I have wanted to post something about Type1 laryngeal clefts for while and am just getting around to it. A Laryngeal Tracheoesophageal Cleft Type 1 (LTC 1) should always be part of a differential diagnosis when it comes to aspiration or symptoms of aspiration. A laryngeal tracheoesophageal cleft is a space between the larynx and the esophagus.
There are 4 basic types:
Type I: is a cleft above the level of the vocal cords and involves failure of interarytenoid muscle development
Type II: cleft extends into the upper cricoid, with the cartilaginous ring remaining intact
Type III: involves a cleft of the entire cricoid cartilage with or without extension into cervical trachea
Type IV: is an extensive cleft affecting the laryngotracheoesophageal structures.
- Coughing and/or Choking with feedings
- Shortness of breath
- Poor weight gain
- Short pauses when breathing (apnea)
- Noisy breathing (stridor)
- Frequent lower respiratory infections (pneumonia)
- Food and / or liquid going into the lungs (aspiration)
- Gastric esophageal reflux
It is a routine part of a feeding therapist’s job to assess for clinical signs and symptoms of aspiration as well as perform objective swallow studies (videofluoroscopic evaluation of swallowing or fiberoptic endoscopic evaluation of swallowing). When aspiration is identified, we make recommendations for the safest diet consistency for the child to prevent aspiration. Sometimes we recommend a follow up swallow study in a few months to look again.
How many of us suspect a structural reason for aspiration? Such as a subtle defect in the larynx like a type 1 LTC or deep intra-arytenoid groove? If we are thinking in those terms, it would mean referring the child to ENT or otolaryngology after identifying the aspiration or symptoms (we have children who pass their swallow studies but are symptomatic and continue to cough on thin liquids) for further evaluation. Diagnosis is made via direct examination by an otolaryngologist using rigid endoscopy. Sometimes the child may need other X-rays or tests to evaluate for aspiration (food / liquid going into the lungs) or other abnormalities.
Modified barium swallow study (MBSS):
“ Symptoms evident on fluoroscopy include a persistent pattern of aspiration that cannot be attributed to oral-motor or other pharyngeal disturbances. It often looks like there’s penetration right between the arytenoids on VFSS.” (Advance article). I would agree with this, sometimes you see aspiration on the posterior tracheal wall. However, we also have children that pass their MBSS but continue to cough, choke or have symptoms with thin liquids.
I have started thinking about this more and more in the last few years working on our hospital based feeding team. We see a lot of children for second opinions and I started to wonder why I was seeing “typically” developing kids on thickened liquid or children that have been on thickened liquids for years, unable to move off of them. Many of these children had never had an airway evaluation.
We met with our ENT’s and began sending these kids for airway evals. In the last year and half, we have identified over 15 children with LTC type 1 or intra-arytenoid groove as a cause for their aspiration. I feel there is comfort in knowing what we are treating. The identification of these airway anomalies will drive treatment. Caregivers will then need to decide how to proceed with their physician- monitor for change, gel injection, or surgical closure. None of which are easy decisions as outcomes vary.
At the end of this post, I have included some research regarding LTC repair. There was a particularly interesting study where the surgeons used gel injection with children with cystic fibrosis who had normal anatomy but were symptomatic of swallowing difficulty. All three children had improved swallowing. This may offer us new options for some of our patients with dysphagia.
Case: a 1 1/2 year old male was referred to our feeding team with feeding difficulties characterized by:
- aspiration of thin liquids (drinking nectar thick liquids)
- g-tube dependence (g- tube placed as young infant)
- oral feeder for small volumes
- severe GERD (history of life threatening reflux events as an infant)
Our team was able to treat the child’s GER and over time transition him from tube feeds to oral feeds. This child was typically developing. He has come to us on thickened liquid and been on this for approximately a year. A repeat MBSS showed no aspiration, however the child continued to have cough and eye tearing with thin liquids. We referred him to ENT who identified a type 1 LTC but recommended monitoring to determine if the child would learn to compensate. We moved the child to small sips of thin liquids. After a 3 months period, this child had recurrent respiratory infections and fevers. A return to nectar thick liquid eliminated these issues. The ENT then agreed to a gel injection of the LTC type 1 with complete resolution of symptoms. This child has been able to move to thin liquids successfully.
I found a video example of gel injection:
Video example is available on the web at: Injection Laryngoplasty for Type 1 Laryngeal Cleft (Published July 23 2013) by Deepak Mehta (Children’s Hospital of Pittsburgh)
Injection Laryngoplasty for type 1 laryngeal cleft is done with first identifying the deep cleft by palpation of the interarytenoid notch. Once a confirmation is made the larynx is suspended with a laryngoscope. Radiesse voice gel is then primed in a laryngeal needle and the needle is placed at the apex of the cleft. The needle is then pushed to palpate the cricoid cartilage with the bevel of the needle pointing towards the esophageal surface. The needle is then slightly retracted and about 0.2 ml of voice gel is injected. Care is taken not to make multiple punctures and the subglottisis watched so that the injection does not inadvertently go into subglottis.
– See more at: http://www.csurgeries.com/video/injection-laryngoplasty-for-type-1-laryngeal-cleft/g5r116zy3n#sthash.GU7etNTQ.dpuf
Cohen MS, Zhuang L, Simons JP, Chi DH, Maguire Rc, Mehta DK. (2011). Injection laryngoplasty for type 1 laryngeal cleft in children. Otolaryngol Head Neck Surg.May;144(5):789-93. Doi: 10.1177/0194599810395082.
Injection laryngoplasty was found to have favorable results in this group of patients with LC-1. Most patients experienced complete resolution of aspiration on MBS, and no patients experienced complications. These findings support the further study of injection laryngoplasty for LC-1 as both a diagnostic and therapeutic technique.
Chiang T, McConnell B, Ruiz AG, DeBoer EM, Prager JD. (2014). Surgical management of type I and II laryngeal cleft in the pediatric population. Int J Pediatri Otorhinolaryngol. Dec;78(12):2244-9. doi: 10.1016/j.ijporl.2014.10.023. Epub 2014 Oct 25.
Twenty patients with LC-1 and 5 with LC-2 were included. Most common presentations were aspiration on modified barium swallow (MBS) (76%) and recurrent pulmonary infections (60%). Gastroesophageal reflux disease (GERD) was a concurrent co-morbidity in 68%. Of the 19 patients who presented with preoperative aspiration on MBS, 52.6% had evidence of improvement on their first MBS. This improved to 78.9% at the time of their most recent MBS (10 with resolution of aspiration, 5 with improved tolerance of consistencies). Two patients failed to improve from surgical repair and two had worsened aspiration on most recent MBS. Given the multidimensional nature of swallowing disorders and aspiration, a comprehensive team approach in the evaluation, management, and surveillance of these patients is a valuable complement to surgical repair since not all aspiration may resolve.
Alexander NS., Liu JZ., Bhushan B., Holinger LD., Schroeder JW Jr. (2015) Postoperative observation of children after endoscopic type 1 posterior laryngeal cleft repair. Otolaryngol Head Neck Surg. Jan;152(1):153-8. doi: 10.1177/0194599814556068. Epub 2014 Nov 5.
summary: The endoscopic surgical repair of a type 1 PLC is successful and has a low morbidity and complication rate. Patients may be safely managed in an observation unit and without postoperative intubation. 54 patients underwent repair of type 1 PLC. Indications for repair included aspiration (72%), chronic bronchitis (24%), and stridor with feeds (4%). Symptoms improved in 41 of the 54 patients (76%). No postoperative complications were noted. Two patients required revision PLC repair.
Horn DL. DeMarre K, Parikh SR. (2014). Interarytenoid sodium carboxymethylcellulose gel injection for management of pediatric aspiration. Ann Otol Rhinol Laryngol. Dec;123(12):852-8. doi: 10.1177/0003489414539129. Epub 2014 Jun 24.
Summary: Chronic aspiration can improve after IL even in patients with normal anatomy. Injection laryngoplasty can be performed to improve selection of PLC-1 patients for definitive endoscopic repair. Further prospective research, with a randomized control group, is needed to understand whether interarytenoid incompetence plays a role in some patients with chronic aspiration, who do not have a PLC-1.Clinical improvement was observed in 57% of patients. Patients with PLC-1 were more likely than noncleft patients to show recurrence of symptoms after initial improvement with IL.
Ojha S., Ashland JE., Hersh C., Ramakrishna J., Maurer., Hartnick CJ.(2014). Type 1 laryngeal cleft: a multidimensional management algorithm.JAMA Otolaryngol Head Neck Surg. Jan;140(1):34-40. doi: 10.1001/jamaoto.2013.5739.
Summary: Age, comorbidity status, severity of aspiration, and the ability to tolerate a feeding regimen should be taken into account when deciding on conservative or surgical management for children with a type 1 laryngeal cleft. A clinical pathway for conservative and surgical management is presented.Success was defined as improving when a child was able to tolerate a feeding without aspirating or resolved when the child had transitioned to tolerating thin liquids. 64% received surgical intervention with a rate of operative success of 78%. Only 6 patients did not demonstrate resolution, 5 of whom had significant comorbidities.
Virbalas J., McMullen C., Cheng J. (2015) Injection laryngoplasty in children with cystic fibrosis and abnormal swallow. Int J Pediatr Otorhinolarngol. Nov;79(11):1856-9. doi: 10.1016/j.ijporl.2015.08.024. Epub 2015 Aug 24.
Summary: Injection laryngoplasty may be effective in normalizing swallowing in children with CF.Three patients with CF underwent injection laryngoplasty for evidence of aspiration or laryngeal penetration on MBS evaluation. Normal laryngeal anatomy was identified intraoperatively in each case. Presenting symptoms included chronic cough, cough with oral liquids, and recurrent pneumonia. Each patient underwent successful injection into the interarytenoid space. Post-operatively, modified barium swallow demonstrated resolution of aspiration or penetration in all patients. No procedure-related complications were encountered.