Problem-Solving: 8 month old with stridor of unknown etiology post VFSS

QUESTION: I would love some advice and guidance. I have a 8 month old that was referred for an outpatient evaluation for feeding/swallowing concerns with a diagnosis of reflux and dysphagia from his pediatrician. Since birth, he has had a persistent stridor as well as reflux. Mom reports having tried multiple formulas, taking Pepcid (as prescribed by his pediatrician) and attempting multiple bottles and nipples prior to finally finding success with Enfamil AR. She reports that only the AR formula has allowed him to gain weight. His BMI was classified as “underweight” at his last appointment with PCP with weight at 20lbs on 6-24-21. The pediatrician recommended that the child see ENT and have a VFSS. No clinical bedside evaluation was conducted first. The ENT saw him in office and recommended a sleep study in addition to the VFSS. The sleep study revealed 4-5 apneic episodes every hour, however, it was ruled as “normal” per parents’ report. They were told that unless he is turning blue then there is nothing to be done at this time. They were very distraught. He wears an owlet, and his mother reports that it goes off continually each night. He is a very restless sleeper. She also reports coughing in his sleep. The VFSS revealed “moderate pharyngeal dysphagia characterized by delayed laryngeal closure resulting in deep laryngeal penetrations with thin (Dr. Brown level 2 nipple utilized) and mildly thick/nectar consistency (Dr. Brown level 3 and 4 utilized) and 1 isolated episode of shallow laryngeal penetration that was trace in volume with moderately thick/honey consistency. Silent aspiration was present with mildly thick/nectar consistency with Dr. Brown level 4 nipple.” It was recommended that he receive all liquids via Dr. Brown Y cut nipple with honey consistency/moderately thick utilized. Specifically, the SLP advised the family to utilize 12ml of Beech-Nut oatmeal cereal to 1 ounce of AR formula. At the time, he was consuming 6-ounce formula bottles so the SLP wrote out for him to consume 72ml of oatmeal to 6 ounces of Enfamil AR (4.75 teaspoons per 6 ounce bottle). He was also encouraged to continue solid age-appropriate diet. Note: Barium was thickening agent utilized during the VFSS and oatmeal not utilized. The flow test confirms this recipe of thickening is greater than moderately thick (honey consistency).

Currently, this child is consuming one 6 oz. bottle every 3 hours. Per his father’s report, he weighs 31lbs. He has gained approx. 10-11 pounds in less than 2 months since his formula has been altered. He is also eating solid cubed and diced foods with no signs or symptoms of aspiration noted. He has to date had no instrumental (laryngoscopy or bronchoscopy) performed with ENT but is scheduled to have completed later this month with ENT citing that he will repair the cleft at that time if present as suspected. I immediately contacted the PCP and requested a dietary consult due to concerns with excessive weight gain and nutrition intake. I’ve also spoken with the ENT to confirm exact plans of scheduled procedure with request to trial a reduction in thickening. The ENT indicated he agreed that he should attempt to decrease intake of added oatmeal.  What would you recommend trialing in office with this child? I want to reduce the amount of oatmeal and thickener utilized as well as aim to alter the nipple utilized to ensure he is as safe as possible pending further instrumental assessment. Thoughts? Advice?

ANSWER:

His clinical and radiological presentation are indeed worrisome.  Sounds like etiology for the stridor isn’t fully clear yet. This is, as Laura suggested,  a rather common presentation that often leads to hospital admission (d/t the events like color change that often co-occur) to expedite a workup. ST is often consulted to assist in the differential (neuro vs. reflux vs cardiac  vs swallowing vs airway). These infants are like small puzzles with lots of moving pieces.

I am always surprised and saddened when parents report to me that the stridor and poor feeding are longstanding and unexplained and unsolved for so long. One infant I was consulted on as an inpatient had such loud inspiratory stridor  that I could hear it when I was coming down the hall, and he had suprasternal and supraclavicular  retractions so deep, he could barely move air. Mother recalled the events of color change and need for CPR with tears as she explained his history to me.  The mother was desperate. The infant was so precarious. Long story short, I had to thoughtfully and respectfully advocate for interventions beyond those initially planned by the team that would have mobilized the infant instead to an earlier discharge. At discharge about 5 weeks later,  he finally could breathe and was feeding, and starting to gain motor and developmental function now that breathing allowed. A few months later, on an unrelated hospital visit, the mother saw me across the hospital lobby,  which is huge,  and called out to me.  When I turned around and then came to her side, she hugged me so tight and cried “You saved my baby’s  life, I just know it. I couldn’t have taken him home that way again”. Whew, tears flowed.  In that moment I knew I was supposed to be where I am. There he was, in her arms, doing so well. Those moments stay with you forever. So your thoughtful advocating for this little guy is something I am sure the parents so appreciate, more than you know.

We don’t know anything else about his history, so unsure if something might be relevant. I am also assuming the stridor is inspiratory, which most often co-occurs with laryngomalacia and/or reflux. Expiratory or biphasic stridor would broaden my thinking into other directions. The inspiratory stridor may be related, at least in part,  to the effects of LPR/EER (based on his coughing with sleep, stridor at non-feeding times), but so far,  we have no UGI to provide data about structures/possible malrotations, and we have no scope yet to give us data about potential co-occurring changes in the larynx/airway r/t LPR/EER that might result in decreased laryngeal sensation. If there were, that could be perhaps part of the etiology(ies) for the events of bolus mis-direction and airway invasion witnessed in radiology.

The stridor may also be due to a primary airway problem such as laryngomalacia with or without co-occurring laryngeal cleft, or both EER/LPR and an airway difference. With no laryngoscopy or bronchoscopy by ENT, we don’t yet know. The correlation between laryngomalacia and airway invasion is quite high in the literature. His WOB, often provoked by multiple reasons for stridor, can often dynamically affect swallowing physiology from moment to moment during a feeding. From the swallow study we know that there was delayed laryngeal closure but no other information about swallowing pathophysiology to help us understand alterations along his swallow pathway better.

In the swallow study I wonder what he would have done with first looking at changing nipple flow rate better manage flow rate, prior to moving to thickening. Given the deep LPs with a level 2,  perhaps  could they have trialed a level 1 nipple with co-regulated pacing with Enfamil AR since it has a bit greater baseline viscosity than typical thin liquids, for example. The high level of thickening prescribed may be affected by an artifact, in that a Y cut nipple will always require more thickening given that it is the highest flowing nipple. Thickening to this degree is I think rare because often we suspect that if this level of thinking is required  to establish a “safe” swallow, is there risk during a true feeding of 30″ (compared to 2-3″ under fluoro), when small changes like the infant sucking a little harder or faster , or change in head/neck position, or taking an extra or prolonged breath r/t likely increased WOB, for example , may indeed alter physiology in the moment, and lead to silent aspiration of this quite thick consistency.

It is possible a different nipple flow with less oats could be safe and more supportive of dietary needs, using data you have from the study. Given the deep laryngeal penetrations and events of silent aspiration, without defined etiology (ies), I wonder if it might be worrisome for this infant to make changes in nipple and thickening level outside the benefit radiology, which would provide objective data about the impact of the change on physiology and airway protection. Perhaps try to schedule a repeat study soon, so you can objectify the changes you make with your interval  plan that appear clinically to be helpful.  Minimizing x-ray exposure is of course important; he just sounds precarious.

 

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