A 17-year-old woman (AB) presents with a 4-year history of dysphagia. She is unable to eat solids. She also 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 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 sportswoman despite her stridor.
Her anesthetic history post-decannulation includes an uneventful laryngeal mask anesthetic for nasal surgery performed last year when she was 16 years old.
Nasal endoscopy reveals an infantile larynx—small with a relatively large, omega-shaped and retroflexed epiglottis. She has a supraglottic stenosis, posterior glottic stenosis secondary to inter-arytenoid scarring, impaired vocal cord motion, circumferential subglottic stenosis, tracheal stenosis, and a pharyngoesophageal stricture (Figure 44–1).
Nasal endoscopic view of the glottis: (A) showing omega-shaped epiglottis; (B) and (C) showing the narrow and distorted glottic inlet; and (D) showing the pharyngoesophageal stricture.
A CT scan confirms supraglottic narrowing at the level of the hyoid bone (0.5 × 1 cm) (Figure 44–2), a transverse diameter across her vocal cords of 1 cm, infraglottic narrowing measuring 1.5 cm in diameter, the transverse diameter of her proximal trachea is 0.8 cm, while her lower trachea and the rest of the airways are widely patent and normal.
CT scan showing the stenotic airway: (A) rotational airway series from preoperative CT scan; (B) and (C) coronal CT views 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 lymphatic malformations are endothelial-lined, lymph-filled cysts caused by an abnormal development in lymphatic channels. Approximately 50% of lymphatic malformations are in the head and neck area, also referred to as cystic hygromas. While fetal cystic hygromas detected by ultrasound before 23 weeks gestation are associated with karyotypic or genetic abnormalities, lymphatic malformations appearing after 30 weeks gestation, as seen in AB's case, tend to be isolated lesions.