how to anesthetize pediatric patient with cleft palate with general anesthesia

1. ANAESTHESIA FOR
CLEFT LIP AND PALATE SURGERY
DR.FAWZI YOUSEF

2. Surgical repair of orofacial clefts (i.e. cleft lip and/or palate)
are commonly performed worldwide in paediatric patients. If
left untreated, the associated facial deformity causes a
multitude of problems ranging from difficulties in feeding,
speech and dental development to psychosocial disturbances.
Hence, a multidisciplinary approach is fundamental in
managing these patients and should comprise of a
combination of specialists (e.g. neonatologists, paediatricians,
surgeons and anaesthesiologists) and allied health care
professionals.

3. Description
Group I (A) Defects of the soft palate only.
Group II (B) Defects involving the hard palate and soft palate extending no further than the incisive
foramen.
Group III (C) Complete unilateral cleft, extending from the soft palate to the alveolus, usually involving the
lip.
Group IV (D) Complete bilateral clefts, resembles Group III but is bilateral. When cleft is bilateral, pre-
maxilla is suspended from the nasal septum.

5. AETIOLOGY
The exact aetiology remains unclear but it is thought to be
multifactorial with a combination of genetic and environmental factors.
Affected parents have a 3-5% chance of producing a child with a cleft
lip and/or palate and if one child is affected then the risk of a sibling
being born with a cleft lip and/or palate is 20- 40%.
Abnormalities can occur during embryological development e.g.
mechanical obstruction as a result of impaired mandibular
development which impedes descent of the tongue and obstructs
fusion of the palatal shelves e.g. Pierre Robin Sequence.
Environmental factors implicated include ethanol use in pregnancy,
infection with the rubella virus, maternal diabetes mellitus, amniotic
band syndrome and an increased paternal age (>40yr).

6. ANAESTHETIC CONSIDERATIONS FOR CLEFT SURGERY
Orofacial cleft surgery is performed worldwide. In developed
countries, specialised teams perform these surgeries at
regional facilities.
But, in developing countries, a lack of appropriately trained
medical personnel and resource constraints may hinder
delivery of a high quality service

7. PRE-OPERATIVE ASSESSMENT
A meticulous pre-operative assessment can decrease the risk of
anaesthesia related morbidity or mortality. The 3 questions that
predominate:
1) Are there other congenital anomalies or syndromes present?
2) Is this going to be a difficult airway?
3) Other considerations

8. The majority of patients present with an isolated cleft lip and/or
palate but approximately 10-20% may have another congenital
abnormality. Defects of branchial arch development (e.g. ear or
upper airway defects), the musculoskeletal and cardiac system are
commonly seen. However, any organ system can be affected
because of the nature of embryological development.

9. The list of syndromes with orofacial clefts as a feature is
exhaustive and many are very rare. As a result, a review of the
literature or an Internet search prior to surgery might be
helpful. Some of the ‘more common syndromes’ are shown in
Table 3 and of particular interest to us are:
1.Pierre Robin Sequence
2.Treacher Collins Syndrome
3.Goldenhar Syndrome
as the difficult airway almost always occurs

10. Syndrome name(s) Features Anaesthesia Implications
Arthrogryposis Multiplex
Congenita
Limb contractures, CHD, stiffness of joints and GU
defects.
Difficult intubation due to limited mouth opening,
position and pad carefully.
Beare-Stevenson Syndrome Craniosynostosis, hydrocephalus, choanal atresia,
midface hypoplasia, proptosis, hypertelorism, cutis
gyratum, tracheal stenosis, and cervical spine defects.
Difficult airway, beware of tracheal stenosis and
caution with neck movements.
Beckwith-Wiedemann
Syndrome
Exomphalos, macroglossia and gigantism. Hypoglycemia. Tongue reduction required
at time of palate repair.
CATCH 22 (Velocardiofacial
Syndrome)
Cardiac defect, abnormal face, thymic hypoplasia, cleft
palate and hypocalcaemia (Di George Syndrome).
Difficult airway. Hypocalcaemia.
Cornelia de Lange Syndrome Growth failure, micromelia, micrognathia, mental
retardation, CHD in 15%.
Airway obstruction, difficult intubation.
Down Syndrome Short stature, mental retardation (variable),
macroglossia, unstable cervical spine, narrow
subglottic space and CHD in 50%
Difficult intubation, airway obstruction and
caution with neck movements.
Goldenhar Syndrome Defect of the 1st and 2nd branchial arches. Hemi facial
and mandibular hypoplasia. Abnormalities of the
cervical spine, external ear and eye.
Difficult airway, laryngeal anomaly, lung
hypoplasia – ventilatory problems, cardiac and
pulmonary complications.
Kabuki Syndrome Craniofacial and skeletal defects, hypotonia, CHD,
visceral and urogenital defects.
Difficult airway.
King Syndrome Congenital myopathy, MH trait. Dysmorphic features. Malignant hyperthermia (MH).

11. Airway Assessment
Paediatric airway assessment might be challenging to perform for
obvious reasons and techniques of airway assessment used in
adults are not always applicable.
As described some syndromes are associated with a difficult
airway but what about the airway of a non-syndromic patient with
an orofacial cleft?
Xue et al, retrospectively evaluated a total of 985 non-syndromic
patients, 1 month to 3 years old, who underwent cleft surgery.

12. Other Considerations
Chronic Rhinorrhoea
This is a common finding due to reflux of food into the nasal
passages resulting in hyper reactive upper airways without
overt infection and needs to be differentiated from an acute
upper respiratory tract infection (URTI) which may require
postponement of surgery

13. Factors implicated in the development of peri-operative
respiratory complications are: infants with bilateral
clefts, wide cleft palates and those with a history of
severe feeding difficulties or symptoms of URTI

14. Chronic airway obstruction/ sleep apnoea
Caregivers may provide collateral history about snoring or
obvious airway obstruction during sleep. These patients may
develop apnoea whilst feeding, have increased feeding times
or poor growth and development. They are very sensitive to
sedatives and opioids and can develop airway obstruction
during induction of anaesthesia and post-operatively.

15. Nutrition
Feeding difficulties are common as the infant cannot create a
seal that is sufficient to suckle. Surgery is often deferred in
severely malnourished or dehydrated infants and the
combination of malnutrition and repeated infections might
exacerbate a physiological anaemia

16. Premedication
The use of a sedative premedication in this group of patients
especially with an obstructed/potentially-obstructed airway is
controversial. Hence, most anaesthesiologists avoid their use.
Whilst every effort should be made to establish rapport with
the patient and caregiver, it is not always feasible especially in
a shrieking, frightened and antagonistic patient (e.g. a
syndromic patient with mental retardation/ behavioural
disorder or a patient presenting for repeat surgeries who has
come to dread the theatre environment and staff

17. In a retrospective study, Zub et al, evaluated patients
who received oral dexmedetomidine as a
premedication before surgery or insertion of an
intravenous (IV) cannula that was used for procedural
sedation (patients with behavioural abnormalities).
Although, it was a small case series, the authors found
that optimal sedation occurred in 85% of patients

18. INTRA-OPERATIVE CONSIDERATIONS
Induction
As always theatre preparation is vital. Generally a volatile
induction allowing the patient to breathe spontaneously is
preferred. Full monitoring (ECG, non invasive blood
pressure, SpO2) is established and intravenous access is
obtained once a suitable depth of anaesthesia is reached
thereafter the ability to mask ventilate the patient needs to
be ascertained.Endotracheal intubation can then be
performed or a muscle relaxant may be added at this stage.
A muscle relaxant should not be administered if the ability
to successfully mask ventilate and hence oxygenate the
patient is in doubt

19. Difficulty with facemask ventilation is not usually
encountered but if unexpected difficulties arise then
anatomical and functional airway problems must be
identified and excluded

20. Causes of unexpected facemask ventilation problems
Exclude and treat anatomical
obstruction
 Re-opening the airway
 Oro ⁄ naso – pharyngeal
airway
 Two-handed technique – jaw
thrust ⁄ open mouth ⁄ chin lift
– facemask ventilation and
two person ventilation
Exclude and treat functional
obstruction
Upper airway
 Inadequate anesthesia
 Laryngospasm
Lower Airway
 Thoracic rigidity
 Bronchospasm
Overinflated stomach (air)

21. A difficult laryngoscopy such as a Cormack and Lehane grade
III/IV is more common and might be further compounded by:
•A large alveolar defect, which can impede laryngoscopy
because the laryngoscope is displaced into the cleft – this can
be prevented by packing the cleft with gauze or using a Miller
blade.
•The vomer or central lip prominence in a patient with a
bilateral cleft lip can hinder midline laryngoscopy - a Miller
blade and a lateral approach may overcome this problem

22. Numerous techniques have been described for
managing difficult airways such as the use of
alternative laryngoscopes, the gum elastic bougie,
paediatric supraglottic airway devices (e.g. as a rescue
airway to maintain oxygenation or as a conduit for a
fibreoptic intubation) etc

23. A difficult airway is a very real possibility therefore, the
difficult airway trolley should be readily accessible for these
cases and familiarity with the available equipment and
knowledge of the algorithms is imperative

26. An oral south facing RAE tube is commonly used, however,
standard or reinforced endotracheal tubes are also
acceptable. Of note, south facing RAE tubes have a preformed
“knee” that might be too long resulting in endobronchial
intubation. Attaching the “knee” lower on the mandible may
overcome this problem.

27. If a Boyle-Davis gag is used for the palate repair, ensure that
the endotracheal tube does not become trapped within the
blades of the gag and kink. Endotracheal tube problems may
arise during any stage of a shared airway and vigilance is
required as the potential for endotracheal tube occlusion,
inadvertent extubation or endobronchial intubation is high.
A throat pack is inserted for cleft lip repair whilst cleft palate
repair does not require one. A head ring and a roll under the
shoulders are frequently placed to extend the neck and the
patient’s eyes must be protected

28. Sevoflurane is commonly used for induction and
maintenance of anaesthesia although there are
concerns about the associated emergence agitation
(EA) or emergence delirium (ED).

29. The use of desflurane for maintenance of anaesthesia is
another interesting prospect as the pharmacokinetics permit
rapid emergence and shorter extubation and recovery times
which may be beneficial in certain patients. But, there is also
concern about the associated emergence agitation (EA) or
emergence delirium (ED) and the cost of this agent

30. Analgesia
A multimodal approach to analgesia is favoured. Opioids such as low
dose fentanyl (1-2ug/kg) for cleft lip repair and a longer acting agent
such as morphine (0.05-0.1mg/kg) for cleft palate repair might be
appropriate.
Remifentanil is also favoured due to its unique pharmacokinetics and
Roulleau et al, compared infants presenting for cleft palate repair who
had received either isoflurane and remifentanil or isoflurane and
sufentanil intra-operatively

31. Lower concentrations of isoflurane and a faster time to extubation
were noted in the remifentanil group and interestingly, there was no
evidence of hyperalgesia or increased morphine consumption in the
first 24 hours post operatively.
Opioids should be used carefully in patients at risk of airway or
respiratory compromise and other alternatives considered

32. POST OPERATIVE CONSIDERATIONS
It is recommended that extubation be performed in the left lateral
position once the patient is awake. This should be preceded by
suctioning the mouth to remove all blood and to ensure that any
surgical packs have been removed. Two major concerns are: acute
airway obstruction and bleeding

33. The surgeon may place a long suture in the anterior tongue which
displaces the tongue forward and away from the posterior pharyngeal
wall to help alleviate airway obstruction
The patient should be carefully monitored post operatively and those
at increased risk should not be discharged to an unmonitored setting.
Multimodal post operative analgesic strategies are very successful and
once bleeding has subsided then oral intake can be initiated, however,
after palate repair, infants may be reluctant to swallow for
approximately 24 hours

34. CONCLUSION
Every 3 minutes, a child somewhere in the world is born with
an orofacial cleft. If left untreated they will face multiple
complex health problems. Whilst these patients may present
a challenge, the associated benefits of surgical correction are
life changing and for those suffering from orofacial clefts
“dignity begins with a smile”.