to close using sutures. Thirty-seven of the 118 newborns in the

study conducted by Jawaid et al. (2013), received a patch. Two of these patients had the

biological patch. The recurrence of herniation was less with the synthetic patch rather than the

biological patch. The two patients with the biological patch had subsequent revisions with the

synthetic patch. After the revision, the patients experienced no reoccurrences. Those that

required a patch were more likely to have further issues, such as, coexisting cardiovascular

disease and thus required ventilator support. This study also noted that patients that required

abdominal herniation tended to have an increased mortality rate. Patients with a lung hypoplasia

and underdeveloped abdominal cavities, were the most severe cases typically. After the

operation, the infant will be on ventilation and stay in the hospital for an extended period. After

the infant is taken off ventilation, supplemental oxygen may be required (Jawaid et al., 2013).

According to Wessel et al. (2015), infants that had treatment complications or other

diseases or lung disorders have a greater morbidity risk in adulthood. During the neonatal period,

20% of reoccurrences occur after two years. Other studies have shown that cerebral changes and

neurological deficits have occurred in children who received ECMO. In adulthood, other

problems such as airway disease, psychomotor delay, chest deformities, gastroesophageal reflux,

and failure to thrive have been seen (O'Mahony, 2012).

ECMO, as mentioned earlier, is used for cardiovascular support in children when all

other less invasive therapies fail or stop working. There are many indications for the use of

ECMO. Indications relating to Evan are as follows: decreased oxygen saturation, poor peripheral

perfusion, heart failure, and congenital diaphragmatic hernia. Patients who are placed on ECMO

need it in order to survive. Only 62% of patients survive to be discharged from the hospital post-

ECMO. Reversible respiratory failure with a history of at least one stable period of PaO2 less



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than 60, weight greater than 2000 g (5 lbs.), greater than 34 weeks of gestation, mechanical

ventilation for less than 28 days, and absence of cerebral injury are all indications for treatment

(Campbell, 2012).

ECMO pump takes over oxygenation and perfusion of the infant’s body while the heart

and lungs are “resting.” It is used as a bridge to surgery in severely compromised patients who

are too ill to undergo repair immediately. It is also used as support for patients awaiting heart,

heart-lung, or lung transplants. ECMO is capable of providing support for several days to at most

a few weeks (Campbell, 2012).

There are two types of ECMO; venovenous and venoarterial. Venovenous is used when

the heart is functioning well and only the lungs need assistance. Blood is removed from a vein,

circulated through a blood pump and artificial lung, then returned to the vein. Venoarterial is

used when both the heart and lungs need assistance. Blood is removed from a vein, circulated

through a blood pump and artificial lung, then returned to an artery (Goodman, 2009).

Following ECMO, there may be severe complications. Infants may suffer from cerebral

infarction, brain hemorrhage, renal failure, or multiorgan system failure. Patients on ECMO must

remain intubated and sedated leading to inability to be mobilized while on support. The child

will have to remain on ventilation to maintain oxygenation. Nitric oxide gas is given through the

ventilator to relax and dilate blood vessels (Kattan et al., 2010).

Kattan et al (2010) looked at the effects of ECMO in newborns with congenital

diaphragmatic hernias (CDH). In few cases, it has been shown to reduce mortality in infants with

respiratory failure. Early intervention of ECMO has shown better results rather than later

intervention, but there is still limited research. After analysis, Kattan et al. (2010) noted that

there is still not enough evidence to suggest that ECMO is beneficial. The survival rate has



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increased with ECMO. Kattan et al. (2010) did conclude that there was an increase with long

term disability. The long term disability did not include cerebral palsy, vision or hearing

impairments, mental delay, or other developmental disabilities. Research should be geared

toward a more selective process for infants that qualify for ECMO in order to reduce a long term

disability. It is proposed that an infant who has had a hernia patch repair and placed on ECMO

has a poorer prognosis. This study concluded that ECMO works best with cardiopulmonary

diseases that are reversible, such as Tetralogy of Fallot.

Tetralogy of Fallot

Tetralogy of Fallot (TOF) is a form of congenital heart disease that results in an abnormal

formation of the heart (Goodman, 2009). It is the most common congenital heart disease found

in infants (Allen et al., 2001). It is characterized as a cyanotic heart defect, meaning that the

blood is not getting enough oxygen to be circulated throughout the body. This is due to tricuspid

atresia, which is a failure of the formation of the tricuspid valve during development. This failure

causes lack of communication between the right atria and right ventricle. Because of this,

deoxygenated blood travels from the right atria to the left side of the heart through a septum wall

defect. This results in de-oxygenated blood being pumped into circulation (Allen et al., 2001).

Prevalence of TOF ranges from 0.26-.48 per 1,000 live births (3.5-9.0%) (Allen et al.,

2001). TOF is classified by four defects: pulmonary artery stenosis, aortic communication with

both ventricles, large ventricle septal defect (VSD), and right ventricular hypertrophy (Goodman,

2009). It is thought that these deformations occur during two phases of embryonic growth,

specifically during the third through fifth weeks of gestation (see Table 2).



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Table 2: Cardiac Normal Embryonic Development (Hill, 2015)

Week 3 - Heart tubes fuse at midline - Heart starts to contract

- Ventricles move caudally reducing the outflow traveled - Atria converge

- Truncus arteriosus is formed

Week 4 - Division of ventricle

Week 5 - Division of the atrioventricular canal and septation

- Endocardial tissue expands creating a separation between the atrium and ventricle, which forms the AV (bicuspid and tricuspid

valves) - Atria separate and a septum is formed in between (foramen ovale

is formed if left open)

- Blood flows from right to left: the pulmonary vein forms off the wall of the left atrium.

- Trabeculations form in ventricle development and eventually become the interventricular septum

Week 6 - Neural crest mesenchymal cells start to form the bulbous cordis,

creating bulbar ridges that collide with the truncus arteriosus - The neural crest migrates to become part of the aortic arch

- Bulbar ridges rotate and create the aorticopulmonary septum forming the semilunar valves

- Bulbus cordis will make up the infundibulum in the right ventricle

and aortic vestibule in the left ventricle.

It is thought that TOF develops from two embryological errors: when the infundibular

growth in the right ventricle develops abnormally resulting in pulmonary artery stenosis and

when the truncus arteriosus divides unevenly resulting in different sized vessels. The

infundibulum is the stalk that lies between the atria and pulmonary artery. If the infundibulum

forms abnormally during atrial separation, it alters the semilunar valve formation resulting in

pulmonary stenosis. The valves become rigid and don’t close or open all the way. This narrowed

opening makes blood passing through more difficult. The right ventricle must pump harder to get



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the same amount of blood through the narrowed opening, resulting in right ventricular

hypertrophy (Allen et al., 2001).

If the truncus arteriosus divides unevenly, the aorta becomes larger and begins overriding

the septum. When the aorta becomes enlarged, it has “communication” with both ventricles. This

means blood from both right and left ventricle are pumped into the aorta rather than only the left

ventricle. This misalignment occurs during the 4th week of gestation where the heart starts to

form (Allen et al., 2001).

The VSD occurs when the septum does not close properly. This results in a gap between

the ventricles (See Figure 3). This deficit occurs during week six, when the trabeculations form

the ventricular wall. When this deformity occurs, deoxygenated blood is mixed with the

oxygenated blood leading to a pressure gradient alteration. The pressure in the ventricle s is now

even when there should be a difference (Allen et al., 2001).

Hemodynamics vary between patients because of the right to left shunting and pulmonary

stenosis. This means deoxygenated blood is not able to get to the lungs efficiently in order to be

oxygenated. Also, due to shunting of the blood and the overriding aorta, the deoxygenated blood

is being pumped into the rest of the body system. All these factors stated can result in cyanosis of

the infant (Allen et al., 2001).



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Figure 3. Blood flow through a normal heart (left) and a heart with TOF (right)

(www.cdc.gov)

Tetralogy of Fallot may be caused by a genetic mutation. The cause of congenital heart

defect is believed to be a disruption of structural formation caused by a mutation within the

genes. The cause of the mutations is thought to be more environmental factors. The missense

mutation of the JAG1 gene has been shown to lead to heart defects in Tetralogy of Fallot and

pulmonic stenosis. The JAG1 gene is believed to cause cardiac development. This occurs early in

embryonic development at 14 days (E14). Donovan et al (2009) looked at the suspected JAG1

gene. which produces a protein in the Notch pathway. This pathway is critical to embryonic

cardiac development. In mice, JAG1 is congruent with Hey2 genes. These genes, specifically,

are found in arterial smooth muscle. When the Hey2 gene sequence was mutated within the

suspected area of the JAG1 and Notch sequence, only 3% of the mice exhibited Mandlian ratios.

The mice litter appeared the same, however, upon necropsy, mutant mice showed characteristics

of heart malformation. They also noted that the most common malformation seen in the Hey2



22

gene mutation showed the characteristics for TOF. This suspicion was confirmed examining

blood flow through the heart by injecting the mutant mice that survived with dye. It was shown

the blood passed from the right ventricle to the left ventricle, through the septal defect and into

the aortic and pulmonary arteries (Donovan et al., 2002).

Nemer et al (2006) also found a correlation between the genes that result in protein

interactions in atrial septal deficits. In other studies, it was concluded that this GATA4 gene

mutation results in abnormal folding within the embryo. However, this is an area for future

investigation and research. Further studies have shown that GATA4 is key in mediating cardiac

morphogenesis. Since this gene is found in cardiac cells during embryogenesis, it is reasonable to

suggest that these genes are responsible for deficits that are seen in TOF (Nemer et al., 2006).

Tetralogy of Fallot can be diagnosed via echocardiography, cardiac catheterization,

cardiac surgery, or autopsy within one year of life (Allen et al., 2001). Tetralogy of Fallot along

with the herniated diaphragm was diagnosed when Evan’s mother had a routine ultrasound.

Echocardiographic studies would show right ventricular dominance and inverted T waves (Allen

et al., 2001). An echocardiogram would reveal VSD, dilated coronary sinus, aortic insufficiency,

and possibly enlarged infundibulum if severe enough (Allen et al., 2001).

Early detection helps the pediatric cardiologist predict outcomes by addressing

hemodynamic problems. This improves neonatal morbidity, long term outcomes, and cost

effective health care management. Outcomes can be determined at 16-37 weeks by measuring

the pulmonary valve, main pulmonary valve ratio, and direction of ductus arteriosus flow.

Pulmonary obstruction can worsen in the second and third trimesters of pregnancy requiring the

prostaglandins to keep open. The pulmonary valve provides a more sensitive identification for

ductal dependence. This increases the need for neonatal surgery. Surgery is based on the



23

adequacy of pulmonary blood flow in the fetus. Fetal echocardiograms performed at 16 weeks

can predict ductal dependence (direction of blood flow) and thus outcome. Fetuses with reversed

blood flow (left to right) were more likely to require surgery to fix the deficit (Arya et al., 2014).

Surgical interventions may be needed if the abnormalities are severe enough. The

approach depends on the severity of the obstruction. Aortopulmonary shunt has the best results

in patients 3 months or older. Shunting has shown to be safe and have reliable outcomes. Patients

had shorter ICU and hospital stays and decreased mortality rates over all. However, some

patients experienced thrombosis or death at the location of the shunt. When the shunt is blocked,

higher in-hospital mortality rate (21.4%) was noted. The occlusions were attributed to a

pulmonary atresia in addition to the small size of the pulmonary arteries. Early surgical

intervention has been shown to avoid shunt related complications, provide early hypoxia relief,

promote normal lung development, avoid further right ventricular hypertrophy and fibrosis, and

comfort the family (Arya et al., 2014).

Another surgery used in TOF is cardiopulmonary bypass. Recent studies have found that

cardiopulmonary bypass has more stable outcomes after initial repair attempts. Primary repairs

are done under hypothermic cardiopulmonary bypass and medically induced heart cessation. The

patient is connected to a bypass machine, similar to one that used in a coronary artery bypass

graft procedure. Once the heart is stopped, a sternotomy is performed. The procedure is then

conducted via a transatrial or transventricular approach. The right side outflow tract is fixed by

patching in a pulmonary stent in order to fix the pulmonary stenosis. This is completed

cautiously taking into consideration the small size of the underfilled and underpressureized

pulmonary artery. The VSD is closed and patched with the patient’s own left auricle. Lee (2014)

noted that no patients experienced junctional tachycardia or AV heart block. Survival rates for



24

this procedure at 6 months, 1 year, and 5 years were shown to be 96%, 92%, and 92%

respectively. A few patients required another procedure for a replacement stent. In addition, the

left and right ventricular systolic function was found to be normal (Lee, 2014).

Typically, normal AV conduction should be seen in infants. However, post operatively, a

right bundle block may be seen. It is common to see arrhythmias after cardiac surgery.

Unfortunately, some of these arrhythmias can be life threatening if left undetected. The common

arrhythmias seen after surgery are atrial fibrillation, atrial flutter, sinus node dysfunction, PVC,

PAC, ventricular tachycardia, various AV blocks, right bundle blocks, and bifascicular blocks

(Kuzevska-Maneva et al, 2005). The septum wall will most likely suffer damage and experience

conduction issues due to the anatomical location (Kuzevska-Maneva et al, 2005).



25

History

Table 3. ICF Model for Evan

Body

Structure and

Function

Activity Participation Environment

al (Internal)

Environmental

(External)

Resources · All senses in tact

· Able to cry and grasp

· Able to turn head to sound

of human voice

· Eager to meet family

· Supportive family

environment · Family has

means to finance best care for Evan

Limitations · Limited

strength, ROM, reflexes

· Muscle tone (hypotonic) ·Extension

patterns (difficulty

achieving and maintaining flexed, midline

orientation) · Inability to

stabilize vitals · Sensory disorganization

· Breathing

· Feeding · Visual and

auditory responsiveness

· Head control and

movement of hands to mouth

· Unable to

socialize with family and be

held by parents

·Overwhelmed

in NICU environment

and sensitive to temperament

· Levels of

lighting and noise in special care

nursery · Number of personnel in

multidisciplinary/ transdisciplinary

team

The Physical Therapy Plan for Examination

Tests and measures in the NICU setting provide unbiased documentation of the neonate’s

function, support for given developmental interventions, documentation of the effectiveness of

treatment, and determination of infants needing developmental follow-up after discharge from

the NICU (Umphred, 2013). The most useful assessments contain components of neurologic

function, neurobehavioral functioning, motor behavior, and oral-motor function. Many of the

assessments used in the NICU setting require certification and training of the individual



26

performing the assessment. In addition, most of the assessments are time consuming and require

close monitoring of the physiologic stability of the infant. If the infant cannot tolerate testing, it

can be postponed or completed within multiple segmented testing periods (Umphred, 2013).

The purpose of examining and evaluating the infant is to identify the impairments in body

structure and function that are contributing to activity limitations and participation restrictions

(See Table 3). It is also used to determine the developmental status of the child and the need for

skilled positioning and handling (see Table 4). Individualized responses to stress and self-

regulation can also be evaluated as well as environmental adaptations to optimize growth and

development (Campbell, 2012).

Table 4. Recommendations for Physical Therapist Examination and Evaluation of Infants

in the NICU (Campbell, 2012)

1) Protect the fragile infant’s neurobehavioral system, especially if they cannot tolerate a standard evaluation

2) Repeat observations over time

3) Utilize the multidisciplinary NICU team with the parents

4) Observe, interpret and communicate the infant’s behaviors to the parents and other NICU team members

States of consciousness in the neonate are assessed as deep sleep, light sleep, drowsy,

quiet awake, active awake, and crying. As an infant matures, they are able to transition smoothly

and predictably between these states of consciousness. Physical therapists play an integral role in

educating parents and staff to identify state transitions. In addition, physical therapy helps create

the optimal environment in order to ease transition to and from sleep (Campbell, 2012).

The goals of physical therapy must be aimed at enabling the infant’s participation in age-

appropriate developmental activities and interactions with family members. Physical therapists



27

create a neurobehavioral profile of the infant based on observations (Umphred, 2013). This

neurobehavioral profile is mainly due to the inability of the infant to tolerate a full standardized

developmental exam. The evaluation describes infant’s successes and difficulties in achieving

and maintaining self –regulation. It also identifies strategies that support the developmental level

of the infant. Physical therapy must be appropriately timed and modulated in order to match the

neurobehavioral competencies of the infant, due to the stress associated with the handling of the

medically frail infant. Any exam or intervention of an infant should include family members in

order to facilitate bonding (Umphred, 2013).

The infant’s heart rate and respiratory rate should be documented and recorded from a

monitor. Neonate heart rate is 120-180 bpm and respiratory rate is 20-40 breaths per minute. It is

important to document respiratory effect and digestive effects during rest, routine care, handling,

and social interaction of the infant. Irregular respirations or paling around the mouth, eyes, and

nose, spitting up, straining, bowel movements, and hiccoughs are indicative of instability and

inability to self-regulate. Smooth respirations, even color, minimal startles, tremors, and

digestive stability indicate that the infant’s ability to self-regulate (Campbell, 2012).

The main component of the motor system exam involves observation of infant’s posture

at rest, active flexion movements during quiet and wake periods, routine care, social interaction,

and feeding. Active flexion begins at approximately 32 weeks for the lower extremities, 35

weeks for the upper extremities, and 37-39 weeks for the head and trunk. Compensations that

can be seen in infants include retracted scapulae, externally rotated and abducted lower

extremities, and extension and rotation postures of cervical spine and trunk. Interestingly, not all

reflexes are tested. Those that are only deemed “functional” are included in the exam. These are

suck, swallow, palmar, and plantar grasp, and early righting reflexes (Campbell, 2012).



28

Physical therapy is critical in the facilitation of visually tracking faces or brightly colored

objects. In addition, alerting the infant to familiar voices is typically seen with compensations

initially (Campbell, 2012). Preterm infants can feel pain, however, they are unable to

communicate their pain level. The Newborn Individualized Developmental Care and Assessment

Program (NIDCAP) is a weekly observation and assessment of the infant’s behavior and

response to handling. It is completed by a developmental specialist or NIDCAP certified physical

therapist (Umphred, 2013). The therapist observes and documents infant behavior before, during,

and after routine caregiving procedures. Other pain assessments include the Premature Infant

Pain Profile (PIPP), which assigns points for changes in three facial expressions (brow bulge, eye

squeeze, and nasolabial fold). The Neonatal Pain, Agitation, and Sedation Scale uses five

indicators to assess pain: cry and irritability, behavioral state, facial expression, extremity

movement and tone, and vital signs. In addition, the Face, Legs, Activity, Cry and Consolability

Behavioral Tool (FLACC) uses a grading system to assess facial expression, leg activity, general

activity, cry, and capability to be consoled (Umphred, 2013).

Review of Systems

A review of systems was obtained from a chart review. Systems reviewed are found in

Table 5.



29

Table 5: Systems Review (McManus, 2012)

System Impairments Impact on Activity

Cardiovascular - TOF - Decreased tolerance for

activity

Pulmonary - Cyanosis - Dyspnea

- Immature lung development

- Small lung volume - Pulmonary hypertension - Mottling

- Decreased tolerance for activity

- Limited awake/alert state - Limited/unable to interact

with environment

Integumentary - Scarring - Limit activity due to pain or discomfort

Neuromuscular - Low tone - Primitive reflexes WNL

- Smaller, less forceful, movements

- Decreased postural control

Musculoskeletal - Decreased ROM retracted scapula,

externally rotated and abducted lower extremities and cervical

spine extension - Decreased strength

- Smaller, cramped movements

- Less forceful movements –may need assistance in positioning

- Decreased self-regulatory skills of hand to mouth and

midline, finger clasping, and foot bracing

Central Nervous

System

- Increased autonomic

response to stimulation

- Decreased tolerance for

activity

Family-Centered Care

Family-centered care (FCC) is the collaboration between families and practitioners to

create a partnership to aid in the care of the family’s infant. Family priorities, learning styles,

emotional stress, and cultural variables are all considered when making critical decisions for the



30

infant. It is an empathetic process that focuses on the infant’s survival and development in

addition to mourning the loss of the family’s ‘imagined” life. This emotional and financial toll on

the family can lead to posttraumatic stress disorder. It is the physical therapist’s duty, along with

NICU health providers, to understand the hardships of the parents (Sweeney et al., 2010).

Family-centered care focuses on the notion that the family is the constant in the child’s

life. It was built upon partnerships between families and professionals. The family is the center

of the services provided and are involved in the decision making process. The family is the

primary decision maker while the professional provides information to help the family make

informed decisions (Law et al., 2003). There are eight core concepts to the FCC, which include

respect, choice, information, collaboration, strengths, support, empowerment, and flexibility

(Campbell, 2012).

Involvement in the grief process with parents is vital for the therapist. It is common for

the parents to initially display an emotional and physical distance from the infant, nevertheless it

is important for the therapist to actively listen and withhold judgment. Peer support groups have

been shown to aid in this process. When an infant passes, it is imperative that the therapist begins

working on closure with the family (Umphred, 2013).

The framework for the FCC model involves three premises or assumptions that form the

basis of family-centered services (See Table 6). Under each premise is a guiding principle that

outlines what the families should expect in terms of a relationship with their service provider.

Finally, the key elements describe the behaviors of service providers and the family’s rights and

responsibilities (Law et al., 2003).

As discussed previously, involvement of the family in the infant’s care is imperative.

Additionally, teaching and education of the family is important for discharge. During education



31

of the family, it is important that the teaching method is adapted to the parent’s learning style. In

addition, the therapist should help parents find and appreciate positive aspects of their infant’s

development. This gives the parents hope and can help them to generate a new found energy

toward to the NICU process (Umphred, 2013). Much research has been conducted on the FCC

model. The variation in the research in how family-centered care is defined, the settings, and

patient population investigated, make it difficult to apply these findings into practice (Harrison,

1993).



32

Table 6. Premises, Principles, and Elements of Family-Centered Care (Law et al, 2003).

Premise

Parents know their children best

and want best for their children.

Families are different and unique. Optimal child functioning occurs

within a supportive family and

community context: The child is

affected by the stress and coping of

other family members.

Guiding Principle

-Each family should have the

opportunity to decide the level of

involvement they wish in decision

making for their child.

-Parents should have the ultimate

responsibility for the care of their

children.

-Each family and family members

should be treated with respect.

-The needs of all family members

should be considered.

-The involvement of all family

members should be supported and

encouraged.

Key Elements

Family

Expectations

Service

Provider

Behavior

Family

Expectations

Service

Provider

Behavior

Family

Expectations

Service Provider

Behavior

-Ultimate

decision makers

-Utilize own

resources

-Receive

information in

order to make

informed

decisions

-Define

priorities of

intervention

-Choose level

and type of

involvement

-Receive

services within

timely manner

-Have access to

information

regarding child

and family

-Encourage

parent

decision

making

-Assist

families to

identify own

strengths

-Inform,

answer, and

advise parents

-Help parents

and children to

prioritize need

-Collaborate

with parent at

all levels

-Provide

accessible

services

-Share

complete

information

about child’s

care on

ongoing basis

-Maintain

integrity and

dignity

throughout care-

giving process

-Be supported in

decisions

-Opinions

listened to

-Receive

individualized

services

-Respect values,

wishes, and

priorities of

families

-Accept and

support family

decisions

-Listen

-Provide flexible

and

individualized

services

-Be

knowledgeable

about and accept

diversity

-Trust parents

-Communicate

in language

understood by

parents

-Needs and

concerns taken

into account

-Feel welcome

and supported in

level of

participation

chosen

-Be sensitive to

psychosocial

needs of all

family members

-Provide an

environment that

encourages

participation of

all family

members

-Respect the

family’s style of

coping without

judging

-Encourage

family-to-family

support and the

use of natural

community

supports and

resources

-Recognize and

build on family

and child

strengths



33

Neonatal Behavioral Assessment Scale (NBAS)

The Neonatal Behavioral Assessment Scale (NBAS) is used to document individual

behavioral and motor differences in infants from 40 weeks post-conceptual age to two months of

age. The NBAS is used as an integrative way to qualify newborn infant behavior on the infant's

ability to recover from stimulus and return to an alert state (Brazelton, 2011). Developed by

Brazelton and Nugent, the scale assesses neuromotor responses in a 30-45 minute examination. It

consists of observation and elicitation. The NBAS scores 28 biobehavioral items on a nine point

scale and 18 reflex items on a four point scale. The scoring is based on the infant's best

performance and the examiner is encouraged to allow the infant time to be alert and ready for

evaluation. The examiner should provide tactile touch and be sure to test the infant in between

feedings to give the infant every advantage. During the NBAS, the newborn is assessed on

interactive ability, motor behavior, behavioral state organization, and physiological organization.

This can be observed through the observation of general qualitative movements (Umphred,

2013). Behavioral state organization is based on state of consciousness in the neonate (Brazelton,

2011).

The NBAS describes general movements (GMs) as a range of behaviors that include

complex motor activities and can be implemented on fetuses, preterm, and term babies. The

nervous system of the fetus generates GMs endogenously, or without being stimulated. GMs

begin as early as 9-12 weeks post-conceptual and is defined by movement patterns such as

startles, limb movements, twitches, stretches, breathing moments, hiccoughs, yawns, head

rotation, head flexion, sucking, and swallowing movements (Brazelton, 2011). GMs are

continued without a significant change in form despite change in the environment. Of these,

stretches and yawns are a key component to analyze due to maintenance of form throughout life.



34

Central pattern generators (CPGs) produce GMs from the higher parts of the developing medulla

and brainstem and can be observed during the first three months of life (Brazelton, 2011). These

endrogenous movements are characterized as breathing, sucking, chewing, eye movements,

swimming, crawling and walking (Brazelton, 2011). GMs are important to observe for their

fluency, variability and complexity.

If the CNS is impaired, GMs will lose these characteristics. These movements can also be

abnormal if they have poor repertoire, cramped-synchronized, or are chaotic or jerky. Poor

repertoire abnormalities indicate a present brain lesion and appear as monotonous and repetitive.

This sequence is the most common abnormality in pre-term and early post-term age babies.

When abnormal GMs are followed by normal fidgety movements, there is a better prognosis.

However, these fidgety movements alone are a predictor for Cerebral Palsy due to its correlation

with severe perinatal asphyxia accompanied by transient phases of hypokinesis (Brazelton,

2011).

General movements of a healthy fetus have a large amplitude, occur at a fast speed, and

occur frequently with the lifting of the pelvis. Preterm age GMs are similar to those of the fetus,

however, exhibit poor repertoire in that they are repetitive and monotonous in sequence.

Increased behavior stress can be demonstrated as sequelae to increased heart rate and mean

arterial pressure such as increased finger splay, arm salute, hiccoughs, yawn, and mottled skin

color. These attributes were demonstrated by infants 30 to 35 weeks post-conception at a higher

rate than infants born at term (Sweeney, 2010). The NBAS is measured on a weekly basis and

takes into account prematurity by beginning at 27 weeks post-conceptual age. Each week is

given a grade of one of the following for observational movement: F+: fidgety movements; F-:



35

absence of fidgety movements; AF: abnormal fidgety movements; CS: cramped-synchronized

GMs; Ch: chaotic GMs; PR: poor repertoire GMs; H: hypokinesis; N: normal GMs.

The strong asset of the NBAS is that it enables examiners to document changes over

time. The NBAS represents a guide that helps examine an understanding the newborn's language.

The NBAS has a 90% interrater reliability rate and is highly sensitive screening for

developmental milestones. This tool for assessment of GMs represents a new paradigm shift

from traditional testing of reflexes and responses to external stimulation. The NBAS, instead,

bases their assessment off of endogenously generated motility. This tool qualifies spontaneous

movement for the detection of specific neurological prognosis that are predictive of

developmental delays and or neurological diagnosis considerations (Brazelton, 2011). The

assessment is not predictive, but gives a good analysis of the infant's strengths and areas of

weaknesses. The NBAS, along with the Neonatal Behavioral Observation (NBO) allow

examiners the ability to evaluate and determine developmental considerations for the newborn.

An example of an examination sequence is outlined in Figure 4. Neonatal physical therapists

determine the willingness of the infant to begin neurological and neuromotor examination. It also

monitors and modifies changes in physiologic and behavioral stability (Sweeney et al., 2010) .

Evan, being born prematurely, will present with probable repetitious movement when

examined on the NBAS. In order to properly examine Evan, we must be sure that he is medically

stable in order to perform the exam. When he is, then he must be fully awake and conscious.

This can be examined in the initial stages of the NBAS where consciousness is considered and

determined on a scale from sleep. Cramped-synchronized GM is the most specific character of a

GM in a preterm baby that we would suspect Evan to repeat when we qualify his movements.



36

Another strong asset of the NBAS is the quality of infant to examiner interaction, which aides in

family involvement in the behavioral styles of the infant.



37

Fig

ure

4.

Exa

min

atio

n S

eque

nce.



38

Newborn Behavioral Observations (NBO)

The Newborn Behavioral Observation is a scale molded after the NBAS that can be used

from birth to the third month of life. While the NBAS focuses on the assessment and diagnosis of

an infant, the NBO is more observational and looks at the developing relationships and describes

behaviors of the high-risk infant. The NBO helps parents understand their infant’s behaviors and

sensitize them to their child’s competencies by promoting positive interactions. There are 18

behavioral observations (See table 7) in the NBO which help understand the newborn’s

development and behavior. There are also seven clinical principles met by the NBO (See table

8). These clinical principles help a clinician make the decision that the NBO is the right

examination procedure.

Tasks observed by the NBO are described by Table 9. These behavioral dimensions

represent developmental tasks infants confront over the first month of development. Table 10

lists response themes one might observe if the behavioral dimensions are disrupted. The NBO is

graded using a traffic metaphor when observing an infant. Green light is indicated self-regulation

and wellbeing, which allows the clinician to continue with the exam. A yellow light indicates the

infants threshold to stimulation and the clinician needs to pause their session before continuing.

Red light indicates a child’s need for support and loss of self-regulation indicating to the

clinician to either stop the NBO or take a prolonged break.

When thinking about Evan and his multitude of pathologies, it is important to postulate

potential observations observed. Based on the NBO, Table 7 shows possible outcomes Evan

would demonstrate (Nugent et al., 2007).



39

Table7: NBO Items

NBO Items Evan’s Predicted Outcome

1. Habituation to light

(flashlight)

Evan might react to light by squirming slightly or blinking. This would be a green

light. If Evan where to show duskiness or is limp, he would be showing signs of a

red light reaction

=3

2. Habituation to sound

(rattle)

A mild startle is a green light reaction where a full body startle is a yellow light

reaction, we would expect Evan to show responsiveness however it might startle

him more than expected due to over stimulation being in the NICU

=3

3. Muscle tone: legs and

arms

Evan probably is not moving too much due to being so sick. He might show signs of

a yellow light which is fussing with active movement but may become fatigued

quickly

=2

4. Rooting Evan probably has good rooting reflexes since there is no neuronal involvement

=3

5. Sucking Evan probably has a good sucking reflex since there is no neuronal involvement

=3

6. Hand Grasp Evan probably has good grasping reflexes since there is no neuronal involvement

=3

7. Shoulder and neck

tone (pull to sit)

Since Evan has a lot going on in his upper body (ECMO, heart issues, etc.), we

would expect him to have head lag, which would be a yellow light

=2

8. Crawling response Like pull to sit, Evan may only have slight arm and leg flexion

=2

9. Response to face and

voice

We would expect to see Evan following either the PT or his parents face as they talk

to him with smooth head and eve pursuits

=3

10. Visual response (to

face)

We would expect to see Evan following either the PT or his parents face with

smooth head and eve pursuits

=3

11. Orientation to voice Evan would probably turn to the sound of his parents as he is in supine

=3

12. Orientation to sound

(rattle)

Evan would probably turn to the sound of a rattle as he is in supine

=3

13. Visual tracking (red

ball)

We would expect Evan track a ball with smooth pursuit

=3

14. Crying



40

15. Soothability Evan’s body is being highly stressed with a lot of stimuli. We would expect him to

have a hard time self-soothing but can be soothed with clinician support

=2

16. State regulation States may be somewhat unpredictable but well defined since Evan has so much

stimuli

=2

17. Response to stress

(color change, tremors,

startles)

Evan might tremor slightly with slight color changes to moderate stimulus

=2

18. Activity level Evan’s body is undergoing a lot so we wouldn’t expect him to have a high level of

activity but moderate would be predicted

=2

Table 8: NBO Clinical Principles

1. Relationship building system

2. Infant focused

3. Individualized development-based system

4. Family-centered system

5. Based on positive-adaptive model

6. Promotes Development of a positive clinician-family partnership

7. Designed to be used to bridge the clinician-family-community gap

Table 9: Behavioral Dimensions Observed via NBO

1. Autonomic

2. Motor

3. Organization of state

4. Responsibility



41

Table 10: Themes for the Pre-term Infant

1. Sleep Issues

2. Touch and handling

3. Feeding problems

a. Low arousal

b. Fatigue

c. poor coordination of swallowing and sucking

d. Poor coordination of breathing with suck-swallow

e. Weak suck and/or hypertonicity

f. Limited tongue and jaw mobility/or hypertonicity

g. Oral hypersensitivity

h. Irritability

i. Poor lip closure

j. Inadequate intra-oral suction

4. Crying

Test of Infant Motor Performance (TIMP)

The TIMP (Test of Infant Motor Performance) is a test of functional motor behavior in

infants between the ages of 34 weeks postconceptional age to four months post-term (Umphred,

2013). The TIMP demonstrates adequate evaluative validity and is able to screen for

developmental delays for infants born prematurely (Spittle, 2008). Developed by Campbell and

colleagues, the 42 item exam evaluates postural control, spontaneous movement, anti-gravity

movement, adaptation to handling, self-regulation, visual reaction, auditory reaction, and

interaction with the caregiver. It also examines head control from 32 weeks post-conceptual age



42

to 16 weeks post-term. The TIMP has a strong construct validity and predictive validity for

developmental screenings.

The TIMP is broken down into two sections: observed and elicited. In the observed

section, the infant gets one point for every movement observed. The observation section should

be performed first, however any of the observed items can be performed throughout the test and

scored appropriately. The elicited section allows the use of visual or verbal prompts to facilitate

the infant to perform the desired motion, however, only three attempts are allowed for each item.

Similar to the other tests, the best possible response is recorded (Campbell, 2001). When the test

is completed, the raw scores are summed and compared to predicted age standards.

According to Lee (2012), infants born less than 1500 grams at birth, before 32 weeks

gestation, or with chronic respiratory disease, had lower TIMP scores than preterm infants with

no medical issues. It was found that the same infants with lower TIMP scores had more severe

developmental delays than infants with initial higher TIMP scores (Lee, 2012). Therefore, when

identifying the risk factor for developmental delays in preterm infants, it is important to perform

the TIMP to predict related problems. Subsequently, intervention plans may be created based of

off the TIMP findings. In clinical practice, it is best to use more than one assessment tool to meet

the needs of the individual being evaluated in order to ensure more valid prognosis and

discriminative prediction for physical therapy intervention (Spittle, 2008).

Evan would be expected to score below average on the TIMP since he is eight weeks

premature. The normal range of scores on the TIMP for his chronological age of two months

should fall between 76-121. His age adjusted range would be equivalent to a newborn, or, 40

weeks postconceptional age. The average range score for this age group is 50-80. Evan had a

combined raw score of 48 which places him in the below average category (35-49). His below



43

average score can be attributed to his past medical history of ECMO and Tetralogy of Fallot.

These pathologies result in fatigue, which would limit his movement and development.

As physical therapists, it is imperative to understand the signs of a self-regulated versus

an agitated infant in order lead an examination and educate the family on what their child is

trying to relay to them. The NBAS allows therapists to discern the general movements of Evan

and his development in the NICU in order to determine his stabilization (Sweeney et al., 2010).

Since Evan was born two months prematurely and presents to us two months term age, it is

suspected that general movements are now present. General movements may be large in

amplitude with a quick pace and frequent pelvic lifts. This type of qualitative analysis is a part of

direct neonatal physical therapy services that are provided to address an infant's musculoskeletal,

neuromuscular, or neurobehavioral needs (Sweeney et al., 2010).

Discussion and Conclusion

The purpose of this case report was to provide information on the examination of a

patient in the neonatal intensive care unit. The pathologies, the NICU setting, and the role of the

physical therapist in this setting were thoroughly presented. The examination strategy presented

is one that can be applicable to other patients in the NICU setting. Other physical therapy

professionals may utilize this case report in examining the preterm infant in the NICU

environment.

The role of the physical therapist includes many aspects of care. The first role is to

examine the infant. This is conducted to determine the need for services in the NICU and

services post-discharge, which include early intervention or out-patient services. The therapist

will also design and perform individualized interventions specific to the infant’s motor,

physiological, neurological, and developmental needs. In the NICU setting, physical therapists



44

and other professionals may follow a multidisciplinary or transdisciplinary model in order to

provide the best line of care for the patient and family.

The NICU setting strives for self-regulation of the infant. In addition, it is essential the

newborn gain weight. The NICU provides the resources for this. However, sometimes these

integral aspects of infant development are not met. Due to this, family-centered care and parental

education right from the beginning of NICU admittance is of the upmost importance.

In NICU clinical practice, the adoption of a more transdisciplinary approach is imperative

in the family-centered care model. It is essential that the newborn be able to respond to their

surroundings with stabilized vitals. This can be achieved through decreasing the stress from this

environment. The less stimulation present in the NICU, the more likely it is that the newborn will

interact with the environment.

In many settings, there are too many untrained personnel in the NICU setting. The role of

the physical therapist is focused on family-centered care and avocation for patients. Thus, it is

important for only essential personnel to be in contact with the infant. This essential listing

should include the mother, father, physician of choice, and developmental specialist of choice. A

developmental specialist can include a physical therapist. With physical therapy being a

doctoring profession, it is within the scope of practice to complete a fellowship or internship

within the NICU setting.

Other areas of research that require further investigation include the genetic causes of

TOF and CDH and the prevention of other congenital disorders. There is much hypothesized

about the genetic mutations of these disorders. However due to the advancement of technology,

more genetic information should be accessible. In addition, exercise guidelines are scarce in the

literature for patients with CDH and TOF. It can be inferred that infants with these disorders



45

would present extremely weak and deconditioned. However, at this time, there is no literature

available to correlate these findings. In future research, the impact of the different models of the

NICU should be considered. More specifically, a transdisciplinary model should be reviewed

against mortality rate and overall development of the child. A single-subject design that is

longitudinal in nature would be imperative to address the uniqueness of each neonate. All of

these suggestions for future research can further advocate for the physical therapy profession.

Specifically, this can be accomplished by providing future generations of neonates in the NICU

with the best quality of care.



46

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