xRead - February 2023

Zalzal & Zalzal

310

Depending on severity, endoscopic lysis of the laryngeal web or open reconstruction is an effective treatment. Severe cases of laryngeal atresia may require an ex utero intrapartum treatment procedure to secure an airway with a tracheotomy upon birth. Laryngeal Cleft and Tracheoesophageal Fistulas Stridor secondary to laryngeal cleft is rare, with an incidence of less than 0.1%. 32 The condition develops as an incomplete partition of the tracheoesophageal septum, resulting in a communication of the posterior larynx and esophagus. The condition can be present in isolation or can be associated with several syndromes, including Opitz-Frias, Pallister-Hall, and VACTERL. There are multiple classification schemes of the laryngeal cleft, all based on the length of the incomplete partition. The Benjamin-Ingles scale is one such classification system, where the laryngeal cleft ex tends deep into the interarytenoid space above the vocal cords (type I), into the cricoid cartilage (type II), through the cricoid cartilage (type III), and into the intrathoracic tra cheoesophageal wall (type IV). 33 Symptoms correlate with the length of the cleft, but some children can be asymptomatic. Children with symptomatic disease will have inspiratory stridor, cyanosis, aspiration, and recurrent pneumonia episodes. The diag nosis is suspected by flexible fiber-optic laryngoscopy and documented by direct laryngoscopy with bronchoscopy, with particular attention to palpation of the interar ytenoid area. A modified barium swallow study not only supports the endoscopic diag nosis but also objectively assesses aspiration. Children with a small type I laryngeal cleft are treated initially with conservative management, including improved feeding positioning, acid reflux control, and liquid thickener in feeds. In patients who have failed conservative management or who have pulmonary sequalae, augmentation with interarytenoid injection or definitive surgical repair, depending on size of the cleft, is recommended. 34 Clefts extending to or through the cricoid (types II, III, and IV) will require surgical repair. Tracheoesophageal fistula (TEF) is the most common congenital anomaly affecting the esophagus when presenting with esophageal atresia (EA). Like laryngeal clefts, TEF also presents as an isolated incomplete partition of the tracheoesophageal septum. There are 5 types of anomalies, with the most common subtype being a distal tracheal fistula with EA (type III), followed by isolated EA without TEF, H-type TEF, EA with proximal TEF, and EA with both proximal and distal TEF. 35 Most patients will pre sent shortly after birth because of the inability to pass a 10F catheter beyond 10 cm in the setting of drooling, respiratory distress, or cyanosis with feeds. 35 Plain chest radio graphs will reveal the presence or absence of a gastric bubble depending on the pres ence of EA with or without TEF, in addition to coiling of a nasogastric tube in patients with EA. Contrast studies are rarely required, owing to risk of pneumonitis, and direct laryngoscopy with bronchoscopy is useful in this setting for visualization of fistula. The condition can be present in isolation or associated with several syndromes, including CHARGE and VACTERL. Treatment involves placement of a gastrostomy tube for definitive feeding management, with repair of the fistula by 3 months of age. 35 Repair can be performed endoscopically or with open repair with esophageal anastomosis and fistula closure. Tracheomalacia Tracheomalacia is a dynamic anomaly of the upper-respiratory tract owing to abnormal weakness of the tracheal walls and supporting cartilage ( Fig. 6 ). The condi tion can be a primary condition, typically owing to gestational prematurity resulting in flattened and shorter tracheal cartilage, or secondary to underlying vascular compres sion, TEF, relapsing polychondritis, or Ehlers-Danlos syndrome. Tracheomalacia can

Downloaded for Anonymous User (n/a) at Geisinger Health from ClinicalKey.com by Elsevier on November 28, 2022. For personal use only. No other uses without permission. Copyright ©2022. Elsevier Inc. All rights reserved.