Cases reported "Esophageal Achalasia"

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1/22. Congenital achalasia of the oesophagus in children.

    Nine cases of congenital achalasia of the cardia in children are reported, of which four were below 1 year old and recording the youngest age of 3 months. A hereditary element is present in four children. association with Hirschsprung's disease of the colon in one case and with hiatal hernia in another case, favours a more generalized neuromuscular pathology than a simple spasm of the cardia.
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ranking = 1
keywords = spasm
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2/22. Treatment of achalasia with botulinum A toxin.

    Achalasia is an idiopathic neuromuscular disorder of the esophagus which is associated with absence of esophageal peristalsis and incomplete relaxation of a normal or raised lower esophageal sphincter (LES). Dysphagia is the most commonly associated symptom. Conventional therapeutic approaches are directed to reducing LES pressure and include orally-administered smooth muscle relaxants, forceful sphincter dilation with balloon dilators, and open or laparoscopic-assisted myotomy of the LES. Pharmacologic therapies have a low success rate. Forceful dilation has a perforation complication rate of 2% to 5%, and myotomies may precipitate significant gastroesophageal reflux, a complication minimized when a partial fundal wrap is employed simultaneously. In recent years, botulinum toxin, utilized widely as a striated muscle relaxant in managing blepharospasm, anal sphincter spasm, and muscle spasm complicating CVAs, and in smoothening facial wrinkles, has been extended to the management of achalasia on the basis that it impairs smooth muscle responsiveness to acetylcholine. Eighty units of Botox (botulinum toxin) are injected directly into the endoscopically (endoscopic ultrasound techniques may facilitate localization) located LES region (20 units into each of 4 quadrants). Symptom relief lasting 6 months on average is experienced in more than 65% of treated patients, and the complication rate is negligible. This therapeutic option is reserved for patients too ill to undergo any surgical procedure and is most effective when the lower esophageal region is hypertonic.
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ranking = 3
keywords = spasm
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3/22. Esophageal motor disorders: achalasia and esophageal spasm.

    PURPOSE: To define the esophageal motor disorders of achalasia and esophageal spasms and describe their presentation in the clinical setting. DATA SOURCES: Selected research-based articles, textbooks, and expert opinion. A case study is presented. CONCLUSIONS: The presentation of esophageal motor disorders may not be clear, particularly when the presenting symptom is chest pain. Determining whether the pain is cardiac or digestive in origin is crucial. IMPLICATIONS FOR PRACTICE: Progressive dysphagia for both solids and liquids is the major symptom of achalasia; other symptoms include regurgitation, chest pain, and nocturnal cough. Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids. Dysphagia related to esophageal motility is characterized by a sensation of swallowed food "sticking" in the throat or chest; there is no problem initiating the act of swallowing.
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ranking = 11894.67751369
keywords = esophageal spasm, spasm
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4/22. esophageal achalasia and hypertrophic pyloric stenosis associated with a phytobezoar in an adolescent.

    esophageal achalasia is an uncommon condition in children. The authors report on a 14-year-old girl who showed a very unusual association of cardiospasm and hypertrophic pyloric stenosis with a gastric phytobezoar.
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ranking = 1
keywords = spasm
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5/22. Three cases of cardiospasm treated successfully with psychotherapy. Catamnestic remarks.

    3 cases of cardiospasm successfully treated by psychotherapy are reported and some catamnestic conclusions and remarks are given after a follow-up of the patients for 10, 9 and 8 years, respectively. Unlike the clinical symptomatology, which was almost identical in all 3 cases, the precipitating factors, the deeper meaning and the unconscious symbolism of the symptoms were quite different but specific for each one of the patients. Some more thoughts are also given in the paper with reference to the clinical criteria to be considered when evaluating the final therapeutic results of the technique of the therapy applied.
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ranking = 5
keywords = spasm
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6/22. Restoration of peristalsis in the esophagus of a patient diagnosed of achalasia.

    We report the case of a patient with dysphagia in whom a clinical and manometric follow-up was carried out. We initially observed a motor disorder of the esophageal spasm type. During manometric follow-up a progression to vigorous achalasia was observed. The patient was treated with pneumatic dilation, which obtained a good response to treatment. In manometries performed after treatment we observed a return of peristalsis in the distal esophageal body. Early treatment and short evolution of disease may allow peristalsis to recover in these cases. peristalsis recovery casts new doubts on achalasia etiology.
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ranking = 1982.4462522816
keywords = esophageal spasm, spasm
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7/22. Familial coexistence of achalasia and non-achalasic oesophageal dysmotility: evidence for a common pathogenesis.

    In five of seven siblings of healthy parents, dysphagia developed during adolescence or early adult life. A barium swallow was normal in one patient but showed appearances considered to be consistent with achalasia in all others. Oesophageal manometry was successfully performed in four of the five patients, including the patient with symptoms but normal radiological appearance. One patient had achalasia, two had oesophageal body motor dysfunction associated with a hypertensive, but normally relaxing lower oesophageal sphincter, and one had diffuse oesophageal spasm alone. The occurrence of three different oesophageal dysmotility disorders within members of a single sibship suggests that these conditions are intimately related and probably genetically determined as an autosomal recessive trait.
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ranking = 1982.4462522816
keywords = esophageal spasm, spasm
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8/22. 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.
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ranking = 9912.2312614082
keywords = esophageal spasm, spasm
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9/22. Triple-A syndrome with prominent ophthalmic features and a novel mutation in the AAAS gene: a case report.

    BACKGROUND: Triple-A syndrome (Allgrove syndrome) is an autosomal recessive disorder characterized by adrenal insufficiency, alacrima, achalasia, and - occasionally - autonomic instability. Mutations have been found in the AAAS gene on 12q13. CASE PRESENTATION: We present the case of a 12 year-old boy with classic systemic features of triple-A syndrome and several prominent ophthalmic features, including: accommodative spasm, dry eye, superficial punctate keratopathy, and pupillary hypersensitivity to dilute pilocarpine. MRI showed small lacrimal glands bilaterally. dna sequencing of PCR-amplified fragments from the 16 exons of the AAAS gene revealed compound heterozygosity for a new, out-of-frame 5-bp deletion in exon 15, c1368-1372delGCTCA, and a previously-described nonsense mutation in exon 9, c938C>T, R286X. CONCLUSIONS: In addition to known ophthalmic manifestations, triple-A syndrome can present with accommodative dysregulation and ocular signs of autonomic dysfunction.
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ranking = 1
keywords = spasm
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10/22. Oesophageal achalasia causing respiratory obstruction.

    A patient with complete respiratory obstruction due to massive oesophageal dilatation is described. Immediate relief of symptoms and disappearance of the oesophageal swelling occurred after administration of sublingual glyceryl trinitrate. nitrates cause a reduction in the lower oesophageal sphincter pressure in patients with oesophageal achalasia and in this case it is presumed that spasm of the lower oesophageal sphincter had been a major factor preventing decompression of the dilated oesophagus.
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ranking = 1
keywords = spasm
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