Pediatric airway obstruction


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Pediatric airway obstruction

  1. 1. Pediatric Airway Obstruction Ibrahim Habib Barakat , MD
  2. 2. The Pediatric Airway• Introduction• Normal Anatomy• Physiology• Airway evaluation• Management of normal vs. abnormal airway• Difficult airway
  3. 3. Introduction• Almost all of pediatric codes are due to respiratory origin• 80% of pediatric cardiopulmonary arrest are primarily due to respiratory distress• Majority of cardiopulmonary arrest occur at <1 year old
  4. 4. Normal Pediatric Airway Anatomy • Larynx composed of hyoid bone and a series of cartilages • Single: thyroid, cricoids, epiglottis • Paired: arytenoids, corniculates, and cuneiform
  5. 5. Pediatric Anatomy cont. Laryngeal folds consist of:• Paired aryepiglottic folds extend from epiglottis posteriorly to superior surface of arytenoids• Paired vestibular folds (false vocal cords) extend from thyroid cartilage posteriorly to superior surface of arytenoids• Paired vocal folds (true vocal cords) extend from posterior surface of thyroid plate to anterior part of arytenoids• Interarytenoid fold bridging the arytenoid cartilages• Thyrohyoid fold extend from hyoid bone to thyroid cartilage
  6. 6. Pediatric Anatomy cont.Sensory Innervation: Recurrent Laryngeal Nerve-supraglottic larynx Internal Branch of Superior Laryngeal Nerve- infraglottic larynxMotor Innervation: External branch of Superior Laryngeal Nerve- cricothyroid muscle Recurrent Laryngeal Nerve-all other laryngeal musclesBlood Supply Laryngeal branches of the superior and inferior thyroid arteries
  7. 7. 5 Differences between Pediatric and Adult Airway• More rostral larynx• Relatively larger tongue• Angled vocal cords• Differently shaped epiglottis• Funneled shaped larynx-narrowest part of pediatric airway is cricoid cartilage
  8. 8. More rostral pediatric larynxLaryngeal apparatus develops from brachial clefts anddescends caudallyInfant’s larynx is higher in neck (C2-3) compared to adult’s(C4-5)
  9. 9. Relatively larger tongue• Obstructs airway• Obligate nasal breathers• Difficult to visualize larynx• Straight laryngoscope blade completely elevates the epiglottis, preferred for pediatric laryngoscopy
  10. 10. Angled vocal cords• Infant’s vocal cords have more angled attachment to trachea, whereas adult vocal cords are more perpendicular• Difficulty in nasal intubations where “blindly” placed ETT may easily lodge in anterior commissure rather than in tracheaImage from:
  11. 11. Differently shaped epiglottis Adult epiglottis broader, axis parallel to trachea
  12. 12. Differently shaped epiglottis Infant epiglottis ohmega (Ώ) shaped and angled away from axis of trachea
  13. 13. Differently shaped epiglottis More difficult to lift an infant’s epiglottis with laryngoscope blade
  14. 14. Funneled shape larynxADULT INFANT • narrowest part of infant’s larynx is the undeveloped cricoid cartilage, whereas in the adult it is the glottis opening (vocal cord) • Tight fitting ETT may cause edema and trouble upon extubation
  15. 15. Funneled shape larynxADULT INFANT • Uncuffed ETT preferred for patients < 8 years old • Fully developed cricoid cartilage occurs at 10-12 years of age
  16. 16. Pediatric Respiratory Physiology• Pulmonary surfactant produced by Type II pneumocytes at 24 wks GA• Sufficient pulmonary surfactant present after 35 wks GA• Premature infants prone to respiratory distress syndrome (RDS) because of insufficient surfactant• Betamethasone can be given to pregnant mothers at 24-35wks GA to accelerate fetal surfactant production
  17. 17. Pediatric Respiratory Physiology• Extrauterine life not possible until 24-25 weeks of gestation• Two types of pulmonary epithelial cells: Type I and Type II pneumocytes • Type I pneumocytes are flat and form tight junctions that interconnect the interstitium • Type II pneumocytes are more numerous, resistant to oxygen toxicity, and are capable of cell division to produce Type I pneumocytes
  18. 18. Pediatric Respiratory Physiology cont.• Work of breathing for each kilogram of body weight is similar in infants and adult• Oxygen consumption of infant (6 ml/kg/min) is twice that of an adult (3 ml/kg/min)• Greater oxygen consumption = increased respiratory rate•• Tidal volume is relatively fixed due to anatomic structure
  19. 19. Pediatric Respiratory Physiology cont.• Minute alveolar ventilation is more dependent on increased respiratory rate than on tidal volume• Lack Type I muscle fibers, fatigue more easily• FRC of an awake infant is similar to an adult when normalized to body weight• Ratio of alveolar minute ventilation to FRC is doubled, under circumstances of hypoxia, apnea or under anesthesia, the infant’s FRC is diminished and desaturation occurs more precipitously
  20. 20. Physiology: Effect Of Edema Poiseuille’s law R = 8nl/ πr4 If radius is halved, resistance increases 16 xImage from:
  21. 21. Normal Inspiration and Expiration turbulenceImage from:
  22. 22. Obstructed Airways turbulence & wheezing
  23. 23. Evaluation of acute upper airway obstruction in children
  24. 24. Airway Evaluation Medical History• URI predisposes to coughing, laryngospasm, bronchospasm, desat during anesthesia• Snoring or noisy breathing (adenoidal hypertrophy, upper airway obstruction, OSA)• Chronic cough (subglottic stenosis, previous tracheoesohageal fistula repair)• Productive cough (bronchitis, pneumonia)• Sudden onset of new cough (foreign body aspiration)
  25. 25. Airway Evaluation Medical History• Inspiratory stridor (macroglossia, laryngeal web, laryngomalacia, extrathoracic foreign body)• Hoarse voice (laryngitis, vocal cord palsy, papillomatosis)• Asthma and bronchodilator therapy (bronchospasm)• Repeated pneumonias (GERD, CF, bronchiectasis, tracheoesophageal fistula, immune suppression, congenital heart disease)• History of foreign body aspiration
  26. 26. Airway Evaluation Medical History• Previous anesthetic problems (difficulty intubation/extubation or difficulty with mask ventilation)• Atopy, allergy (increased airway reactivity)• History of congenital syndrome (Pierre Robin Sequence, Treacher Collins, Klippel-Feil, Down’s Syndrome, Choanal atresia)• Environmental: smokers
  27. 27. Signs of Impending Respiratory Failure • Increase work of breathing • Tachypnea/tachycardia • Nasal flaring • Drooling • Grunting • Wheezing • Stridor
  28. 28. Supraglottic Glottic Subglottic TracheaSound Sonorous, gurgling Biphasic stridor High-pitched stridor Coarse, Inspiratory stridor expiratory stridor,Structures Nose / Pharynx / Epiglottis Larynx Subglottic trachea Vocal cordsCongenital Micrognathia ,Pierre Robin Laryngomalacia Subglottic stenosis Macroglossia, Vocal cord paralysis Tracheomalacia Down syndrome Laryngeal web Tracheal stenosis Storage disease Laryngocele Vascular ring Choanal atresia Hemangioma cyst Lingual thyroid Thyroglossal cystAcquired Adenopathy Papillomas Croup Tonsillar hypertrophy Foreign body Bacterial tracheitis Foreign body Subglottic stenosis Pharyngeal abscess Foreign body Epiglottitis Causes of Stridor: Anatomic Location, Sound, and Etiology
  29. 29. Signs of Impending Respiratory Failure • Head bobbing • Use of accessory muscles/retraction of muscles • Cyanosis despite O2 • Irregular breathing/apnea • Altered consciousness/agitation • Inability to lie down • Diaphoresis
  30. 30. Causes of acute upper airway obstruction that are commonly life-threatening Epiglottitis Retropharyngeal abscess Bacterial tracheitis Croup Foreign body Anaphylaxsis Neck trauma Burns thermal or caustic
  31. 31. Airway Evaluation Physical Exam• Facial expression• Nasal flaring• Mouth breathing• Drooling• Color of mucous membranes• Retraction of suprasternal, intercostal or subcostal• Respiratory rate• Voice change• Mouth opening• Size of mouth
  32. 32. Airway Evaluation Physical Exam• Mallampati• Loose/missing teeth• Size and configuration of palate• Size and configuration of mandible• Location of larynx• Presence of stridor (inspiratory/expiratory)• Baseline O2 saturation• Global appearance (congenital anomalies)• Body habitus
  33. 33. Diagnostic Testing• Laboratory and radiographic evaluation extremely helpful with pathologic airway• AP and lateral films and fluoroscopy may show site and cause of upper airway obstruction• MRI/CT more reliable for evaluating neck masses, congenital anomalies of the lower airway and vascular system
  34. 34. Imaging may be useful in identifying the location and nature of the airway obstruction but should never interferewith the stabilization of a child with a critical obstruction.
  35. 35. Diagnosis …. ? Retropharyngeal abscess
  36. 36. Abnormal retropharyngeal space: Retropharyngeal space : >7 mm @ C2 Retrotracheal space : 14 mm@ C6 .. Ped 22 mm @ C6 .. Adult
  37. 37. F. B. ingestion
  38. 38. MRI/CTUsually not useful in an acute settingMore reliable for evaluating neck masses and congenital anomalies of the lower airway and vascular system
  39. 39. Diagnostic Testing• Perform radiograph exam only when there is no immediate threat to the child’s safety and in the presence of skilled personnel with appropriate equipment to manage the airway• Intubation must not be postponed to obtain radiographic diagnosis when the patient is severely compromised.• Blood gases are helpful in assessing the degree of physiologic compromise; however, performing an arterial puncture on a stressed child may aggravate the underlying airway obstruction
  40. 40. Flexible Laryngoscopy: Proper Equipment Assess nares/choanae Assess adenoid and lingual tonsil Assess TVC mobility Assess laryngeal structures
  41. 41. Airway Management: Normal Airway • Challenging because of unique anatomy and physiology • Goals: protect the airway, adequately ventilate, and adequately oxygenate • Failure to perform any ONE of these tasks will result in respiratory failure • Positioning is key!
  42. 42. Bag-Mask Ventilation •Clear, plastic mask with inflatable rim •Place fingers on mandible to avoid provides atraumatic seal compressing pharyngeal space •Hand on ventilating bag at all times to •Proper area for mask application-bridge monitor effectiveness of spontaneous breaths of nose extend to chin •Continous postitive pressure when needed •Maintain airway pressures <20 cm H2O to maintain airway patencyImage from:
  43. 43. Oropharyngeal Airway PROPER SIZE POSITIONImage from:
  44. 44. Oropharyngeal Airway Placement Image from:
  45. 45. Nasopharyngeal Airway •Distance from nares to angle of mandible approximates the proper length •Nasopharyngeal airway available in 12F to 36F sizes •Shortened endotracheal tube may be used in infants or small children •Avoid placement in cases of hypertrophied adenoids - bleeding and traumaImage from:
  46. 46. Nasopharyngeal Airway •Distance from nares to angle of mandible approximates the proper length •Nasopharyngeal airway available in 12F to 36F sizes •Shortened endotracheal tube may be used in infants or small children •Avoid placement in cases of hypertrophied adenoids - bleeding and traumaImage from:
  47. 47. Sniffing PositionPatient flat on operating table, the oral (o),pharyngeal (P), and tracheal (T) axis passthroughthree divergent planesA blanket placed under the occiput aligns thepharyngeal (P) and tracheal (T) axesExtension of the atlanto-occipital joint aligns theoral (O), pharyngeal (P), and tracheal (T) axes Image from:
  48. 48. Selection of laryngoscope blade: Miller vs. Macintosh• Miller blade is preferred for infants and younger children• Facilitates lifting of the epiglottis and exposing the glottic opening• Care must be taken to avoid using the blade as a fulcrum with pressure on the teeth and gums• Macintosh blades are generally used in older children• Blade size dependent on body mass of the patient and the preference of the anesthesiologist
  49. 49. Endotracheal Tube Age Wt ETT(mm ID) Length(cm)Preterm 1 kg 2.5 6 1-2.5 kg 3.0 7-9Neonate-6mo 3.0-3.5 106 mo-1 3.5-4.0 111-2 yrs 4.0-5.0 12
  50. 50. Endotracheal Tube New AHA Formulas: Uncuffed ETT: (age in years/4) + 4 Cuffed ETT: (age in years/4) +3 ETT depth (lip): ETT size x 3
  51. 51. Complications of Endotracheal Intubation • Postintubation Croup • Incidence 0.1-1% • Risk factors: large ETT, change in patient position introp, patient position other than supine, multiple attempts at intubation, traumatic intubation, pts ages 1-4, surgery >1hr, coughing on ETT, URI, h/o croup • Tx: humidified mist, nebulized racemic epinephrine, steroid
  52. 52. Complications of Endotracheal Intubation• Laryngotracheal (Subglottic) Stenosis • Occurs in 90% of prolonged endotracheal intubation • Lower incidence in preterm infants and neonates due to relative immaturity of cricoid cartilage • Pathogenesis: ischemic injury secondary to lateral wall pressure from ETT edema, necrosis, and ulceration of mucosa, infx • Granulation tissues form within 48hrs leads to scarring and stenosis
  53. 53. Cuff vs Uncuffed Endotracheal Tube• Controversial issue• Traditionally, uncuffed ETT recommended in children < 8 yrs old to avoid post-extubation stridor and subglottic stenosis• Arguments against cuffed ETT: smaller size increases airway resistance, increase work of breathing, poorly designed for pediatric pts, need to keep cuff pressure < 25 cm H2O• Arguments against uncuffed ETT: more tube changes for long-term intubation, leak of anesthetic agent into environment, require more fresh gas flow > 2L/min, higher risk for aspiration
  54. 54. Cuff vs Uncuffed Endotracheal Tube -Concluding Recommendations-• For “short” cases when ETT size >4.0, choice of cuff vs uncuffed probably does not matter• Cuffed ETT preferable in cases of: high risk of aspiration (ie. Bowel obstruction), low lung compliance (ie. ARDS, pneumoperitoneum, CO2 insufflation of the thorax, CABG), precise control of ventilation and pCO2 (ie. increased intracranial pressure, single ventricle physiology)
  55. 55. Laryngeal Mask Airway• Supraglottic airway device developed by Dr. Archie Brain• Flexible bronchoscopy, radiotherapy, radiologic procedures, urologic, orthopedic, ENT and ophthalmologic cases are most common pediatric indications for LMA• Useful in difficult airway situations, and as a conduit of drug administration (ie. Surfactant)• Different types of LMAs: Classic LMA, Flexible LMA, ProSeal LMA, Intubating LMA• Disadvantages: Laryngospasm, aspiration
  56. 56. Laryngeal Mask AirwayLMA size Weight Max cuff volume (mL) ETT (mID) 1 .0 Neonate/Infants ≤ 5kg 4 3.5 1.5 Infants 5-10kg 7 4.0 2.0 Infants/children 10-20kg 10 4.5 2.5 Children 20-30kg 14 5.0 3.0 Children/small adult > 30kg 20 6.0 cuff 4.0 Normal/large adolescent/adult 30 7.0 cuff 5.0 Large adolescent/adult 40 8.0 cuff
  57. 57. Other Supraglottic Devices• Laryngeal tube • Latex-free, single-lumen silicone tube, which is closed at distal end • Two high volume-low pressure cuffs, a large proximal oropharyngeal cuff and a smaller distal esophageal cuff • Both cuffs inflated simultaneously via a single port • Situated along length of oropharynx with distal tip in esophagus • Sizes 0-5, neonates to large adults (only sizes 3-5 available in US) • Limited data available for its use in children
  58. 58. Other Supraglottic Devices• Cobra Perilaryngeal Airway • Perilaryngeal airway device with distal end shaped like a cobra-head • Positioned into aryepiglottic folds and directly seats on entrance to glottis • Inflation of the cuff occludes the nasopharynx pushing the tongue and soft tissues forward and preventing air leak • Available in sizes pediatric to adult ½ to 6 • No studies currently available evaluating this device in children
  59. 59. Difficult Airway Management Techniques • Rigid bronchoscopy • Flexible bronchoscopy • Direct laryngoscopy • Intubating LMA • Lighted stylet • Bullardscope • Fiberoptic intubation • Surgical airway
  60. 60. TracheotomyCricothyroidotomy is difficult b/c of small membrane and flexibilityEarly complications Pneumothorax, bleeding, decannulation, obstruction, infectionsLate complications Granuloma, decannulation, SGS, tracheocutaneous fistula
  61. 61. Airway ManagementClassification of Abnormal Pediatric Airway • Congenital Neck Masses (Dermoid cysts, cystic teratomas, cystic hygroma, lymphangiomas, neurofibroma, lymphoma, hemangioma) • Congenital Anomalies (Choanal atresia,tracheoesophageal fistula, tracheomalacia, laryngomalacia, laryngeal stenosis, laryngeal web, vascular ring, tracheal stenosis) • Congenital Syndromes (Pierre Robin Syndrome, Treacher Collin, Turner, Down’s, Goldenhar , Apert, Achondroplasia, Hallermann- Streiff, Crouzan)
  62. 62. Airway ManagementClassification of Abnormal Pediatric Airway • Inflammatory (Epiglottitis, acute tonsillitis, peritonsillar abscess,retropharyngeal abscess, laryngotracheobronchitis,bacterial tracheitis,adenoidal hypertrophy,nasal congestion, juvenile rheumatoid arthritis) • Traumatic/Foreign Body (burn,laceration,lymphatic/venous obstruction,fractures/dislocation, inhalational injury, postintubation croup (edema),swelling of uvula • Metabolic (Congenital hypothyroidism, mucopolysaccharidosis, Beckwith-Wiedemann Syndrome,glycogen storage disease, hypocalcemia laryngospasm)
  63. 63. Congenital Neck Masses
  64. 64. Congenital Neck Masses
  65. 65. Congenital AnomaliesTracheoesphageal Fistula
  66. 66. Congenital Anomalies Tracheoesphageal Fistula• Feeding difficulties (coughing, choking and cyanosis) and breathing problems• Associated with congenital heart (VSA, PDA, TOF), VATER, GI, musculoskeletal and urinary tract defects• Occurs in 1/ 3000-5000 births• Most common type is the blind esophageal pouch with a fistula between the trachea and the distal esophagus (87%)
  67. 67. Congenital AnomaliesTracheoesphageal Fistula Radiograph of a neonate with suspected esophageal atresia. Note the nasogastric tube coiled in the proximal esophageal pouch (solid arrow). The prominent gastric bubble indicates a concurrent tracheoesphageal fistula (open arrow)
  68. 68. Congenital Anomalies Choanal Atresia• Complete nasal obstruction of the newborn• Occurs in 0.82/10 000 births• During inspiration, tongue pulled to palate, obstructs oral airway• Unilateral nare (right>left)• Bilateral choanal atresia is airway emergency• Death by asphyxia• Associated with other congenital defects
  69. 69. Congenital Anomalies Choanal Atresia
  70. 70. Clinical manifestations• - unilateral : Asymptomatic for along period till the first attack of upper respiratory tract infection,diagnosis may be suggested by nasal discharge or persistent nasal obstruction .• - bilateral : Difficulty with mouth breathing make vigerous attempts to inspire , often suck in their lips ,and develop cyanosis . Distressed children then cry ( which relieve the cyanosis ) and become more calm with normal skin colour , only to repeat the cycle after closing their mouths . Those who are able to breath through their mouths at once experience difficulty when suckling and swallowing , becoming cyanotic when they attempt to feed .
  71. 71. diagnosis• - inablity to pass afirm cather 3 to 4 cm into the nasopharynx through each nostril .• - the atritic plate can be seen directly with fibro optic rhinoscopy .• -the anatomy is best evaluated by using high resolution CT scan .
  72. 72. treatment• - bilateral : -• - An oral airway or intubation .• - trasnasal endoscopic surgical repair .• - stents are usually left in place after the repair for weeks to prevent restenosis or closure .• - tracheostomy should be considered in child has other potentially life threatening problems and in whom early surgical repair of the choanal atresia may not be appropriate or feasible .
  73. 73. treatment• - unilateral :-• - operative correction may be deffered for several years• - in both nuilateral or bilateral cases , restenosis necessitating dilatation or reoperation , or both , is common .• - mitomycin-c has been used to help prevent the development of granulation tissue and stenosis .
  74. 74. Congenital Syndromes Pierre Robin Sequence• Occurs in 1/8500 births• Autosomal recessive• Mandibular hypoplasia, micrognathia, cleft palate, retraction of inferior dental arch, glossptosis• Severe respiratory and feeding difficulties• Associated with OSA, otitis media, hearing loss, speech defect, ocular anomalies, cardiac defects, musculoskeletal (syndactyly, club feet), CNS delay, GU defects)
  75. 75. Congenital SyndromesPierre Robin Sequence
  76. 76. Congenital SyndromeTreacher Collins Syndrome• Mandibulofacial dysotosis• Occurs in 1/10 000 births• Cheek bone and jaw bone underdeveloped• External ear anamolies, drooping lower eyelid, unilateral absent thumb• Respiratory difficulties• Underdeveloped jaw causes tongue to be positioned further back in throat (smaller airway)• Associated with OSA, hearing loss, dry eyes
  77. 77. Congenital SyndromeTreacher Collins Syndrome
  78. 78. Congenital Syndrome Down’s Syndrome• Trisomy 21• Occurs in 1/660 births• Short neck, microcephaly, small mouth with large protruding tongue, irregular dentition, flattened nose, and mental retardation• Associated with growth retardation, congenital heart disease, subglottic stenosis, tracheoesophageal fistula, duodenal atresia, chronic pulmonary infection, seizures, and acute lymphocytic leukemia• Atlantooccipital dislocation can occur during intubation due to congenital laxity of ligaments
  79. 79. Congenital Syndrome Down’s Syndrome
  80. 80. Inflammatory (Epiglottitis)• Etiology: Haemophilus influenzae type B• Occurs in children ages 2-6 years• Disease of adults due to widespread H. influenza vaccine• Progresses rapidly from a sore throat to dysphagia and complete airway obstruction (within hours)• Signs of obstruction: stridor, drooling, hoarseness, tachypnea, chest retraction, preference for upright position• OR intubation/ENT present for emergency surgical airway• Do NOT perform laryngoscopy before induction of anesthesia to avoid laryngospasm• Inhalational induction in sitting position to maintain spontaneous respiratory drive (Sevo/Halothane)• Range of ETT one-half to one size smaller
  81. 81. Inflammatory
  82. 82. The treatment of epiglottitis.Instrumentation: Avoid instrumentation. In suspectedepiglottitis.Specialist consult: An anesthesiologist or otorhinolaryngologistshould be involved early in the management of epiglottitis.Monitoring: Patients must be monitored for respiratory fatiguevisually and with continuous pulse oximetry. Accessibility toequipment and expertise for immediate intubation is requiredin the event of respiratory failure. If endotracheal intubation isnot possible, cricothyroidotomy may be required.Oxygen: Oxygen is administered according to pulse oximetryresults. Dry air may worsen inflammation. Use of humidifiedoxygen or a room humidifier is recommended.Antibiotics: Presumptive intravenous antibiotics are indicated,tailored to results from blood cultures.
  83. 83. The treatment of epiglottitis.Glucocorticoids: Either intravenous or inhaled glucocorticoids are sometimes given to reduce inflammation. However, controlled trials of the effectiveness of this approach in epiglottitis are limited.Volume deficits: Correct volume deficits with intravenous fluids.Sedatives: Avoid sedatives that may suppress the respiratory drive.Other medications: In patients with croup, aerosolized racemic epinephrine is sometimes used to reduce mucosal edema; however, the role of this drug in persons with epiglottitis is not defined. Adverse events have been reported in patients with epiglottitis.24 Beta-2 agonists are not typically used in patients who do not have asthma
  84. 84. The treatment of epiglottitis.Glucocorticoids: Either intravenous or inhaled glucocorticoids are sometimes given to reduce inflammation. However, controlled trials of the effectiveness of this approach in epiglottitis are limited.Volume deficits: Correct volume deficits with intravenous fluids.Sedatives: Avoid sedatives that may suppress the respiratory drive.Other medications: In patients with croup, aerosolized racemic epinephrine is sometimes used to reduce mucosal edema; however, the role of this drug in persons with epiglottitis is not defined. Adverse events have been reported in patients with epiglottitis.24 Beta-2 agonists are not typically used in patients who do not have asthma
  85. 85. Inflammatory• Etiology: Parainfluenza virus• Occurs in children ages 3 months to 3 years• Barking cough• Progresses slowly, rarely requires intubation• Medically managed with oxygen and mist therapy, racemic epinephrine neb and IV dexamethasone (0.25-0.5mg/kg)• Indications for intubation: progressive intercostal retraction, obvious respiratory fatigue, and central cyanosis
  86. 86. Inflammatory
  87. 87. Croup / steepling of the subglottic trachea
  88. 88. MetabolicBeckwith-Wiedemann Syndrome • Occurs in 1/13000-15000 births • Chr 11p.15.5 • Autosomal dominant • Macroglossia, Exomphalos, Gigantism • Associated with mental retardation, organomegaly, abdominal wall defect, pre- and postnatal overgrowth, neonatal hypoglycemia, earlobe pits, Wilms tumor
  89. 89. MetabolicBeckwith-Wiedemann Syndrome
  90. 90. Management
  91. 91. Management of complete airway obstruction in children
  92. 92. Management of severe upper airway obstruction in children