Guidance

Newborn and infant physical examination (NIPE) screening programme handbook

Updated 28 April 2021

This handbook informs and supports best clinical practice and should be used in conjunction with the NIPE programme standards and the NIPE screening pathways. It relates to the screening elements of the top-to-toe physical examination of the baby.

1. NIPE screening examination

The NHS newborn and infant physical examination (NIPE) programme’s main aims are to:

• identify and refer all children born with congenital abnormalities of the eyes, heart, hips and (in males) testes, where these are detectable, within 72 hours of birth
• to further identify those abnormalities that may become detectable by 6 to 8 weeks of age, at the infant physical examination
• reduce morbidity and mortality

These ages are recommended based on best practice and current evidence and should facilitate a prompt referral for early clinical assessment.

The NIPE programme screens babies (ideally) within 72 hours of birth (NIPE newborn screening examination) and again at 6 to 8 weeks of age (NIPE infant examination) for conditions relating to the:

• eyes
• heart
• hips
• testes (if applicable)

All references to the NIPE examination in this document relate to the 4 screening elements above. References to parent(s) also relate to carer(s), if appropriate.

1.1 NIPE infant screening examination

The infant examination should be performed at 6 to 8 weeks of age for all 4 conditions (as some conditions can develop or become apparent after the newborn screen). This is usually undertaken in a primary care setting.

There are no NIPE national standards for the infant screening examination, as there is no way of systematically measuring them. This document includes good practice guidance and recommended referral timescales for the infant examination until there is a reliable method of recording, measuring and reporting on this part of the NIPE programme.

Please note that the infant examination is not a formally managed part of the national programme and local commissioners will provide scrutiny (as required) to oversee this part of the examination. It is recommended that all screening results are recorded on the GP IT system and in the personal child health record (PCHR), the ‘red book’.

1.2 NIPE screening pathways

For further information please refer to the NIPE newborn and infant screening pathways.

2. Information for parents

It is important that all parents receive Public Health England’s (PHE’s) screening tests for you and your baby information about antenatal and newborn screening. PHE has more guidance on how to provide information to the parents effectively, including a range of resources to support health professionals which can be requested from the PHE screening helpdesk.

This information should be given:

• during the antenatal period
• before offering the NIPE newborn and infant screening examination

Screening tests for you and your baby: babies in special care units has more information for parents of babies in:

• neonatal units
• neonatal intensive care units
• paediatric intensive care units

Healthcare professionals should inform parents of findings at each examination and advise them to report any concerns about their baby’s wellbeing at any time.

2.1 NIPE examination declined

When the NIPE examination is declined:

• parent(s) should be fully informed of the NIPE newborn and infant screening examinations and why screening is being offered and undertaken
• consent should be obtained and recorded

Parent(s) have the right to decline all or part of the NIPE newborn or infant examination.

If they decline all or part of the NIPE newborn screening examination, this should be documented on the NIPE national IT system (SMaRT4NIPE (S4N)), communicated to the GP and health visitor and recorded in the PCHR. The parent(s) should be reminded that their baby will remain eligible for NIPE newborn screening until 6 weeks of age and will be offered NIPE infant screening at 6 to 8 weeks of age. Contact details should be provided for the parent(s) from the discharging service, in case they change their mind.

If they decline all or part of the NIPE infant screening examination, the reason should be documented in the GP IT system and in the PCHR. The examination can be undertaken later. See section 7: Babies who have missed screening below for clinical information regarding screening older children.

3. Screening tests

3.1 NIPE newborn screening examination

This examination should ideally take place before 72 hours of age. For babies born in hospital, it is recommended that the examination is completed before transfer home. This will maximise the opportunity for completing the examination within 72 hours of age.

There must be a local follow-up pathway to ensure screening takes place, ideally within 72 hours of age or as soon after this as possible.

Local arrangements should be in place to meet national timescales when babies are:

• born at home
• transferred home before NIPE newborn screening is completed

This could be by attending a designated NIPE clinic or arrangements being made for the examination to take place at home or in the primary care setting. It is the responsibility of the care provider to ensure the NIPE newborn screening is completed.

Local arrangements should also be in place to meet national timescales when babies are transferred from one acute provider to another before NIPE newborn screening is completed or there are screen positive results requiring follow-up.

Babies in neonatal units

If a baby is receiving care in a neonatal unit, this is not an automatic reason to delay screening. NIPE newborn screening should be undertaken as soon as the baby is well enough and, or has reached 34 weeks plus 0 days (34⁺⁰) weeks corrected age (see below).

‘Too young’ for NIPE newborn screening

If a baby is born before 34⁺⁰ weeks gestation, screening can be delayed to 34⁺⁰ weeks corrected age. The baby continues to remain eligible for NIPE newborn screening and the examination should be performed as soon as the baby is deemed old enough and is well enough. The screening of babies before 34⁺⁰ weeks corrected age is a local clinical decision. The result should be recorded on S4N.

Please note that the terminology used has changed from ‘too preterm’ to ‘too young’ for NIPE newborn screening in national standards and guidance.

‘Too ill’ for NIPE newborn screening

Screening may be delayed if a baby is too ill for screening. These babies should be accounted for in key performance indicator (KPI) data, giving the reason for delay as a mitigation against the screening by the 72 hours of age performance threshold.

Possible clinical reasons for this delay include:

• respiratory support (other than low flow oxygen), including the presence of chest drains for the first 72 hours
• any cardiovascular support, for example, inotropes, prostin
• ventilated infant until extubated
• baby on continuous positive airway pressure (CPAP)
• therapeutic hypothermia
• intense phototherapy (double or more, need for immunoglobulin or exchange transfusion)
• chest drain in place (without additional respiratory support)
• umbilical lines and, or arterial lines in place
• post-operative, until off analgesia
• unstable hypoglycaemia until off intravenous dextrose
• where active reorientation of care to comfort or palliative care is taking place

Trusts can use the above reasons to mitigate against performance thresholds for KPI NP1.

This is not an exhaustive list and clinical judgement should also be used when assessing suitability for newborn NIPE screening.

Screening results – NIPE newborn screening examination

All screening and outcome results should be recorded on S4N and in the baby’s PCHR.

Screen negative

Following the NIPE newborn screening examination, the parent(s) should be informed of the results. For babies who have transferred into the area or between care providers with a screen negative result, no further action is required.

The parent(s) should be informed that the NIPE infant screening examination will be undertaken in the primary care setting at 6 to 8 weeks of age (as some conditions can develop or become apparent later).

Screen positive

The parent(s) should be informed of the results and of any referral process that may be required, including expected appointment timescales.

If a baby was not brought for any follow-up appointment, further appointments should be made in line with the trust did not attend (DNA) or was not brought (WNB) policy.

If the baby is in a treatment pathway for one of the screening elements, the NIPE infant 6 to 8-week screening will still need to be completed for the remaining screening elements.

Where there is a screen positive result and NIPE newborn screening examination has been undertaken after the target timescale, a specialist appointment should be arranged without delay.

3.2 NIPE infant 6 to 8-week screening examination

Information about the NIPE infant screening examination should be given to the parent(s) during the postnatal period and again before the NIPE infant screening examination is offered and undertaken. This examination typically takes place in a primary care setting.

There should be timely checks to ensure that:

• all eligible babies are offered screening (including those who move into the area)
• the examination takes place between 6 and 8 weeks of age
• any required referrals are made within the nationally recommended timescales
• referrals are followed up so that appropriate interventions take place in line with national guidance

If the baby is screen positive for any element of the NIPE newborn screening examination, the practitioner undertaking the NIPE infant screening examination should check the progress along the care pathway to ensure required actions have taken place.

Screening results – NIPE infant screening examination

All screening and outcome results should be recorded on the GP IT system and in the baby’s PCHR.

Screen negative

Following the NIPE infant screening examination the parent(s) should be informed of the results. For babies who have transferred into the area or between care providers with a screen negative result, no further action is required.

The parent(s) should be advised that the baby will follow the Healthy Child Programme.

Screen positive

The parent(s) should be informed of the results and of any referral process that may be required, including expected appointment timescales.

Where there is a screen positive result and NIPE infant screening examination has been undertaken after the target timescale, a specialist appointment should be arranged without delay.

4. Record keeping

Providers should obtain verbal consent for screening from parent(s), which should be documented, and a record should be made of the screening result and any referrals following screen positive result(s). The above information should be recorded in:

• S4N (NIPE newborn screening examination) or GP IT system (NIPE infant screening examination)
• baby’s clinical notes
• baby’s PCHR
• local clinical data collection system (where appropriate)

There should be:

• local arrangements to ensure all babies with screen positive results are referred and seen in line with national standards
• regular feedback of attendance at appointment or specialist review after screen positive referrals to enable recording of outcomes in S4N
• a local process to follow up all non-attendance of appointments after screen positive referral
• a process to record all screening results and outcomes (including post referral outcomes on S4N)

Access to S4N can be given to ultrasound practitioners, orthopaedic services or ophthalmology services to support timely input of post referral outcomes for the NIPE hip and eye screening pathways. This can be discussed and agreed locally with the NIPE lead.

5. Babies who move in and out of area

5.1 Babies who move into the area

Babies who move into the area (move into the country or county) and are unscreened are the responsibility of the current care provider.

5.2 Transfer of unscreened baby to another care provider

If a baby is transferred to another care provider before the NIPE newborn screening has been undertaken or completed, the transferring care provider should formally hand over responsibility and communicate this to the receiving care provider both verbally and using S4N.

The responsibility for screening eligible babies remains with the birth unit until responsibility is formally passed and accepted by another provider, whether acute or primary care. Ideally this should be managed using S4N. Newborn screening results should be recorded on S4N if the NIPE newborn screening examination is undertaken by the maternity service.

After transfer, the receiving care provider should undertake the NIPE examination. This may be a maternity, neonatal or paediatric service. Screening results for these babies should be recorded on S4N wherever possible.

5.3 Babies with screen positive results who transfer between care providers

The transferring provider is responsible for communication with the receiving provider to ensure completion of any outstanding elements of the screening pathway.

If there is an agreement, the record is formally transferred on S4N. The receiving provider is then responsible for follow-up and recording of post referral outcomes. If the birth unit chooses to keep the responsibility for follow-up appointment (due to local decision) they should ensure outcomes are known, recorded and communicated to the responsible care provider.

If there is not a prior agreement and formal transfer of care, the assurance of the completion of the screening pathway and recording of the outcome remains the responsibility of the birth unit.

6. Communication pathways following bereavements

There should be local systems to identify deceased babies and inform other relevant screening and clinical services without delay. If a baby dies before NIPE newborn screening has been completed, this should be marked on the baby’s S4N record.

7. Babies who have missed screening

The following clinical guidance outlines the appropriate screening tests depending on age for babies found to have missed NIPE screening. If the missed screen is due to a screening pathway failure, then actions should be taken in line with national screening incident guidance.

7.1 Classification of missed NIPE newborn screening examination

If the NIPE newborn screening examination is not completed by 72 hours of age, it should be done as soon as possible. The reasons for the delay beyond 72 hours of age should be recorded on S4N and used locally to collate mitigations against NP1 coverage performance target. Babies remain eligible for NIPE newborn screening examination until 6 weeks of age. Reasons for delayed screening should be locally audited and investigated if appropriate.

All babies should have all elements of the NIPE newborn screening examination before being fully discharged from the care of any maternity, neonatal or paediatric inpatient services.

A NIPE newborn screening examination is classified as ‘missed’ if it is not completed:

• before full discharge from maternity, neonatal or paediatric inpatient services (without provision for completion within the community setting)
• by 6 weeks of age for unscreened babies who remain in maternity or neonatal inpatient care
• by 6 weeks of age for unscreened babies who have moved into an area

At 6 weeks of age, babies become eligible for the NIPE infant screening examination.

7.2 Infants who have missed NIPE screening

Up to and including 3 months of age

NIPE newborn screening examinations not undertaken by 72 hours of age should be completed as soon as possible after this time by the current care provider (see the NIPE newborn screening examination section above for reasons screening can be delayed).

Babies remain eligible for NIPE newborn screening examination until 6 weeks of age, but the examination should be completed as close to the 72-hour target as possible. This includes unscreened babies who ‘move in’ to an area or between care providers.

Eligible babies who move into the area who have not been screened should be offered screening by the responsible service provider. This may be by the maternity, neonatal or paediatric services or in primary care depending on the age and condition of the baby. For these babies, screening results will, in practice, only be recorded on S4N if the NIPE newborn screening examination is undertaken by the maternity or neonatal service.

If NIPE newborn screening is performed at or after 6 weeks of age, it is not necessary to do the NIPE infant screening examination.

Older than 3 months of age

If the NIPE infant screening examination is overdue (after 6 to 8 weeks of age), it should be done as soon as possible.

The screening examination for unstable hips using the Barlow and Ortolani manoeuvres is no longer accurate at this age. Any asymmetry of leg length or restricted hip abduction should be assessed, and the child’s gait observed, if walking.

In line with advice in the PCHR, the parent(s) should be advised to contact their GP or health visitor if they have any concerns regarding their child’s wellbeing.

7.3 Completing a missed NIPE examination

The relevant examination or observation must be undertaken by a NIPE qualified practitioner.

8. Screening examination of the eyes

Eye development is complex, and a wide range of structural abnormalities may occur if the process is disturbed either due to genetic, infective or sporadic causes.

The primary purpose of the NIPE newborn and infant eye screening examination is to identify congenital cataract(s). Some abnormalities, particularly congenital cataract, are treatable but require early detection, rapid referral and management to prevent lifelong visual impairment.

8.1 Incidence

Around 2 to 3 in every 10,000 babies (200 per year in England) are born with cataracts. In over 50% of these, both eyes will be affected. One fifth of affected babies will have a family history of cataracts. Cataract is the most common treatable cause of blindness in childhood in the UK and worldwide.

A congenital cataract is an opacity within the lens of the eye, which is located behind the pupil. It can occur in one or both eyes and range in severity. A severe cataract blocks light and images from reaching the retina, preventing visual pathway development at a critical stage. Although rare, it is the most common, treatable, visually significant condition identified during NIPE eye screening.

The screening practitioner should be alert to risk factors, such as genetic syndrome, pre-natal infection, sensorineural hearing loss, neurodevelopmental issues or a family history of an eye condition with onset in infancy or early childhood.

Babies with other eye abnormalities, incidental findings or risk factors identified at the NIPE newborn or infant screening examination should be referred for specialist review according to locally agreed pathways.

Please note the guidance below relates to both the NIPE newborn and infant eye screening examinations unless otherwise stated.

8.2 Before the NIPE eye screening examinations

Before the examinations, practitioners should:

• discuss the screening test and gain consent from the parents to undertake the examination
• establish the mother’s recent obstetric history
• establish if there are any clinical risks for eye disorders (see below)

Risk factors for congenital cataracts

Babies with a family history of bilateral congenital or hereditary cataracts in a first-degree relative are at risk of developing early cataracts. Even if the NIPE screening examination is normal, these babies may be considered for referral for early specialist opinion, via locally agreed pathways.

Risk factors for other eye or visual problems

Risk factors include:

• a first-degree relative with an ocular condition which was congenital or developed in early childhood, for example aniridia (absent iris), coloboma (malformation of the eye) or retinoblastoma (malignant retinal tumour) childhood
• prematurity
• 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 (deafness caused by abnormal nerve function in the inner ear)

Although practitioners should be aware of these risk factors, they do not alter the NIPE national eye screening pathway. Babies identified with such risk factors should be managed via locally agreed pathways.

8.3 The eye examination

NIPE newborn eye screening examination

The primary aim of the NIPE eye screening examination is the detection of congenital cataracts. In addition, although not part of the screening programme, it is considered good practice to include assessment of the:

• eyelids, to exclude malformation and skin abnormality, such as extensive port wine stain involving the eyelids, which can cause glaucoma and affect the baby’s ability to fully open the eyelids
• presence of both eyes
• symmetry of eye size
• symmetry and clarity of the cornea (the cornea is the circular transparent window of the eye through which the iris and pupil can be seen – its diameter in a term baby should be similar to the width of the practitioner’s little finger-tip, the roundness and symmetry of the pupils)

NIPE infant eye screening examination

In addition to the assessment described for the newborn eye screening examination, the 6 to 8-week infant eye screening examination should also include checking:

• if the parents have any concerns about the baby’s visual behaviour, for example asking if the baby looks at them steadily and has started smiling back at them
• the ability of the baby to fixate the practitioner’s face steadily, without nystagmus (wobble of the eyes)
• the ability of the baby to fix and follow a large, bright target by moving their eyes (not just by moving their head)
• the alignment of the eyes, which can be variable at this age but a consistently and significantly deviated eye is not normal

The red reflex examination

The red reflex should be assessed. This 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 and is like the red-eye effect seen on flash photography.

To undertake the examination, the practitioner should:

• dim the overhead lights and make sure that the baby is settled
• hold the eyepiece of the ophthalmoscope up to his or her eye, at arm’s length from the baby’s face
• direct the circle of light from the ophthalmoscope towards the baby’s eye while gently parting the baby’s eyelids, if necessary
• view the red reflex through the ophthalmoscope eyepiece – the colour, brightness and presence of any shadows on the red reflex is noted in each eye

The examination can be repeated by an experienced practitioner if the examination is equivocal. This should ideally be within the 72-hour guideline period of the NIPE newborn examination.

The normal red reflex varies in hue depending on the baby’s ethnicity. White babies have an orange-red reflex. The reflex can be less bright and appear magnolia in colour in black, Asian or minority ethnic babies. If the assessment is difficult, it can help to assess the baby’s parents’ red reflexes to determine the expected reflex colour.

Examples of normal red reflex by ethnicity of baby (from left to right: black, Asian, white)

Congenital cataracts

Congenital cataracts cause a central shadow, completely obscure the red reflex or may make the reflex in one eye appear duller than the other. A severe cataract can make the pupil appear white when viewed with the naked eye.

The red reflex is abnormal if it is completely or partially obscured, is abnormal in shape (iris coloboma or aniridia), white or asymmetrical in colour or brightness to the other eye.

Examples of partially (left) and completely (right) obscured red reflexes

An example of a white ‘red reflex’

8.4 Screen negative (normal red reflex assessment)

Babies with screen negative eye results for the NIPE newborn screening examination should have the NIPE infant screening examination at 6 to 8 weeks of age. Infants with screen negative eye results for the NIPE infant screening examination should follow the Healthy Child Programme.

Babies with screen negative eye results but with risk factors described above may be referred via locally agreed pathways.

8.5 Screen positive (abnormal red-reflex assessment)

Babies with screen positive eye results following the NIPE newborn screening examination should be urgently referred via the NIPE pathway to an ophthalmologist within 2 weeks of the screening examination. However, if there are significant concerns at the time of the NIPE newborn screening examination, discussion with the ophthalmology service before the baby’s discharge home should be considered.

Surgery for severe cataract is usually undertaken between 6 to 10 weeks of age for optimal visual outcome.

Babies with screen positive eye results following infant screening examination should be referred promptly and seen by a consultant ophthalmologist or paediatric ophthalmology service by 11 weeks of age.

8.6 Parental concerns

Parents should be advised to contact their midwife, GP or health visitor whenever they have any concerns about their baby’s eyes or visual behaviour, including:

• inability of their baby to fully open their eyes or if eyelid opening appears asymmetrical
• apparent deterioration of visual interest
• a wobbling of the eyes
• a consistent eye misalignment
• an abnormal appearance of the eyes
• a white reflex, consistently seen on flash photography
• asymmetry of the red reflex, consistently seen on flash photography

Note that a family history of an eye or vision condition developing later in life does not require a neonatal referral to ophthalmic services.

Other eye abnormalities should be managed according to local referral pathways.

9. Screening examination of the heart

The purpose of screening is early identification of congenital heart problems. Congenital heart disease (CHD) is a term used to describe a problem with the heart’s structure and function that is present at birth.

Note that the NIPE heart screening pathway will be reviewed in the tax year 2021 to 2022.

9.1 Incidence

The overall incidence of CHD ranging from non-significant to major and critical lesions, 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:

• CCHD, which includes all potentially life-threatening duct-dependent conditions and those conditions that require procedures within the first 28 days of life
• major serious CHD, which includes 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 NHS fetal anomaly screening programme (FASP), during the 20-week ultrasound scan. The acceptable FASP standard target detection rate for specific cardiac abnormality is at least 50%.

9.2 Risk factors for CHD

Risk factors for CHD include:

• 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 antenatal scan
• maternal exposure to viruses, for example, rubella during early pregnancy
• maternal conditions, such as diabetes (type 1), epilepsy, systemic lupus erythematosis (SLE)
• teratogenic drugs taken during pregnancy

Although practitioners should be aware of these risk factors, they do not alter the NIPE national heart screening pathway.

9.3 Undertaking the heart examination

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

Parents should be asked if their baby:

• ever gets breathless or changes colour at rest or while feeding
• is not feeding well or ever too tired to feed, quiet, lethargic, or has poor muscle tone

9.4 Observation

Observation includes reviewing the baby’s:

• 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 or grunting)

9.5 Palpation

Palpation involves examination of the baby’s:

• 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)

9.6 Auscultation

Auscultation includes identification of a murmur, either systolic or diastolic or loudness. It also includes the assessment of the 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 fourth intercostal space (tricuspid area)
• apex (mitral area)
• midscapulae (coarctation area)

9.7 Screen negative results

Babies with screen negative heart results following the NIPE newborn screening examination should have the NIPE infant screening examination at 6 to 8 weeks of age.

Infants with screen negative heart results following the NIPE infant screening examination should follow the Healthy Child Programme. Parents should be advised to contact their healthcare professional or emergency services if they have any concerns about their baby.

9.8 Screen positive results

Babies with screen positive heart results following the NIPE newborn screening examination should be seen by a senior paediatrician with expertise in cardiology in the early neonatal period as required. The urgency will depend on suspected condition. Please note that confirmation of assessment within 24 hours of the examination will constitute an outcome in S4N.

If a suspected major or critical heart condition is found on the NIPE newborn screening examination, the baby should be seen as a matter of urgency and definitely before discharge home.

Babies with screen positive heart results following the NIPE infant screening examination should follow the locally agreed referral process. Urgency will depend on the clinical condition of the baby.

Signs and symptoms that suggest critical or major congenital heart abnormality

These include:

• tachypnoea at rest
• episodes of apnoea lasting longer than 20 seconds 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 or extra heart sounds

Significant murmurs

These are:

• usually loud
• usually heard over a wide area
• usually with a harsh rather than soft quality
• possibly 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.

The examining practitioner should discuss findings with a senior paediatrician or a paediatrician with expertise in cardiology and refer as appropriate. Urgency will depend on the assessment of the clinical condition of the baby.

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). However, cardiac murmurs may be absent in babies with a significant cardiac defect.

9.9 Heart screening standards

Although there are no NIPE heart screening standards, it is recommended that the national clinical pathway above is followed. Practitioners should be familiar with, understand and follow any existing local policies, guidelines and referral pathways.

10. Screening examination of the hips

The guidance below relates to both NIPE newborn and infant screening examinations unless otherwise stated. Note that the hip screening standards and pathways changed in April 2021. National hip risk factors and suspected abnormality on clinical hip examination all now follow the same pathway.

The primary purposes of screening are:

• early identification of a dislocated or a dislocatable hip(s)
• the identification of sonographic pathological hip dysplasia through selective ultrasound scan (USS)
• minimising the risk of long-term complications through:
  • timely hip ultrasound scan
  • further expert assessment
  • early intervention

10.1 Incidence

The incidence of babies requiring treatment is:

• 3 to 5 per 1,000 live births that may require a Pavlik Harness
• 1 to 2 per 1,000 live births that may require surgery

Undetected unstable hip(s) 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 should improve health outcomes and reduce the need for surgical intervention.

10.2 NIPE hip risk factors

NIPE national hip risk factors include:

• first-degree family history of hip problems in early life – this includes baby’s parents or siblings who have had a hip problem that started as a baby or young child that needed treatment with a splint, harness or operation
• breech presentation at or after 36 completed weeks of pregnancy, irrespective of presentation at birth or mode of delivery – this includes babies who have had a successful external cephalic version (ECV)
• breech presentation at the time of birth between 28 weeks gestation and term

10.3 Multiple pregnancy

All babies from a multiple pregnancy where any of the NIPE hip risk factors listed above are present should have a hip ultrasound. This is because if one of the babies meets the criteria of breech presentation described above during pregnancy, it may be difficult to accurately identify which baby was affected.

10.4 Undertaking the hip screening examination

Before the examination, practitioners should establish:

• a mother’s recent obstetric history
• a baby’s family history
• the presence of any NIPE hip risk factors

The examination should take place in a warm environment and on a firm, flat surface with the baby undressed and settled.

10.5 Observation and manipulation

Observation covers:

• symmetry of leg length
• level of knees when hips and knees are bilaterally flexed
• restricted abduction of the hip in flexion

Please note that observation of skin creases for symmetry is no longer part of the NIPE screen. This is no longer regarded as a screen positive finding.

You should undertake both the Barlow and Ortolani test manoeuvres on each hip separately to assess hip stability. The Barlow manoeuvre is used to screen for a dislocatable hip (a dislocatable hip will be displayed out of the joint by this manoeuvre). The Ortolani manoeuvre is used to screen for a dislocated hip (a dislocated hip will be felt clunking into place).

10.6 Screen negative

Babies with screen negative hip results on NIPE newborn clinical examination and no national hip risk factors present should have the NIPE infant screening examination at 6 to 8 weeks.

Infants with screen negative hip results on NIPE infant examination (and no national hip risk factors – unless they have not been previously referred), should follow the Healthy Child Programme.

10.7 Parental concern

Parents should be advised to contact their healthcare professional at any time if they have any concerns about their baby’s hip(s). They should observe if:

• one leg cannot be moved out sideways as far as the other when changing the baby’s nappy
• one leg seems to be longer than the other
• one leg drags when their baby starts crawling
• their child walks with a limp or has a ‘waddling’ gait when they start walking

10.8 Screen positive

A screen positive hip result is an abnormal clinical hip examination (with or without risk factors) and, or presence of NIPE hip risk factors.

A suspected abnormality on clinical examination is defined by:

• difference in leg length
• knees at different levels when hips and knees are bilaterally flexed
• 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 or Barlow manoeuvre

Babies with screen positive hip results following NIPE newborn screening examination should be managed as outlined below.

10.9 Referral and outcome decision

The standards S03 (NP2) and S04 are effective from 1 April 2021 and replace all previous versions.

Standard 3 (NIPE S03 – NP3)

For babies with screen positive hip results, NIPE standard 3 requires the hip USS to be undertaken within the target timescale.

For babies who are born at <34⁺⁰ weeks gestation, hip USS should be undertaken between 38⁺⁰ and 40⁺⁰ weeks corrected age.

Referral to orthopaedic services after hip USS will then depend on the scan result and local management policy in place.

Please see the Good practice guidance section below for further guidance.

Standard 4 (NIPE S04)

For babies who attend for ultrasound scan of the hips after screen positive newborn hip referral, standard 4 requires an outcome decision to be made within the target timescale. This is to ensure that babies who require treatment enter the treatment pathway by 6 weeks of age.

For babies who are born ≥34⁺⁰ gestation, an outcome decision should be made by 6 weeks of age.

For babies who are born <34⁺⁰ weeks gestation, an outcome decision should be made by 40⁺⁰ weeks corrected age.

An outcome decision is either:

• discharge from the hip screening pathway after review of normal hip USS results
• attendance for clinical assessment by orthopaedic specialist (it is required that hip USS has been undertaken in advance of the decision)

Infants with screen positive results following NIPE infant 6 to 8-week screening examination should be referred directly to paediatric orthopaedic surgeon for urgent expert opinion and be seen by 10 weeks of age.

10.10 Good practice guidance and additional information

A ‘one stop shop’ model can be provided, in which ultrasound and review by orthopaedic specialist or specialist practitioner is undertaken on the same day, during the same care episode. In order to meet the national standards associated with the pathway, this joint care episode should be between 4 and 6 weeks of age (or between 38⁺⁰ and 40⁺⁰ weeks corrected age for babies born <34⁺⁰ weeks gestation).

If hip USS and orthopaedic specialist assessment are undertaken separately, the scan will need to be routinely done soon after 4 weeks of age (or equivalent corrected age) to give time for orthopaedic appointment to be arranged, outcome decision to be made and, if required, treatment to be started by 6 weeks of age (or equivalent corrected age).

In line with national standard S04 and after local agreement, babies can be discharged by ultrasound services after normal hip scan. However, please note that some areas may wish review of all hip scan results by an orthopaedic specialist.

The term ‘orthopaedic specialist’ (standard S04) encompasses anyone with delegated responsibilities to initiate treatment decisions based on local policy or practice arrangements. These may include roles such as specialist paediatric physiotherapists, specialist orthopaedic nurse specialists and experienced radiographers.

If a hip rescan is required for technical reasons, it should, wherever possible, take place before 6 weeks of age (or equivalent corrected age). This will ensure that the final outcome decision is known and, if necessary, treatment can start within the optimum timescales (within 6 weeks of age or equivalent corrected age).

If a hip rescan is required for findings of immature hips (Graf 2a), it is acknowledged that this will be required at a timescale exceeding 6 weeks of age (or 40⁺⁰ weeks corrected age).

In both instances, the need for rescan should be recorded on S4N with the specific reason added in the comments box. Comments should include the keywords ‘technical’ or ‘immature’.

The national programme will be reviewing the hip screening pathway. It is vital that S4N data is accurate and complete to enable full analysis when the hip screening pathway is reviewed. A performance threshold will be set for standard 4 (outcome decision) in due course. A hip rescan undertaken after 6 weeks of age (or equivalent corrected age) will not count as an outcome decision as the screening pathway is not complete and an outcome decision is still required.

A local agreement should be in place to ensure that the results of the hip ultrasound scan and orthopaedic specialist or specialist practitioner appointment are recorded on S4N. This can be supported by enabling ultrasound and orthopaedic services access to S4N so that outcomes can be added.

10.11 Confirmation of breech presentation

It is important that NIPE practitioners recognise and act on identification of national NIPE risk factors. In response to queries regarding what constitutes breech presentation (abdominal palpation and, or ultrasound scan) and to make sure national NIPE hip risk factors are accurately defined, please see nationally agreed recommendations below.

Note that it may not always be feasible to arrange for a same day USS.

If the practitioner is sure of the findings on abdominal palpation (at or after 36 weeks gestation) and has recorded breech presentation in the woman’s records, this would count as a national NIPE hip risk factor.

If the practitioner is unsure of the findings on abdominal palpation (at or after 36 weeks gestation) and has recorded them as ‘? breech’ in the woman’s records, this initial finding of ‘? breech’ would not count as a national NIPE hip risk factor. Ultrasound at the earliest opportunity or senior practitioner assessment would be required.

In both these scenarios, if the woman gives birth before confirmation ultrasound and the presentation at birth is cephalic, the NIPE examiner should seek local senior practitioner advice to assess whether the risk factor would apply (this would be a local decision).

10.12 Management of ‘clicky hips’

Hip screening aims to identify hip instability. The significance of isolated ‘clicks’ is debated. On balance and after review of evidence, isolated clicks are not considered clinically significant by the NHS NIPE screening programme.

Isolated clicks without any other relevant clinical findings should therefore not be classified as screen positive and do not require referral for ultrasound.

If the examiner is unsure of their findings, confirmation of the screening outcome should be sought by an experienced clinician. After a second opinion, and if the screening outcome is still unclear, an ultrasound scan may be considered. This would be a local clinical referral and not part of the national NIPE hip screening pathway and should be recorded as an ‘other’ hip finding on S4N.

Local audits may be carried out to assess clinical competency and feedback to practitioners about ‘clicky hip’ findings.

Relevant studies related to ‘clicky hips’ include Kamath and others, 2005, Nie and others, 2017 and Marson and others.

Please contact the PHE screening helpdesk if further references regarding hip pathway changes are required.

11. Screening examination of the testes

The primary purpose of screening is to identify bilateral undescended testes or unilateral undescended testis.

Note that the NIPE testes screening pathway will be reviewed in the tax year 2021 to 2022.

11.1 Incidence

Undescended testes affect approximately 2 to 6% of male babies born at term. One in 100 of these babies will have testes that stay undescended unless treated.

Undescended testes are associated with:

• an increased 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 later testicular torsion

Bilateral undescended testes in the newborn may be associated with ambiguous genitalia or an underlying endocrine disorder. Congenital adrenal hyperplasia must be excluded in a baby with bilateral impalpable gonads.

Early diagnosis and treatment at an early age can reduce the risk of fertility problems and reduce the risk of testicular cancer.

11.2 Clinical risk factors

Clinical risk factors include:

• a first-degree family history of undescended testes (baby’s father or sibling)
• low birth weight
• small size for gestational age or preterm birth

Although practitioners should be aware of these risk factors, they do not alter the NIPE national testes screening pathway.

11.3 Undertaking the examination

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

11.4 Observation

Observe the scrotum for symmetry, size and colour.

11.5 Palpation

Carry out palpation of scrotal sac to determine location of testes bilaterally. Undertake palpation of the inguinal canal and perineum if testes are not located in the scrotal sac. Where testes are felt bilaterally but high in the inguinal canal, this should be managed as screen positive.

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

11.6 Screen negative

Babies with screen negative results following the NIPE newborn screening examination should have the NIPE infant screening examination at 6 to 8 weeks of age.

Infants with screen negative results following the NIPE infant screening examination should then follow the Healthy Child Programme. Parents should be advised to contact their healthcare professional or emergency services if they have any concerns about their baby.

11.7 Screen positive

A screen positive result is the absence or incorrect position of one or both testes.

Babies with screen positive findings of bilateral undescended testes following NIPE newborn examination should be seen for assessment by a senior paediatrician within 24 hours of the examination to rule out metabolic and disorders of sex development conditions.

Babies with screen positive findings of unilateral undescended testis following NIPE newborn examination should be reviewed by the GP during the NIPE infant examination at 6 to 8 weeks of age. Please note that confirmation of referral to GP will constitute an outcome in S4N.

Infants with screen positive findings of bilateral undescended testes following NIPE infant screening 6 to 8-week examination should be referred and seen by a senior paediatrician within 2 weeks of the examination.

Infants with screen positive findings of persistent unilateral undescended testis following NIPE infant screening 6 to 8-week examination should be reviewed by a GP between 4 and 5 months of age, then be referred to a surgeon if testis still absent or not descended (to be seen no later than 6 months of age).

12. Training and maintenance of competency

All healthcare professionals have a personal and professional responsibility to maintain their clinical competency. There is also an organisational responsibility to ensure a safe and competent workforce.

Those who undertake NIPE examinations must work in a framework of professional accountability and code of conduct. Each practitioner is responsible for maintaining their own competence to carry out the examination to the highest standard and to identify gaps in their knowledge and any training needs.

To provide duty of care, an appropriately trained health professional must carry out NIPE examinations.

12.1 NIPE newborn screening examination

This must be completed by a trained practitioner who is competent to undertake all elements of the newborn screening examination and who has undergone relevant training and a locally agreed competency assessment by a practising NIPE examiner. This can be a midwife, nurse or health visitor who has successfully completed a university accredited ‘examination of the newborn’ programme of study or a doctor.

Any newly qualified midwife who has undertaken the module as part of their pre-registration midwifery training must have completed both the theoretical and clinical components of the module before they can be deemed competent to undertake the examination. When employing newly qualified midwives (who are trained in examination of the newborn), providers need to be diligent to ensure all required elements of the course have been successfully completed as pre-registration course content varies across the country.

Nursing associates

The national programme considers that nursing associates would not be eligible to undertake examination of the newborn training or the newborn examination (as agreed by the NIPE Education and Training working group January 2020).

12.2 NIPE infant (6 to 8-week) screening examination

Doctors

Doctors can undertake all elements of the 6 to 8-week NIPE examination. However, not all trainee GPs undertake formal paediatric training, so it is important that NIPE is covered in their GP attachment (ST3 year). Practitioners need to take responsibility for competence and continuing professional development (CPD).

It is suggested that any GPs who have not undertaken the 6 to 8-week exam for some time or for whom it is a new activity should firstly refresh by working through the NIPE e-learning module. In addition, a number of universities undertake one-off updates and clinical study days. These could be accessed by contacting the NIPE course leader at local university for details.

If the GP or practice feels it necessary, they may wish to consider the GP working with a colleague who is confident and competent to undertake NIPE practitioner in the practice to offer some mentorship or observed practice.

Physician’s associates

The ability to undertake the NIPE newborn or infant screening examination will depend on the training and qualification achieved. Physicians associates who require training should contact the relevant university provider to discuss enrolment for the examination of the newborn course.

Health visitors or nurses

The examination can also be carried out by a health visitor or nurse with suitable competency. To be considered competent, a health visitor or nurse is expected to:

• successfully complete a university-accredited examination of the newborn course, as elements of this would also be relevant for the infant examination
• undergo a locally agreed competency assessment by a practising NIPE examiner

Currently there are no courses that specifically focus on training for the 6 to 8-week NIPE infant examination. Current national guidance is that health visitors or nurses should undertake an examination of the newborn training, as some elements of the course are transferrable and relevant to undertake the infant screening examination.

12.3 Maintenance of competency

A local competency assessment process should be in place. The focus should not be on a minimum number of examinations, but on the quality of the examination performed. Local providers may choose to determine and set minimum numbers of examinations, but this is not a programme requirement.

A local mandatory assessment of clinical competencies for all healthcare professionals who conduct the newborn and infant screening examinations should be undertaken. A local annual update on NIPE screening pathways and local referral processes should be provided for all those who undertake the NIPE examination (including midwives, nurses, doctors and other NIPE practitioners). This may also include practical and theoretical assessment.

The NIPE e-learning module should be completed annually. Registration and a password are required to access the resource on the e-Learning for Healthcare platform.

12.4 Non-university accredited training courses

If practitioners undertook a ‘short unaccredited examination of the newborn course’ (prior to 2016) then in line with national guidance at the time, practitioners need not undertake another course. In such cases, trust(s) would need to ensure they have a process to assess the practitioner competence and CPD and manage any risk associated with this approach (NIPE Education and Training working group January 2020).

Trusts should seek university accreditation or a formal university quality assurance process for any in-house NIPE practitioner training courses. In order to ensure appropriate expertise of assessors, any assessment should be undertaken by a university which provides an accredited examination of the newborn practitioner course. A quality assurance process should assess course content in line with required competencies, documentation (including teaching materials and lesson plans) and assessment processes. An annual review should include evaluation strategies, including feedback from students and inform future course planning.

Trusts are responsible for managing any risk associated with the approach where they enable practitioners to undertake the NIPE screening elements of the newborn examination by:

• offering, commissioning or accessing a locally delivered examination of:
  • the newborn practitioner training course
  • an unaccredited course

Note that short courses or in-house training may not be recognised by all providers, so this may pose a problem if practitioners were to transfer from one trust to another.

13. Quality assurance

The NIPE screening programme has a defined set of standards that providers must meet to ensure local services are safe and effective. Quality assurance (QA) is the process of checking these standards are met and encouraging continuous improvement.

It includes:

• advising on the development of national quality standards
• monitoring how services meet (or fail to meet) standards
• providing expert screening advice for incident management
• facilitating quality review of services, including peer advice in supporting those involved in commissioning or providing screening services

Formal QA visits provide the forum for a peer review of the whole multidisciplinary screening pathway.

Screening quality assurance service (SQAS) teams make recommendations to providers and commissioners about what is needed to make sure the service meets the national service specifications and standards. They monitor and provide expert advice on the management of screening incidents and promote shared learning and examples of good practice at national, regional and local levels.

Participation in a formal process of QA is the responsibility of each local screening provider. The performance of local services is monitored in a variety of ways including the national data reports, at local trust meetings and within screening programme boards.

13.1 Key performance indicators (KPIs)

KPIs provide a way of measuring screening programme performance in specific areas. They contribute to the overall quality assurance of screening programmes but are not sufficient to fully quality assure, or performance manage, screening services. KPIs help local screening services identify potential or actual problems so remedial actions can be taken.

The 2 KPIs for the NIPE screening programme are NP1 and NP3 (S03).

13.2 Coverage data and failsafe

Coverage performance data helps ensure safe and inclusive local delivery of the NIPE programme. This data is reported using S4N. Accurate and timely use of S4N supports complete cohort identification, failsafe and reporting.

For babies screened by non-NHS services (independent or private), there is no obligation to complete S4N. However, it is good practice to record that screening has taken place by a private provider if this information is known.

13.3 NIPE KPI process guidance

Reporting focus NHS Trust KPI data for NIPE newborn screening is taken directly from S4N. Before data submission, providers will have the opportunity to review, sense check and provide mitigation information before data is submitted.

The NIPE programme produces quarterly reports that are available on S4N.

Trusts should regularly review S4N failsafe reports and follow up babies identified as ‘not screened’ or who have not attended for post screen positive referral appointments. These reports should also be reviewed and discussed at local screening programme boards.

13.4 Screening safety incidents

Health professionals should use the managing incidents guidance if there are any problems during screening.

13.5 NIPE standards

NIPE screening programme standards provide a defined set of measures that providers must meet to ensure local programmes are safe and effective.

The NIPE national standards are available.

Trusts should make sure there are local systems for entering all relevant coverage and outcome data on to S4N including date of post screen positive referral appointment or review.

Trusts should make sure all NIPE screening and outcome data is inputted onto S4N to:

• assure failsafe
• reflect completion of the whole screening pathway
• reflect programme performance

Where NIPE newborn screening examinations have taken place in primary care, local feedback mechanisms should be developed and agreed so screening results can be entered on S4N in a timely fashion.

Summary S4N data reports are produced quarterly and sent to screening and immunisation teams and SQAS to provide a useful overview and quarter by quarter comparison. PHE Screening publishes KPI data reports quarterly and shares an extended KPI data set with NHS England.

We gratefully acknowledge professionals involved (including members of the NIPE expert working groups) for their contribution to the content of this handbook. If you would like further information, please contact the PHE screening helpdesk.