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DR.DEEPAK SOLANKI
M.D. ANAESTHESIOLOGY dr.dsolanki@gmail.com
Embryological Development
Cleft lip unilateral / bilateral # unilateral left sided common # bilateral protruded premaxilla palate incomplete / complete
Cleft
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
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
# 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
# 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
Airway Need
obstruction
Intubation Plain
radiograph of mandible
Cleft
palate repair delayed till 18 months palate any time till 4- 5 years
hard
soft
early
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
Monitoring
Monitoring
Precordial stethoscope monitor heart rate, breath sounds
NIBP
ECG
Blood loss
Kinking of ETT
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
EMBRYOLOGICAL DEVELOPMENT
EMBRYOLOGIC DEVELOPMENT
Ectodermal neural crest deepening neural groove
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
Myelomeningocele neural dysfunction below the level of MNC Lumbosacral flaccid paraplegia, loss of pinprick and loss of anal and bladder sphincter tone
MANAGEMENT
Management
Antibiotics
Early
Delay
Increased
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
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
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
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
# 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
SHUNTS
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
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
Anaesthetic considerations
PRE-MEDICATION ICP pre-operative sedation (narcotics, sedatives) avoided hypoxia / hypercarbia raise ICP ICP sedation used judiciously
Increased
Normal
Anaesthetic considerations
ICP
Local
Needle
Anaesthetic considerations
ICP
Raised
ICP steps to prevent further rise in ICP Prevent gastric acid aspiration, preoxygenation, intravenous induction with cricoid
Anaesthetic considerations
Anaesthetic considerations
MAINTENANCE Anaesthetic consideration for maintenance are
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
Adequate
Adequate
Gastric
Fully
Post-operative management
# oxygen, adequate ventilation # maintenance of body temperature # analgesia judicious use of opioids # intra-operative infiltration of skin
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