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CLEFT LIP AND PALATE

DR.DEEPAK SOLANKI
M.D. ANAESTHESIOLOGY dr.dsolanki@gmail.com

Embryological Development

Cleft lip and palate

Cleft lip unilateral / bilateral # unilateral left sided common # bilateral protruded premaxilla palate incomplete / complete

Cleft

# incomplete hard palate # complete both hard and soft palate

Pathophysiology

Pathophysiology

Uncorrected cleft lip sucking and feeding problems failure to thrive anemia

Uncorrected cleft palate regurgitation into nose and pharynx URI middle ear infection

In older children delayed speech development nasal speech psychological problems

Co-existing problems
Associated congenital anomalies # Congenital heart disease # Craniofacial abnormalities Pierre Robin syndrome, Goldenhaars syndrome

Problems of deglutition

Pulmonary aspiration

Anemia - poor nutrition, feeding problems Increased incidence of URI, chronic otitis media

Pierre Robin Syndrome


COMPONENTS :

# retrognathia posterior positioning of mandible # micrognathia hypoplasia of the mandible # glossoptosis due to retroposition of mandible # cleft palate # arched palate # airway obstruction due to falling back of tongue

Treacher Collin Syndrome


COMPONENTS

# notching of lower eyelids # antimongoloid slant of eyelids # hypoplasia of facial, malar and mandibular bones # malformation of external ear # macrostomia with high palate # skeletal defects clefts of palate and face

Importance of cranio-facial abnormalitites

Airway Need

obstruction

to maintain airway pre-op. and post-op. problems

Intubation Plain

radiograph of mandible

Management Cleft lip repair

Cleft

lip repair between 3 weeks and 3 months

Rule of ten 10 lb ; 10 weeks ; 10 gm % Hb

Inadequate tissue hampers earlier repair

Cleft palate repair


Cleft

palate repair delayed till 18 months palate any time till 4- 5 years

hard

soft

palate before phonation

early

repair not recommended ossification of maxilla at 12 18 months

Pre-operative assessment and preparation

Pre-operative assessment and preparation


Assess nutritional status Improve anemia

Evidence of URI T/S culture and sensitivity

Evidence of chest infection Antibiotics

Airway difficult intubation Associated craniofacial abnormalities Protruding premaxilla

Anaesthetic prerequisites

Anaesthetic prerequisites

Adequate depth during surgery recovery at the end of surgery when patient fully awake

Quick

Extubation

Induction

Induction
Intravenous or inhalational

in patients with suspected difficult airway # inhalational induction # intubation in sp. Breathing

in patients with large palatal cleft # slipping of blade into cleft # insert sponge to reduce gap

ETT fixed on the lower lip (midline) Types of ETT used : non kinkable tubes # Oxford # Preformed RAE # Reinforced (flexometallic)

Maintenance of Anaesthesia

Maintenance of Anaesthesia
Nitrous IPPV CHD Use

oxide and volatile anaesthetics

, using muscle relaxant determines choice of the relaxant

of epinephrine determines volatile agent

Extubation when child fully awake

Monitoring

Monitoring
Precordial stethoscope monitor heart rate, breath sounds

NIBP

Pulse oximetry mandatory

ECG

Blood loss

Capnography detects accidental dislodgement

Problems during surgery

Problems during surgery


Sharing of airway

Arrythmias use of adrenaline for infiltration

Accidental dislodgement of ETT

Kinking of ETT

Aspiration of secretions pack around ETT

Conjuctivitis or corneal abrasion

Post- operative

Post- operative
Close monitoring for 24 hours

Airway obstruction # surgical oedema # falling back of tongue tongue sutured to cheek # inadvertently left pack # aspiration of on going bleed

Dehydration - limited oral intake

Pain management # over sedation airway compromise # intra-orbital nerve block

MENINGOCELE & MYELOMENINGOCELE

EMBRYOLOGICAL DEVELOPMENT

EMBRYOLOGIC DEVELOPMENT
Ectodermal neural crest deepening neural groove

Fusion of margins of the neural groove neural tube

Developmental defects # Spina bifida failure of vertebral arch bony growth & fusion # Meningocele bulging of meninges # Myelomeningocele herniation of neural elements # Encephaloceles herniation of brain

CLINICAL FEATURES

Clinical Features
Depend on the site and the contents of the sac

Lumbosacral most common

Meningocele no neurological deficit

Myelomeningocele neural dysfunction below the level of MNC Lumbosacral flaccid paraplegia, loss of pinprick and loss of anal and bladder sphincter tone

Clinical Features contd.


Loss of skin covering inc. risk of infection Associated congenital anomalies congenital cardiac defects, Arnold Chiari malformation, hydrocephalus, club foot Urinary tract infection Obstructive uropathy Corrective orthopaedic procedures

MANAGEMENT

Management
Antibiotics

Early

closure within 24 hours in closure of defect

Delay

Increased

incidence of infection Increased neural damage and motor dysfunction


Pre-operative evaluation

Pre-operative evaluation

Evaluation for increased intracranial pressure Arnold Chiari malformation, hydrocephalus Neurological deficits depending on level of lesion # 75% in lumbosacral region
lesions above T4 - paraplegia lesions below S1 - allow ambulation lesions between L4 and S1 legs severely affected

Assessment of volume status Large third space losses from exposed myelomeningocele

Anaesthetic Considerations

Anaesthetic Considerations
Age related problems

High association with hydrocephalus # Potential for increased ICP # Brainstem herniation

Intubation problems encephalocele Problems due to positioning # Supine position during induction pressure on sac # Prone position for surgery

Fluid and blood loss exposed myelomeningocele

Prolonged indwelling urinary catheter # Increased incidence of latex allergy Intra-operative bronchospasm and hypotension

Induction

Induction
Patients with lumbosacral or -thoracic MMC # Supine position sac protected by cushioned ring # Lateral position prevents pressure on sac # Inhalational or I/V induction # Intubation with succinylcholine

Patients with large nasal encephalocele # Airway obstruction # Difficult to obtain good mask fit # Intubation under sedation with fibreoptic technique

Maintenance

Maintenance
Prone position after securing airway chest and hip rolls free abdomen Facilitates ventilation Reduce intra-abdominal pressure Reduce bleeding from epidural plexus Excess rotation of neck avoided Nitrous oxide in oxygen with intermediate acting muscle relaxants Long acting muscle relaxants avoided nerve stimulation often required to assess neural function

Emergence
Patients to be extubated fully awake

# at risk for apnoea # with severe central neurologic deficit # undergoing craniotomy for encephalocele

Those with nasal encephalocele


# airway obstruction # may require post-operative intubation

HYDROCEPHALUS

Hydrocephalus
Increased CSF within the ventricles and around brain CLASSIFICATION A. Non obstructive / communicating # No obstruction to circulation # Over production # Abnormal absorption B. Obstructive / non communicating # Obstruction to circulation which may be - within the ventricles - structures connecting ventricles - within subarachnoid space - at the site of absorption

Hydrocephalus
CAUSES

# congenital anomalies( Arnold Chiari malformation) # neoplasms # inflammatory conditions

Clinical Features

Clinical Features

Signs and symptoms depend on : # age of the child # rate of increase in ICP

Clinical Features
Children < 2 years , cranial sutures not fused # abnormal enlargement of head # prominent frontal area of the skull # bulging anterior fontanelle # widely separated cranial sutures # trans-illumination of affected area of cranial vault # resonant skull # sunset eye eye deviated inferiorly, lower margins of iris sink below margin of lower eyelid # dilated scalp veins , thin shiny skin

Clinical features Older children


# may not have enlarged head # signs of raised ICP - morning headache - vomiting - papilledema - sixth nerve palsy internal deviation of eye - lower cranial nerve dysfunction - swallowing abnormalities - stridor - drowsiness , coma

Investigations

Investigations
To assess the magnitude

# Serial head circumference measurement # Skull radiographs # Trans-cranial sonography patent ant. fontanelle # # CT scan closed ant. Fontanelle MRI

Management

Management
Shunt

Treatment of underlying pathology procedures :

# Ventriculo peritoneal, atrial, pleural # Ventriculo cisternostomy, ventriculo cholecystostomy, lumboperitoneal

SHUNTS

VENTRICULO ATRIAL SHUNTS obstructive and non-obstructive

For

Problems

: # thrombosis IJV, IVC # pulmonary embolism # pulmonary hypertension # septicemia # migration of catheter into SVC neccesitating revision or conversion of shunt to

SHUNTS
VENTRICULO PERITONEAL Complications less serious, easily managed Most popular

VENTRICULO CISTERNOSTOMY Connecting ventricles to cisterna magna Obstructive hydrocephalus - bypasses intraventricular obstruction

Repeat procedures in children for placement Revision of shunt infection, obstruction and raised ICP

Pre anaesthetic assessment

Pre anaesthetic assessment


Focus

on signs of raised ICP - level of consciousness Increased ICP in patients presenting for primary shunting, revision or malfunction Stomach- Increased ICP - delayed gastric emptying precautions to prevent aspiration related pathophysiology

Full

Age Airway

Anaesthetic considerations

Anaesthetic considerations
Presentation

with broad spectrum of symptoms and sign # an apparently healthy child

# seriously comatosed urgent surgery

Anaesthetic considerations

PRE-MEDICATION ICP pre-operative sedation (narcotics, sedatives) avoided hypoxia / hypercarbia raise ICP ICP sedation used judiciously

Increased

Normal

Anaesthetic considerations

PRE-INDUCTION reduced: needle tapping through scalp infiltration: minimal trauma

ICP

Local

Needle

can be left in place to monitor intracranial pressure during induction

Anaesthetic considerations

INDUCTION normal inhalational induction intravenous induction

ICP

Raised

ICP steps to prevent further rise in ICP Prevent gastric acid aspiration, preoxygenation, intravenous induction with cricoid

Anaesthetic considerations

INTUBATION WITH MUSCLE RELAXANTS


Intravenous succinyl choline # controversial transient rise in ICP # rapid onset of action # excellent relaxation # drug of choice Rocuronium 1.2 mg/kg equally useful Without i/v cath i/v access with small butterfly minimal trauma Failing i/v access skillful inhalational induction is better than repeated pricks which are more traumatic and cause increase in ICP

Anaesthetic considerations
MAINTENANCE Anaesthetic consideration for maintenance are

Positioning Ventilation Anaesthetic

agents Muscle relaxants Fluid management Maintenance of body temperature

Maintenance

Maintenance
POSITIONING

# supine, head turned excess rotation and flexion of head avoided impede jugular venous drainage # head up tilt to 30 degrees improves cerebral venous drainage

Maintenance
VENTILATION

# IPPR hyperventilation # Pa CO2 maintained at 25-30 mm Hg # Spontaneous ventilation must be avoided - hypoxia, hypercarbia increase ICP - air embolism ( pleuro-atrial )

ANAESTHETIC

Maintenance

AGENTS # nitrous oxide in oxygen # low concentration of volatile anaesthetics isoflurane sevoflurane

# narcotic supplementation in minimal doses at the time of subcutaneous passage of catheter # opioids of short duration

Maintenance

MUSCLE RELAXANTS # intermediate acting # histamine releasing relaxants avoided BLOOD LOSS # no significant blood loss # maintenance fluids to replace pre-operative deficits (vomiting) and intra-operative loss MAINTENANCE OF BODY TEMPERATURE # tendency for hypothermia as large surface area exposed

Emergence from anaesthesia

Emergence from anaesthesia

Anaesthetic considerations for emergence


time for elimination of anaesthetics reversal of blockade

Adequate

Adequate

Gastric

suction mandatory before extubation awake patient with recovery of

Fully

Emergence from anaesthesia



Post-operative management
# oxygen, adequate ventilation # maintenance of body temperature # analgesia judicious use of opioids # intra-operative infiltration of skin

THANK

YOU

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