Complicated Pediatric Anesthesia Part II – Flashcards
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Spina Bifida
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-A defect of the vertebral column due to the imperfect union of the paired arches at the midline through which the meninges and the cord may protrude -Most common congenital vertebral column abnormality -Covers abnormalities of: Vertebrae, Vertebral arch, Spinous process
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Spina Bifida Occulta
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-Boney abnormality seen only by radiography and nerves may be involved when associated with skin changes or hairy patch (NO PROTRUSION OR CYST!).
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Spina Bifida Aperta (Cystica)
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-Usually seen at birth. There is a boney abnormality plus it involves the NERVES, CSF, and MENINGES (protrusion or cyst).
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Spina Bifida Occulta: diagnosis
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-Since there is no opening, the diagnosis can only be made by X-ray or MRI - Clinical findings: Hairy patch, Sacral dimple in skin
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Spina Bifida Occulta: Treatment
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-None-if abnormality is limited to the bone -Deformities usually found at L5-S1
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Spina Bifida Aperta (Cystica): diagnosis
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-Also known as Cystica -Diagnosis made/found with prenatal ultrasound screening or at birth -CSF involvement!! -Mass at base of spine: meningocele (meninges), myelocele (SPINAL CORD), meningomyelocele (both)
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Spina Bifida Aperta: Meningocele
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-mass at the base of the spine that DOES NOT contain NERVE material -mass can either be open or covered with a thin membrane (meninges)
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Spina Bifida Aperta: Meningomyelocele
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-mass at the base of the spine that contain NERVES and meninges -mass can either be open or completely covered with a thin layer of membrane (meninges)
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Spina Bifida Aperta: Diagnosis & Treatment
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-Diagnosis made with prenatal ultrasonography -Treatment involves neurosurgery w/n 24-48hrs -Goal is to preserve nerves and to restore normal anatomy -Reduce the risk of infection -Reduce the progression of long-term disability
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Spina Bifida Aperta: Important associated anomalies
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-Hydrocephalus (80%)*** -Tethered spinal cord at caudal level involving orthopedic/GI/urologic symptoms -Arnold- Chiari malformation Type II* -Short trachea
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Spina Bifida Aperta: Special anesthesia considerations
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-Two IV's -Continue Glucose infusions -Positioning the patient for intubation -Good head and neck support -Considerable BLOOD LOSS! -Hypothermia -Post/op ventilatory depression or apnea
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Spina Bifida Aperta: Pre-op management
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-Cover lesion with sterile dressing and keep moist -Type and cross for blood in O.R* -Keep O.R. warm -Emergency drugs -Avoid Latex contact; 70% chance of developing allergy or sensitivity
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Spina Bifida Aperta: Anesthesia Considerations
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-Positioning for induction and intubation: Lateral left position;Supine -Supine: Provide sterile/clean cushion ring for defect if using supine position -Avoid Succinylcholine! (Hyperkalemia assoc.) -Avoid NMB; consult with surgeon first -Position patient on bolsters in prone position -Recommend Sevoflurane/ Remifentanil -Closely monitor blood loss -No pre-op medication!
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Spina Bifida Aperta: Post-op management
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-Observe respirations after tight skin closure -Position patient prone or lateral -Observe for increased ICP! -Avoid narcotics when possible -Check Hgb/Hct -Check glucose
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Arnold-Chiari Malformation
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-Elongated and flattened medulla with cerebellar vermis that herniates below the plain of the foramen magnum (4th ventricle) with associated compression of the BRAIN STEM -Dysplastic brainstem with a characteristic "kink" -Hypoplastic tentorium with a small posterior fossa
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Arnold-Chiari Malformation: Present with
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-Vocal cord paralysis (Tracheostomy) -Swallowing dysfunction (G Button) -Recurrent pulmonary aspiration -Stridor -Depressed gag reflex -Possible apnea episodes
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Arnold-Chiari Malformation: Special anesthesia considerations
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-Position same as for posterior fossa exploration -Pre-op ventilation may be required and post-op apnea may occur -Impaired pulmonary function d/t aspiration Stridor may occur -Prone position with neck flexion -NASOTRACHEAL INTUBATION PREFERRED!!!
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Positioning for Surgical Procedure: Prone
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-Access to patient and airway; have emergency plan -Rolls at chest and pelvis; frees up the abdomen -Pad all extremities and hands, fingers, feet, toes as well as lines, EKG -Maintain stable hemodynamics -Position of head: Flexion of neck, Kink in ETT,Cervical Spinal Ischemia -Eye protection -ETT tube: Oral vs. Nasal** -Avoid oral airway!
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Encephalocele
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-Congenital defect of the neural tube with a sac like protrusion -Local herniation of NEURAL TISSUE through a defect in the skull covered with a membrane (Can appear anywhere from frontal area to occipital area of the head; Cysts can appear be as large as head (Brain material and CSF filled)
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Encephalocele: Special anesthesia considerations
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-Challenging airway/intubation -Positioning can be challenging -Keeping the patient warm
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Encephalocele: Anesthesia management
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-Position same as for posterior fossa exploration -Adequate IV access and Arterial line -Closely monitor blood loss (T&C)
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Craniofacial Reconstruction: Special anesthesia considerations
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-Difficult airways and intubations -May require pre-op tracheostomy -Excessive blood loss (seen as oozing) -Special precautions for positioning for long surgeries -Surgical manipulation may induce OCULAR-CARDIAC reflex -Unintentional perioperative extubation -May require RAPID emergence -Prepare to deal with extensive POST-OP SWELLING
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Craniofacial Reconstruction: Pre-op management
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-Thorough history required -Consider associated anomalies of syndromes -Thorough examination of AIRWAY* -Order and check required lab work -Ensure adequate blood is available -Order PRE-OP sedation: Midazolam (IV, POV, G-Button)
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Craniofacial Reconstruction: Anesthesia management
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1. Induction management -Anticipated Difficult Intubation -Anticipated Difficult Ventilation -Unanticipated Ventilation -Suture (oral) ETT in place -Armored tube/Reinforced tube (most secure) 2. Anesthesia maintenance: Minimal inhalational agent*; Narcotic/Steroid/NMB
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Craniofacial Reconstruction: Perioperative management
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-Controlled ventilation with invasive blood gas monitoring -10-15 degree head-up position -Pad pressure areas well (long cases) -Eye ointment and tape (Non-petroleum based if possible) -Reinforce monitor placement (opsites) -Prepare for MASSIVE BLOOD LOSS* -Assist in brain mass reduction -Assist with induced hypotension -Plan for an awake extubation in the operating room if needed
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Craniofacial Reconstruction: Monitor
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-capnography -CVP -ABG -temperature -blood loss -coagulation studies -urine output -precordial doppler -arterial line
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Craniofacial Reconstruction: Post-op management
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-Follow post craniotomy precautions -Extubate fully awake with no danger of airway obstruction due to edema -Use narcotics with caution -Check Hgb/ Hct/ Glucose/ Coagulation Studies -Observe for signs of increased ICP
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Craniosynostosis
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Premature ossification of the sutures of the skull.
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Prognathism
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Abnormal forward projection of one or both jaws. (Mandible and Maxilla)
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Macro/ Microsomia
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A condition of either small or large developed BODIES.
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Glossoptosis
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Retraction-downward placement of TONGUE
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Macro/Micrognathia
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Over or underdevelopment of the jaw. (Mandible)
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Scaphocephaly
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Congenital malformation of the skull; premature closure of skull results in abnormal NARROWING and LENGTHENING of SKULL.
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Macro/ Microglossia
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Over or undersized TONGUE
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Coloboma
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deformity of the OCULAR TISSUE affecting iris, ciliary body or choroid forming a cleft the extends inferiorly
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Apert's Syndrome
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Maxilla- midfacial hypoplasia Exophthalmus* Craniosynostosis Full forehead- flat occipital Strabismus Cleft lip- palate with a narrow palate Cervical fusion at C5-C6
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Apert's Syndrome: Description
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-Bamboo Trachea (narrow trachea with fused rings) -Progressive increased ICP -Congenital heart defects -Mental retardation -Large fontanels/late closure -Fusion of 2-3-4 fingers (Syndactyly)* -Short limbs
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Apert's Syndrome: Anesthesia considerations
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-Difficult mask fit -Difficult oral and nasal intubations: Poor dentation,Narrow nares -Respiratory complications: History of recent URI
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Crouzon's Syndrome
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Craniosynostosis* Hypoplastic maxilla Mandibular prognathism* High arched palate/cleft palate/lip "Parrot Beak" nose Frontal bossing*
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Crouzon's Syndrome: Description
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-Increased ICP (corrective surgery needed) -Shallow orbits-ocular proptosis -Exophthalmos; secondary to premature closures of the cranial sutures -Strabismus-Nystagmus -Conductive loss of hearing -Mouth breather
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Crouzon's Syndrome: Anesthesia considerations
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-Difficult mask ventilation -Eye protection -Post-op airway obstruction common -Elective pre-operative tracheostomy considered -Usually normal intubations unless external fixators are in place; limited opening of the mouth
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Down Syndrome(Trisomy 21)
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-Most frequent chromosomal aberration -Maternal age is factor -Result from Trisomy 21 or balanced/unbalanced chromosome 21
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Down Syndrome: Anomalies
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-CHD: (ASD,VSD, PDA, TOF) -Pulmonary hypertension secondary to CHD or chronic hypoxia d/t upper airway obstruction
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Down Syndrome: Description
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-Smaller for normal age -Microbracycephaly -Short necks -Epicanthal folds -Small low set ears -Macroglossia -Mandibular micrognathia -Broad flat nose -Generalized hypotonia
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Down Syndrome: Airway considerations
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-Obese "fluffy" -Difficult IV access: Small vessel sizes -Bradycardia/systemic hypotension on induction -Hypertrophic lymphatic tissue -Subglottic stenosis -Cervical spine disorders with vertebral and ligamentous instability (C1-C2) -Cervical spine injury -Predominate tongue -Post-op stridor
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Hallermann-Streiff Syndrome
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Mandibular- craniofacial dysostosis Mandibular hypoplasia Mandibular joint dislocation "Beak Like" narrow nose* High narrow palate Dental abnormalities Frontal bossing
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Hallermann-Streiff Syndrome: description
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-Abnormalities of skull -Frontal- Occipital- Parietal bossing -Delayed fontanel closure -Small facial features -Micrognathia -Microphthalmia (80%) -Milky cataracts (94%)
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Hallermann-Streiff Syndrome: Anesthesia considerations
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-Difficult mask airways -Difficult oral and nasal intubations -Normal tongues in small mouths -Eye protection -Brittle teeth* -TMJ dislocation -Prone to frequent URI
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Goldenhar Syndrome
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Anomalies are unilateral or asymmetrical facial muscles and bones (70%) Characterized by ear and eye abnormalities Maxillary hypoplasia Limited mouth opening Abnormal tongue and high cleft palate
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Goldenhar Syndrome: description
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-Micrognathia -Cervical spine synostosis -CHD -Strabismus/ microphthalmia -Subconjunctival lipomas -TEF -Preauricular skin tags* -Branchial cleft remnants on neck
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Goldenhar Syndrome: anesthesia considerations
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-Difficult mask airways -Oral intubation difficult especially with bilateral lesions -Limited mouth opening -Cervical spine defects -Problems with cardiac diseases -Awake extubation encouraged
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Hurler's Syndrome
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Macrocephaly Large lips Hypertrophic adenoids and tonsils Wide spread teeth - Short necks Mouth breathers
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Hurler's Syndrome: description
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-Scaphocephaly -Valvular cardiac disease -Respirations effected by deformed ribs, scoliosis and hepatomegaly -Corneal opacities -Hearing loss and increased middle ear infections -Increased ICP -Mental retardation
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Hurler's Syndrome: anesthesia considerations
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-Thorough assessment of cardiac status -Mask airway obstruction (lymphoid tissue) -Difficult airways and intubations -Possible Atlantoaxial subluxation -Possible tracheostomy -Possible post obstructive pulmonary edema -Prophylactic antibiotics for cardiac history -Administer Glycopyrrolate to dry airway
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Pierre-Robin Syndrome
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-Etiology defect early in utero- sporadic -Triad: Micrognathia, Glossoptosis, Mandibular dysplasia -Cleft palate or round contour palate
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Pierre-Robin Syndrome: description
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-Obstructive apnea may lead to Cor Pulmonale -Some mental retardation secondary to hypoxia or suffocation -CHD
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Pierre-Robin Syndrome: anesthesia considerations
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-Assess cardiac defects -Prophylactic antibiotics for cardiac defects -Neonates: upper airway obstruction leading to Cor Pulmonale -Difficult intubations -May require tracheostomy -May require tongue suture -Awake extubation
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Treacher-Collins Syndrome: description
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-CHD -Antimongoloid obliquity of the palpebral fissures (89%) -Microphthalmia -Coloboma of lower lids (67%) -Choanal atresia -Dwarfism -External ear defects and deafness (77%)
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Treacher-Collins Syndrome: Anesthesia considerations
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-Thorough assessment of cardiac function and airway -Antibiotic prophylaxis -Possible pre-op tracheostomy -Possible very difficult mask airway and tracheal intubation* -Awake extubation -Eye protection
