Many children require plastic surgery to correct congenital deformities. The head and neck are commonly affected, which may introduce special problems for the anesthesiologist. In addition, some children undergo plastic surgery for acquired lesions, such as burn scars and contractures or dog bites.
General Principles
- 1.
Many of these children have psychological upsets stemming from both the deformity and multiple surgical procedures. A careful, considerate approach by the anesthesiologist is essential. Review of prior anesthetic records is essential so as to adequately assess approaches to premedication, airway management, venous access, and other associated medical or surgical issues.
- 2.
Smooth general anesthesia with quiet emergence lessens the risk of damage to grafted areas and delicately sutured repairs.
- 3.
Many children undergoing plastic surgery have potentially serious airway problems that require careful assessment and special management.
- 4.
Congenital structural anomalies commonly affect more than one body system. The child with defects requiring plastic surgery may also have disease affecting other systems. If congenital heart disease is present, consider the possibility that prophylactic antibiotic therapy is indicated (see Chapter 14, page 462 ) and carefully review the cardiac surgical history and the most recent cardiac evaluations; seek consultation with the cardiologist when indicated.
CLEFT LIP AND PALATE
Cleft lip and palate (CLP) are present in various combinations in as many as 1 of every 1000 live births. CLP defects may be isolated or occur as part of a syndrome or association of defects (Syndromic CLP). Infants with these lesions may be both malnourished and anemic as a result of feeding difficulties and may have a history of repeated respiratory infections. The treatment of CLP is optimally managed within a multidisciplinary team; the pediatric anesthesiologist is an essential member of this team.
The surgical management of CLP is evolving and debates continue about the optimal age for stages in the repair and the use of such procedures as preoperative orthodontic maneuvers to mold the bony structure of the palate. An orthodontic appliance (e.g., Latham appliance) is sometimes applied to the neonatal palate with the object of progressively molding the bony alveolar ridge into alignment before soft tissue repairs.
- 1.
Congenital heart disease—not specifically associated with isolated cleft palate, but may be present as part of a syndrome or association.
- 2.
Airway anomalies—for example, Pierre Robin syndrome or Treacher Collins syndrome that may make intubation extremely difficult.
- 3.
Syndromes associated with CLP may have other specific anesthesia implications; some of these are listed in Table 12-1 .
TABLE 12-1
Some Syndromes Associated With Cleft Palate
Syndrome Name(s) Implications
Features
Anesthesia
Arthrogryposis multiplexCongenita
Limb contractures, CHD in 10%
Stiffness of joints, GU defects
Difficult intubation due to limited mouth opening, antibiotics for CHD, position and pad carefully.
Beare-Stevenson syndrome
Craniosynostosis, hydrocephalus choanal atresia, midface hypoplasia proptosis, hypertelorism, cutis gyratum
Difficult ventilation (Choanal atresia) difficult intubation, beware of tracheal stenosis, caution with neck. Cervical spine defects.
Beckwith-Wiedemann syndrome
Exomphalos, macroglossia, gigantism, hypoglycemia
Danger of hypoglycemia, infuse glucose and monitor level, tongue reduction may be required at time of palate repair.
CATCH 22
Cardiac defect, abnormal facies, thymic hypoplasia, hypocalcemia, (Di George syndrome)
Difficult airway. Antibiotics for CHD. Treat hypocalcemia.
Cornelia de Lange syndrome
Short stature, developmental delay (variable), CHD in 15%
Airway obstruction, difficult intubation, antibiotics for CHD.
Down syndrome
Short stature, developmental delay (variable), macroglossia, unstable cervical spine, narrow subglottic space. CHD in 50%
Difficult intubation, caution with neck, caution with ETT size, antibiotics for CHD, prone to airway obstruction.
EEC syndrome
Ectrodactyly, ectodermal dysplasia, hypohidrosis. Chronic respiratory infections
Malnutrition, anemia, hypohidrosis, temperature control problems. Difficult intubation, protect eyes, position and pad carefully. Caution with atropine.
Kabuki syndrome
Craniofacial and skeletal defects, hypotonia anomalies, CHD, visceral and urogenital defects, increased susceptibility to infections.
Difficult airway, caution with relaxants, antibiotics for CHD, care with asepsis.
King syndrome
Congenital myopathy, MH trait. Dysmorphic features (Noonan-like).
Malignant hyperthermia precautions.
Miller syndrome
Mandibular defects, limb anomalies, renal defects.
Difficult airway
Multiple pterygium syndrome
Webbing of skin, syngnathia, ankyloglossia, web neck.
Difficult airway—more severe with age
Nager syndrome
Malar hypoplasia, micrognathia, CHD, radial hypoplasia, absent thumbs, vertebral anomalies
Very difficult airway, limited mouth opening, cervical spine anomalies. Antibiotics for CHD.
Oto-palatal-digital syndrome
Skull deformity, hearing loss, cervical spine defect, (Arnold Chiari), limb defects, possible thoracic hypoplasia.
Possible brain-stem compression causing postoperative respiratory depression.
Patau syndrome (Trisomy 13)
Microcephaly, developmental delay, micrognathia. CHD, usually fatal in infancy.
Difficult airway, antibiotics for CHD
Pierre-Robin sequence
Micrognathia, cleft palate
Difficult airway, postoperative ventilatory obstruction.
Seckel syndrome
Birdlike facies, dwarfism microcephaly, possible glottic narrowing; post-op apnea reported.
Difficult airway, caution with ETT size. Monitor ventilation postoperative.
Smith-Lemli-Opitz syndrome
Growth failure, microcephaly, developmental delay, CHD, renal defects, hypotonia, GE reflux. Thymic hypoplasia—prone to infection.
Possible difficult airway, intraoperative muscle rigidity, temperature control problems, antibiotics for CHD.
Spondyloepiphyseal dysplasia
Congenita
Dwarfism, C1-C2 instability
Caution with neck during intubation and positioning (use vacuum splint).
Stickler syndrome. (Pierre-Robin variant)
Midface hypoplasia, cleft palate, retromicrognathia, “Moon Face” one third of “Pierre Robin” patients
Difficult airway (mask ventilation and intubation)
Walker-Warburg syndrome
Micrognathia, hypotonia, hydrocephalus, developmental delay, GU anomalies.
Difficult intubation, postoperative hypoventilation.
18 trisomy
Cleft palate, lung hypoplasia, macrognathia, CHD.
Difficult airway, ventilatory failure, antibiotics for CHD.
The surgical care of the patient with cleft lip/palate now comprises many possible stages, not all of which will be required by all children.
Possible Surgical Procedures
- 1.
Application of orthodontic splints or devices to the palate.
- 2.
Cleft lip repair—usually performed at 10 to 12 weeks sometimes earlier.
- 3.
Cleft palate repair—usually performed at 12 to 18 months, but sometimes in younger infants.
- 4.
Alveolar bone graft.
- 5.
Pharyngoplasty—for velopharyngeal incompetence —usually performed at 5 to 15 years.
- 6.
Maxillary advancement (Le Fort procedure) required for some adolescent patients.
Special Anesthesia Problems
- 1.
Airway problems, including difficulty with intubation (sometimes extreme). Intubation with isolated micrognathia tends to be difficult in the young infant and becomes easier with age. This may not apply if a syndrome is present.
- 2.
Problems related to associated conditions (see previous discussion).
- 3.
As plastic surgery involves soft tissue dissection, coagulopathy causes significant bleeding. Blood loss during cleft palate repair is usually insignificant but very occasionally may require transfusion.
Anesthesia Management
Preoperative
- 1.
Carry out a very careful preoperative assessment.
- a.
Direct special attention to the airway, lungs, and other systems that may be affected in congenital syndromes (see Appendix 1, page 539 ).
- b.
Check especially carefully for upper respiratory tract infection; if such an infection is present, surgery should be postponed.
- c.
Check for anemia.
- a.
- 2.
Check for any history of bleeding tendency. Check medication history (salicylates (eg., aspirin), NSAIDs, gingko, garlic, or ginseng). If positive, determine the bleeding time; if it is prolonged, surgery should be deferred.
- 3.
Blood is rarely needed for cleft palate surgery, but check that the child has been typed for blood and serum saved.
Perioperative
- 1.
If there is any doubt about the ease of tracheal intubation, perform an inhalation induction and manage as for “difficult airway” see Chapter 4, page 103 . Consider starting an IV before anesthesia as tolerated.
- 2.
For inhalation induction, administer nitrous oxide (N 2 O) and oxygen (O 2 ) with sevoflurane or halothane until the child is anesthetized adequately for laryngoscopy. Then discontinue N 2 O and continue inhalational anesthesia in O 2 . Establish IV access. Give lidocaine 1.5 mg/kg IV (or propofol 1 to 2 mg/kg) before insertion of the laryngoscope to minimize the risk of coughing or laryngospasm.
- 3.
If the cleft is large or bilateral, consider packing it with moist sterile gauze to prevent trauma during laryngoscopy and intubation.
- 4.
For orotracheal intubation, use a RAE® *
* RAE = Ring-Adair-Elwyn
preformed tracheal tube. Check carefully that bilateral ventilation of the lungs is present after the mouth gag is positioned. Insertion of the gag and flexion of the neck may advance the tip of the tube in the trachea, so that it may pass into a bronchus. Extension of the head tends to withdraw the tip of the tube and a leak may occur— especially in small infants if the tube has a “Murphy Eye.”
- 5.
For cleft lip repair, tape the tube carefully to the mandible and ensure that the upper lip is free and not distorted by the surgical tape.
- 6.
Monitor air entry and ventilation continuously during surgery, paying special attention each time the gag is repositioned or the child is moved.
- 7.
Maintain anesthesia with N 2 O, a relaxant and controlled ventilation and a low concentration of sevoflurane or isoflurane. The inhalational agent should be discontinued before the end of the operation so that the child awakens promptly once the relaxant has been antagonized. Otherwise a TIVA regime may be used.
- 8.
Monitor blood loss carefully and replace if indicated. The infiltration of a local anesthetic with 1:200,000 epinephrine reduces blood loss in cleft palate surgery and also provides some analgesia postoperatively.
- 9.
Inspect the mouth and pharynx gently at the end of surgery; use a laryngoscope and remove all blood and clots. Extubate when the child is fully awake. After palate repair, acute swelling of the tongue causing obstruction has been reported as a complication of the use of the tongue blade on the mouth gag. Therefore examine the mouth carefully; if any signs of tongue swelling exist, the trachea should be left intubated.
- 10.
A long suture is often inserted into the tongue to exert traction and facilitate immediate postoperative control of the airway in the PACU after cleft palate repair. This is usually removed when the child is fully awake but may be left in place overnight until a clear airway is assured.
Postoperative
- 1.
Cleft lip surgery is often performed in the day surgery unit because the surgery is superficial and postoperative problems are rare. The use of an infraorbital nerve block (see Chapter 5, page 163 ) provides good postoperative analgesia for this type of surgery and should be routinely used.
- 2.
All children are admitted overnight after cleft palate surgery. Ensure constant observation for 24 hours because airway problems or bleeding may occur. A small percentage of children may have to return to the operating room for control of bleeding and some may require reintubation.
- 3.
Order analgesics with caution to avoid excessive respiratory depression as upper airway obstruction is a particular risk in the postoperative period.
Application of Palatal Splints or Devices in the Neonate
This is sometimes performed to mold the palate and alveolar ridge and improve the aesthetic and dental results of subsequent surgery. All provisions for neonatal anesthesia should be observed. General endotracheal anesthesia is preferred; use an oral RAE tube. After the procedure the infant is usually uncomfortable and requires a mild analgesic.
Alveolar Bone Grafting
An alveolar bone graft may be required to close a gap in the bony alveolar ridge; this procedure may be performed on an ambulatory basis. The operation is preferably performed as the permanent dentition erupts so that teeth may be guided into the grafted area. Bone is often harvested at the iliac crest in which case preincisional infiltration of the donor site with bupivacaine may significantly reduce postoperative pain. For the ambulatory patient, prophylactic administration of dexamethasone and antinausea medication significantly decrease PONV and speed return to normal activity.
Pharyngoplasty
Pharyngoplasty is performed to reduce velopharyngeal incompetence and improve speech. The procedure inevitably increases resistance to ventilation. Postoperative airway problems are common. These children require close monitoring postoperatively.
Special Anesthesia Problems
- 1.
Postoperative airway obstruction is a particular danger and may occur early in the PACU.
- 2.
Chronic airway obstruction may persist after the operation and may lead to obstructive sleep apnea and possibly to later pulmonary hypertension.
Anesthesia Management
As for cleft palate (see previous discussion).
Postoperative
- 1.
Observe closely in the PACU or PICU for airway obstruction and/or bleeding for at least 12 hours.
- 2.
A nasopharyngeal airway, placed by the surgeon and left in situ for 24 hours, is effective in reducing serious postoperative respiratory complications.
- 3.
Do not order heavy opioid sedation.
- 4.
Continuing supervision for signs of obstruction during sleep is suggested, and a postoperative sleep study is recommended.
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