32 Ophthalmology
Many ophthalmologic diagnoses can be confirmed only by the ophthalmologist examining a cooperative infant or child. An examination under anesthesia (EUA) often is essential for an accurate diagnosis and evaluation of many processes, including trauma, tumors, infiltrative diseases, coloboma, glaucoma and other vascular diseases of the retina, Coats disease, and incontinentia pigmenti. Inpatient preterm infants often require serial EUAs to monitor the development and progress of retinopathy of prematurity (ROP) and the response of the disease to surgery. These examinations may be performed in the neonatal intensive care unit or operating room and may require sedation or general anesthesia.1 Other inpatient trauma victims may require serial EUAs to monitor the development of glaucoma or retinal injury. Serial EUAs are also necessary to monitor progress during outpatient retinoblastoma radiation treatments. The anesthesiologist is essential to the provision of pediatric ophthalmologic diagnostic and therapeutic techniques.
The child in need of ophthalmologic surgery typically requires general anesthesia or deep sedation rather than exclusive use of local or regional anesthesia. Many infants and children cannot cooperate for anything beyond a brief eye examination. Although the ophthalmologist may be able to tolerate small movements by the child during an EUA, unnecessary head or eye globe movement during an ophthalmologic procedure should be prevented. Retrobulbar block is sometimes performed for postoperative analgesia in children before emergence from general anesthesia2; all complications associated with a retrobulbar block identified in adults may also occur in children.3,4
Preoperative Evaluation
The perioperative environment should be welcoming to the child and family.5,6 All team members should be comfortable with the anesthesia considerations for infants and children for ophthalmologic procedures.6 Anticipation and prevention of postoperative nausea and vomiting (PONV) and understanding of anesthesia emergence and postoperative analgesia are essential.
Before an elective ophthalmologic procedure, the patient undergoes a thorough physical examination, including a review of all systems, and a complete medical history is obtained, including a surgical and anesthesia history, list of current medications, known allergies, and family history.7 Because many ophthalmologic diagnoses are commonly associated with systemic conditions, all implications of the systemic illness are a concern for the anesthesiologist.
Common Ophthalmologic Diagnoses Requiring Surgery
Common pediatric diagnoses and surgical procedures are listed in Table 32-1. A diagnosis may exist in isolation or be one aspect of a more complex group of diagnoses. Many involve systemic illnesses, and the anesthesiologist should be familiar with the implications of ophthalmologic disease in these settings.
TABLE 32-1 Common Ophthalmologic Procedures in Children
Communication and flexibility are mandatory in anesthesia planning. Anesthesia may start with a plan for a brief EUA using an inhalational anesthetic with a facemask and no intravenous catheter. However, if corrective surgery becomes necessary, airway control may require placement of a laryngeal mask airway (LMA) or tracheal tube. Intravenous access is required if the airway is instrumented to administer medications, including those to prevent or treat the oculocardiac reflex (OCR), postoperative pain, and nausea and vomiting.8
For procedures of brief or intermediate duration, the use of an LMA allows excellent access to all periorbital structures and provides good airway control in most circumstances. Compared with mask anesthesia, the LMA has the advantage of decreasing environmental contamination by inhalational anesthetics. It is relatively easy to insert and remains secure while avoiding the need for the anesthesiologist to hold a facemask near the surgical site. Compared with the tracheal tube, the LMA does not increase the heart rate, blood pressure, and intraocular pressure (IOP) to the same degree.9
Some of the more common ophthalmologic presentations and associated systemic illnesses or syndromes are listed in Table 32-2. Some systemic conditions have significant cardiorespiratory or central nervous system implications for perioperative management, and they should be fully evaluated before anesthesia (see Chapter 4). Many procedures are performed on preterm infants or formerly preterm infants, and prematurity and its complications have some of the most clinically important implications for anesthesia management.
TABLE 32-2 Ophthalmologic Conditions Associated with Systemic Syndromes and Illnesses
| Syndrome or Illness | Ophthalmologic Conditions |
|---|---|
| Fetal alcohol syndrome | Strabismus, optic nerve hypoplasia |
| Galactosemia | Neonatal cataracts |
| Mucopolysaccharidoses | Corneal involvement; may require transplantation |
| Retinitis pigmentosa | Heart block |
| Sturge-Weber syndrome | Glaucoma |
| Prematurity | Retinopathy of prematurity, strabismus |
| Fabry disease | Whorled corneal opacities |
| Tay-Sachs disease | Cherry-red macular spot |
| Osteogenesis imperfecta | Blue sclerae |
| Craniofacial syndromes (e.g., Crouzon, Apert, Pfeiffer) | Proptosis, strabismus, glaucoma |
Ophthalmologic Conditions Associated with Systemic Disorders
Prematurity
The preterm infant may present for many surgical procedures early in life. ROP, congenital cataracts, and glaucoma may require surgery even when the infant weighs less than 1000 g. The preterm infant may have significant systemic illnesses. Common complications of prematurity include acute and chronic pulmonary disease,10 respiratory failure and pulmonary hypertension, congenital heart disease (unrepaired or with a limited palliative repair), and intraventricular hemorrhage,11 with or without obstructive hydrocephalus.
Acutely ill preterm infants and those younger than 1 year of age are at greater risk than older children and adults for perioperative complications.12 Careful attention to airway management, assisted ventilation, and titration of oxygen therapy with specified goals are essential for success.13 In preterm infants whose airways are already intubated and whose lungs are ventilated mechanically, the anesthesiologist should confirm the position of the tube, transport the infant safely to the operating room, and limit the exposure to high concentrations of oxygen. Although institutional goals are not uniform regarding supplemental oxygen therapy,14 communication with the neonatal team is often helpful in gauging the infant’s previous oxygen requirement and current targeted goals (e.g., hemoglobin-oxygen saturation levels of 90% to 95%). Because most inhalational anesthetics impair hypoxic pulmonary vasoconstriction, a greater fraction of inspired oxygen (Fio2) may be necessary to maintain the targeted hemoglobin saturation.
Infants with extremely low birth weight require many weeks to grow and develop to a weight of approximately 1800 g and to maintain normothermia without special environmental control. These infants commonly have a history of short-term or intermediate-term assisted ventilation and may not require supplemental oxygen before an EUA or planned operative procedure for ROP.15 Many of these infants undergo ophthalmologic examinations while their lungs are ventilated in the neonatal intensive care unit.16 During surgical therapy (i.e., laser or cryosurgical stabilization) for ROP, these infants require anesthesia to provide optimal conditions (Fig. 32-1). Perioperative apnea may preclude tracheal extubation or require close postoperative monitoring after anesthesia.
Perioperative apnea in the preterm infant is widely described.17,18 Whether the child is still hospitalized or presenting for elective surgery as an outpatient, the preoperative assessment should determine the pattern and frequency of apnea before the planned surgical procedure and anesthesia. If the child has been discharged, the current use of respiratory stimulants (i.e., caffeine or theophylline) and oxygen should be determined. Is an apnea monitor being used, or has it been discontinued? Guidelines have not been developed to manage some of the scenarios, but infants who continue to require supplemental oxygen, who are younger than 60 weeks postconceptional age, or who are monitored for apnea or bradycardia should have continuous cardiorespiratory and oxygen saturation monitoring postoperatively for at least 12 hours or until they are apnea free (see Chapter 4). The risk of apnea after general anesthesia and sedation decreases with advancing gestational age at birth and with advancing postnatal-postconceptual age. The risk of apnea is independent of opioid use; its multifactorial origins include the presence of general and neuraxial anesthetics and the immature central nervous system and respiratory center in the preterm infant. Flexible planning for possible postoperative ventilatory support is essential, and families should be informed of this possibility preoperatively.
Although chronic lung disease due to prematurity is prevalent, its intensity has been significantly reduced with the routine use of surfactant and advances in ventilator management strategies. Long-lasting respiratory effects from prematurity may include reactive airway disease, subglottic stenosis from prolonged intubation, and alveolar or interstitial disease with an oxygen requirement lasting weeks to years.10 Because many anesthetics impair hypoxic pulmonary vasoconstriction, an increased oxygen requirement in the perioperative period should be anticipated. Tracheal intubation, a light level of anesthesia, or topical use of β-adrenergic antagonists may exacerbate reactive airway disease, requiring further treatment to reduce air trapping and hypercarbia.
Along with assessing for airway and alveolar diseases, the anesthesiologist should determine whether pulmonary hypertension or right ventricular dysfunction is or was present.19 Some infants may be receiving continuing oxygen therapy as treatment for pulmonary hypertension and to reduce intermittent episodes of hypoxemia due to crying or while sleeping. If pulmonary hypertension was diagnosed previously, an updated evaluation is warranted. Because pulmonary hypertension is exacerbated by hypoxia and hypercarbia, tracheal intubation should be considered to ensure control of ventilation and oxygenation. At emergence, pulmonary hypertension may be exacerbated as hypercapnia develops, causing physiologic or anatomic shunting with systemic hemoglobin-oxygen desaturation. Immediate and continued evaluation of the airway is imperative to rule out an independent respiratory contribution to the systemic hypoxia.
Congenital cardiac disease may be diagnosed in the preterm neonate, infant, or child who presents for ophthalmologic procedures. A patent ductus arteriosus may not close spontaneously or after administration of cyclooxygenase inhibitors. This may lead to persistent congestive failure, reduced pulmonary compliance, and complications of fluid management. Congenital cardiac anomalies require assessment before elective surgery. The various complex congenital cardiac lesions have a wide spectrum of interactions with multiple anesthetic agents.20 Many cardiac conditions require surgery or palliation (e.g., systemic-to-pulmonary shunts) before elective ophthalmologic procedures. Correction of congenital heart disease is limited by the difficulty of using cardiopulmonary bypass in infants weighing less than 2 kg. There may be an urgent need for ophthalmologic evaluation (e.g., congenital tumor, cataract, glaucoma) before repair of the congenital cardiac condition. Preoperative consultation with the infant’s pediatric cardiologist can provide useful information on the infant’s current ventricular function and the risk of dysrhythmias associated with cardiac defects. Medical management must be optimized before undertaking anesthesia and surgery.
Intraventricular hemorrhage is a major source of morbidity and mortality for preterm infants.11 Obstructed hydrocephalus may occur and require cerebrospinal fluid diversion procedures to decompress the obstructed ventricular system and treat the associated increased intracranial pressure. Many of these infants require ophthalmologic surgery for repair of strabismus due to a neurologic insult. If a ventriculoperitoneal shunt is in place, its proper function should be determined by direct evaluation. The anesthesiologist should assess whether there is inappropriate macrocephaly or a bulging or tense fontanelle. Obstructed hydrocephalus may occur after the infant’s discharge from hospital, even though a ventriculoperitoneal shunt was not required previously. The preoperative assessment should include the child’s developmental and neurologic status at the time of surgery. Because intraventricular hemorrhage is associated with long-term morbidity, any history of seizures should be elicited, and the antiepileptic drugs being used should be documented.
Preterm and small infants rapidly lose heat when anesthetized. Prevention of hypothermia is essential in the perioperative environment. Hypothermia can decrease metabolism of most drugs and depresses respiratory drive in preterm infants (see Chapters 35 and 36).
Down Syndrome
Children with Down syndrome (i.e., trisomy 21) frequently present for ophthalmologic surgery because of associated pathologic processes such as neonatal cataracts, significant refractive errors (e.g., hypermetropia, astigmatism), strabismus, glaucoma, keratoconus, nasolacrimal duct obstruction, and nystagmus.21–23 Infants with trisomy 21 should have an ophthalmologic evaluation in the neonatal period. If this requires an EUA, the anesthesiologist should be prepared for the extensive medical implications associated with trisomy 21.24,25 Almost half of these infants are born with congenital heart disease, including septal defects, complete or partial atrioventricular canal, tetralogy of Fallot, transposition of the great arteries, and valvular insufficiency or stenosis. Any child with a left-to-right shunt may develop pulmonary hypertension, and children with trisomy 21 develop irreversible pulmonary hypertension at an earlier age. Bradycardia (25% to 60%) and hypotension (12% to 73%) have been reported during sevoflurane anesthesia in these children.26,27 Complete understanding of the child’s cardiac defects is essential to planning anesthesia (see Chapters 14, 16, and 21).
Airway abnormalities such as narrowed nasopharyngeal passages, macroglossia, pharyngeal hypotonia, and subglottic stenosis are frequently observed in children with trisomy 21. These abnormalities may contribute to development of chronic intermittent hypoxia, further exacerbating pulmonary hypertension, and these children should be expected to demonstrate exacerbations of airway obstruction and hypoxia after general anesthesia.28
Children with trisomy 21 have a wide spectrum of developmental delays. Cervical spine instability occurs, and occiput-C1 and C1-2 instabilities have been described.29,30 Subluxation of the cervical spine has rarely been reported in these children during anesthesia. Nonetheless, the anesthesiologist and the surgeon should avoid extremes of neck flexion and extension and lateral rotation during head positioning for laryngoscopy and surgery. While the child is awake, the range of motion of the neck in flexion and extension should be assessed, along with complaints of numbness or tingling in the hands or feet in a particular position. Previous spine and neck investigations or operations should be reviewed. These infants and children may have ligamentous laxity of the cervical spine and other locations. No consensus exists for the radiologic workup of children who are asymptomatic for cervical disease, although many children have been evaluated radiologically before about 5 years of age.
Children with trisomy 21 may be born with congenital hypothyroidism, or it may develop at any time during their life span. They may develop junctional bradycardia during sevoflurane anesthesia.31 The bradycardia may be associated with or result from occult hypothyroidism. If the child is found to have a goiter on examination or has symptoms consistent with hypothyroidism (i.e., prolonged jaundice, hypothermia, constipation, dry skin, macroglossia, or relative bradycardia), thyroid function studies should be obtained before anesthesia for an elective procedure.
Alport Syndrome
Alport syndrome (i.e., progressive hereditary nephritis) is one of the disorders in a group of familial oculorenal syndromes. This classification includes Lowe (oculocerebral) syndrome and familial renal-retinal dystrophy. Alport syndrome involves sensorineural hearing loss, progressive renal disease, and multiple ophthalmologic disorders, including cataracts, retinal detachment, and keratoconus.32,33 Development of myopathy and renal failure constitutes the major anesthesia concern. If the patient has myopathy, it is prudent to avoid succinylcholine and the risk of a hyperkalemic response. Renal insufficiency may alter the choice of pharmacologic agents to very-short-acting agents or agents that are not renally excreted.
Marfan Syndrome, Ehlers-Danlos Syndrome, and Homocystinuria
Marfan syndrome is caused by a defect in the fibrillin 1 gene (FBN1), which affects elastic and nonelastic connective tissues. These children have an increased risk of retinal detachment, lens dislocation, glaucoma, and cataract formation (E-Fig. 32-1).34 They may have significant pulmonary (scoliosis) and cardiovascular problems,35 which may include aortic, mitral, or pulmonic valve insufficiency. Preoperative cardiovascular evaluation is indicated to determine the progression of cardiovascular abnormalities that inevitably occur. Blood pressure control is essential to prevent aortic dissection.
Homocystinuria has at least three forms and different inborn errors. Enzymatic deficiency of the metabolism of sulfur-containing amino acids causes the intermediate metabolite, homocysteine, to accumulate. These children suffer from cataracts, retinal degeneration, optic atrophy, glaucoma, and lens dislocation. The cardiovascular pathology includes coronary artery disease at a young age. Thromboembolic phenomena occur more frequently because the children may be hypercoagulable.36 Nitrous oxide should be avoided because it inhibits methionine synthase, further limiting the conversion of homocysteine to methionine.
At least 10 forms of Ehlers-Danlos syndrome have been described. Not all forms express significant ocular pathology. From the anesthesiologist’s perspective, positioning is important to avoid trauma to the skin because these children develop hemorrhages with minor trauma and experience delayed wound healing. A thorough preoperative assessment should be performed for cardiac lesions. Hypertension should be avoided to reduce the risk of rupturing an aneurysm. The duration of effect of local anesthetics in patients with class III Ehlers-Danlos syndrome may be less than that in normal patients, and contingency plans should be in place to address these possibilities, including retrobulbar block or general anesthesia.37
Mucopolysaccharidoses
The systemic complications of these disorders are sufficiently severe that even well-planned anesthesia management for ophthalmologic procedures may cause death.38 Airway management can be extremely difficult with poor mask fit, dynamic airway obstruction with narrowed passages, a floppy epiglottis, and difficulty visualizing the larynx.39 Infiltrative material may be deposited in the laryngeal inlet and pretracheal tissues; an LMA is particularly useful in maintaining a patent airway. Tracheal intubation may require the use of advanced airway management techniques, and the anesthesiologist should have several plans for airway management (see Chapter 12). Cardiac evaluation should be considered before an elective procedure to assess ventricular function and arrhythmias. Intravenous access may be difficult due to subcutaneous deposition of mucopolysaccharides.
Craniofacial Syndromes
Craniofacial syndromes may manifest as craniosynostosis or have only middle and lower facial structure involvement.40 Apert and Crouzon syndromes are both disorders of craniofacial development, but syndactyly occurs only in the former (E-Fig. 32-2). They share the potential for many ocular disorders, including severe proptosis, and mask airway management may be difficult.41 Other mutations of the fibroblast growth factor receptor 2 gene (FGFR2) cause Antley-Bixler and Pfeiffer syndromes. These children may develop chronic airway obstruction, and some have complete tracheal rings. Tracheal narrowing should be anticipated, and smaller tracheal tube sizes should be used.
Children with asymmetry of facial and mandibular bone growth may present with limited mouth opening. Children with Goldenhar syndrome (i.e., hemifacial microsomia), Treacher Collins syndrome, and Pierre Robin sequence can be expected to present a challenge to airway management (see Chapters 12 and 33).42 Children with craniosynostosis have an increased risk of congenital heart disease, and a cardiac evaluation should be performed before anesthesia.43 Neurologic morbidity and seizure disorders occur more frequently in this group of patients.
Phakomatoses
The phakomatoses are neurocutaneous syndromes with multiple ocular pathologic processes. These syndromes include neurofibromatosis,44,45 encephalotrigeminal angiomatosis (i.e., Sturge-Weber syndrome),46 tuberous sclerosis,47,48 incontinentia pigmenti, and ataxia telangiectasia. Central nervous system involvement varies with each of these diseases. Patients may have developmental delay, seizures, and significant neurologic morbidity. Anticonvulsant drugs should be continued in the perioperative period. Electrolyte and hepatic function studies should be done, and plasma levels of antiepileptic agents should be evaluated preoperatively.
Ophthalmologic Physiology
Intraocular Pressure
IOP is the pressure exerted by the internal components of the globe on the covering (i.e., sclera and conjunctiva). The normal IOP is 12 to 15 mm Hg. An IOP greater than 20 mm Hg is considered abnormal. Aqueous humor is a clear fluid that is secreted by the ciliary body and released into the anterior chamber of the globe. It traverses the anterior chamber and bathes the iris. It flows through the canal of Schlemm into the pores of Fontana and then drains into the episcleral veins (Fig. 32-2). The posterior chamber, which is larger than the anterior chamber, is composed of a gelatinous mix known as vitreous humor. The sclera and globe that encase the intraocular constituents are relatively noncompliant and are protected by the bony orbit. However, intraorbital masses may impinge on the globe and increase the relative IOP or alter the flow of aqueous humor, resulting in increased IOP. Any obstruction to the drainage of aqueous humor causes fluid to build up within the anterior chamber and increases IOP.49 Increased central venous pressure (e.g., Trendelenburg position, coughing, Valsalva maneuver, straining, increased intrathoracic pressure) attenuates the drainage of aqueous humor from the eye. Arterial pressure does not directly effect changes in IOP. However, as arterial blood pressure increases beyond the normal range, approximately 30% of the increase in systolic blood pressure is reflected in IOP increases. Aqueous humor formation is described in the following equation:
K is the coefficient of outflow, OPaq is the osmotic pressure of aqueous humor, OPpl is the osmotic pressure of plasma, and Pc is the capillary pressure. These variables allow calculation to intervene by increasing the plasma osmolality acutely with mannitol to lower the IOP. This increases the gradient of osmolality and draws water out of the aqueous humor, thereby reducing the IOP.
The effect of succinylcholine on IOP is well documented.50,51 Succinylcholine increases IOP 6 to 10 mm Hg, an effect that begins within 1 minute after administration and continues for up to 10 minutes, at which time the IOP returns to normal. This effect has been attributed to four possible mechanisms:
