Diffuse esophageal spasm: the surgical approach.

Diffuse esophageal spasm (DES) is a rare primary motility disorder of unknown cause, that can be found in patients complaining of chest pain and dysphagia and in whom ischemic heart disease and GERD have been excluded. The manometric hallmark of DES is the presence of simultaneous contractions in the distal esophagus alternating with a normal peristalsis. Even at specialized esophageal motility laboratories, DES is considered an uncommon diagnosis. In this review, the authors discuss the clinical and diagnostic aspects of this disease, as well as the possible therapeutic options (medical, endoscopic or surgical therapy). Surgery (esophageal myotomy performed through a thoracotomy or with a thoracoscopic access) seems to have a better outcome than medical or endoscopic treatment, and it is considered "the last resource" in these patients. However, satisfactory results are reported, from highly skilled centers, in only about 70% of treated cases, certainly inferior to those achieved in other esophageal disorders. The role of surgery in this disease requires therefore further study, even if controlled trials are probably difficult to perform, due to the rarity of the disease.

Botulinum toxin in the treatment of diffuse esophageal spasm.

Diffuse esophageal spasm is a primary esophageal motility disorder. The prevalence is 3-10% in patients with dysphagia and treatment options are limited. This review summarizes the treatment of diffuse esophageal spasm, including pharmacotherapy, endoscopic treatment, and surgical treatment with a special focus on botulinum toxin injection. A PubMed search was performed to identify the literature using the search items diffuse esophageal spasm and treatment. Pharmacotherapy with smooth muscle relaxants, proton pump inhibitors, and antidepressants was suggested from small case series and uncontrolled clinical trials. Endoscopic injection of botulinum toxin is a well-studied treatment option and results in good symptomatic benefit in patients with diffuse esophageal spasm. Surgical treatment was reported in patients with very severe symptoms refractory to pharmacologic treatment. This article summarizes the present knowledge on the treatment of diffuse esophageal spasm with a special emphasis on botulinum toxin injection. Endoscopic injection of botulinum toxin is presently the best studied treatment option but many questions remain unanswered.

Use of computerized manometry for the detection of pharyngoesophageal spasm in tracheoesophageal speech.

OBJECTIVE: To evaluate the utility of computerized manometry (CM) to identify pharyngoesophageal segment (PES) spasm during tracheoesophageal speech. STUDY DESIGN: Prospective clinical, controlled study. SUBJECTS AND METHODS: Intraluminal pressures of the PES were collected in 12 tracheoesophageal speakers without spasm and 8 tracheoesophageal speakers with PES spasm before and after localized injection of botulinum toxin to the PES. All subjects underwent voice analysis and videofluoroscopy in addition to CM before and after treatment. RESULTS: All tracheoesophageal speakers with PES spasm presented with mean intraluminal pressures greater than 16 mmHg (mean, 25.36 mmHg). In contrast, mean intraluminal pressures of subjects without spasm was 11.76 mmHg (P<0.05). The negative predictive value associated with the use of 16 mmHg as a threshold value for spasm was 100%. CONCLUSION: CM is a clinically useful tool to aid in speech rehabilitation for tracheoesophageal speakers. Intraluminal pressures of greater than 16 mmHg was highly predictive for PES spasm.

Reabilitação fonatória do laringectomizado total: utilização de toxina botulínica na voz tráqueo-esofágica com prótese fonatória / Botulinum toxin in speech rehabilitation with voice prosthesis after total laryngectomy

Na punção tráqueo-esofágica(PTE) é realizada miotomia do músculo constritor da faringe, mas sua necessidade é entre 9 por cento a 79 por cento dos pacientes. Sua realização pode aumentar as taxas de fístula salivar no pós-operatório. A aplicação da TB é ambulatorial. OBJETIVO: Análise da eficácia da aplicação de toxina botulínica (TB), na reabilitação do laringectomizado total com voz tráqueo-esofágica(VTE) com espasmo(E) do segmento faringo-esofágico (SFE) sem miotomia. MATERIAL E MÉTODOS: Análise de oito pacientes submetidos à laringectomia total (LT), reabilitados com VTE com prótese fonatória (PF), esforço para emissão de voz devido à E do SFE. Todos submetidos a tratamento dessa alteração motora com injeção de 100 unidades de TB no SFE. A avaliação constituiu-se de análise perceptiva de voz, videofluoroscopia (VF) do SFE, análise acústica de voz e manometria computadorizada (MC) do SFE, todos antes e após aplicação de TB. DESENHO DE ESTUDO: Estudo prospectivo. RESULTADOS: Houve diminuição na pressão à MC do SFE, após a injeção de TB. Análise acústica demonstrou melhora na qualidade de harmônicos após o tratamento. Houve emissão de voz sem esforço e melhora do E após o uso da TB. CONCLUSÃO: Todos os pacientes com E do SFE apresentaram melhora vocal após aplicação da TB neste SFE.

In tracheo esophageal puncture (TEP), we carry out a myotomy of the pharynx constrictor muscle; however, about 9 to 79 percent of patients need such procedure. The consequence of such procedure is an increase in salivary fistula rates in the postoperative. Botulin toxin is used in an outpatient basis. AIM: analyzing the efficacy of botulin toxin (BT) use in the rehabilitation of totally laryngectomized patients with tracheo-esophageal voice (TEV) with spasms (S) of the pharyngo-esophageal segment (PES) without myotomy. MATERIALS AND METHODS: We analyzed eight patients submitted to total laryngectomy (TL), rehabilitated with TEV, with speech prosthesis (SP) and struggle to utter voice because of PES spasms. They were all submitted to treatment of such motor alteration with the injection of 100 units of BT in the PES. The evaluation was based on perceptive voice analysis, video fluoroscopy (VF) of the PES, acoustic voice analysis and computerized manometry (CM) of the PES, all before and after BT injection. STUDY DESIGN: prospective. Results: There was a reduction in PES CM pressure after BT injection. Acoustic analysis showed an improvement in harmonics quality after treatment. There was smoother voice utterance and spasm improvement after BT. CONCLUSION: all patients with PES spasms presented vocal improvement after BT injection in the PES.

[Injection of botulinum toxin for diffuse esophageal spasm]. / Endoskopische Injektion von Botulinumtoxin A bei einem Patienten mit diffusem Osophagusspasmus.

HISTORY AND ADMISSION FINDINGS: A 67-year-old man complained of recurrent retrosternal pain for 3 - 5 minutes as the only symptom for 3 - 4 years. Only when dysphagia occurred in the course of the disease was a gastroenterologist consulted. INVESTIGATIONS: The diagnosis of diffuse esophageal spasm was confirmed by esophageal manometry and radiology. Upper gastrointestinal endoscopy revealed no further pathological findings. TREATMENT AND COURSE: 100 units of botulinum toxin were injected in 10 divided doses, into the tubular esophagus, 1 cm apart, beginning at the z-line of the gastroesophageal junction, deeply into the muscular coat of the posterior part of the esophageal wall. This achieved improvement of symptoms as well as esophageal transit time measured by scintigraphy. The symptomatic benefit has now lasted for several months. CONCLUSION: The treatment of patients with diffuse esophageal spasm with botulinum toxin is a promising therapeutic option, which can be safely performed in such patients. But this regime needs evaluation in controlled prospective clinical studies.

Diffuse oesophageal spasm masking achalasia.

Several reports have suggested that esophageal motility disorders may progress from one type to another. A 41-year-old female patient underwent thoracoscopic esophagomyotomy for diffuse esophageal spasm (DOS) with normal resting pressure and complete relaxation of the LOS; findings were confirmed in two preoperative esophageal manometries. Postoperatively, she developed severe dysphagia, and a new esophageal manometry concluded achalasia. She underwent a laparoscopic Heller's myotomy and a posterior (180 degrees) Toupet's fundoplication. Since the second operation, she remains asymptomatic and does not experience any difficulty in swallowing. We concluded that DOS and achalasia might coexist in this case. Through multiple synapses and several nervous roots in the esophageal wall, the inhibitory neurons at the level of LOS were effective before esophagomyotomy and did not show symptoms and manometric findings suggestive for achalasia. Esophagomyotomy, causing disruption of these synapses and lost of inhibitory innervation, finally resulted in symptoms and manometric findings of achalasia.

Treatment of symptomatic diffuse esophageal spasm by endoscopic injections of botulinum toxin: a prospective study with long-term follow-up.

BACKGROUND: Diffuse esophageal spasm is a rare esophageal motility disorder for which there are no satisfactory pharmacologic alternatives for treatment. The aim of this study was to investigate whether botulinum toxin (BTX) injection is an effective short- and long-term treatment for patients with symptoms caused by diffuse esophageal spasm. Whether recurrence of clinical symptoms can be successfully retreated by BTX injection was also studied. METHODS: Nine symptomatic patients (6 women, 3 men; 57-86 years) with manometrically proven diffuse esophageal spasm underwent BTX injection. One hundred IU BTX were diluted in l0 mL of saline solution and injected endoscopically at multiple sites along the esophageal wall beginning in the region of the lower esophageal sphincter and moving proximally in 1- to 1.5-cm intervals, and into endoscopically visible contraction rings. Symptom scores based on an analogue scale for dysphagia, regurgitation, and noncardiac chest pain were assessed before and after therapy, 1 day thereafter, and at 1 and 6 months. RESULTS: Symptoms improved immediately in 7 (78%) patients after 1 injection session. After 4 weeks 8 (89%) patients were in remission with a decrease in total symptom score. The total symptom score decreased from a median 8.0 (interquartile range: 6.75; 9.0) before treatment to 2.0 (1.5; 3.75) after 1 day (p < 0.01) and to 2.0 (interquartile range: 0.75; 3.0) after 1 month (p < 0.01). After 6 months all 8 patients with a response at 1 month still had a symptom score of 3 or less without further treatment. Subsequently 4 patients required reinjection 8, 12, 15, or 24 months after the initial treatment with similarly good results. No serious adverse effects were observed. CONCLUSIONS: BTX injection at several levels of the tubular esophagus is an effective treatment for patients with symptoms caused by diffuse esophageal spasm. Symptom relapse can be effectively treated by repeated BTX injection.

Peppermint oil improves the manometric findings in diffuse esophageal spasm.

BACKGROUND: Diffuse esophageal spasm (DES) is an uncommon condition that results in simultaneous esophageal contractions. Current medical treatment of DES is frequently unsatisfactory. We hypothesized that, as a smooth muscle relaxant, peppermint oil may improve the manometric findings in DES. STUDY: Eight consecutive patients with chest pain or dysphagia and who were found to have DES were enrolled during their diagnostic esophageal manometry. An eight-channel perfusion manometry system was used. Lower esophageal sphincter pressure and contractions of the esophageal body after 10 wet swallows were assessed before and 10 minutes after the ingestion of a solution containing five drops of peppermint oil in 10 mL of water. Each swallow was assessed for duration (seconds), amplitude (mm Hg), and proportion of simultaneous and multiphasic esophageal contractions. RESULTS: Lower esophageal sphincter pressures and contractile pressures and durations in both the upper and lower esophagus were no different before and after the peppermint oil. Peppermint oil completely eliminated simultaneous esophageal contractions in all patients (p < 0.01). The number of multiphasic, spontaneous, and missed contractions also improved. Because normal esophageal contractions are characteristically uniform in appearance, variability of esophageal contractions was compared before and after treatment. The variability of amplitude improved from 33.4 +/- 36.7 to 24.9 +/- 11.0 mm Hg (p < 0.05) after the peppermint oil. The variability for duration improved from 2.02 +/- 1.80 to 1.36 +/- 0.72 seconds (p < 0.01). Two of the eight patients had chest pain that resolved after the peppermint oil. CONCLUSIONS: This data demonstrates that peppermint oil improves the manometric features of DES.

Emprego da toxina botulínica no tratamento do espasmo difuso esofagiano. Relato de caso / Use of botulin toxin in the treatment of esiohageal spasm. Case report

O espasmo difuso do esôfago destaca-se dos demais distúrbios motores pela gravidade dos sintomas e falta de resposta satisfatória ao tratamento conservador. Esta condiçao requer indicaçao de tratamento cirúrgico ou dilatador. Nos pacientes de alto risco cirúrgico, esta situaçao configura grave problema, pela falta de outra opçao terapêutica. Em paciente nessa condiçao utilizamos a injeçao endoscópica de toxina botulínica na cárdia com bons resultados após seguimento de três meses. Apesar do curto período de observaçao, acreditamos que a infiltraçao de toxina botulínica na cárdia constitui opçao terapêutica válida no tratamento do espasmo difuso do esôfago.

[Perforation of the esophagus after esophageal manometry]. / Osophagusperforation nach Osophagusmanometrie.

HISTORY AND FINDINGS: A 75-year-old man was admitted for oesophageal manometry because of dysphagia for the past 2 years and retrosternal burning sensation unrelated to exercise. His general condition was appropriate for his age. INVESTIGATIONS: An oesophagogram showed corkscrew-like deformation of a diffuse oesophageal spasm. The first, but incomplete, manometry recorded clearly propulsive contractions with markedly raised and prolonged pressure, as in "nutcracker oesophagus". The lower oesophageal sphincter could not be demonstrated initially. Subsequent pH measurements provided no evidence for increased gastrooesophageal reflux. TREATMENT AND FURTHER COURSE: After the first manometry conservative treatment was initiated with molsidomine, nifedipine and nitrospray sublingual, but the dysphagia was not significantly improved. A second manometry was performed before a planned surgical exploration. Placing of the catheter was again difficult and mild resistance experienced. Endoscopy revealed only minimal, presumably superficial, mucosal lesions. 2 days later bilateral pleural effusions together with mediastinitis occurred. Conservative treatment was continued until finally a distal oesophageal perforation was demonstrated. At surgery the perforation was seen and a oesophagectomy with gastric pull-through and intrathoracic anastomosis performed. However, the patient died of septic multi-organ failure. CONCLUSIONS: Oesophageal manometry is a safe but invasive method with few complications for measuring oesophageal motility. Although this has not previously been reported, oesophageal perforation with mediastinitis may end fatally, if the particular circumstances are unfavourable. In addition to special anatomical features, type and state of the manometric catheter may present a risk factor.

Using botulinum toxin A to improve speech and swallowing function following total laryngectomy.

OBJECTIVE: To evaluate a technique to reduce dysfunctional spasm in the pharyngoesophageal segment (PES) in patients after laryngectomy. DESIGN: Pharyngoesophageal segment function related to voice and/or swallowing in patients who had undergone a laryngectomy was evaluated before and after the injection of botulinum toxin A. SETTING: Academic referral medical center. PATIENTS: Eight outpatients with voice and/or swallowing complaints after undergoing a total laryngectomy. INTERVENTIONS: Videofluoroscopic contrast examination was completed to identify stricture vs spasm in the PES in patients with voice and/or swallowing complaints after undergoing a laryngectomy. Lidocaine hydrochloride injection under fluoroscopic guidance was completed to facilitate immediate relaxation of spasm. After positive results with lidocaine, botulinum toxin was injected into the same area to facilitate longer-lasting benefit. MAIN OUTCOME MEASURE: Patient report of benefit and videofluoroscopic evaluation of PES function. RESULTS: Six of 8 patients demonstrated improved function within the PES after lidocaine injection. Five of these 6 received transcutaneous injection of botulinum toxin. Four of the 5 patients demonstrated improved swallowing and/or voice function, and 3 of these 4 received subsequent injections of botulinum. No serious complications were encountered. CONCLUSIONS: Transcutaneous injection of botulinum toxin in the PES under videofluoroscopic guidance provides improvement in voice and/or swallowing function without significant complications. Additional clinical study will be required to evaluate dose and technique influences on degree and duration of benefit and complications.

The diagnostic and therapeutic contribution of calcium channel inhibitors (nifedipine) in esophageal spasm.

The effect of nifedipine (10-20 mg) on esophageal motility was tested in 18 patients with clinical esophageal syndrome and radiologic aspect suggesting esophageal spasm. The drug was administered 20-30 min prior to the second radiologic examination. In 3 cases of achalasia the drug was administered in doses of 10 mg/day for 10 days before the second X-ray examination. Fiber esophagoscopy was performed in all the cases. The initial radiologic aspect was favourably influenced by nifedipine administration in 12 cases confirmed as esophageal spasm. The other 6 patients who did not benefit by the test were cases of organic diseases: 2 post caustic stenoses and 4 cases of eso-cardio-tuberal neoplasm. In the 3 cases of achalasia, treatment with nifedipine led to clinical improvement with relaxation of inferior esophageal contraction on radiologic examination. The study demonstrated the favourable contribution of calcium channel inhibitors to the diagnosis and treatment of esophageal motility disturbances.