CLEFT LIP in anesthesia considerations required

cleft lip anaesthetic implications
post graduate residency
authentic
surgical considerations during cleft lip surgery
pediatrics anesthesia

1. Cleft lip and cleft palate anesthetic consideration MODERATOR : Dr Shikha Sharma SPEAKER : Dr Tanvi Sharma

2. INCIDENCE A cleft lip or palate (CLP) is the second most common congenital abnormality after club foot highest amongst Asians(1:500) live births Cleft lip alone: 25% (60% male) Cleft palate alone: 25% (59% females) Lip + palate: 50% (70% males)

3. EMBRYOLOGY 4 th week : facial prominences begin to appear. 5 th week: the nasal pits form from the frontonasal prominence Nasal pits : nostrils with the medial and lateral nasal prominences Next 2 weeks the maxillary prominences increase in size and grow medially pushing the medial nasal prominences to the midline. The cleft between these prominences fuses and this becomes the upperlip Lateral nasal prominences : nasal alae. Mandibular prominences: lower lip and jaw . Two medial nasal prominences merge : philtrum ,the upper jaw with 4 incisor teeth triangular primary palate. 6 th week: secondary palate is formed from two shelf-like outgrowths of the maxillary prominences. 7 th week shelves move up as the tongue moves down and fuse to form the secondary palate.

4. The nasal septum grows down to join the cephalic part of the newly formed palate. By 10–12 weeks, the development of the face is complete. Cleft lip results from partial or complete lack of fusion of the maxillary prominence with the medial nasal prominence on one or both sides. Cleft palate results from failure of fusion of the palatine shelves .

5. CLEFT LIP cleft lip (CL): UNILATERAL OR BILATEREAL Complete or incomplete. – Complete CL – involves the entire lip going into the nostril Incomplete CL – ranges from small to large defect

6. CLEFT PALATE Primary cleft palate (prepalatal) involves anterior palate, alveolus, lip, nostril floor, and ala nasi. They can be complete or incomplete. Secondary cleft palate ( postpalatal cleft) : posterior to incisive foramen. (complete / incomplete) . Submucosal cleft in which a bone defect exists without a mucosal defect

7. CAUSES Familial predisposition Chromosomal abnormalities Chromosomal abnormalities - trisomy21 Drugs- phenytoin, sodium valproate and methotrexate , steroids, excessive maternal vitamin A intake , folic acid deficiency Maternal tobacco / alchohol abuse Maternal diabetes mellitus Maternal age <20 or >39 yrs Some syndromes associated with cleft lip ans palate: Pierre Robin Syndrome , Treacher Collins Syndrome , GOLDENHAR SYNDROME

8. Pierre Robin Syndrome Micrognathia Glossoptosis (posterior displacement of the tongue into the pharynx,) Acute upper airway obstruction Cleft palate Acute upper airway obstruction Feeding problems, failure to thrive, and cyanotic episodes Congenital heart disease

9. TREACHER COLLINS SYNDROME Autosomal dominant Micrognathia results in early airway problems Associated cleft palate 30% Ventricular septal defect Malar hypoplasia, colobomas (notching of lower eyelids) Antimongoloid slant of the palpebral fissures Ear tags and gross deformities of the external ear canals and ossicular chain

10. Unilateral mandibular hypoplasia Eye, ear, and vertebral abnormalities on affected side Ease of tracheal intubation variable Nager syndrome Nager syndrome-- rare form of acrofacial rare form of acrofacial Ease of tracheal intubation variable Nager syndrome Nager syndrome-- rare form of acrofacial rare form of acrofacial dysostosis dysostosis - craniofacial abnormalities craniofacial abnormalities (malar hypoplasia, severe micrognathia) GOLDENHAR SYNDROME

11. Syndrome Associated anomalities Velocardiofacial syndrome Microcephaly and microstomia Flat nasal bridge, small ears, short stature Immune deficiency Congenital cardiac disease, Laryngeal and tracheal anomalies Velopharyngeal incompetence with/ without cleft palate 22q 11 deletion (FISH test) Stickler Syndrome Micrognathia and flat face Eye, ear and joint abnormalities Congenital cardiac disease Progressive connective tissue disorder Down syndrome Microstomia and relative macroglossia Atlantoaxial subluxation and instability Epicanthic folds, simian crease Congenital cardiac disease Fetal Alcohol Syndrome Smooth philtrum, thin vermillion Small palpebral fissures Growth retardation CNS abnormalities Klippel-Feil syndrome 15% associated with cleft palate Congenital cardiac disease Short, webbed neck and fused cervical vertebrae Renal anomalies Scoliosis

12. SYMPTOMS Feeding : unable to suckle need feeding device or insertion of a nasogastric tube. recurrent chest infections due to repeated episodes of aspiration . Swallowing : unable to coordinate the ‘ breathe, suck, swallow ’ reflex which results in frequent nasal regurgitation of feeds or intermittent choking spells. Hearing : Eustachian tube dysfunction which results in middle ear effusions and conductive hearing loss Speech : A chid with an unrepaired cleft palate will never speak normally due to excessive nasal escape of air. Denta l: A child with a cleft of the alveolus will require bone grafting and orthodontics. Psychological : Cleft patients may have a number of psychological problems due to the abnormal looking face, incomprehensible speech or just repeated visits to hospital.

13. TIMING OF SURGERY Early infancy : this will reduce parental anxiety better cosmetic and functional result and increase the chances of better feeding. ‘Rule of tens’ : 10 weeks of age, 10 lbs in weight and 10 g/dl haemoglobin , WBC : <10,000 3 months of age gives time to detect most congenital abnormalities and allows for anatomical and physiological maturation. Developing countries: hb of 8 g/dl , weight : 3kg for a cleft lip repair in an otherwise healthy child is acceptable .

14. ANESTHETIC CONSIDERATIONS   1. Difficulty with intubation  2. Inadverent extubation during the procedure  3. Postoperative airway obstruction.  4. Age of the patient  5. The availability of intraoperative monitoring equipment  6. Anaesthetic drugs and expertise  7. Shared airway  8. The level of postoperative care .

15. Preoperative assessment HISTORY Presence Of Other Congenital Anomalies , Snoring and episodes of choking ( ACC TO POSITION ) Feeding difficulties ( nutritional impairment can lead to difficulty in wound healing) SYPTOMS: eustachian tube dysfunction and chronic serous otitis URI – Difficult to control URI in preoperative period in children with CP . antibiotics may be needed ( REACTIVE AIRWAY )

16. PREOPERATIVELY -----INVESTIGATIONS Routine – CBC and urine examination ( anaemia , renal involvement ) Chest X-ray if there is fever, running nose, purulent secretions and noisy chest Sweating during feeding, cyanotic spells and repeated chest infections , murmur ( CARDIAC ANOMALY ) echo and ECG should be done Routine NPO guidelines have to be followed 6 hours for solid food and formula feeds, 4 hours for breast milk and 2 hours for clear fluids.

17. INTRAOPERATIVELY MAC values in infants are higher than in adults. Intraoperative analgesia : acetaminophen rectally or I/V, local infiltration of the operation site by the surgeon, infraorbital nerve blocks NSAIDS can also be used in children over 6 months of age with no other contraindications

18. INDUCTION Induction of anaesthesia is most safely performed by inhalational anaesthesia with halothane or sevoflurane  Intravenous access is gained when an adequate depth of anaesthesia is achieved . No neuromuscular blocking agents should be given until one is sure that the lungs can be ventilated with a mask. An oral, preformed RAE tube is usually chosen. For palatal surgery, a mouth gag that fits over the tube is used to keep mouth open and the tongue out of the way.  After the patient has been finally positioned for surgery, check the patency and position of the endotracheal tube by auscultation and by gentle positive pressure ventilation to assess airway resistance .

19. INDUCTION In patients with hypoplastic mandibles or wide cleft palate, tongue can prolapse into nasopharynx leading to airway obstruction during the induction of anaesthesia  During laryngoscopy, place lubricated dental roll/gauze in the cleft to prevent trauma to underlying tissue Oral pack should be inserted to absorb blood and secretions. 

20. There should be communication between the anaesthetist and the surgeon Pulse oximetry, capnography, ECG, NIBP and a precordial stethoscope

21. Maintainence Isoflorane / Sevoflorane + N2O + Fentanil + Vecuronium/ Atracuronium   Fluid - Holliday-Segar formula  Normothermia, normocapnia, homeostasis  Surgeons use Adrenaline - ensure dose/concentration (1 ml/kg of 1:200000) Remove throat pack at the end & examine oral cavity for edema or bleeding  At the end of the surgery, the pharynx and the oral cavity should gently suctioned, if possible under vision using the laryngoscope Do not exert pressure on the suture line

22. EXTUBATION Insert a nasopharyngeal airway or endotracheal tube nasally just prior to extubation with the tip just above the epiglottis so as to overcome the obstruction can be kept for 24 to 48 hrs

23. EXTUBATION Acute airway obstruction is a very real risk at the end of the procedure following extubation  . The surgeon needs to remove the throat packs and ensure that the surgical field is dry.  Suctioning should be kept to a minimum to avoid disrupting the surgical repair.  Oropharyngeal airways are best avoided, if possible. Extubation should be undertaken only after the return of consciousness with protective reflexes intact.  A tongue stitch will often be placed in patients with preoperative airway obstruction. This pulls the tongue forward away from the posterior pharyngeal wall as a treatment for postoperative airway obstruction

24. Infraorbital nerve is a branch of the maxillary division of the trigeminal nerve Infraorbital nerve block can be performed in 2 different ways: Direct cutaneous injection : Infraorbital foramen should be palpated, and approximately 1–2 mL of 0.25 or 0.5% bupivacaine is injected near to surround the nerve, but not in the foramen Intraoral injection : during palpation of the foramen with the non dominant hand, the needle is inserted into the superior labial sulcus at the apex of the canine fossa. Approximately 1–2 mL of 0.25 or 0.5% bupivacaine is injected around the infraorbital foramen. INFRAORBITAL NERVE BLOCK

25. Post operative airway obstruction 1. After palatal surgery, reduction in the size of the airway 2. Edema of the tongue due to pressure from the mouth gag. 3. Residual effect of anesthesia and/or opioids. 4. Laryngeal edema following endotracheal intubation. All patients at risk of delayed airway obstruction should monitored closely in the postoperative period