achalasia

Moyamoya disease is a condition caused by the obstruction of intracranial vessels, which can lead to ischemic or hemorrhagic vascular events. Most patients have underlying risk factors that contribute to the development of this vascular disease. This article presents a case of Moyamoya disease associated with esophageal achalasia in an 11-year-old boy with a family history of the condition. Comprehensive examinations of this patient did not reveal any complications other than achalasia as a risk factor.

Heller myotomy has been considered the standard surgical treatment for patients with achalasia. Since the initiation of peroral endoscopic myotomy (POEM), it has represented an alternative for treating patients with achalasia. Over the years, numerous prospective and retrospective studies with POEM use for achalasia have been published. We performed a systematic review and meta-analysis to evaluate the efficacy and safety of POEM in patients with achalasia.

Publications investigating the safety and efficacy of POEM in patients with achalasia were searched in Medline, Ovid Journals, Medline non-indexed citations, and Cochrane Central Register of Controlled Trials and Database of Systematic Reviews. Pooling was conducted by both fixed and random effects models.

The initial search identified 328 reference articles; of these, 34 relevant articles were selected and reviewed. Data was extracted from 20 studies (n=1753) which met the inclusion criteria. In pooled analysis, the clinical success of POEM at 3 months was 94% (95% CI=93–95). The pooled clinical success of POEM at 12 months was 91% (95% CI=90–92). The pooled rate of gastroesophageal reflux disease (GERD) was 21% (95% CI=19–23), esophagitis was reported in 16% (95% CI=15–18), pneumomediastinum in 4% (95% CI=3–6), cervical emphysema in 12% (95% CI=10–13), pneumoperitoneum in 8% (95% CI=7–10), pneumothorax in 5% (95% CI=4 – 6), pleural effusion in 3% (95% CI=2–3), post-operative bleeding in 4.29% (95% CI=1.91 –7.61) and aspiration pneumonia in 3.08% (95% CI=1.13–5.97) of the patients after POEM.

This meta-analysis suggests that POEM is a highly effective and safe endoscopic treatment for patients with achalasia and a reasonable alternative to Heller myotomy.

Pneumatic Balloon Dilation (PBD) as an achalasia treatment is painful procedure because of tearing the lower esophageal sphincter (LES) muscle fibres. Recently, two sedation methods including the moderate sedation and deep sedation are used for increasing the convenience of the patients and gastroenterologist.

To compare the efficacy of moderate and deep sedation in PBD in treatment of Idiopathic Achalasia (IA).

We prospectively assessed 200 IA patients. The first 100 patients (group A) underwent PBD by the moderate sedation using diazepam or midazolam and meperidine injections. The patients in the group B (100 patients) received midazolam, fentanyl and propofol as a deep sedation.  The pulse rate (PR), systolic and diastolic blood pressure (SBP, DBP), respiratory rate (RR), and oxygen saturation were monitored before, during and after PBD and achalasia symptom scores (ASS) were collected before and 1.5 months after treatment.The mean PR during and after procedures in the group B were significantly lower compared to the group A (p 0.001, 0.028). The patients in group B revealed less SBP and DBP after PBD versus group A (p 0.004, 0.002). The mean psi for 30 mm and 35 mm balloon dilators were significantly increased in group B compared to group (p 0.0001, 0.002).

We concluded that the deep sedation of achalasia patients in the PBD process can improved the efficacy of PBD and decreased the complications (transient chest pain) of the procedures. The patients with the deep sedation revealed less tachycardia and blood pressure rising and tolerated more pressure in balloon dilators.

 Achalasia is a rare esophageal motility disorder that can require surgical intervention in some cases. This retrospective cross-sectional study aims to evaluate the clinical symptoms of patients with advanced achalasia who underwent laparoscopic Heller myotomy (LHM) and Dor fundoplication.

 The study included 86 patients (38 men, 48 women) diagnosed with achalasia between 2010 and 2020, of which 20 patients with advanced achalasia underwent LHM and Dor fundoplication. The median follow-up time was 48 months.

 The study found that LHM and Dor fundoplication surgery improved dysphagia in 12 patients, with four patients showing improvement in solid food dysphagia and two patients showing improvement in semi-solid dysphagia. Nocturnal cough and slow emptying sensation also improved in 16 cases. Additionally, barium stasis decreased significantly in 14 patients. However, two patients who underwent esophagectomy had hospital mortality.

 This study highlights the effectiveness of LHM and Dor fundoplication in reducing dysphagia, nocturnal coughing, regurgitation, and other obstructive symptoms in patients with advanced achalasia. However, the study also underscores the potential risks associated with esophagectomy, suggesting that surgical treatment for achalasia should be carefully considered on a case-by-case basis.

Laparoscopic Heller myotomy (LHM) is considered the standard surgical approach in patients newly diagnosed with achalasia worldwide. However, proceeding to fundoplication after LHM remains controversial due to the observed postoperative symptoms, including dysphagia and regurgitation. This study was conducted to compare the postoperatively experienced regurgitation and dysphagia between those undergoing LHM with fundoplication and those with mere LHM.

This four-year controlled clinical trial was performed on adult patients with esophageal type two achalasia, referring to the Shariati Hospital, who gave their written informed consent to enroll. The diagnosis of achalasia was confirmed using manometric assessments. The control group underwent LHM with fundoplication, while the cases received LHM without fundoplication. The validated Achalasia Patients Questionnaire was used for assessing the experienced symptoms pre- and postoperatively. Also, esophagography was used to investigate the alterations of the symptoms pre- and postoperatively.

A total of 48 patients were evaluated. 23 were assigned to the case group, while 25 were considered the controls (male to female ratio: 25 to 23). The mean age of the patients was 36.94 years, and the average disease duration was 6.22 years. Cases and controls were matched demographically. There was no statistically significant difference between the cases and controls regarding postoperative active or passive regurgitation or dysphagia to either solids or fluids. Also, the mean score of total clinical symptoms after the surgery was not significantly different between cases and controls. Lastly, esophagography revealed significant improvement regarding all the symptoms postoperatively (P = 0.001); however, no statistically significant difference existed in this regard between cases and controls.

Our results indicate no significant difference regarding the postoperative achalasia-related symptoms, namely regurgitation and dysphagia, between those patients undergoing LHM with and without fundoplication. However, further studies are required to thoroughly investigate the effects of various fundoplication techniques in relation to all achalasia-related symptoms to confirm these results.

The TBS-derived image processing method, based on the observer's diagnosis, has been developed in the current investigation. Image parametrization is proposed for both novel description and convergent shreds of evidence.

Condensed X-ray images of the esophageal timed barium swallow (TBS) provide substantial implications for elucidating the pathophysiological dimensions of esophageal motility disorders.

Throughthe simultaneous study on TBS and high-resolution manometry (HRM) findings, we performed a retrospective cohort study on 252 patientsfrom March 2018 to October 2019. Interventions, irrelevant information, and insufficient patient data were excluded. Only subjects with adequate data and acceptable test accuracy were considered for participation. We reviewed 117 Dicom (digital imaging and communications in medicine) X-ray images from patients with confirmed diagnoses of achalasia type II, esophagogastric junction outflow obstruction (EGJOO), or non-achalasia.

The results suggested a cut-off level of 47% in DDi (dilated diameter index) as a sign of the dilated body. In achalasia type II patients (n=66 images), the mean DDi was 55.6%. Our method presented a sensitivity of 95% and a specificity of 93% compared to images of the non-achalasia findings. The mean DDi in EGJOO patients was 50.4%, according to the 27 images. Moreover, results from EGJOO patients provided a sensitivity of 85% and specificity of 87%.

TBS is an efficacious method and a prominent component in the process of achalasia diagnosis. Standard parametrization might develop radiological exports proposed by DDi. Our method could assist in obtaining a non-invasive medical diagnosis and help advance diagnostic reports to identify achalasia subtypes somewhat earlier. To the best of our knowledge, this interface is an innovative parametrization for TBS image review.

Esophageal motility disorders (EMDs) are common in patients with dysphagia and are effectively diagnosed with high-resolution manometry (HREM). In this study, we aimed to evaluate the prevalence of different types of primary EMDs in patients referred for HREM and to further investigate the factors associated with EMDs.

In this cross-sectional study, all patients referred to the endoscopy section of Shariati Hospital during 2018-2019 (279 patients) were subjected to HREM and were evaluated according to their diagnosis, and the effect of each factor and each symptom on motility disorders was investigated.

84.5% (235) of the participants were diagnosed with at least one esophageal motility disorder; of them, achalasia was the most common form (52.6%). None of the predictive factors showed a statistically significant correlation with EMDs. However, regarding the symptoms, regurgitation and nocturnal cough were significantly more common in patients with EMD (P=0.001 and 0.009, respectively).

This study demonstrates the high prevalence of EMDs in patients undergoing manometry. None of the factors studied, such as age, sex, diabetes, hypothyroidism, smoking, and alcohol and opium consumption, had a statistically significant correlation with EMDs.

Pneumatic balloon dilation (PBD) is a first line treatment for idiopathic achalasia. Here we report the safety and efficacy of graded gradual PBD on short and long-term follow-up.

We evaluated 1370 idiopathic achalasia patients over a period of 24 years (1994-2018), prospectively. 216 patients did not undergo PBD due to comorbid diseases. Ultimately, 1092 achalasia patients were enrolled. All patients underwent graded gradual PBD, with repeat dilation if symptoms relapsed. Response to treatment was evaluated by Vantrappen scoring system.

Of 1092 achalasia patients, 937 patients were treated by PBD and 155 patients were treated by combined therapy (PBD 1 month after Botulinum toxin injection). In short-term follow-up, 728 of 1092 patients underwent one PBD and 77.3% of them had excellent or good response (responders), 163 patients (58.6%) who underwent two PBDs were responders, and 44 (51.2%) patients who underwent three PBDs were responders. Overall, 2193 balloon dilations were performed on 1092 patients (mean 2 PBDs/patient). Of 786 patients with long-term follow-up, 259 patients had excellent or good response with one PBD. The responders with two, three, and four or more dilations were 149, 67, and 67, respectively. The overall response rate was 69%. No any serious complications were noted by using the graded gradual method.

Our results show that graded gradual PBD is a safe and effective method for treatment of achalasia patients, and achieves sufficient short and long-term symptomatic remission with high cumulative success rate.

With the appearance of enhancing high-resolution manometry (HRM), realizing the difference of achalasia symptoms between classified groups by HRM is an outcome of interest in areas with remote access to this device.

All patients newly diagnosed with achalasia from January 2019 to March 2020 were enrolled in the study. All the patients were diagnosed via HRM after undergoing endoscopy to rule out pseudo-achalasia, and grouped based on the Chicago classification criteria and answered a questionnaire assessing relevant symptoms.

We included 62 patients recently diagnosed with achalasia with a mean age of 53.66 in males (n=30), and 45.4 in females (n=32). Mean time of diagnosis was 24 months. Thirty-seven percent were in type I, 50% in type II, and 13% in type III. Dysphagia and weight loss were higher in type III, while all other relevant symptoms were higher in type II, none of which, however, was statistically significant. Weight loss was reversely associated with duration of symptoms (Spearman correlation= -0.3, P=0.01), and this reverse association was more prominent in females (Spearman correlation= -0.47, P=0.009), type III (Spearman correlation= -0.74, P=0.03), and in the first clinical stages (Spearman correlation= -0.55, P=0.04) in sub analysis.

Type II is the most common type of achalasia in this study. Unlike HRM classification, clinical manifestations alone cannot be used to group patients into different types. However, significant weight loss of the newly diagnosed subjects can become an indicator of on-time diagnosis of the patients.

Three manometric patterns are seen in high-resolution manometry (HRM). Response to treatment has been reported to be different in these subtypes. We aimed to investigate the frequency and response to treatment in subtypes of achalasia.

306 patients between 15 to 60 years old, naïve to treatment with idiopathic achalasia (IA) were evaluated prospectively in a cohort study for 8 years. The patients were treated with pneumatic balloon dilation (PBD), and evaluated before and one month after PBD with Achalasia Symptom Score (ASS) and timed barium esophagogram (TBE) and then every 6 months with ASS. The primary study outcome was defined as a reduction in ASS (equal to or less than 4) and a reduction greater than 80% in the volume of barium in TBE at 1 month after PBD compared with baseline values.

According to HRM, 57 were classified as type I (18.62%), 223 as type II (72.9%), and 26 as type III (8.5%). The mean lower esophageal sphincter (LES) residual pressures before treatment were 34.05 ± 31.55, 32.99 ± 17.90, and 37.47 ± 14.07 mmHg in types I, II, and III, respectively (p = 0.18). The mean ASS values before treatment were 12.23, 11.50, and 11.50, for types I, II, and III, respectively (p = 0.29). The ASS dropped to 2.50 in type I, 2.40 in type II, and 2.12 in type III at 1 month after treatment (p = 0.83). Eventually, at the end of follow-up, 24 patients with type I (83%), 82 patients with type II (67%), and five patients with type III (83%) showed sustained good responses (p = 0.528).

Manometric subtypes of achalasia did not have an important role in clinical success in the long term. Achalasia has no definite cure, but with current treatment modalities, palliation of symptoms is possible in over 90% of patients.

one of the most common reported chief complaints in visits to pediatricians is constipation. Although in a majority of cases with no anatomical defect, dietary manipulation, stool softeners, and oral laxatives are successful, some patients fail to respond.Internal Anal Sphincter (IAS) Achalasia is one of the causes of constipation.It results in failure of IAS relaxation and has similar clinical presentation to Hirschsprung’s disease with absence of rectosphincteric inhibitory reflex on anorectal manometry (ARM) and presence of ganglion cells on rectal biopsy.Although posterior internal anal sphincter myectomy (ISM) is considered the standard treatment for IASA, some cases fail to respond and present with intractable constipation which may be associated with soiling.This research aims to assess the role of botox injection for treatment of patients who presented with intractable constipation and have already beentreated by posterior IAS myectomy.

Internal anal sphincter Botox injection was performed (with a dose of 20 U/Kg) in 14 patients with internal anal sphincter achalasia (IASA); who had presented with intractable constipationafter being treated by posterior internal anal sphincter myectomy. Patients were followed for 2 years after injection.

of all 14 patients with persistent constipation (resistant to oral laxatives), 12 patients (85.7%) had regular bowel function for more than 6 months after botox injection therapy (P < 0.05).Of all 14 patients that had needed rectal enema for defecation, no patient needed rectal enema after botox injection therapy (P < 0.05). Of 5 patients with fecal soiling before botox injection therapy (4 had occasionalsoiling, and 1 had soiling every day without social problems) only a single patient experienced transient fecal soiling for 2 weeks after botox injection therapy (P < 0.05).No patient needed another botox injection in 2 years of follow up.

IAS Botox injection therapy (BIT) was successfully used to manage IASA patients who had presented with intractable constipation after posterior myectomy. This method significantly reduces the need for laxatives and rectal enema; and improves constipation, fecal soiling and bowel movements at the same time.

A few cases with esophageal bezoar have been reported in achalasia. We describe here a rare case of esophageal pharmacobezoar after ingestion of ferrous sulfate capsules in a patient with achalasia.
A 29-year-old woman presented with severe dysphagia since five days earlier. She had history of achalasia since 3 years ago but had refused any treatment option. After about 3 weeks of ferrous sulfate capsules ingestion, she developed severe dysphagia and was referred to a gastroenterologist. Physical examination was unremarkable. A barium swallow revealed dilated esophagus and bird's beak appearance. Esophagogastroduodenoscopy (EGD) showed dilated esophagus and soft black color bezoar in distal part of esophagus. The bezoar was retrieved with basket. In the next endoscopic session, achalasia balloon dilation was successfully applied.
Ferrous sulfate capsules can cause pharmacobezoar in patients with achalasia. Esophageal bezoar should be considered in differential diagnosis of untreated achalasia and acute exacerbation of dysphagia.

Achalasia is the most well known esophageal motility disorder. Laparoscopic Heller myotomy (LHM) is the most effective treatment for achalasia. The aim of this study was to review our results on LHM for achalasia. In this cross-sectional study all patients undergoing LHM between 2015 and 2017 were studied. The myotomy was followed by an anterior or posterior partial fundoplication. All patients were followed up for at least six months. We conducted this prospective study on 36 consecutive patients who underwent LHM over 3 years. The mean age of the patients was 36.64 ± 13.47 years. 30 patients (83.3%) underwent Toupet and 6 patients (16.7%) received Dor fundoplication. 11 patients (30.6%) developed reflux after the procedure. According to the Eckardt Symptom Scoring (ESS), the symptoms improved in 74.2% of the patients and remained unchanged in 25.8% of the patients. Analysis of the ESS, indicated a significant change in regurgitation and retrosternal pain, dysphagia, and weight loss after the surgery (p = 0.001, p = 0.002, p = 0.046, and p = 0.001, respectively). LHM with anterior or posterior partial fundoplication is safe and achieves a good outcome in the treatment of achalasia, especially in patients who have not responded to other methods while no serious complication was reported despite several prior endoscopic interventions.

BACKGROUNDAchalasia, as an incurable disease is defined by the lack of normal esophageal peristalsis and loss of lower esophageal sphincter relaxation due to impaired myenteric neural plexus. The exact cause of myenteric neural cells degeneration in achalasia is still unknown. One hypothesis is that certain neurotropic viruses and autoimmune factors cause the inflammatory response in myenteric network, which consequently destroy neural cells. This study was designed to find the evidence of viral causes of achalasia.
METHODSIn this case-control study, 52 patients with achalasia and 50 controls referred to Shariati Hospital, were evaluated for the genome of neurotropic viruses, HPV, and adenovirus by polymerase chain reaction (PCR) and reverse transcription (RT) PCR techniques.
RESULTSGenome assessment of neurotropic DNA viruses turned out negative in the patients, however, the genome of HSV-1 (Herpes simplex virus) was found in tissues of six controls. No neurotropic RNA viruses were observed in the tissue samples and whole blood of both the patients and controls. Among non-neurotropic viruses, adenovirus genome was positive in tissues of two out of 52 patients and three out of 50 controls. In addition, one out of 52 patients and two out of 50 controls were positive for HPV infection in tissues.
CONCLUSIONWe could not detect any significant relationship between achalasia and HPV, adenovirus, and neurotropic viruses in the cases. Nevertheless, it does not exclude the hypothesis of either an alternate viral species or resolved viral infection as the etiology of achalasia.

Achalasia is a medical condition presenting with dysphagia to both liquids and solids. Although it is usually a primary disease, there are cases in which achalasia occurs secondary to other conditions. Despite its presentation as a secondary condition in a number of diseases, it is very rare for achalasia to be associated with multiple myeloma especially as the presenting symptom when the patient’s disease has not been diagnosed yet. Initial manifestations of multiple myeloma include array of symptoms such as back pain, bone lesions, and anemia, but as mentioned before it is extremely rare for it to initially present with achalasia. Here we describe a man who initially presented with dysphagia and further studies confirmed the diagnosis of achalasia. Later he was diagnosed as having multiple myeloma following other presentations of the disease, thus making the dysphagia caused by achalasia his presenting symptom of multiple myeloma.

Aim: The aim of this study was to evaluate and compare the functional results before and after laparoscopic Heller myotomy for Iranian patients with achalasia.
Achalasia is a severe neuromuscular disorder of the esophagus, characterized by the loss of peristalsis and an inability of the lower esophageal sphincter (LES) to reach optimal relaxation.
In this cross sectional study, patients who underwent Heller myotomy for achalasia via laparoscopy in Taleghani Hospital Tehran, Iran were evaluated. Symptoms including pressure of residual, integrated relaxation sphincter (IRP), pressure of free drinking, pressure of LES, dysphasia score and peristalsis movement was measured and recorded by manometry before and after (2 months) treating with Heller myotomy.
In this study, 23 patients with achalasia (12 females and 11 males) with a mean age of 30±3.5 years (minimum 20, maximum 44 years) who met the inclusion criteria of the study were examined. Results of this study showed that, all the diagnostic criteria that were measured before the treatment was significantly different from after the treatment (P Considering the results of Heller myotomy surgery can be as a treatment of choice for achalasia. Free Drinking pressure can be a suitable criteria after treatment for evaluation and prediction of the reducing the dysphasia score after the surgery.

Although Heller myotomy is one of the most effective treatments for achalasia, it may be associated with early or late symptom relapse in some patients. Therefore, additional treatment is required to achieve better control of symptoms. To evaluate the safety and efficacy of pneumatic balloon dilation (PBD) in patients with symptom relapse after Heller myotomy. Thirty six post-myotomy patients were evaluated from 1993 to 2013. Six patients were excluded from the analysis because of comorbid diseases or epiphrenic diverticula. Thirty patients were treated with PBD. Primary outcome was defined as a decrease in symptom score to 4 or less and a reduction greater than 80% from the baseline in the volume of barium in timed barium esophagogram in 6 weeks. Achalasia symptom score (ASS) was assessed at 1.5, 3, 6, and 12 months after treatment and then every six months in all patients and PBD was repeated in case of symptom relapse (ASS>4). The mean age of the patients was 45.5±13.9 years (range: 21-73). Primary outcome was observed in 25 patients (83%). The mean ASS of the patients dropped from 7.8 before treatment to 1.3±2.0 at 1.5 months after treatment (p=0.0001). The mean volume and height of barium decreased from 43.1±33.4 and 7.1±4.7 to 6.0±17.1 and 1.1±2.2, respectively (p =0.003, p =0.003). The mean duration of follow-up was 11.8±6.3 years. At the end of the study, 21 patients (70%) reported sustained good response. No major complications such as perforation or gross bleeding were seen. PBD is an effective and safe treatment option for achalasia in patients with symptom relapse after Heller myotomy.

The purpose of this study was to determine the efficacy of Heller myotomy for the treatment of achalasia in a referral center in Tehran, and investigate the clinical characteristics, manometric results and treatment responses among three achalasia subtypes in Iranian patients. Esophageal achalasia is an unusual swallowing disorder, characterized by high pressure in the lower esophageal sphincter (LES) on swallowing, failure relaxation of the LES and the absence of peristalsis in esophageal. Patients and In this cross sectional study, clinical symptom and esophageal manometry before and 2 months after treating with Heller myotomy in 20 patients with achalasia who were referred to Taleghani Hospital, Tehran, in 2013 were evaluated. Patients’ demographic, clinical features and response to treatment were analyzed using SPSS software (version 20, Chicago, IL, USA). All the diagnostic criteria measured after the treatment were significantly different (P<0.05) before and after the therapy. The average decline in the length of the esophagus was 1.8 cm and dysphasia score was 7.25 units. Also an average decline in LES Resting Pressure, LES Residual Pressure, PIP, and IRP were 23.2 mmHg, 14.3 mmHg, 3.4 mmHg and 17.8 mmHg, respectively. Results of this study showed that the Heller myotomy is highly effective in relieving dysphasia in patients with achalasia. Also, type II achalasia is the most common subtype of achalasia with a better response to Heller myotomy compared to the other types.

Here, we present a case of a 78-year-old man that underwent gastrointestinal endoscopy because of one- month history of dysphagia to liquids and solid foods with accompanying weight loss. On endoscopy, there was distal esophageal stenosis. Multiple biopsies were obtained. Histologic examination of the samples revealed normal tissue. The stenosis was treated by dilatation and abdomino pelvic computed tomography scanning was performed to search for an underlying malignant lesion that showed a mass adjacent to distal esophagus. We did endosonography- guided fine needle aspiration of the mass. It was a squamous cell carcinoma (SCC). Malignancy is a challenging diagnosis in patients with dysphagia and near normal endoscopy. To our knowledge, there are a few reports of SCC to cause it.