4/18/2015 Laryngomalacia Diagnosis & Management Prof. Alaa Gaafar, MD Otolaryngology – H&N Surgery Department A l e x a n d r i a U n i v e r s i t y, E g y p t Infantile Larynx Adult Larynx 1 4/18/2015 Laryngomalacia Laryngo = larynx, malacia = abnormal flaccidity. Collapse of the supraglottic components during inspiration: Epiglottis. Aryepiglottic folds. Arytenoids. Types Infantile Laryngomalacia: Symptoms occur early in life. Improve by the age of 2 years Late onset Laryngomalacia: Children > 2 years. Present during feeding, exercise or sleep. Presented by snoring or OSA. State Dependent Laryngomalacia: Older age group / Adults. Occurs only during sleep. Diagnosed by sleep endoscopy. 2 4/18/2015 Incidence • Most common cong laryngeal anomaly (60%). • Most common cause of chronic stridor in infants. • More common in males. • Associated with GERD in 75 - 80% of cases. • Synchronous airway anomalies in 15 - 20% of cases. • Associated with congenital anomalies in 10% of cases. Pathophysiology Anatomical Abnormalities 1. Omega shaped epiglottis. 2. Short AE fold: AE/Glottic length 0.5 = N 0.3 = severe LM 3. Redundant mucosa over arytenoids. *Manning et al, Arch Otolaryngol H N Surg 2005;131(4):340-3. 3 4/18/2015 GERD & Laryngomalacia GERD can be invited by laryngomalacia as a result of increased negative intrathoracic Laryngomalacia pressure during inspiration. The presence of GERD can aggravate laryngomalacia. *Gastroesophageal Reflux Disease in Infants by Stuart Morgenstein, DO (CME – online offering) GERD Classification of Infantile Laryngomalacia* Primary Laryngomalacia Secondary Laryngomalacia Anatomical anomalies narrow Reflux induce edema and the laryngeal inlet. narrowing of the larynx. Reflux 2ry to –ve ITP. Reflux is the inciting factor. Usually need surgical Usually respond to medical treatment. treatment. *Kelly PE, et al. Operative Techniques in Otolaryngology (2005) 16, 198-202 4 4/18/2015 Clinical presentation Stridor: High pitched, cyclic inspiratory stridor. Starts few weeks after birth increase in severity over 6-9 months Resolves spontaneously 18 – 24 months in 80 - 90% of cases. Increased on supine, feeding & agitation. Decreased on neck extension & prone position. Voice = normal. Feeding = normal (interrupted in severe cases/ ass. with reflux). Laryngoscopy Local or General anesthesia No muscle relaxant. No endotracheal tube. 5 4/18/2015 Endoscopic findings Omega shaped epiglottis. Short AE fold. Redundant mucosa over arytenoids. Signs of GERD. Collapse of supraglottic structure:* Type A: Posterolateral collapse = AE folds & arytenoids. Type B: Complete collapse = AE folds + Arytenoids + Epiglottis. Type C: Anterior collapse = Epiglottis. *Holinger & Konior, 1989. Laryngoscopy Type A 6 4/18/2015 Laryngoscopy Type B Laryngoscopy Type C 7 4/18/2015 Treatment of Laryngomalacia Conservative Surgical 90% 10% Conservative Treatment Assurance of parents. Anti reflux measures: Type & position of feeding. Lansoprazole = 0.5 mg/Kg once daily. Domperidone = 1 mg/kg/day before feeding. Short course of CS (e.g. URTI) Follow up: Monitor growth. Respiratory distress. Blood gases. 8 4/18/2015 Surgical Treatment Indications: Severe stridor, cyanotic attacks. Weight loss, failure to thrive. Recurrent chest infection. Chest deformity. Cor pulmonale. Hypoxia, Hypercarbia. Surgical Treatment I) Tracheostomy. Only in severe cases associated with other cong. anomalies Side effects: High morbidity. Affects phonation. Infection. Psychic Disturbance. II) Supraglottoplasty 9 4/18/2015 Surgical Treatment II) Supraglottoplasty: (microscissor, laser, microdebrider) Aryepiglottoplasty = Division of AE folds. Trimming of epiglottis. Vaporization of arytenoid mucosa. Epiglottopexy. Preoperative tips Do we need to do laryngobronchoscopy ? What type of supraglottoplasty ? Cold instruments or Laser ? Which type of Laser ? Unilateral or bilateral ? How to start ? 10 4/18/2015 Do we need to do laryngobronchoscopy ? Synchronous Airway Lesions in 18.9 % Clinically significant SALs in 4.7 % SALs need intervention in 3.9 % Mancuso et al, 1996 What type of surgery ? LM Type A AE fold lysis Type B Epiglottoplasty Type C Epiglottopexy+AE *Holinger & Konior, 1989. 11 4/18/2015 Cold instruments or Laser ? Cold Instruments: No thermal trauma. Risk of bleeding. Laser: Precise cutting. No bleeding. Thermal trauma fibrosis. Which type of laser ? CO2 laser. Diode KTP. Nd: YAG. 12 4/18/2015 Unilateral or Bilateral ? Bilateral: Better results. Postoperative supraglottic stenosis (3%) Unilateral: May need revision session (10%). No supraglottic stenosis. How to start ? 13 4/18/2015 Supraglottoplasty – Diode Laser Laser Supraglottoplasty 14 4/18/2015 Postoperative management Postop. endotracheal intubation ?? AE cutting no need for intubation. Epiglottoplasty intubation for one day. Ward admission for 2 days. Postoperative antibiotic, CS and pain killer for 5 days. Anti-reflux measures. Prognosis 15 4/18/2015 Prognosis Patients without associated anomalies: Immediate improvement of symptoms in 70% of cases. Improvement of symptoms within one week in 100% of cases. Patients with associated anomalies: Downs, CHARGE syndrome Delayed or failure of improvement. Complications Mild: Transient aspiration. Intraoperative bleeding. Granuloma, edema formation. Severe: Supraglottic stenosis. Persistence of symptoms 8% (4% needs revision surgery) 16 4/18/2015 Conclusion Although it has a typical presentation, Laryngoscopy is the main diagnostic tool for laryngomalacia. Treatment of GERD is mandatory for control of symptoms. Surgical treatment is indicated in minority of cases. Supraglottoplasty is an effective and safe procedure for relief of symptoms of severe laryngomalacia. Prof.. Alaa Gaafar 17