INTRODUCTION- Cleft of lip and palate is
the most common congenital defects which affect the orofacial regions. A cleft
is a congenital space or gap created in the upper lips, alveolus, nasal floor
or palate. In most cases children, who have a cleft
lip with a cleft palate or without it usually seen in 1 in 600 populations. A
cleft palate alone considered as a separate entity, which means its occurrence
in 1 out of 2000 live births [1]. Therefore, current estimates of
around 35,000 children had cleft lip and palate congenital disorder every year
in India [2]. Failure to fuse or space is created while fusion of
nasal and maxillary processes within the palatine shelves arises during 8th
week of the embryonic development may create a cleft of varying extent through
the nasal, alveolus and upper portion of lips, it may be complete or
incomplete. Majority of 275 congenital
distributions [3] had left lip (CL)
and cleft palate (CP) syndrome. That's why multiple
co-morbidities coexist in these patients. Anaesthetists are mainly concerned
with cardiac and airway abnormalities.
Syndrome
patients with mandibular hypoplasia make airway management difficult and 5-10%
may present with congenital heart disease [1]. Those Infants have orofacial deformities are generally concerning
with number of additional abnormalities such as dentition/hearing defect,
recurrent ear/upper respiratory tract infection (URTI), as well as pulmonary
aspiration with malnutrition problems. Patients with orofacial abnormalities
need special attention and adopted recent criteria to repair cleft associated
problems especially in infants who had 10 pounds of weight, 10 weeks of age and haemoglobin of 10g% [4]. This recent concept of
early cleft repair in newly born children is based on the number of
specialities factors such as improvements in parent-infant relation, feeding
behaviour, infant-growth and speech/ language development [4],
oral/ maxillofacial surgery, genetics and other factors.
A neonatal surgery is avoided in children with CLP syndrome unless it is for non-cleft operations. Most congenital
abnormalities can be detected if we wait for 3 months which allows anatomical
and physiological maturation5. This cleft deformity needs to be
examined as earliest as possible or early age to achieve functional and normal
well-being. But CLP defects treatment is usually needed a medical
expertise team involving a team of experts of surgeons and physicians, speech/
language experts and Ear Nose Throat (ENT). Because of the treatment process is
quite complex and intensive care is needed during surgery of patients with a CLP and it is performed in between the 1-2 years age of children [6].
As we already discussed the children with CLP had the
additional disease of the respiratory tract with microcytic anaemia or
associated with a high risk of malnutrition, all these events are just because
of difficulties facing during the feeding [7,8] of breast milk due
to abnormalities developed in upper lip portion. The reason behind
this event is due to the creation of negative intra-oral pressure while feeding
of milk and is predisposed to regurgitation and aspiration due to the palatal defect [8]. Delayed
wound healing is usually observed in the postoperative period because of malnutrition
and anaemic status while respiratory-tract infection.
It may predispose off these patients to develop the laryngospasm, bronchospasm
and hypoxia conditions during the intake of general endotracheal anaesthesia.
This situation arises because of the hyperactivity of the airway [9]
passage. Therefore, it is crucial to treat such patients medically before
underwent to surgery or intake of anaesthesia doses before surgery, to avoid
blockage of the respiratory tract. Care of a cleft patient requires lots
of attention against serious factors. In the early stages of the disease, they need
parents attention, then feeding and then with the surgery and finally speech
therapy and possibly orthodontics. The pre and post-operative care of these
patients are crucial to their survival and the success of the surgery. Surgery
for a cleft lip/palate is elective as a baby with a cleft lip/palate can live
to old age without surgery so it is imperative to learn to take care of these
patients and guide them through the perioperative course safely. This review
aims to enumerate the preoperative and postoperative concerns and management of
a CLP child.
Pre-operative
Preparation- Surgical
operation to treat the cleft lip defects within the 3-6months and within the 12
to 18 months to repair the cleft palate. The surgical procedure depends on the
surgeon's personal preferences and techniques. Actual aim to reduce the cleft
width size, align the cleft lip and alveolar segments in an ordered way and
correct the cleft-specific nasal airways [10].
Several enlisted
goals of pre-operative nursing care:
1.
Safe preparation
of the child for surgery
2.
Making
the child stress free
3.
Counselling
parents regarding surgery and post-operative care
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Fig.
1: Diagrammatic
way of Representation of Pre-operative nursing care
In
addition to the standard preoperative history requires medical examination with
coordinated attention is needed to access the complex congenital abnormalities:
Associated congenital abnormalities- CLP combined linked with 150 known syndromes, therefore; a careful clinical
examination should be needed to access the deformity level. The three main
characteristics with cleft palate, micrognathia blocked an
upper-respiratory-tract causing airways and feeding is seriously blocked which
constitute the Pierre-Robin Syndrome. Other associated facial anomalies are included:
Goldenhar and Treacher-Collins Syndrome [11].
An array of preoperative problems in cleft lip and
Palate children is displayed here:
Upper Respiratory Tract Infection (URTI)-
CLP patients have chronic rhinorrhea with URTI due
to difficulties in food reflux into the nasal passages. Incidence of
postoperative respiratory complications (PRC) is reduced with the
administration of preoperative antibiotics. Significantly higher rates of PRC
(9%) are seen in infants with bilateral CLP as compared to unilateral CL or in CLP with a 2 to3% PRC rate
respectively, even with no clinical sign of scoring pre-operatively which indicate
the no clinical sign of infection [12].
Chronic
Airway-Passage Obstruction- Snoring, apnoea like signs appeared during milk feeding or protracted
feeding time clearly may indicate a chronic airway-passage obstruction. Increased risk of airway obstruction due to
oversensitivity to sedatives may be observed in the peri-operative period. Older children and adults may have chronic hypoxia, right
ventricular hypertrophy and corpulmonale like symptoms arise which leads to
congenital heart disease in 5-10% patients. Other recommended techniques like ECG
and ECHO should be considered if cardiac problems were suspected in patients [13].
Nutrition
and Hydration- An optimal current nutritional status of children
can be solved by accurate measurement of supportive feeding. Height and weight
measurement can be done accurately to appropriate dosages of drugs and fluid,
being administered to CLP patients. A child with cleft lip and palate (CLP) defects can have difficulty to suck breast milk. Usually, this feeding
difficulty is commonly observed in these children and it should be recommended
surgery because of deferred malnourished or in dehydrated children. Nutritional
or physiological anaemia mayoccur at 9-weeks interval that's why haemoglobin
(Hb) measurement should be checked frequently. Ideally, all studied patients
have haemoglobin (>10g/dl) concentration. Clear fluids intake can be prescribed
in 2-hours preoperatively and exclusively in breast-feeding infants until
4-hours preoperatively [14].
Pre-medication therapy- Sedatives may be prescribed to precipitate airway passage obstruction,
otherwise, it should be avoided. Atropine is an effective anticholinergic and
recommended drug whenever any difficulties is in the upper respiratory tract or
dosing of anaesthesia in combination with ketamine (20 µg/kg) is recommended
either through an intramuscularly for 30 minutes pre-operatively or through an
intravenously dosing (10-20 µg/kg) at induction phase of administration of
drugs [15].
Anticipated difficult intubation- A difficulties in airways passage to be
operated are especially observed in those patients who have <6 months aged
with either having retrognathia (receding lower jaw) like problems or with
bilateral clefts. In addition to this, about known allergies can also be
described in this report. Pre-operative fasting must be prescribed by
physicians for 2 hours to clear up liquids in all the age groups, while at
least 4hours for breast milk and 6hrs for solids feed intake. The timing and
nature of last oral intake should be noted. And all these problematic issues
must be identified first and then treated. Also, check for any chronic ailment or genetic problems. Ensuring that
all documentation and checklists are complete before the child leaves the ward.
Because the children and their parents are very apprehensive, psychological
care of younger children focuses on limiting painful procedures and anxiety
caused by separation from carers [16].
If the
hospital policies can allow children with their parents, the presence of the
parent in the anaesthetic room or operation theatre during induction of
anaesthesia in children may help reduce their distress situations. It may be
appropriate to introduce them to another family whose child is recovering from
the same surgery. This will help carers understand the changes that they can
expect to see in the early postoperative period.
Post-operative Care- Under the postoperative situation,
patients were under close observation to avoid blood losses or airway passage
obstruction and promote to easy access of recovery from this critical
situation, and only those one returned to the ward in a fully awake condition.
Each patient should be given sufficient supplemental oxygen until the child is
in a fully awake condition with additional analgesia (i.e. intravenous route administration of morphine) can be carefully
administrated to these patients [17].
Intra-operative and postoperative
analgesia treatment can be administrated to patients with infiltration of local
anaesthetic and adrenaline either through surgically or prescribed to use
infra-orbital blocks paracetamol syrup is recommended regularly in the ward
postoperatively. Some safety
precaution must be operative for those CLP patients
who are under close observation under the Jam pack situation, as usually
observed in hospitals or limited staffs inwards; the usage of opioids is
avoided. To avoid the situation of the high incidence of postoperative airway
complications, it should be recovered safer or adjacent them to the operation
theatre until they are in fully awake [14,16] situations.
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Fig. 2: Potential Post-operative nursing issues
Many
potentially devastating complications can occur during emergence and recovery
from anaesthesia. Monitoring is essential for the early detection of
post-operative complications. CLP surgeries show better outcomes with optimum
pain management. Multimodal therapy with Opioids, non-opioids and infra-orbital
nerve blocks are mainly used. The infra-orbital nerve provides sensory
innervation into the skin and upper lip mucous
membranes, cheek, lower eyelids and nasal alveolus. Blockage of the
infra-orbital nerve provides relief from pain in most of the tissues affected
by a (CL) repair. In the 2016 year published Cochrane review on infra-orbital
nerve blockage during the (CL) repair studies, which demonstrated its efficacy
in terms of lowering the opioid consumption during the surgery and counting the
lower pain scores in the post-anaesthesia care unit [18].
Recognition and Management of Post-operative
Complications- Following
problematic complications arise frequently in cleft children while operating
surgery in the nasal-airway passage:
Residual
sedation- CLP Patients were
recommended to anaesthetic drugs along with potent sedative drugs. Few of these
children may metabolise the drugs slower than expected. This may result in
prolonged sedation following surgery. This treatment is purely supportive.
Ensure the airway is clear, do a chin lift and jaw thrust and place the patient
appropriate position. Additionally, if required give oxygen either via a face
mask or nasal prongs [19,20].
Forgotten pack- Throat pack is aseptically removed
before the child is in fully awake mode. If missed it will obstruct the airway and the child will have difficulty
breathing and might activate the gag reflex. Sometimes the child will have
complete obstruction of their airway and have a respiratory arrest. It is
important to diagnose the problem emergently and facilitate removal of the pack
[21-23].
Laryngospasm- Laryngospasm during recovery occurs sometimes if
the child is in lighter planes during extubation. Any blood or sputum near the
vocal cord acts as an irritant leading to reflex adduction of vocal cords. This
may lead to airway obstruction and hypoxemia. Immediately call for help,
support airway and give oxygen. A forcible jaw thrust and pressure on Larson's
point sometimes relieves the laryngospasm. Unexpectedly, still, the condition
does not improve in the child, then it may need intubation of following a dose
of propofol or succinyl choline [20,21] drugs.
Airway obstruction- Airways blockage can occur due to a combination of
factors such as edema in the tongue, laryngospasm, retained throat pack or blood
clotting. The lateral position with CPAP for a short period may be considered
as an effective one. Nasopharyngeal airways (NPA) are
especially observed to well tolerate in those patients who are at a high risk
of postoperative airway complications or they may be inserted before emergence.
The NPA can usually be removed immediately the following day once the swelling
has subsided and the child has mastered to inhale through the mouth. The only
option is to avoid operating oropharyngeal airways while surgery because of the
high chances of disrupting air passage. Instead of this, an only small number
of infants needed re-intubation and possibly tracheostomy [19-23] like
advanced technologies.
Bleeding- An excess of bleeding is examined after the cleft
palate surgery from children who had continued dribbling of blood from the
mouth or it may restrict the airway passage. In this condition, additional
support and care are provided to those children who needed excess quantity of
oxygen with a face mask, with fluid resuscitation and place them in the left lateral position and stay head down to allow
the blood to drain and prevent them to aspiration. Suction the mouth
cautiously, entering closely to 1cm asides of the mouth. Compress any visible
bleeding points with gauze, ensuring that it does not cause any further airway
obstruction [21-23].
Laryngeal Oedema- This type of anomaly develops when the true or false
vocal cords, both are in the swollen state. It happens due to difficulties
intubation or placement of an oversized endotracheal tube. This is an
emergencyand needs the assistance of the anaesthetist. In the meantime, it
supports the airway passage and releases out oxygen additionally equipped with
a well-sealed mask (Ambu-bag). Sometimes, nebulized epinephrine (adrenaline)
intake of (0.5 mg/kg) in 3 ml of normal saline will relieve from oedema
temporarily. Dexamethasone dose of 0.1-0.25 mg/kg is given intravenously if it
has not already been given in operation theatre at induction
[21-23].
Bronchospasm-
Bronchospasm is due to constriction of the muscle
around the bronchioles. It is usually exacerbated by using certain drugs.
Children with upper respiratory tract infection are more susceptible to
post-operative bronchospasm. This may be avoided spontaneously with oxygen via
a face mask. However, if the child develops an audible wheeze, a tracheal tug
or sub-costal recession and looking restless then it is operated with nebulized
(2.5 mg) salbutamol in a 3 ml of normal saline. An intake of Dexamethasone of
0.1-0.25 mg/kg is given intravenously to them [20-22].
Aspiration-
A baby with a cleft palate has an abnormal
swallowing mechanism and hence is susceptible for aspiration of saliva, blood
and feeds. This makes the baby prone to developing a chest infection. They may
have added breath sounds and have low oxygen saturation on room air. Baby is to
be oxygenated. Antibiotics are to be started as per protocol. Feeding is to be
commenced once the baby is awake and can control their airway. Mothers must be
supervised and taught how to feed a baby safely post cleft palate repair [19-21].
Fever-
Majority of children develop a fever postoperatively
after administration of atropine drugs given in an operation theatre. This usually
resolves spontaneously. If the fever persists, start tepid sponging and
antipyretic drug treatment is prescribed to them. Appropriate antibiotics can
be commenced [18,19,22] to avoid this situation.
Hypothermia
and Shivering- Children
usually lose the ability to conserve their body temperature because of the low
temperature and anaesthetic drugs affect. Before the age of 3 months, children
don't have a shivering mechanism and therefore is more liable to become
hypothermic. In this situation, they release oxygen, warm up with a blanket and
avoid using cold fluids [20,24].
Post-operative
nausea and vomiting-Post-operative
vomiting in children can be distressing one. To avoid this situation, one
should administrate the proper supply of oxygen, delay in the oral intake and
continue intravenous fluids. If found the very severe situation, an antiemetic
can be recommended [25,26].
Agitation-
Agitation should be operative in hypoxia, pain, hunger,
and separation from care, un-comfortable environment situations or certain
children reaction against the drugs like sedatives. Fully access clinically
those patients and reason for causing it [19, 21,27].
CONCLUSIONS- The present study concludes the vigilant
post-operative care is essential for good surgical outcomes. It starts in
recovery and continues in the ward. It requires vigilance and careful regular
observation of the child. All observations must be documented clearly to
appropriate management and measurement can be implemented easily. Airway
complications constitute the most common postoperative complication arises
especially in children of less than 12 months of age. Children and babies with
clefts are susceptible to more to upper respiratory tract infections which
impaired airways and increase the chances to develop the post-operative
respiratory complications. Children with this disorder have high chances of
risk so rapidly, that's why they are recommended to recover them as soon as
possible to monitor regularly. Careful selection of the patient undergoing cleft
surgery to gain a favourable outcome. Expertise
anaesthesiologists should be preferred choice to manage the kinds of critical
cases and hospitals should be equipped with
all the facilities resources to handle the cases with care.
CONTRIBUTION OF AUTHORS
Research concept-
Dr. Shiv Shanker Tripathi, Dr. Suruchi Ambasta
Research design- Dr.
Shiv Shanker Tripathi, Dr. Suruchi Ambasta
Supervision-
Dr. Shiv Shanker Tripathi, Dr. Anurag Agarwal
Materials-
Dr. Shiv Shanker Tripathi, Dr. Suruchi Ambasta
Data collection-
Dr. Shiv Shanker Tripathi, Dr. Suruchi Ambasta
Data analysis and Interpretation- Dr. Shiv Shanker Tripathi , Dr. Suruchi Ambasta
Literature search- Dr. Shiv Shanker Tripathi
Writing article-
Dr. Shiv Shanker Tripathi, Dr. Suruchi Ambasta
Critical review-
Dr. Shiv Shanker Tripathi, Dr. Anurag Agarwal
Article editing-
Dr. Shiv Shanker Tripathi
Final approval-
Dr. Shiv Shanker Tripathi, Dr. Anurag Agarwal
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