Otolaryngologist Approach

As with all patients presenting with dysphagia, a thorough otolaryngologic examination is critical. This would include assessment of general appearance for any syndromic features, nasal patency, and all oral structures. Breathing should be examined/auscultated before, during, and after feeding. Flexible fiber-optic nasopharyngoscopy should also be included to assess pharyngeal and laryngeal anatomy. Though the physical exam of patients with cricopharyngeal achalasia is generally normal, there may be clues present including pooling of secretions in the hypopharynx.

While modified barium swallow study is considered the “gold standard” for diagnosis of cricopharyngeal achalasia, there are a few other studies that may be beneficial in the overall work-up of these patients. These include (1) upper GI endoscopy, (2) pH probe studies, and (3) esophageal manometry.

Upper endoscopy is likely to reveal a tight upper esophageal sphincter but otherwise no other findings specific to cricopharyngeal achalasia. Endoscopy is also useful to rule out any obstructive lesions, as well as to evaluate for reflux and/or eosinophilic esophagitis – factors that could potentiate UES hyperactivity and hypertonicity [9]. Similarly, pH probe studies can help assess for reflux.

Esophageal manometry can be utilized to better define the swallow dysfunction as it relates to the UES. Manometry would be expected to demonstrate high baseline UES pressures with limited relaxation of the UES during swallowing. Manometry – particularly high-resolution manometry – can also assess for abnormal timing and dyscoordination of pharyngeal contraction in relation to UES relaxation, which would result in premature closure of the UES and decreased time available for bolus transit.

Finally, if cricopharyngeal achalasia is diagnosed, further evaluation for the etiology of this issue is recommended. Specifically, a thorough neurologic evaluation should be undertaken, with strong consideration of head MRI to assess for posterior fossa abnormalities such as Chiari malformation.

Options for management of cricopharyngeal achalasia range include simple observation, dilation, Botox injection, and surgical (open or endoscopic) myotomy. In all cases, treatment to prevent reflux should be included, as exposure of the UES to refluxate is thought to aggravate muscle spasm and hyperactivity.

Nitrates and calcium channel blockers – such as nifedipine – have shown promise in the treatment of lower esophageal sphincter disorders; however, these do not have clear and consistent effects at the UES and, therefore, are not considered viable treatment options for cricopharyngeal achalasia.

Though difficult to predict , there are reports of spontaneous resolution of cricopharyngeal achalasia, particularly in neonates and infants. Thus, treatment options that are temporary (such as dilation or Botox injection) may be reasonable in the hopes of being able to avoid the risks involved with myotomy.

In some cases, observation alone – with provision of an alternative feeding modality such as a gastrostomy tube – might be considered appropriate for treatment of cricopharyngeal achalasia when there are significant additional neurologic and functional deficits that would otherwise preclude development of normal feeding and swallowing. In cases where the child is otherwise normally developing, however, observation without specific treatment is not ideal. Cricopharyngeal achalasia typically results in severe swallow impairment that is not likely to improve with a change in feeding technique or food consistency. And while the natural history of this condition is unpredictable (and spontaneous resolution has been described), placement of a nasogastric or gastric feeding tube to merely temporize during a period of observation has the additional disadvantage of inhibiting development of normal feeding behaviors.

Operative Approaches