A patient's story: An interdisciplinary approach to a child with dysphagia
by Joan Arvedson, PhD; Robert Chun, MD; Richard Noel, MD, PhD; Cecille Sulman, MD; and Neelesh Tipnis, MD
Joan C. Arvedson, PhD, is program coordinator of Feeding and Swallowing at Children's Hospital of Wisconsin, a clinical professor of Pediatrics (Gastroenterology) at The Medical College of Wisconsin and a member of Children's Specialty Group.
Robert Chun, MD, is a pediatric otolaryngologist at Children's Hospital of Wisconsin, an assistant professor of Pediatric Otolaryngology at The Medical College of Wisconsin and a member of Children's Specialty Group.
Richard Noel, MD, PhD, is a pediatric gastroenterologist and program director of Feeding at Children's Hospital of Wisconsin, an assistant professor of Pediatrics (Gastroenterology) at The Medical College of Wisconsin and a member of Children's Specialty Group.
Cecille Sulman, MD, is a pediatric otolaryngologist and medical director of Otolaryngology at Children's Hospital of Wisconsin, an assistant professor and interim chief of Pediatric Otolaryngology at The Medical College of Wisconsin and a member of Children's Specialty Group.
Neelesh Tipnis, MD, is a pediatric gastroenterologist and program director of Endoscopy at Children's Hospital of Wisconsin, an assistant professor of Pediatrics (Gastroenterology) at The Medical College of Wisconsin and a member of Children's Specialty Group.
|The Airway, Digestive and Voice Center at Children's Hospital of Wisconsin provides a multidisciplinary approach to patients with complex aerodigestive disorders. Members of the team (from left) include Joan Arvedson, PhD; Robert Chun, MD; Richard Noel, MD, PhD; Neelesh Tipnis, MD; and Cecille Sulman, MD.|
He otherwise met all other developmental milestones in speech and fine motor skills, had a normal brain MRI, but still was failing to thrive. He had one episode of RSV at 15 months and no symptoms of upper or lower airway obstruction.
Previous diagnostic testing
An upper gastrointestinal (UGI) contrast study was concerning for cricopharyngeal achalasia. Esophagogastroduodenoscopy (EGD) showed no abnormalities except a narrow upper esophageal sphincter that did not allow expected passage of a 9 mm gastroscope.
A bronchoscopy showed normal vocal cord function and normal glottic and tracheal anatomy. A video fluoroscopic swallow study (VFSS) was performed to assess the upper aerodigestive anatomy and dynamic function. It demonstrated a posterior cricopharyngeal bar and aspiration on thin and thickened consistencies, typically after swallows, on residue in the pharynx due to inability to clear the bolus through the upper esophageal sphincter (UES).
Cricopharyngeal musculature was injected with botulinum toxin with a repeat UGI showing resolution of the cricopharyngeal bar and no aspiration, but within 10 weeks his dysphagia returned and he resumed his NPO status. Due to continued dysphagia, aspiration and failure to thrive, a gastrostomy tube was placed at 8 months.
The patient's care was then transferred to Children's Hospital of Wisconsin.
Differential diagnosis of dysphagia etiology
Interdisciplinary approach to patient care
Upper endoscopy (EGD) was useful in ruling out eosinophilic esophagitis and any other inflammatory problem that can present with infantile feeding disorders and dysphagia. Contrast studies and EGD identified a narrow cricopharyngeal luminal diameter, which subsequently improved after injection with botulinum toxin. Recurrence of symptoms may simply indicate cessation of the botulinum toxin effect. While positive response to botulinum toxin suggests cricopharyngeal achalasia, prior to a definitive operative treatment, manometric studies can functionally confirm a diagnosis of cricopharyngeal achalasia.
High-resolution esophageal manometry was performed and noted elevated resting pressure, poor relaxation of the cricopharyngeal musculature and poor function of the muscle. Pharyngeal contraction pressure and esophageal body function was normal. These findings confirmed the diagnosis of cricopharyngeal achalasia. Previous brain MRI failed to identify posterior fossa abnormalities that can be associated with cricopharyngeal achalasia.
Despite the feeding disorder, this patient was well nourished and growing appropriately. The benefits of supplemental gastrostomy nutrition in this case cannot be understated, as it allows the medical and surgical focus to remain strictly on resolution of dysphagia without worries about malnutrition and its impact on development.
Children with aerodigestive pathologies almost always have poor respiratory health. The maintenance of respiratory health is a challenge and often involves diagnosing the airway and lung disease. A combined assessment of the airway by both otolaryngology and pulmonology are complementary. Rigid bronchoscopies are most useful in identifying anatomic abnormalities such as laryngeal clefts and subglottic stenosis. Flexible bronchoscopy is a useful dynamic assessment of the lower airway, especially for lesions such as tracheobronchomalacia.
Management often includes focusing on airway clearance in addition to inhaled and oral anti-inflammatory medication. Due to our patient's history of aspiration and RSV bronchiolitis, there were long-term concerns for his respiratory morbidity with continued feeding difficulty and aspiration. In light of his resolving bronchiolitis, inhaled steroids and chest physical therapy were recommended.
When our patient presented at 16 months, VFSS revealed intermittent silent trace aspiration events with very thin (juice) and thickened (nectar) liquid. Aspiration was seen more frequently, but very small volume per bolus, after swallows of smooth puree (pudding) because of the reduced opening of the UES noted by cricopharyngeal (CP) bar with increased pressure at the UES, consistent with manometry findings. Specific goals to advance oral feeding were established in light of the global physiologic findings.
The follow-up VFSS was completed about 24 hours after the myotomy. A CP bar was not prominent. Boluses moved through the UES with no restriction. Trace aspiration on very thin liquid was noted one time just prior to initiation of a pharyngeal swallow due to a timing problem, not a CP deficit. A small ÒpouchÓ was noted at the posterior UES with a very small amount of barium contrast seen in the superior retropharyngeal space. Overall, this child showed remarkable improvement in swallowing just 24 hours following surgery.
Communication with parents a few weeks after discharge indicated that functional oral feeding gains were occurring with gradual increases in volume of oral feeding and expansion of textures. Pharyngeal pressures had been reported within normal limits via manometry preoperatively, which was an important finding to support a positive prognosis for oral feeding gains anticipated following surgery.
Given that the botox temporarily resolved the child's symptoms and the desire for definitive treatment, surgical options included external versus endoscopic cricopharyngeal myotomy. The cricophayrngeus muscle forms the upper esophageal sphincter and under tonic contraction prevents food passage.
External approach risks include external incision, restenosis or scarring, salivary fistula, esophageal perforation, mediastinitis, vocal cord paralysis and hoarseness.
Endoscopic approach risks include restenosis or scarring, retropharyngeal space infection and mediastinitis, thermal injury, fistula formation and subcutaneous emphysema.
Treatment and outcomes
The patient underwent a coordinated EGD, bronchoscopy and endoscopic cricopharyngeal myotomy. A postoperative VFSS demonstrated resolution of the cricopharyngeal bar and minimal aspiration. There was a pharyngeal defect secondary to the surgery, and the patient developed no signs or symptoms of infection. The patient was discharged to home with continued oral feeding and swallow therapy.
- Differential diagnosis for dysphagia is broad and diverse.
- Children with cricopharyngeal achalasia typically have multiple issues in their swallowing difficulties, not a simple UES dysfunction.
- Botox injection can provide temporary alle viation of symptoms.
- Surgical management may be considered for long-term management.
- With complex patients with dysphagia, an interdisciplinary approach is best to evaluate and manage swallowing, feeding and breathing disorders. Professionals make the best decisions when interdisciplinary team members communicate directly among themselves and with parents at every step of the diagnostic workup, decision-making and follow-up treatment.
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