Question:

I have an infant (2-week-old) coming in for a feeding eval next week (mother reported difficulty latching on breast and bottle, feeds last 30-40 min, infant not gaining back birth weight), referred by ENT, script says micrognathia and glossoptosis. I did a quick research scan and didn’t find a lot regarding micrognathia besides osteogenesis. Open to suggestions or any research articles you can point me to regarding this topic… who to refer to? any other treatment you’d recommend?

Answer:

This clinical presentation in infants can be quite complex. Sucking, swallowing and the swallow-breathe interface can all be affected, as can overall postural control and sensory-motor learning. Once you see the infant, can begin your differential about what relevant areas are impacting swallow function, and what other consults or diagnostics might be helpful.

Good that you already have an ENT involved who has evaluated the infant and provided impressions. Do you know if there was a flexible scope done as part of the ENT consult? You may want to check, as that will also be helpful to better understand the functional dynamics of the base of tongue on this infant’s swallow. Due to the glossoptosis, you will likely hear inspiratory stridor created by intermittent obstruction of the base of the tongue against the posterior pharyngeal wall. This can interfere with swallow-breathe interface and alter swallowing physiology. An elevated swaddled sidelying position will help to utilize the effects of gravity to help bring the mandible forward. An instrumental assessment of swallowing physiology will likely be a part of your differential, as there is often altered oral and pharyngeal phase physiology that creates risk for both silent and symptomatic airway invasion with this clinical presentation.

It is also likely that there may be associated tethered oral tissues, as the timing of developmental emergence of structures in utero predisposes these correlations. As mentioned already, the tethering, if it is present, can actually help the infant to compensate for the glossoptotic forces and therefore help keep the tongue from falling farther into the hypopharynx.  This reminds us of how important it is to not “signal react” to one aberration (e.g., tongue tie) , without considering its dynamic influence on the big picture, i.e., releasing the tethering would cause more problems, given this infant’s co-morbidities. So, compensating for the adverse impact of possible tethering on the suck may be a focus. The infant may have a “compression-only” sucking pattern r/t the glossoptosis in and of itself, as it often leads to a thickened and bunched lingual body (intrinsic and extrinsic tongue muscles often don’t develop properly in the setting of this malformation). The altered lingual control can affect not only establishment of suction but also oral bolus control, and cause “problems down the line” (i.e., with pharyngeal function). The Dr. Brown’s nipple with specialty valve may work but sometimes the tongue may be too far back in the mouth to even permit an effective latch with that system. An option would be the Haberman, but without any squeezing of the teat; increasing flow rate would be contraindicated with this clinical presentation, as it would increase bolus size and adversely affect bolus control and airway protection

Co-regulated pacing and resting will be essential, as the infant’s WOB (work of breathing) will be increased. Breathing is typically adversely affected by challenges with airway maintenance created by the need for further retraction of the tongue base that is part of the swallow process. For this infant, that will make it difficult for the infant to maintain the airway as it reconfigures from a respiratory tract to an alimentary retract dynamically during feeding.

Mother will likely need your support to focus on infant-guided feeding (versus volume), learning about what the infant’s behaviors during feeding mean (what infant is trying to communicate), waiting for the infant to root (that suggests he is then, at that moment, better managing his airway and is ready to suck), and thoughtfully and contingently offering interventions to optimize safety.

This wonderful paper by my friend and colleague Laura Brooks at Children’s Healthcare of Atlanta provides valued additional insights for my rationale to consider a swallow study to objectify physiology, given this infant’s co-morbidities:

Brooks, L., Evans, S., Alfonso, K. et al. The Role of Dysphagia Assessment in the Identification of Upper Airway Obstruction in Infants. Dysphagia (2022). https://doi.org/10.1007/s00455-022-10433-5

This pathophysiology Laura describes is not uncommon when an infant presents with these challenges. The alterations along the swallow pathway created by glossoptosis may be part of the reason for poor intake. Perhaps the infant is trying to protect his airway when he purposefully disengages during feeding. I appreciate Laura’s adding to our evidence base and reinforcing that “co-morbidities matter,” as I always like to say.

Question:

I have a question about an NICU patient who is currently 39-3, formerly 25-2. BPD, chronic respiratory, very prolonged intubation. I did pre-feeding milk drops, etc but she never even began cueing until 35 weeks when on 2Liters. I used a Dr. Brown’s Ultra Preemie and progressed PO very slowly due to increased WOB. She has now had steroids and was just weaned to room air this past week. She takes full volume and is doing much better pacing herself and I have upgraded her to a Dr. Brown’s Preemie.  She has always had spells and apnea but for the past 3 weeks every time you go to burp her, she bears down like she is trying to maybe protect her airway from reflux, she then holds her breath and desats. I have tried smaller volumes and burping more frequently but no luck getting her burp until further into the feeding. The desats are not as low as they once were but I just feel like I see so many babies that are suffering from large volumes and experience reflux. She does have occasional choking spells and I offered to do MBSS but was shot down. The neo that day wanted to thicken with rice. We haven’t been thickening very much since I came to the unit and when the neos want to thicken they choose oatmeal. There is also a SIM spit up formula for term babies that is thicker. What should I do in this situation? I fear I am missing aspiration but most of the babies I take for fluoro don’t aspirate and I just see the retrograde flow (reflux). I have read laying them on their right side may help. What are our research proven reflux tools – I know a lot of the answer is -time to mature but is there anything else I should be doing?

Answer:

Sounds like you have offered her thoughtful preparation for infant-guided, neuroprotective feeding. I’d be extra careful about letting her set her own pace, given her respiratory hx. Even healthy term NBs don’t “pace themselves” until closer to 2 months of age per the research. Though she may take breaths on her own with PO, given her hx it is likely the breaths may be even a bit latent or a bit insufficient, or she may have some element of gulping that results in air ingestion. Have you tried burping in elongated sidelying? I find that better takes the pressure off the abdomen than semi-upright burping in caregiver’s lap (since increased abdominal pressure which can provoke EER/LPR). The choking could be co-occurring EER/LPR or events of airway invasion/t mistiming of swallow-breathe interface, which is what we typically see if we were to do a VFSS on an infant with her hx and diagnoses. We rarely thicken too. Seems with Dr Brown’s nipples and contingent co-regulated pacing with resting and elevated elongated sidelying we can minimize EER/LPR events as much as possible and avert need to thicken for reflux. At the same time, we are optimizing the swallow-breathe interface with the same interventions that also minimize/inhibit gulping (and air swallowing). Sim Spit up has a slightly increased viscosity which may help – but I’d try the more strict/frequent pacing. Yes, our neos are strict about VFSS understandably but this hx is worrisome and is the classic presentation that is a silent aspirator when objectified.  Studies show that L side down the for first hour post feeding then R side down the second hour post feeding is optimal based on gut anatomy –but RNs must balance head shaping and “changing position with cares” as well, so it cannot be a perfect world. This is a fabulous paper on reflux! Does not include newer meds due to publication date but is otherwise so wonderful!— Schurr, P., & Findlater, C. K. (2012). Neonatal mythbusters: evaluating the evidence for and against pharmacologic and nonpharmacologic management of gastroesophageal reflux. Neonatal Network, 31(4), 229-241.

Question:

I work in a level 2 NICU.  We are a small unit with anywhere from 0 to 9 babies at a time.  We don’t often have infants with respiratory compromise nor do we have many infants born prior to 30 weeks’ gestation.  My question is in relation to this population:  premature, occasional resp problems that often are managed and then resolve by discharge.

I had an infant recently with difficulty with getting a proper latch on the bottle when presented.  Infant was a 38 weeker, SGA, initial hypoglycemia, fetal cephalhematoma (no fracture or IVH), and initial hyperbilirubinemia.  He has no known drug exposure and MOB was allowed to breastfeed.  Infant appeared to latch well and was satisfied to breast feed as he would be calm and latch immediately to mom’s breast.  However, since mom’s milk supply was still low, and she was unable to feed every feeding, and infant weight gain was important due to SGA, the infant required bottle feeding.  When presented with the nipple the infant was eager, almost frantic.  Despite palatal placement with gentle pressure the infant continued to root for the nipple.  Nursing reported, “I know that I’m not supposed to use chin support but I did.  I felt like it helped him to find and maintain the latch on the bottle.”  I also noticed that the mother had a similar approach to bottle feeding her infant, also including cheek support.  My approach was to swaddle the infant in a cradled upright position.  Doing that along with palatal placement with gentle pressure as well as providing pressure to the corners of the mouth to increase “feedback” helped on occasion but the feeding was still laborious as this still resulted in a lengthy feeding.  My thought was that, I noticed that when the infant breastfed 1. MOB was so quick that the infant did not have time to work up into a frenzy, 2. infant was well wrapped and contained, 3. the infant mouth and face were receiving pressure against the breast.  I was unable to successfully replicate this for bottle feeding.  Additionally, nursing staff decided to try a faster flow.  I watched the mother feed the infant in an upright cradle position, using cheek support as well.  Initial look on the infant’s face was that the flow was fast, but was able to get into a rhythm with feeding.  So, my question is, is cheek and chin support appropriate in this situation?  What should I try in the future for similar situations of excessive rooting?

Answer:

You’re doing a good job trying to sort out what is going on. The population in your Level II NICU sounds very typical, mostly GA 30+ weeks and typical respiratory issues associated with preterm birth, that can indeed affect coordination but are not enduring. May often see increased WOB with intermittent tachypnea due to the aerobic demands of feeding. So, breathing regulation is a component we would want to specifically support.

This little guy due to being SGA likely has some challenges with state regulation and can look/get “disorganized” due to more rapid movement to active alert state. One neo years ago told me the altered state regulation in SGA infants is likely related to the increase in catecholamines provoked when one is born SGA that creates this altered state regulation; this then can create poor latch at times, rapid state change to crying, contributing to an ineffective latch and ineffective feeding. Vestibular input (gentle slow rocking, one per second gentle patting to calm the regulatory system) would be important prior to and contingently during feeding, perhaps starting with a few pacifier dips to entrain the oral-sensory-motor system and promote organization without flow at first.

Of course, as you know, “poor latch” can be for so many reasons, sometimes more than one for an infant. Was managing flow rate at breast well but mom’s supply low. Not latching to nipple readily …my first thought is… was the flow from the man-made nipple too fast so he could not organize suck even to start? Or was it hard for him being SGA to transition to a rubber nipple especially if mom’s breasts are larger? Is odd that sensory input to palate (from, I hope, a drained man-made nipple), did not elicit his root-to-latch sequence. Mom likely learned cheek support from the RN – it of course creates flow without active suction/latch perhaps used to “get him infant going”?? But meanwhile he is not learning to establish the motor mapping for an effective root-to-latch sequence for sucking form the bottle—it’s being bypassed. The fast flow nipple selected by RN would be faster than the breast flow – and combined with cheek support and “pressure to the corners of the mouth”, the flow is likely too much, uncouples swallowing and breathing, and interferes with swallow-breathe coordination, in effect provoking less sucking drive and less intake, longer feedings. Agree with secure swaddle for containment and organization, humeral flexion with hands at midline near face. I’d try elevated side lying with capital flexion, eliciting rooting towards midline/towards chin (i.e., into forward flexion) as it can help with organization and support optimal latch by recruiting the proper motor mapping. The infant’s facial expression as if flow was too fast was real, and he might have then tried to accommodate but has limited capacity to do so, which can lead to subtle effects on coordination and likely learning, sometimes maladaptive behaviors.  I suspect with the faster flow there were still subtle occasions of adaptive respiratory behavior such as slight nasal flaring/blanching, slight chin tugging, slight blinking in an attempt to compensate for the faster flow and its impact on the fine coordination of suck-swallow-breathe. No overt events but still a level of physiologic stress, if observed closely. Cheek support would not be helpful based on what I understand about this infant and his presentation. If you see excessive rooting in another infant, focus on “why” that infant with his unique history might be showing that maladaptive behavior and as you unpeel the layers, you will select an intervention clearly mapped to the etiology. Creating flow via a faster flow rate nipple as RNs selected, or passive increase in flow (via cheek support), may be more like band aids, than an etiology-based solution, which I find can be more effective and at the same time promote learning on the infant’s part, with neuroprotection.

I hope this is helpful.

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.

Question:

I have a curious case question to put to you. I just saw a 9-year-old boy with a dx of myotonic dystrophy for a swallow evaluation. However, his mother reports no actual difficulty with swallowing food and liquid, but that he has experienced 4-5 acute episodes of gagging/choking behavior with no oral intake at all, over the last 6 months or so. Apparently, with no liquid or solid material being consumed, he has begun to have episodes where he will cough/gag/choke with such severity that he has difficulty breathing, and it’s very frightening to his teachers/family.

I did a bedside swallow eval and observed very low muscle tone in his tongue, lips and face, but no drooling and no anterior/lateral spillage of bolus textures. There is poor coordination and mastication of solid textures, and often food is swallowed not-fully-masticated, but he showed no s/s of aspiration or penetration with the liquid and solid textures I gave him. I never saw this coughing/gagging behavior today, and don’t quite know what to tell the parents.

My suspicion is that he is aspirating on his own saliva, which does pool in the anterior portion of his oral cavity frequently. But why would this just start to affect him to such an extent, when that has been his baseline for years?

Have any of you heard of this happening with MD? Any ideas a) what is causing the episodes and b) any way to help?

Answer:

Sounds like there are multiple pieces to this puzzle. The systemic hypotonia both distal and proximal makes me wonder about overall motility and integrity throughout the aerodigestive pathway, both oral, pharyngeal, and esophageal and beyond. The lack of adverse overt events reported by family may be an artifact, given that typically hypotonia and hyposensation often go hand in hand. That may be why, along with a likely delay r/t ineffective supra and infrahyoid function, that he does not sense the excess saliva in his oral cavity which you described. The incompletely masticated foods can then be swallowed, likely pharyngeal constrictors are lax to some degree, and the lack of bolus clearance is not sensed until a sufficient volume triggers the altered sensory system, or it eventually migrates toward the airway post feeding.

The likely co-occurring etiology of esophageal dysmotility (EER/LPR) and/or structural/emptying alterations of the esophagus or lower GI tract, or an outpouching, may also be part of the differential. It’s not uncommon for proximal hypotonia to affect the entire body, which would include motility throughout the GI system. Children with postural hypotonia often are high risk for constipation due to poor GI motility and that can create havoc for digestion, provoke reflux and create or exacerbate maladaptive swallowing and feeding behaviors.

Sounds like a VFSS with UGI and small bowel follow through would be very helpful to explore the aerodigestive pathway and objectify his swallowing physiology. I would not be surprised if results indeed raise concerns about silent airway invasion and multiple competing etiologies. I like Amanda’s idea of then pulling in neurology, as his myotonic dystrophy may indeed be worsening and be another part of your differential.

You are doing a great job sorting out a very complex presentation. Our kids like to keep us thinking outside the box.

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