Laryngomalacia

It is the most frequent cause of stridor in newborns. More common in males, it constitutes 60% of the airway congenital anomalies, and it is usually a benign and self-limiting disease. Symptoms generally start between weeks 2 and 4, with progression until months 2 and 3. Most patients improve spontaneously after 12 and up to 24 months of age, if the treatment is conservative. Less than 15% correspond to severe cases. In them, supraglottoplasty is the method used, which is performed with microlaryngeal instruments or by using CO₂ laser. This surgery is performed endoscopically and requires general anesthesia. The patient remains intubated 24 hours and is extubated in an intensive care unit (Fig. 71.1).

Congenital Subglottic Stenosis

It is a congenital narrowing of the subglottic space of less than 4 mm in term newborn infants and 3 mm in preterm newborn infants. It is the third larynx congenital malformation (10%), and it is a consequence of a recanalization failure of the laryngeal lumen during the embryonic period. In its embryological process, it is related to laryngeal atresia and congenital laryngeal diaphragms. Symptomatology is variable and is related to the degree of stenosis. It can appear as mild stridor at birth or severe obstructive symptomatology that requires an emergency tracheotomy (Fig. 71.2). In the case of severe stenosis, it is usually required to perform a tracheotomy to ensure proper ventilation. A final reconstructive surgery is performed at around 1 year of life and consists of a partial cricoid resection with trachea-thyroid anastomosis. The tracheotomy is removed. In cases of congenital subglottic stenosis the cricoid is malformed, with a narrower lumen and a characteristic elliptical shape.

Laryngeal Diaphragm or Webs

Diaphragms or congenital laryngeal webs are the result of an incomplete recanalization of the primitive larynx. They appear as an obstruction of the upper airway, and characteristically with stridor. Newborn’s crying is abnormal and can even cause aphonia. There are two types of congenital diaphragms: one is a thin web that is delimited to the glottis; the other is a thick diaphragm that extends into the subglottis. The latter is thought to correspond to a lower degree of laryngeal atresia rather than a real web. They are infrequent and constitute 5% of the congenital anomalies of the larynx. There is a genetic association with DiGeorge syndrome, which is characterized in some cases by a microdeletion of chromosome 22q11. The acquired webs are usually postintubation and produce a variable degree of synechia in the vocal cords. Most laryngeal diaphragms have associated subglottic stenosis and thickening of the cricoid cartilage, especially grades III and IV. According to Cohen’s classification, they can be grouped into four types depending on the morphology and the degree of laryngeal obstruction they cause (Figs. 71.3 and 71.4).

Initial diagnosis is made with a nasolaryngoscopy conducted while the patient is awake, which will rule out other possible diagnoses, such as laryngomalacia or vocal cord paralysis. A final evaluation requires direct laryngoscopy. Different therapeutic options are available: a newborn with laryngeal diaphragm diagnosis and severe airway obstruction during the first hours of life characteristically corresponds to a case of diaphragm with severe obstruction of the glottic space, and in many cases emergency surgery is required to secure the airway (tracheotomy). In patients with thin and anterior diaphragms, an endoscopic cut can be performed. In thick diaphragms, with mild to moderate symptoms, later repair is suggested, because it is easier in technical terms. In children with more severe symptoms, the intervention must be done early. To relieve obstruction, tracheotomy is also endorsed as an option while the child grows, in order to repair at a later age.

In the majority of type III and IV diaphragms, open surgery involves a two-stage repair that includes the division of the diaphragm and enlargement of the subglottic space. The current recommended technique consists of performing anterior laryngofissure with a siliconized laryngeal stent placement plus a temporary tracheotomy. The purpose of this laryngeal prosthesis is to allow an adequate epithelization of the medial border of the cords that was congenitally adhered and thus prevent them from adhering again. Between 6 and 8 weeks later, the laryngeal prosthesis is removed endoscopically, and then it is possible to remove the tracheotomy. As mentioned, it is advisable to expand the airway using an anterior or posterior graft of costal cartilage due to the frequent association that this pathology has with subglottic space stenosis. In grade III patients, having to enlarge the subglottic space in the posterior plate and the anterior wall occurs frequently. In mild forms, the approach is endoscopic surgery conducted by experts, and requires cutting off the web using a laser, and also a mucosal flap in the cords to prevent posterior synechiae and recurrence. The insertion of a keel endoscopically, between the medial borders of the separated cords, is another endorsed alternative in older patients.

Subglottic Hemangioma

It is a benign vascular tumor located in the subglottic space, specifically in the left posterolateral region, close to the vocal cord on the same side. It corresponds to 1.5%–3% of all benign lesions of the larynx, and its evolution is self-limited, starting to remit after 18 months. Hemangiomas of posterior localization, left and bilateral, are less common. In our experience, we have evaluated eight subglottic hemangiomas, one of them posterior bilateral associated with congenital narrowing of the subglottic space. Because initial treatment with propranolol was not enough, surgery had to be done, resecting the hemangioma, removing the cricoid posterior mucosa, and performing a partial cricoid resection and a primary thyrotracheal anastomosis without tracheotomy.

Currently, the treatment of choice is the administration of propranolol; however, there are other treatment options that are second-line therapy that should be analyzed in each patient, considering the size of the lesion, age, degree of obstruction, and response to steroids and propranolol. Observation is appropriate for older patients, in whom there is little or minimal associated symptomatology of respiratory obstruction.

In cases where airway obstruction caused by hemangioma is less than 50% of the lumen and the symptoms are mild, the alternative to observe and re-evaluate is valid. Endoscopic techniques of intralesional steroids injection, submucosal dissection with microdebrider, and laser ablation can only achieve partial removal of the lesion and cause some degree of damage to the mucosa or cricoid cartilage, so currently there is controversy regarding these therapies. Temporary tracheotomy offers a stable airway during the growth and involution phase of the hemangioma. In case of opting for this alternative, the presence of the stoma should be considered for a period of approximately 10–30 months. The advantage of this option is that the involution of the lesion is achieved without a surgical intervention that may be associated with cord injury. However, it should also be considered that tracheotomy in infants has associated morbidity and mortality and requires special care. Open surgical resection is the treatment of choice in symptomatic patients with large hemangiomas that grow quickly. It would also be indicated in patients in whom the involution of the hemangioma has not occurred within 2 years (and where a tracheotomy or observation was chosen). Endoscopic excision with CO₂ laser, open resection, endoscopic resection with microdebrider, and tracheostomy are the main surgical alternatives. However, propranolol treatment currently prevails in most hemangiomas.

Tracheomalacia-bronchomalacia

Surgical alternatives, such as aortopexy, bronchopexy, intraluminal supports (stents), partial resections, and tracheotomy are reserved for severe cases: apneas, respiratory obstruction episodes during feeding, and recurrent respiratory infections.

Stents

Intraluminal supports that keep the airway lumen expanded (Figs. 71.5 and 71.6). Its effectivity has been confirmed. The insertion is done by endoscopy. The types of stents available are mainly metallic (Palmaz, nitinol, wallstents) and silicone (Dumon, Montgomery). Currently, self-expandable and heat-moldable stents are available, thus achieving a better resistance and effectiveness to solve a severe airway collapse. They can be inserted into the tracheal or bronchial lumen unilaterally or bilaterally as required. Once in situ, it can remain for a long time or be removed as needed.

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