Laryngeal Cleft (from Pezzettigotta et al.

Oto Clinics of NA, 2008) rare, congenital laryngeal anomaly o fissure between the laryngotracheal and the pharyngoesophageal systems 60% are seen in conjunction with other abnormalities o tracheoesophageal fistula o tracheomalacia

Embryology Primitive foregut gives off respiratory primordium o respiratory primordium forms primitive trachea, bronchi, and lung buds o a tracheoesophageal septum (mesenchyme between respiratory and digestive tubes) forms o Controlled apoptosis and involution of tracheoesophageal setpum necessary for normal formation of division between larynx, trachea, and esophagus Theories for formation of laryngeal cleft o intraembryonic - pressure causing displacement of esophagus o epithelial occlusion lack of recanalization of the esophagus o vascular occlusion abnormal persistence of aberrant vessel o differential cell growth

Epidemiology 0.5% to 1.6% of laryngeal abnormalities (1/2000) Rare but probably underestimated: o minor laryngeal clefts are asymptomatic o endoscopic diagnosis is difficult o severe clefts can be fatal before diagnosis Males > Females Classification based on level of cleft Benjamin and Inglis Classification Type I supraglottic interarytenoid cleft ABOVE level of TVC Type II cleft extends below the TVC into the upper cricoid cartilage Type III cleft extends through the cricoid cartilage +/- extension into cervical trachea (extrathoracic) Type IV cleft extends into the thoracic trachea and extends toward the carina (intrathoracic) Type O = Submucosal Clefts

Symptoms Three main types of symptoms: o Swallowing aspiration, cyanosis during feeding, chronic cough o Pharyngolaryngeal stridor, toneless cry or weak voice, pharyngeal hypersecretions o Respiratory dyspnea, recurrent aspiration, respiratory distress Milder with milder clefts o Stridor, toneless or hoarse cry, pharyngeal hypersecretions, swallowing disorders, cough, dyspnea, and cyanosis during feeding o NO impact on pulmonary tract (no recurrent aspirations) Type II and III clefts have more aspiration, recurrent pneumonias Type IV present with early respiratory distress at birth, bronchial flooding, difficulty maintain proper ventilation Differential Diagnosis Must rule out: o TEF o Laryngomalacia o Laryngeal mobility disorder

o GER o Central neurogenic swallowing disorders Associated conditions (co-existing) o laryngomalacia 5% to 33% o tracheobronchial dyskinesia (flattening of trachea by >50% of size during inspiration) o tracheobronchomalacia o GER (19% to 70%) o Syndromes: G syndrome Pallister Hall syndrome VACTERL CHARGE

Diagnosis Endoscopy o Office-based flexible scope can detect type I clefts along with laryngomalacia o Definitive microlaryngoscopy/direct laryngoscopy + bronchoscopy needed Allow spontaneous ventilation to detect tracheobronchial dyskinesia or tracheobronchomalacia 1% xylocaine is applied topically laryngeal cleft may be hidden by redundant mucosa between the esophagus and trachea Other investigations o genetic consultation o karyotype o cardiac ultrasound o renal ultrasound o radiographs of the spine Management Goals o Prevent pulmonary complications Reduce aspiration of gastric contents Antireflux treatment (PPI) Thickening of feedings Upright position during feeding May need NG feeding for severe clefts Type III and IV clefts may need TPN, G-tube insertion, fundoplication o Address existing respiratory/pulmonary symptoms ventilatory assistance

chest physiotherapy antibiotics Surgical Management o Endoscopic Repair Benefits: avoid skin incision, avoid laryngofissure or pharyngotomy Setting: general anaesthesia, spontaneous ventilation, topic xylocaine Type I can have endotrcheal tube in place, but spontaneous ventilation offers better exposure Equipment suspension laryngoscopy Endoscopic needle driver and knot pusher 4-0 to 6-0 absorbable suture 6 8 mm half curve needle Procedure: denude mucosal margin of cleft (CO2 laser) Develop mucosal flaps on either side using microscissors Double layer closure with sutures from distal to proximal Can extubate immediately Feed after 5 to 14 days o Open Technique (mainly for Type III and IV clefts) lateral approach with lateral pharyngotomy lateral approach with posterior pharyngotomy (rarely used) anterior translaryngotracheal approach (procedure of choice for Type III and IV) Thoracic approaches right posterolateral thoracotomy combined with right cervical approach partial upper stenotomy combined with cervical incision anterior cervicothoraci approach