Diffuse refractory esophageal spasm with excellent response to treatment with sildenafil

Puya Gamarro, Marina; López Vega, María del Carmen; Méndez Sánchez, Isabel María

doi:10.1016/j.medcle.2020.04.056

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He had no remarkable clinical history, and the physical examination revealed no significant findings. Given the above, he was prescribed omeprazole 20 mg/12 h and underwent an esophagogastroduodenoscopy (EGD) with esophageal biopsies. The EGD revealed no macroscopic or histological alterations. Because of the lack of improvement after 6 weeks of treatment, we requested the conduct of a manometry and esophageal pH-metry, which revealed signs of physiological gastroesophageal reflux. The manometry showed a lower esophageal sphincter (LES) with a normal tone and >20% premature contractions, compatible with diffuse esophageal spasm (DES) according to the Chicago 3.0 classification. Based on these findings, treatment with nifedipine 10 mg/8 h was prescribed, achieving only a mild improvement. Given that a subsequent esophagogram revealed multiple areas of uncoordinated esophageal contractions, the patient’s treatment was changed to isosorbide dinitrate 20 mg/8 h. However, due to his poor response to this regimen, it was later replaced by amitriptyline 25 mg/day. Owing to his refractory symptoms, sildenafil 50 mg/day was added to his treatment, observing a significant subsequent improvement. During the last check-up, the patient reported only occasional dysphagia, but no regurgitation or pain (Eckardt-1).DES is caused by alterations in the inhibitory innervation that result in dysphagia and retrosternal pain. The Chicago 3.0 classification defines it as the presence of premature contractions in at least 20% of deglutitions, together with a normal relaxation of the LES. Distal latency demonstrates a stronger correlation with these symptoms, with most patients with a distal latency <4.5 s being diagnosed with DES or type III esophageal achalasia.<a class="elsevierStyleCrossRef" href="#bib0005">1</a>The combined prevalence of DES, spastic achalasia, and esophageal hypercontractility is 2%. In the event of suspected DES, other causes of chest pain should be ruled out,<a class="elsevierStyleCrossRef" href="#bib0010">2</a> including, in the first place, heart diseases. Then, in addition to an adequate history taking, it is important to rule out other gastroesophageal diseases, to which end a gastroscopy, manometry, and esophageal pH-metry are all useful. Nevertheless, detecting this condition can be hard owing to its intermittent nature.<a class="elsevierStyleCrossRef" href="#bib0015">3</a>Because the underlying neuropathological process cannot be cured, therapeutic approaches are aimed at relieving the physiological obstruction by surgical or endoscopic disruption of the muscle, or with the aid of smooth muscle relaxants targeting the LES, thus reducing the occurrence of spastic contractions.<a class="elsevierStyleCrossRefs" href="#bib0005">1,2</a>Treatment remains a challenge due to an insufficient understanding of the pathophysiology of the disease and the lack of controlled clinical trials studying this condition.The first drugs used to treat this ailment were smooth muscle relaxants, including nitrates and calcium channel blockers, the most commonly used ones being isosorbide dinitrate and nifedipine. Other less frequently used drugs include anticholinergics, amyl nitrite, nitroglycerin, theophylline, beta-2 agonists, and, recently, phosphodiesterase inhibitors (sildenafil).<a class="elsevierStyleCrossRef" href="#bib0020">4</a>The latter block the degradation of nitric oxide, thus resulting in a more prolonged relaxation, relieving the esophageal symptoms, and improving the manometric findings.<a class="elsevierStyleCrossRef" href="#bib0020">4</a> The limitations of these drugs are their side effects and high price.<a class="elsevierStyleCrossRef" href="#bib0010">2</a> The most frequently reported adverse reactions are headache, flushing, dyspepsia, nasal congestion, dizziness, nausea, and visual alterations.<a class="elsevierStyleCrossRef" href="#bib0025">5</a>The chest pain associated with DES occasionally responds to anti-reflux treatment. If this treatment fails, other available alternatives include tricyclic antidepressants, selective serotonin reuptake inhibitors, and trazodone, as anxiety control is extremely important in this condition.<a class="elsevierStyleCrossRef" href="#bib0020">4</a>Although both the Heller myotomy and pneumatic esophageal dilation are established treatments for achalasia, response to these procedures is poor in the case of spastic disorders, as the disease not only affects the LES, but also the esophageal body, therefore generally requiring a more extensive myotomy.<a class="elsevierStyleCrossRef" href="#bib0010">2</a>Finally, per-oral endoscopic myotomy (POEM) has recently been proposed as another treatment option and is associated with a high success rate. Its associated side effects are acceptable, and it offers the advantage of allowing the length of the myotomy to be adjusted according to the manometric findings and to achieve a prolonged, sustained benefit.<a class="elsevierStyleCrossRef" href="#bib0005">1</a> Although the results of this procedure are promising, the number of patients with DES who are treated with POEM is low.To conclude, we must insist on the low prevalence of these disorders, which pose a diagnostic and therapeutic challenge. In our particular case, prior to applying invasive therapies lacking sufficient evidence for this type of disorder, we chose to use sildenafil considering that it is a pharmacological treatment with acceptable associated side effects. However, given the patient’s age, we cannot rule out the need for other treatments in the future, including the POEM technique, which has demonstrated promising results in this context." 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Kahrilas" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.gtc.2012.11.002" "Revista" => array:6 [ "tituloSerie" => "Gastroenterol Clin North Am" "fecha" => "2013" "volumen" => "42" "paginaInicial" => "27" "paginaFinal" => "43" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23452629" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Espasmo esofágico" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. Pérez Lasala" 1 => "N. Mañas Gallard" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Rev Esp Enferm Dig" "fecha" => "2014" "volumen" => "106" "paginaInicial" => "425" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25361455" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Esophageal motility disorders medication" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "A. Eric" 1 => "M.D. Gaumnitz" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "WWW" => array:2 [ "link" => "https://emedicine.medscape.com/article/174783-overview" "fecha" => "2017" ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:1 [ "referenciaCompleta" => "Agencia Europea del Medicamento. Ficha técnica de la Viagra®. 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Vol. 157. Issue 2.

Pages 94-95 (July 2021)

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