The Newborn and Infant Physical Examination programme (NIPE) Handbook

Patient information leaflet
Screening providers should use information in Screening tests for you and your baby to inform parents about the newborn and 6 to 8-week physical examinations

Aim

The NIPE programme screens newborn babies within 72 hours of birth, and then once again between 6 and 8 weeks for congenital conditions affecting their:
eyes, heart, hips and testes

Record keeping

1. Providers should obtain verbal consent for screening.
   Clearly record each screening examination and the outcome of each examination in the:
   NIPE screening management and reporting tool SMaRT4NIPE (S4N) IT system (newborn examination)
   or
   GP IT system (infant examination)

2. baby’s clinical notes

3. personal child health record (PCHR) – commonly known as the red book

4. local clinical data collection system, where appropriate

Quality Assurance

1. Local arrangements to ensure all screen positive babies are referred and seen in line with national standards

2. Regular feedback of results from screen positive referrals to enable recording of outcome on S4N to support failsafe processes

3. A local process to follow up all non-attendance of appointments after screen positive referral

4. Screen positive babies who are born in hospital and need senior paediatrician referral for heart and bilateral undescended testes should have this completed within 24 hours of the examination or before discharge (whichever is sooner). The outcome needs to be set on S4N.

NIPE newborn examination (within 72 hours of age)

This examination should take place ideally within 72 hours of age.
We recommend this is before transfer home, unless it is a home birth
Delay screening if it is not practical, for example if the baby is too premature or too ill.

Results

• Following the newborn examination, record all screening results and give parents an explanation of any referral process that may be required, including expected appointment timescales.

• Inform parents that the NIPE infant examination will be undertaken at 6 to 8 weeks of age as some conditions can develop or become apparent later.

• Babies less than 32 weeks gestational age (up to 31 weeks and 6 days) or less than 1,500g birthweight should be screened for retinopathy of prematurity (ROP).

Screen positive

Outcomes that should be recorded for screen positive babies are:

1. eye screen positive – seen at ophthalmology appointment by 2 weeks of age

2. heart screen positive – senior paediatrician review (urgency depends on suspected condition), but review is recommended before discharge home

3. hip screen positive (clinical examination) – undergo hip ultrasound by 2 weeks of age
   presence of NIPE hip risk factors – undergo hip ultrasound by 6 weeks of age

4. bilateral undescended testes – consultant paediatrician/ associate specialist assessment within 24 hours of the newborn examination
   unilateral undescended testis – GP informed regarding need for review at 6 to 8-week examination

Examination of the eyes

The prime purpose of screening is to identify congenital cataracts which may require urgent management.

Approximately 2 or 3 in 10,000 babies have congenital cataracts in one or both eyes.
A cataract is an opacity within the lens of the eye, which is located just behind the pupil.

Risk factors for eye or visual problems include:

• a family history of bilateral congenital or hereditary cataracts affecting a first-degree relative

• a first-degree relative with an ocular condition which was congenital or developed in early childhood, for example aniridia (absence of the iris), colobomata (a hole in one of the structures of the eye) or retinoblastoma (a rare malignant tumour of the retina)

• genetic syndromes, such as trisomy 21, associated with eye and vision disorders

• extensive port wine stain involving the eyelids, which can cause glaucoma

• maternal exposure to viruses during pregnancy, including rubella and cytomegalovirus

• neurodevelopmental conditions or sensorineural hearing loss (a type of hearing loss, or deafness, in which the root cause lies in the inner ear)

• prematurity

Although the primary purpose of screening is to identify congenital cataracts, local referral pathways should be followed if major abnormalities of the eyes, clinical risk factors or other incidental eye abnormalities are identified during the examination.

Undertaking the newborn eye examination

Before the examination, practitioners should establish:

• the mother’s recent obstetric history

• the family history of a first-degree relative with an ocular condition which was congenital or developed in early childhood (particularly congenital cataracts)

Assessment of the appearance of the eyes (external examination) should include:

• the ability to fully open the eyelids

• both eyes the same size

• symmetry of eye size and clarity of the cornea

• roundness and symmetry of the pupils

• The red reflex examination
  The red reflex is the normal reflection of white light from the back of the eye which is seen as a red glow in the pupil on ophthalmoscopy.
  The red reflex is viewed through the ophthalmoscope eyepiece.
  The colour, brightness and presence of any shadows on the red reflex should be noted in each eye.
  Caucasian babies have a bright, pinky-red reflex.
  The reflex can be less bright and of yellow/brown hue in non-Caucasian babies.
  If the assessment is difficult, it can be helpful to assess the parents’ red reflexes to determine the expected reflex colour.

Screen positive at newborn examination
Absence of any red reflex suggests congenital cataract
Presence of a white reflex (leukocoria) suggests retinoblastoma
Other abnormal findings: abnormalities of the iris, small or absent eye
Babies require assessment by paediatric ophthalmology service by 2 weeks of age.

6 to 8-week infant eye examination

In addition to the assessment described for the newborn screen, the 6 to 8-week examination includes checking:

1. if the parents have any concerns about the baby’s visual behaviour, for example asking if the baby looks at the them steadily or if the baby has started smiling back at them

2. the ability of the baby to fix on the practitioner’s face steadily, without nystagmus (wobble of the eyes)

3. the ability of the baby to fix and follow a large, bright target by moving their eyes (and not just by moving their head)

4. the alignment of the eyes (bearing in mind that although alignment can be variable at this age, a consistently and significantly deviated eye is not normal)

5. lack of ‘red eye’ on one eye in a photograph of their baby

Screen positive at 6-8w infant check
Dark area within the red reflex is present or if the red reflex is dim or absent (cataract).
Assessment by paediatric ophthalmology should be carried out by 11 weeks of age.

Screen negative at 6-8w infant check
If the NIPE eye examinations are normal, care should be transferred to the Healthy Child Programme with routine vision screening at 4 to 5 years of age.

Examination of the heart

The purpose of screening is early identification of congenital heart problems.

Ranging from non-significant to major and critical lesions, the overall incidence of congenital heart disease (CHD) is about 8 per 1,000 (range 6 to 12 per 1,000 live births).

Critical congenital heart disease (CCHD) accounts for 15% to 25% of these and is a leading cause of morbidity and mortality.

Congenital heart abnormalities can be categorised as:

• critical CHD (CCHD), life-threatening duct-dependent conditions and those conditions that require procedures within the first 28 days of life

• major serious CHD, defects not classified as critical but requiring invasive intervention in the first year of life

Some critical and major cardiac lesions may be detected during pregnancy as part of the fetal anomaly screening programme (FASP) during the fetal anomaly ultrasound scan. The acceptable FASP standard target detection rate for specific cardiac abnormality is ≥50%.

RISK FACTORS

• family history of CHD (first-degree relative)

• fetal trisomy 21 or other trisomy diagnosed (these babies have high risk of cardiac defects and require continued surveillance)

• cardiac abnormality suspected from the 18+0 to 20+6 antenatal scan.

• maternal exposure to viruses, for example, rubella during early pregnancy,

• maternal conditions, such as diabetes (type 1), epilepsy, systemic lupus erythematosis (SLE)

• drug-related teratogens during pregnancy, for example, antiepileptic and psychotrophic drugs (drugs that affect a person’s mental state)

HISTORY

Parents should be asked if their baby:

• ever gets breathless or changes colour at rest or with feeding

• has normal feeding behaviours and energy levels

• is ever too tired to feed, quiet, lethargic, or has poor muscle tone

Before the examination practitioners should establish relevant information regarding:

• mother’s medical and recent obstetric history, including any medication

• baby’s family history

• baby’s immediate postnatal health

EXAMINATION

Observation

• general tone

• central and peripheral colour

• size and shape of chest

• respiratory rate

• symmetry of chest movement, use of diaphragm and abdominal muscles

• signs of respiratory distress (recession/grunting)

Palpation

• femoral and brachial pulses for strength rhythm and volume

• assessment of perfusion through capillary fill time

• position of cardiac apex (to exclude dextrocardia)

• palpation of liver to exclude hepatomegaly (may be present in congestive heart failure)

• vibratory sensation felt on the skin (+/- thrill)

Auscultation

Any cardiac murmur, either systolic or diastolic and its loudness.

It also covers quality of heart sounds at:

• second intercostal spaces adjacent to the sternum: left (pulmonary area)

• second intercostal spaces adjacent to the sternum: right (aortic area)

• lower left sternal border in the 4th intercostal space (tricuspid area)

• apex (mitral area)

• midscapulae (coarctation area)

Signs and symptoms that suggest critical or major congenital heart abnormality

• tachypnoea at rest

• episodes of apnoea lasting >20s or associated with colour change

• intercostal, sub-costal, sternal or supra-sternal recession, nasal flaring

• central cyanosis

• visible pulsations over the precordium, heaves, thrills

• absent or weak femoral pulses

• presence of cardiac murmurs/extra heart sounds

Significant murmurs

• loud

• heard over a wide area

• have a harsh rather than soft quality

• associated with other abnormal findings

Benign murmurs

These are typically short, soft, systolic, and localised to the left sternal border.
They have no added sounds or other clinical abnormalities associated with them.

Many babies will have cardiac murmurs in the first 24 hours of life in the absence of a cardiac defect (linked to physiological changes at birth).

Screen positive at Newborn or 6-8w infant check
Discuss/examine by senior paediatrician
Urgency will depend on circumstances.

Screen negative at 6-8w infant check
If no abnormality suspected, transfer care to the Healthy Child Programme

Examination of the hips

The guidance below relates to both newborn and 6 to 8-week infant examination unless otherwise stated.

Approximately 1 or 2 in 1,000 babies have hip problems that require treatment.

Undetected unstable hips with delayed treatment may result in the need for complex surgery and/or long-term complications such as:

• impaired mobility and pain

• osteoarthritis of the hip and back

Early diagnosis and intervention in infants with developmental dysplasia of the hips will improve health outcomes and reduce the need for surgical intervention.

NIPE hip risk factors—> NIPE hip clinical examination + Hip Ultrasound

1. First degree family history of hip problems in early life

2. Breech presentation at or after 36 completed weeks of pregnancy, irrespective of presentation at birth or mode of delivery (i.e. includes breech babies who have had a successful external cephalic version ECV)

3. Breech presentation at the time of birth between 28 weeks gestation and term

4. Multiple pregnancy where any of the NIPE hip risk factors listed above is present. All babies from that pregnancy should have a hip ultrasound.

Management of CLICKY HIPS
Isolated clicks without any other relevant clinical findings should not be classified as screen positive and do not require referral for ultrasound.
Confirmation of the screening outcome by an experienced clinician should be sought if the examiner is unsure,
After a second opinion and if a screening outcome is still unclear, an ultrasound scan at 6 weeks of age may be considered.
This would be a local clinical referral and not part of the national NIPE screening pathway.

Recording clicky hips
Hip screening is to identify instability. Isolated clicks are not clinically significant and should be recorded as an ‘other’ hip finding on S4N.
Babies with isolated clicks should not be included as part of the NIPE screening programme KPI data NP2.

Clicky hip alone is not a true risk factor for developmental dysplasia of the hip

Undertaking the examination

This is to assess for unstable hips.

ESTABLISH HISTORY

• a mother’s recent obstetric history

• a baby’s family history

• the presence of any NIPE hip risk factors

OBSERVATION

• symmetry of leg length

• level of knees when hips and knees are bilaterally flexed

• restricted abduction of the hip in flexion

Undertake both the Barlow and Ortolani test manoeuvres on each hip separately to assess hip stability

Barlow manoeuvre is used to screen for dislocatable hip—> aims to dislocate hip posteriorly.

Adduct hip to midline, femur vertically pushed downwards aiming to posteriorly dislocate hip.
POSITIVE if femur heads slips out of posterior rim of hip joint (thus confirming the hip is dislocatable and has instability)

Ortolani manoeuvre is used to screen for a dislocated hip—> aims to relocate a posteriorly dislocated hip.

Abduct hip, pressure on greater trochanter to elevate femur upwards
POSITIVE if femur head relocates anteriorly into acetabalum (thus confirming the hip was dislocated)

Actions

1. Screen negative If no abnormality is detected, transfer care to the Healthy Child Programme.

2. Screen negative newborn examination but positive NIPE hip risk factors—> hip ultrasound by 6 weeks of age.

3. Screen positive newborn examination —> hip ultrasound within 2 weeks of age

   Screen positive results are:
   difference in leg length
   knees at different levels when hips and knees are bilaterally flexed (positive Galeazzi sign)
   restricted unilateral limitation of hip abduction (with a difference of 20 degrees or more between hips)
   gross bilateral limitation of hip abduction (loss of 30 degrees abduction or more)
   palpable ‘clunk’ when undertaking the Ortolani manoeuvre
   from 2018, asymmetrical skin creases has been removed from the list of screen positive criteria.
   

4. Screen positive following 6 to 8-week infant examination—> paediatric orthopaedic review and seen by 10w of age

Examination of the testes

The term ‘undescended’ applies for clinical findings of either ‘absence’ and ‘incorrect position’.

Cryptorchidism affects approximately 2% to 6% of male babies born at term.

It is associated with:

• a significant increase in the risk of testicular cancer (primarily seminoma, a germ cell tumour of the testicle)

• reduced fertility when compared with normally descended testes

• other urogenital problems such as hypospadias and testicular torsion

• Bilateral undescended testes in the newborn may be associated with ambiguous genitalia or an underlying endocrine disorder such as congenital adrenal hyperplasia.

Early diagnosis and intervention improves fertility, reduces the risk of torsion and may help earlier identification of testicular cancer.

Clinical risk factors

• a first-degree family history of cryptorchidism (baby’s father or sibling)

• low birth weight

• small size for gestational age or preterm birth

Undertaking the examination

Before the examination, practitioners should review mother’s recent obstetric history and baby’s family history.

Observation Observe scrotum for symmetry, size and colour.

Palpation

1. Carry out palpation of scrotal sac to determine location of testes bilaterally.

2. Undertake palpation of the inguinal canal if testes are not located in the scrotal sac.

3. Where testes are felt bilaterally but high in the inguinal canal, this should be managed as screen positive and recorded on S4N.
   In line with national guidance, these babies should be seen within 24 hours of the examination

Screen negative

If no abnormality is detected, transfer care to the Healthy Child Programme.

Screen positive newborn examination

bilateral undescended testes —> senior paediatrician review within 24 hours (to rule out metabolic and intersex conditions).

unilateral undescended testis —> These babies should be reviewed at 6 to 8-week examination.

Screen positive following 6 to 8-week examination

bilateral undescended testes —> senior paediatrician review within 2 weeks

unilateral undescended testis: GP to review between 4 and 5 months of age; refer to surgeon if testis still absent (to be seen no later than 6 months of age)