A 17-year-old woman (AB) presents with a 4-year history of dysphagia and is unable to eat solids. She has poor voice with low pitch and stridor at rest and would like these symptoms improved as well.
AB has a complicated airway history. As a neonate, she had a congenital right-sided neck lymphatic malformation (LM) that was surgically excised and then treated with a sclerosing agent. At 1 month of age, a tracheotomy was performed because of airway obstruction secondary to supraglottic swelling. As a child, she had repeated laryngeal procedures with a CO2 laser. She was decannulated at age 8 after an anterior tracheal augmentation with rib graft.
AB is otherwise well. She is well-nourished, despite her dysphagia, and is an active athlete despite her stridor.
Her anesthetic history postdecannulation includes an uneventful anesthetic using a laryngeal mask airway (LMA®) for nasal surgery performed last year when she was 16 years old.
Nasal endoscopy reveals a small larynx with a relatively large, omega-shaped epiglottis that is fixed in a retroflexed position. She has a supraglottic stenosis, posterior glottic stenosis secondary to interarytenoid scarring, impaired vocal cord motion, circumferential subglottic stenosis, tracheal stenosis, and a pharyngoesophageal stricture (Figure 44.1).
Flexible laryngoscopic examination findings: (A) the valleculae and retroflexed epiglottis; (B) the omega-shaped epiglottis; (B) and (C) the narrow and distorted supraglottic airway; and (D) the hypopharynx and postcricoid region of the larynx (larynx is in the upper half of the clinical photograph).
A computerized tomography (CT) scan confirms supraglottic narrowing at the level of the hyoid bone (0.5 cm × 1 cm) (Figure 44.2), a transverse diameter across her vocal cords of 1 cm, infraglottic narrowing measuring 1.5 cm, the transverse diameter of her proximal trachea is 0.8 cm, while her lower trachea and rest of the airways are widely patent and normal.
Preoperative CT Scan of the stenotic airway: (A) 3D reconstruction of airway; (B) and (C) coronal CT images demonstrating numerous sites of airway stenosis.
AB will need multiple laryngeal procedures under general anesthesia to improve her swallowing, voice, and airway. However, her first priority is to be able to eat better, and she presents for microlaryngoscopy, tracheoscopy, and pharyngoesophageal balloon dilation.
What Is a Congenital Lymphatic Malformation?
Congenital LMs are endothelial-lined, lymph-filled cysts caused by an abnormal development of the lymphatic system. Approximately 50% of LMs are in the head and neck area. While fetal LMs detected by ultrasound before 23 weeks of gestation are associated with karyotypic or genetic abnormalities, LMs appearing after 30 weeks of gestation, as seen ...