September 2019 HSC Section 1 Congenital and Pediatric Problems

J.C. Yeung et al. / International Journal of Pediatric Otorhinolaryngology 101 (2017) 51 e 56

laryngeal clefts, in the absence of strong evidence to support spe- ci fi c diagnosis and management strategies.

Where applicable, the relevant peer-reviewed literature that pro- vides the basis for recommendations is cited. Any person seeking to apply or consult the report is expected to use independent medical judgment in the context of individual patient and institutional circumstances.

2. Target population

Pediatric patients with signs concerning for pharyngeal dysphagia and aspiration due type I laryngeal clefts.

6. Recommendations and justi fi cation

3. Intended users

The recommendations are outlined in the following appendices.

These consensus recommendations are intended to provide comprehensive care recommendations for otolaryngologists who manage patients with dysphagia/aspiration and type I laryngeal clefts.

Section 1 : Diagnostic Considerations 1.1 : Consultations 1.2 : Direct Laryngoscopy and Bronchoscopy Section 2 : Medical Management Section 3 : Surgical Management 3.1 : Pre-Operative Considerations 3.2 : Intra-Operative Considerations 3.3 : Post-operative Management and Follow-up

4. Methods

Consensus guidelines based on review of the literature and expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method was used to establish expert recommendations on the diagnostic con- siderations, multi-disciplinary approach, medical management, and operative considerations, with respect to type I laryngo- tracheoesophageal clefts. An online survey investigating the diag- nostic and management considerations for type I LTECs was designed by the primary and senior authors (J.Y. and R.R.). The survey was distributed to the members of the IPOG. The survey responses were collected and collated. The results were anony- mized and redistributed; respondents were given the opportunity to comment on the format and content of the survey, as well as revise their responses in order to achieve consensus. Consensus was de fi ned as > 80% agreement among respondents and no consensus was de fi ned as < 50% agreement [1] . Twenty members completed the survey. This guideline, developed around the survey, is based on input from 23 attending faculty at 17 tertiary care pe- diatric hospitals, representing seven countries including Australia, Canada, France, Ireland, Portugal, the United Kingdom, and the United States of America (see Fig. 1 ). Subsequent use of the term ‘ member ’ refers to members of the IPOG. CXR, chest x-ray; FEES, fi ber-optic endoscopic evaluation of swallowing; FFL, fl exible fi ber-optic laryngoscopy; IPOG, Interna- tional Pediatric Otolaryngology Group; LTEC, laryngo-tracheo- esophageal cleft; MBS, modi fi ed Barium swallow, also known as video fl uoroscopic swallow study; TEF, trachea-esophageal fi stula. 4.1. Abbreviations

6.1. Section 1: diagnostic considerations

Risk factors for aspiration should be elicited. Patients at high risk for aspiration with or without concomitant LTEC are de fi ned as those with a history of recurrent pneumonia, neuromuscular dis- order, prematurity, genetic syndrome, and/or other congenital anomaly [3] . There are several ancillary tests used in the work-up of patients presenting to the otolaryngologist with dysphagia and aspiration. These include fl exible fi ber-optic laryngoscopy (FFL), chest X-ray (CXR), modi fi ed barium swallow (MBS) study, and fi ber-optic endoscopic evaluation of swallowing (FEES) [4,5] . The severity of aspiration and degree of feed thickening required to prevent aspi- ration is of importance. The indications and frequency of use of these tests is described in Table 1 . Generally, both MBS and FEES can be used to diagnose laryngeal penetration/aspiration. Their speci fi c use is institution-dependent and each test has advantages and disadvantages. The advocates for FEES reason that penetration of the fl uid bolus in the intra- arytenoid area is more readily identi fi ed in FEES compared to MBS. Fluid penetration between the arytenoid complexes or “ pos- terior aspiration ” can also be diagnosed with MBS. FEES is also advantageous in that it does not expose the child to radiation. Contrastingly, the advantages of MBS include the ability to assess the oral and pharyngeal phases of swallowing, lack of white-out phase, and the patient does not need to cooperate with FFL. In select cases, when consistent and persistent interarytenoid pene- tration is present, one may potentially infer the presence of symptomatic cleft/deep intra-arytenoid groove without need for con fi rmation with DLB and associated anesthetic risk. Thus, in these selected cases, a trial of medical management can be pro- posed to the family. However, it is important to note that de fi nitive diagnosis of type I LTEC should be based solely on endoscopic evaluation under general anesthesia.

4.2. Classi fi cations

Benjamin-Inglis Classi fi cation of laryngotracheoesophageal clefts (LTECs) [2] .

Type I e Interarytenoid cleft Type II e Partial cleft of the cricoid cartilage Type III e Complete cleft of the cricoid cartilage with or without cervical tracheal involvement Type IV e Involvement of the thoracic trachea

6.2. Section 1.1: consultations

Patients presenting with dysphagia and aspiration bene fi t from a multidisciplinary evaluation and management. The primary ser- vices involved in the care of patients with dysphagia/aspiration include the speech language pathologist/feeding team, gastroen- terologist, pulmonologist and neurologist. Patterns and indications for referral are highlighted in Table 2 .

5. Disclaimer

Members of the International Pediatric Otolaryngology Group (IPOG) prepared this report. Consensus recommendations are based solely on the collective opinion of the members of this group.

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