Several grading classifications for LTCs have been proposed, primarily based on the craniocaudal extent of the cleft. Such classifications are mainly descriptive, help in therapeutic choices, and have to some degree prognostic value. The most widely used classification is the one proposed by Benjamin and Inglis in 1989 [3]. Benjamin’s classification was modified and updated by Sandu and Monnier [2] in 2006, in order to introduce parameters with therapeutic and prognostic implications previously overlooked (Table 10.1).

Unlike previous classifications, Sandu and Monnier introduce type 0 clefts (“occult” or more accurately submucosal), which appear as a consequence of a posteriorly defective cricoid cartilage accompanied by the absence of transverse interarytenoid muscles [6]. Type I clefts are manifest interarytenoid clefts with the absence of interarytenoid muscles which do not involve at all the cricoid cartilage. Type II clefts extend partially through the cricoid cartilage. By definition, type III and type IV clefts extend above and below the thoracic inlet, respectively. Sandu and Monnier’s classification pays particular attention to two specific features in these severe forms of disease: (1) whether the cricoid plate is partially or completely involved by the LTC in type III clefts and (2) whether the cleft is supra- or infracarinal in type IV LTCs (Fig. 10.1).

The classical diagnostic triad of LTC consists of husky cry, aspiration pneumonia, and swallowing disorders in a newborn child with associated congenital anomalies [7]. While such scenario should immediately prompt active search for LTC, disease spectrum can often complicate and delay LTC diagnosis. Indeed, LTCs are congenital laryngotracheal disorders which can present as submucosal closure defect in the mildest cases versus complete laryngotracheoesophageal clefts (LTEC) in some other severe cases. Breathing difficulties are typically due to prolapsing of retroarytenoid mucosa caused by the absence of the aerodigestive party wall and obstruction of the posterior respiratory glottis (Fig. 10.2). Therefore, presenting signs and symptoms related to LTC may vary depending on disease severity [7, 8].

LTCs without involvement of the posterior cricoid plate (type 0 and I) may be asymptomatic or in the case of type I clefts present with mild occasional episodes of aspiration, hoarse cry, aspiration, cough, or in some cases dyspnea or cyanosis during feeding [8–10]. Due to their rarity, diagnosis of type 0 and I clefts requires a high-suspicion index.

Regarding clefts with cricoid (with or without tracheal involvement), the outstanding issue is severe aspiration and subsequent lower pulmonary tract infection, as well as respiratory problems in some cases [9, 11]. Finally, type IV LTCs have a dim prognosis due to respiratory distress, poor airway tone, and difficulties to maintain a patent airway even when using invasive procedures [11–13]. Table 10.2 summarizes the clinical presentation of LTCs [14].

While the mainstay of LTC diagnosis is endoscopic (discussed below), some imaging exams may be contributive. Plain chest X-ray is often performed in children with persistent respiratory symptoms, whether or not such symptoms are secondary to an underlying LTC. Patients with LTC may present aspiration pneumonia or peribronchial cuffing, but chest X-ray is normal in up to 25 % of type I and around 10 % of type II LTCs [15].

Modified barium swallow (MBS) performed under the supervision of a speech and swallowing pathologist, testing diverse food consistencies, may help identifying patients with discoordinated swallow or aspiration. It has been previously pointed out that aspiration in an otherwise healthy child most often correlates with an underlying anatomic abnormality [16].

It is important to keep in mind that children with an undiagnosed LTC presenting with swallowing disorders may have undergone several MBSs in the past, and the cumulative radiation dose can be substantial.

FEES provides an extremely accurate dynamic assessment of the laryngeal function during swallowing. FEES is usually possible in children older than 1 year but is otherwise difficult to perform in younger children. During FEES, several food consistencies should be tested. Hypopharyngeal stasis and/or laryngeal penetration may be visualized in cases of early-stage LTCs [17].

Like MBS, FEES can be normal in children with grade I cleft with only intermittent aspiration.

Definitive LTC diagnosis relies on endoscopic examination. Due to the high incidence of associated malformations, complete endoscopy of the upper aerodigestive tract is mandatory, and should include:

Associated airway findings in LTC include:

Since the late 1970s, various endoscopic approaches have been successfully used primarily for types I and II clefts [25]. Injection laryngoplasty using different products (Gelfoam, bioplastic) has been reported [26, 27]. Endoscopic repair has progressively become the standard of care for types I and II clefts that remain symptomatic despite adequate medical treatment after an observation period of 4–6 months [14, 16, 17].

Sandu and Monnier reported a small series of patients with types IIIa and IIIb LTCs managed endoscopically [2]. In their reported experience carbon dioxide laser is used in ultrapulse mode to incise the cleft from caudal to cranial direction – cleft apex up to the cuneiform cartilages. The CO₂ laser is a precise cutting tool giving a bloodless field and causes no mucosal charring. Two layers of mucosae are created: laryngotracheal and pharyngoesophageal. Starting caudally, a set of inverted Vicryl 5.0 sutures are placed on the tracheal aspect of the cleft and the knots tied facing the pharyngoesophageal side. The second mucosal layer is sutured in similar fashion in distal to proximal direction with knots facing the esophagus while gradually withdrawing the suspension laryngoscope (Fig. 10.4). The procedure is performed under spontaneous respiration and without endotracheal intubation. At the end of the procedure, it is important to maintain an adequate posterior commissure and avoid posterior glottic stenosis (Fig. 10.5). Special endoscopic suturing instruments are a must and their role needs emphasis. The details of such instruments are described in related articles [2].