Early care services for babies born with cleft lip and/or palate

This Technical Report specifies recommendations for the care of babies born with cleft lip and/or cleft palate at time of diagnosis (ante- and/or postnatal) and the year following birth or diagnosis (whichever is later), including referral processes, establishment of feeding, parental support and care pathways.
Recommendations on all aspects of surgery, including timing and the use of pre surgical orthopaedics is excluded.

Fürsorgedienstleistungen für Babies mit Lippen-, Kiefer- und Gaumenspalten

Services de prise en charge précoce des bébés nés avec une fente labiale et/ou palatine

Zgodnja nega otrok s prirojenim razcepom ustnice, čeljusti in/ali neba

To tehnično poročilo določa priporočila za nego otrok s prirojenim razcepom ustnice in/ali neba v času diagnoze (pred rojstvom in/ali po njem) in v letu po rojstvu ali diagnozi (kar je pozneje), vključno z napotitvenim postopkom, vzpostavitvijo hranjenja, starševske podpore in načinov nege.
Ne zajema priporočil o vseh vidikih operacij, vključno s časom operacije in uporabo predoperativne ortopedije.

General Information

Status
Published
Public Enquiry End Date
04-Feb-2015
Publication Date
19-Apr-2015
Technical Committee
Current Stage
6060 - National Implementation/Publication (Adopted Project)
Start Date
19-Mar-2015
Due Date
24-May-2015
Completion Date
20-Apr-2015

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SLOVENSKI STANDARD
SIST-TP CEN/TR 16824:2015
01-maj-2015
=JRGQMDQHJDRWURNVSULURMHQLPUD]FHSRPXVWQLFHþHOMXVWLLQDOLQHED
Early care services for babies born with cleft lip and/or palate
Fürsorgedienstleistungen für Babies mit Lippen-, Kiefer- und Gaumenspalten

Services de prise en charge précoce des bébés nés avec une fente labiale et/ou palatine

Ta slovenski standard je istoveten z: CEN/TR 16824:2015
ICS:
11.020.10 Zdravstvene storitve na Health care services in
splošno general
SIST-TP CEN/TR 16824:2015 en

2003-01.Slovenski inštitut za standardizacijo. Razmnoževanje celote ali delov tega standarda ni dovoljeno.

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SIST-TP CEN/TR 16824:2015
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SIST-TP CEN/TR 16824:2015
TECHNICAL REPORT
CEN/TR 16824
RAPPORT TECHNIQUE
TECHNISCHER BERICHT
March 2015
ICS 11.020
English Version
Early care services for babies born with cleft lip and/or palate

Services de prise en charge précoce des bébés nés avec Fürsorgedienstleistungen für Babies mit Lippen-, Kiefer-

une fente labiale et/ou palatine und Gaumenspalten

This Technical Report was approved by CEN on 7 March 2015. It has been drawn up by the Technical Committee CEN/TC 424.

CEN members are the national standards bodies of Austria, Belgium, Bulgaria, Croatia, Cyprus, Czech Republic, Denmark, Estonia,

Finland, Former Yugoslav Republic of Macedonia, France, Germany, Greece, Hungary, Iceland, Ireland, Italy, Latvia, Lithuania,

Luxembourg, Malta, Netherlands, Norway, Poland, Portugal, Romania, Slovakia, Slovenia, Spain, Sweden, Switzerland, Turkey and United

Kingdom.
EUROPEAN COMMITTEE FOR STANDARDIZATION
COMITÉ EUROPÉEN DE NORMALISATION
EUROPÄISCHES KOMITEE FÜR NORMUNG
CEN-CENELEC Management Centre: Avenue Marnix 17, B-1000 Brussels

© 2015 CEN All rights of exploitation in any form and by any means reserved Ref. No. CEN/TR 16824:2015 E

worldwide for CEN national Members.
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Contents Page

Foreword ..............................................................................................................................................................4

Introduction .........................................................................................................................................................5

1 Scope ......................................................................................................................................................6

2 Terms and definitions ...........................................................................................................................6

3 Diagnosis and referrals .........................................................................................................................7

3.1 Antenatal diagnosis ...............................................................................................................................7

3.1.1 Recommendations on making the diagnosis .....................................................................................7

3.1.2 Limitation of ultrasound ........................................................................................................................7

3.1.3 Suspected cleft lip and/or palate ..........................................................................................................7

3.1.4 Recommendations on referral to the cleft team .................................................................................8

3.2 Postnatal diagnosis ...............................................................................................................................8

3.2.1 Recommendations on making the diagnosis .....................................................................................8

3.2.2 Recommendations on referrals ............................................................................................................9

4 Immediate Postnatal Care .....................................................................................................................9

4.1 Clinical assessment ..............................................................................................................................9

4.1.1 General ....................................................................................................................................................9

4.1.2 Pierre Robin Sequence – managing airway obstruction ................................................................ 10

4.2 Early parental involvement ................................................................................................................ 10

5 Feeding ................................................................................................................................................ 10

5.1 Knowledge and skills of person providing feeding advice ............................................................ 10

5.2 Clinical assessment ........................................................................................................................... 10

5.3 Feeding plan ........................................................................................................................................ 11

5.4 Growth Measures ................................................................................................................................ 11

6 Monitoring the baby and preparation for surgery ........................................................................... 12

6.1 Monitoring ........................................................................................................................................... 12

6.2 Preparation for Surgery ..................................................................................................................... 12

7 Recommendations for involving and supporting parents ............................................................. 12

7.1 Involving parents ................................................................................................................................ 12

7.2 Parent to parent support .................................................................................................................... 13

8 The longer term care pathway ........................................................................................................... 13

8.1 Scope of a care pathway .................................................................................................................... 13

8.2 Long term care pathway .................................................................................................................... 14

9 Recommendations for the cleft unit team members and facility requirements ........................... 16

9.1 Team members .................................................................................................................................... 16

9.2 Education and training ....................................................................................................................... 16

9.3 Team management and responsibilities .......................................................................................... 16

9.3.1 Communication and coordination .................................................................................................... 16

9.3.2 Referral and communication with other professionals .................................................................. 16

9.3.3 Research .............................................................................................................................................. 16

10 Recommendations for organization of the cleft service, including clinical governance and

audit ..................................................................................................................................................... 16

10.1 Service requirement ........................................................................................................................... 16

10.2 Facilities for parents and children .................................................................................................... 17

10.2.1 Out-patient medical and dental care ................................................................................................. 17

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10.2.2 In-patient care ...................................................................................................................................... 17

10.3 Clinical governance ............................................................................................................................. 17

10.4 High quality standards ........................................................................................................................ 17

10.5 Audit, outcome measures and comparative studies ....................................................................... 17

10.5.1 Monitoring short-term and long-term treatment outcomes ............................................................ 17

10.5.2 National data sets — National Registers and Databases ................................................................ 18

10.5.3 Future developments .......................................................................................................................... 18

11 Information and education needs ...................................................................................................... 18

11.1 Families/Caregivers ............................................................................................................................. 18

11.1.1 General ................................................................................................................................................. 18

11.1.2 At time of diagnosis (pre and post natal) .......................................................................................... 18

11.1.3 Preparation for surgery ....................................................................................................................... 19

11.1.4 Longer term care pathway .................................................................................................................. 19

11.2 Health professionals ........................................................................................................................... 19

11.3 Social services and education providers .......................................................................................... 20

11.4 Governments, healthcare service providers and the general public ............................................. 20

12 Information production ....................................................................................................................... 20

Annex A (informative) Types of cleft lip and/or palate .................................................................................. 21

A.1 General ................................................................................................................................................. 21

A.2 Incomplete cleft lip .............................................................................................................................. 21

A.3 Cleft of the soft palate ......................................................................................................................... 21

A.4 Complete cleft lip ................................................................................................................................. 22

A.5 Cleft of the soft and hard palate ........................................................................................................ 22

A.6 Unilateral cleft lip and palate (alveolus involved) ............................................................................ 22

A.7 Bilateral cleft lip and palate (alveolus involved) .............................................................................. 22

Annex B (informative) Pierre Robin Sequence and treatment options (Subclause 4.1.2) ......................... 23

B.1 Pierre Robin Sequence ....................................................................................................................... 23

B.2 Suggested treatment options ............................................................................................................. 23

Annex C (informative) Bottles and teats used for assisted feeding of babies with cleft........................... 25

C.1 Example of a squeezable bottle ......................................................................................................... 25

C.2 Example of squeezable teat................................................................................................................ 26

C.3 Example of a sipper spout .................................................................................................................. 27

Annex D (informative) Description of the role of a nurse specializing in cleft care ................................... 28

Annex E (informative) Sample form used for feeding assessment of babies with cleft ............................ 29

Annex F (informative) Recommendations on treatment records ................................................................. 33

F.1 General ................................................................................................................................................. 33

F.2 Minimum treatment records ............................................................................................................... 33

F.3 Timing of minimum records ............................................................................................................... 34

Bibliography ...................................................................................................................................................... 36

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Foreword

This document (CEN/TR 16824:2015) has been prepared by Technical Committee CEN/TC 424 “Project

Committee - Care services for cleft lip and/or palate”, the secretariat of which is held by ASI.

Attention is drawn to the possibility that some of the elements of this document may be the subject of patent

rights. CEN [and/or CENELEC] shall not be held responsible for identifying any or all such patent rights.

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Introduction

In Europe around 1 in 700 babies is born with cleft lip and/or palate, the most common congenital anomaly of

the head and neck region. The incidence is approximately 1,6 per 1 000 live births, but there is some variance

across Europe [1]. Estimates indicate there are over 900 000 individuals (babies, children and adults) with

clefts in Europe [2] - a significant figure, especially when one considers that not only the patients but also their

families are affected in terms of psychosocial adjustment and having to endure the burden of a long treatment

pathway.

In round figures the incidence by type of cleft may be summarized as follows [3]:

Table 1 — Incidence of Type of Cleft
Type of Cleft Percent of Total
Cleft palate only 50 %
Cleft lip (±alveolus) only 20 %
Cleft lip and palate 20 %
Bilateral cleft lip and palate 10 %

In some cases the cleft may be associated with other problems which need specialist management and these

need to be identified early [4]. Accurate diagnosis (antenatal or post natal), the provision of appropriate

information and support for the family, and the establishment of a structured care pathway, especially in the

early months, will ensure that these infants thrive and develop like all other children. Access to good treatment

varies widely throughout Europe, meaning that many children born with clefts are never given the opportunity

to realize their full potential. The concept of a comprehensive specialist-team approach to care is not

universal. Furthermore babies with clefts are still institutionalized in some countries in Europe [5].

The aim of this report is to provide an informative document which can be used by those countries where

national protocols need to be established.
1) For further information on different types of cleft see Annex A.
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1 Scope

This Technical Report specifies recommendations for the care of babies born with cleft lip and/or cleft palate

at time of diagnosis (ante- and/or postnatal) and the year following birth or diagnosis (whichever is later),

including referral processes, establishment of feeding, parental support and care pathways.

Recommendations on all aspects of surgery, including timing and the use of pre surgical orthopaedics is

excluded.
2 Terms and definitions
For the purpose of this document, the following terms and definitions apply.
2.1
assisted feeding

use of a soft, squeezable, bottle and/or adjusted teat and/or sipper spout to allow delivery of milk to the infant

who is unable to generate suction to extract fluid independently

Note 1 to entry: It enables the infant to feed, effectively and safely, the required volume within an acceptable time

frame.

Note 2 to entry: For further information on types of bottles and teats used for assisted feeding of babies born with

clefts see Annex C.
2.2
cleft centre
hospital with a designated cleft team and paediatric facilities
2.3
cleft surgeon

surgeon trained in cleft surgery with a major commitment to cleft care and who practices cleft surgery on a

regular and frequent basis
2.4
cleft team

multidisciplinary team which comprises the following members with proven competence in their field of

expertise, paediatric experience and a major commitment to cleft care: a care coordinator/manager of the

service; a surgeon trained in primary cleft surgery; a surgeon specializing in secondary cleft surgery such as

bone grafting and orthognathic surgery; an orthodontist; a speech and language therapist; a nurse specializing

in cleft care; a psychologist with recognized clinical training; an audiologist; an ENT surgeon; a geneticist; a

restorative dentist; a paediatric dentist; a dental technician

Note 1 to entry: While not all specialities will be required for every patient, access to all these practitioners is available

when needed.

Note 2 to entry: If patients receive some aspects of care nearer home (e.g. orthodontics, speech and language

therapy) they receive care by trained specialists working in collaboration with the cleft team.

2.5
Eurocleft

Eurocleft Project 1996 – 2000 funded by the European Commission having the aim to improve management

and understanding of cleft lip and palate and create a network of European researchers and clinicians to

facilitate information exchange
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2.6
cleft support organization

non-medical group with paid staff and/or volunteers offering advice and support to families affected by cleft

2.7
nurse specializing in cleft care

specialist responsible for planning and coordination of early cleft care, including feeding assessment,

development of a feeding plan, providing support to the family and liaising with other health care professionals

Note 1 to entry: In the absence of a nurse, these services are provided by another trained professional with similar

skills.

Note 2 to entry: An illustrative role of a nurse specializing in cleft care is provided in Annex C.

2.8
palatal plate
presurgical orthopaedics

intraoral appliance which can be used in the treatment of infants with cleft palate

Note 1 to entry: It can help with feeding and can be used to improve physiological tongue position and to influence

maxillary growth in preparation for surgery.

Note 2 to entry: In newborns with Pierre-Robin-Sequence a plate with velar extension can be helpful to treat upper

airway obstruction and avoid invasive surgical procedures, e.g. tracheostomy.
2.9
Pierre Robin Sequence

sequence of symptoms present in a newborn; micrognathia (small lower jaw), cleft palate, and glossoptosis

(posteriorly placed tongue) that combine to cause airway obstruction

Note 1 to entry: The back of the tongue falls back, particularly when the baby is supine, and occludes the airway.

3 Diagnosis and referrals
3.1 Antenatal diagnosis
3.1.1 Recommendations on making the diagnosis

The foetal face can be studied with ultrasound very early in gestation. Examination of the foetal face should be

a component of guidelines for second trimester ultrasound examination [6].
3.1.2 Limitation of ultrasound

Although the correct ultrasound examination technique should enable clefting of the upper lip to be identified,

it is difficult to detect an isolated cleft palate antenatally [7]. What is often described as a ‘cleft palate’ on

ultrasound may be just a cleft alveolus. Training of the professional undertaking the ultrasound diagnosis

should include information on basic embryology of clefting and how treatment of clefts is managed.

3.1.3 Suspected cleft lip and/or palate

If a cleft has been suspected, there should be a referral to a specialist in ultrasound diagnostics to confirm the

diagnosis. As a cleft lip and/or palate may be associated with other anomalies, early assessment and

diagnosis is necessary.
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Clear information about cleft lip and palate should be given to families if a cleft is suspected. Genetic

counselling for patients and families should be available on request. The main topic for discussion is the

association with other anomalies and the recurrence risk for following pregnancy.

If the cleft is part of a complex syndrome, specific information about it and about the foetus viability should be

given to parents during the meeting.

In the absence of other anomalies, a foetus with a cleft does not require a change in standard obstetric care.

3.1.4 Recommendations on referral to the cleft team
The following recommendations should be followed:

— a referral from the antenatal diagnostics unit to a multidisciplinary cleft team and to a cleft support

organization (where it exists) should be made as early as possible after diagnosis; the referring unit

should ensure it maintains up to date contact information on the local or regional cleft team(s);

— parents should be contacted by a member of the cleft team as soon as possible after receiving the

referral; and

— parents should be offered a face to face meeting with a member of the cleft team within one month of the

confirmed diagnosis.

The aim of the referral to the cleft team is to provide parents with support and counselling, giving clear

information about treatment and successful management of cleft lip and palate. Families do not generally

expect the diagnosis or know anything about cleft lip and palate and they have a lot of questions and concerns

about bringing up a child with a cleft. Some families may be thinking about terminating the pregnancy.

Psychosocial support for parents who are expecting a child with a cleft should be available. Parents may need

help in normalizing the pregnancy and understanding that the cleft does not define the baby. The following

topics may need addressing:
— grief adjustment process;
— possible feeling of guilt;
— impact on the family;
— how to handle reactions from others; and
— whether or not it will be possible to breast feed.

Parents should be given contact details for the cleft team and be informed that the team is able to provide

additional information at any stage during the pregnancy. The information needs of families after a diagnosis

of a cleft are provided in Clause 11.
3.2 Postnatal diagnosis
3.2.1 Recommendations on making the diagnosis

Diagnosis of a cleft palate is often missed. Proper assessment of the palate is of paramount importance:

— a palpation of the palate is not sufficient on its own. Routine assessment of all newborn babies should

include a visual inspection of the palate with a torch and spatula to depress the tongue;

— every newborn baby with a cleft should be seen by an experienced paediatrician as soon as possible.

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3.2.2 Recommendations on referrals

Each maternity unit should designate a staff member to liaise with the cleft team to ensure that all staff are

familiar with the contact arrangements and maintain an up to date knowledge and understanding of current

practice in providing care for children with a cleft lip/palate.
Neonatal intensive care should be used only where specific needs justify it.

The maternity unit should provide basic information on cleft care such as general explanation of the condition,

the reasons for treatment, treatment options, location of the cleft team, and details of support groups. This

may be supplemented by leaflets, booklets or other kinds of information – websites, posters, and phone

numbers.

Involvement of the cleft team around the birth will be necessary whether or not co morbidities are present,

such as Pierre Robin Sequence (see Annex B).

All babies should be referred by relevant professionals to the cleft team as soon as possible after diagnosis. A

cleft team member (a nurse specializing in cleft care where they exist) should visit at the earliest opportunity

after receiving the referral. The team member will visit the mother at the maternity unit to observe the child

during a feed, offer advice and supply any feeding equipment considered necessary.

4 Immediate Postnatal Care
4.1 Clinical assessment
4.1.1 General

It is important that a baby with a cleft lip/palate has a full clinical assessment by a neonatologist or

paediatrician to rule out any possible comorbidities or associated syndromes. As a guide, the likelihood of co-

morbidities according to cleft type are shown in Table 2:
Table 2 — Co-morbidities associated with cleft type [8]
Cleft type Association with co-morbidities (%)
cleft palate only 42,3
cleft lip and palate 23,5
cleft lip only 7,6
Cleft types are described in Annex A.

Most commonly these are airway obstructions (Pierre Robin Sequence) or cardiac abnormalities

If co-morbidities are present there needs to be a consideration as to whether the baby should be referred to

neonatal intensive care. A referral to a genetics team at this stage may also be considered. The

appropriateness of early feeding should be assessed and further investigations should be undertaken if

required.

All children with cleft palate only should be assessed for airway obstruction and possible Pierre Robin

Sequence prior to commencing oral feeding (see 4.1.2).

In the presence of a congenital heart anomaly consider 22q11 deletion syndrome, the second commonest

syndrome after Pierre Robin Sequence associated with cleft palate only.
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If no co-morbidities are found, the child can be treated as a well baby.
4.1.2 Pierre Robin Sequence – managing airway obstruction

The airway obstruction in these babies can be managed in a variety of ways including positioning (e.g. nursing

the baby in a side lying position), use of intra oral plates and insertion of a nasopharyngeal airway – see

Annex B). Tracheostomy and jaw distraction or other invasive surgical procedures in the absence of additional

anomalies should be avoided. Pierre Robin Sequence may be associated with Sticklers Syndrome so it is

recommended that these babies have a genetic, ENT and ophthalmological assessment to exclude this.

4.2 Early parental involvement

The post natal period provides a window for parent/child bonding. This important aspect of early care should

not be overlooked in the prioritizing of clinical care and support for a child born with a cleft. The following

recommendations should be respected:

— the child should be kept with the mother on the postnatal ward unless there are co-morbidities

necessitating a neonatal intensive care environment;

— both parents should be involved in the care of the child and they should be kept informed of their child’s

diagnosis and treatment; and

— parents may need additional support to adjust to their child’s diagnosis and help with the bonding

process.
5 Feeding
5.1 Knowledge and skills of person providing feeding advice
These should include the following items:
— paediatric skills;
— counselling skills; and
— knowledge of normal and abnormal feeding mechanisms.
5.2 Clinical assessment

Feeding should only be commenced after a full clinical assessment has been performed (see 4.1).

Premature babies may not manage oral or assisted feeding and may require nasogastric feeding initially. Oral

feeding from the breast or a normal bottle can be commenced if there are no co-morbidities.

A feeding assessment should be undertaken by a health care professional with special knowledge of feeding

mechanisms and skills within cleft before commencing assisted feeding. Children without co-morbidities

...

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