Congenital esophageal stenosis presenting as noncardiac,esophageal chest pain

Summary A case of a 31-year-old female with congenital esophageal stenosis presenting with symptoms of chest pain caused by esophageal dysmotility is described. The involved segment in congenital esophageal stenosis has a characteristic thickening of the muscularis propria layer, as seen by EUS examination. In these patients, symptoms of dysphagia can be managed with esophageal dilation and noncardiac esophageal chest pain responds to pharmacotherapy with diltiazem.The opinions and assertions contained herein are the private ones of the authors and are not to be construed as official policy or reflecting the views of the Army or the Department of Defense.     

Adult case of squamous cell carcinoma arising on congenital esophageal stenosis due to fibromuscular hypertrophy

This study relates to an adult case of squamous cell carcinoma arising on congenital esophageal stenosis. The patient was a 65-year-old man who had suffered from dysphagia and vomiting since birth and was diagnosed as having congenital esophageal stenosis. The patient had not received any treatment because his symptoms were mild. The patients suffered from severe dysphagia since he was 20 years old and had received balloon therapies several times; however, the effects were transient. After admission to our hospital, he underwent a transhiatal esophagectomy without thoracotomy. Histopathological examination of the resected specimen revealed a thick muscular mucosae associated with hypertrophic fibromuscular components and poorly to moderately differentiated squamous cell carcinoma in the region of stenosis. This case report is the first of a patient with squamous cell carcinoma arising on congenital esophageal stenosis.     

Novel Biodegradable Stents for Benign Esophageal Strictures Following Endoscopic Submucosal Dissection

The application of metallic stents for benign stenosis is limited due to long-term complications. We report here the results of the implantation of a novel biodegradable poly-l-lactic acid (PLLA) esophageal stent in two patients with benign esophageal stenosis after endoscopic submucosal dissection (ESD). Case 1 was a 64-year-old man who received ESD for an early squamous esophageal cancer in the middle esophagus. The mucosal defect was seven-eighths of the circumference, and the distal margin of the resection scar formed the stenosis. After balloon dilatation, the PLLA esophageal stent was endoscopically placed; for 6 months, he has not experienced any symptoms of re-stenosis. Case 2 consisted of a 62-year-old man who developed an early squamous esophageal cancer in the middle esophagus. The lesion was resected by ESD, and the mucosal defect was seven-eighths of the circumference. The resection scar formed the stenosis, and the PLLA esophageal stent was endoscopically placed. He also has not experienced any symptoms of re-stenosis for 6 months. In conclusion, the PLLA esophageal stent provides a new possibility for the management of benign esophageal strictures after ESD. Due to the biodegradable features of this stent, longer term studies are necessary to investigate the relationship between the expected disappearance of the stent and the patency of the stricture.     

Report on a case of Rothmund-Thomson syndrome associated with esophageal stenosis

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis. While its incidence is unknown, approximately 300 cases have been reported in the literature. The syndrome typically presents with a characteristic facial rash (poikiloderma), its diagnostic hallmark, and heterogeneous clinical features including congenital skeletal abnormalities, sparse hair distribution, juvenile cataracts, and a predisposition to osteosarcoma. Gastrointestinal symptoms, such as pyloric stenosis, anal atresia, annular pancreas, and rectovaginal fistula, have also been reported sporadically. This is a report describing a patient diagnosed with RTS referred to us because of dysphagia caused by esophageal stenosis. Long-term results of endoscopic dilation are also presented.     

Congenital esophageal stenosis in adults

OBJECTIVE: Congenital esophageal stenosis is thought to be a rare disease confined to infancy and childhood with only a few case reports in adults described. METHODS: We report five patients between the ages of 19 and 46 yr who presented with this disorder over a 2-yr period. RESULTS: Patients had been labeled with reflux strictures, webs, or as idiopathic in the past. All patients had chronic solid food dysphagia, some since early childhood. The location of the stricture varied, occurring in the mid or proximal esophagus in four, but throughout the esophagus in one. Radiographic and endoscopic appearance was a smooth concentric stricture or multiple rings, sometimes tracheal in appearance. Endosonography was performed in two patients, both of whom had focal circumferential hypoechoic wall thickening with disruption of the normal layer pattern corresponding to the areas of luminal narrowing. All patients dilated had good symptomatic response, with resolution of symptoms up to 6 months in follow-up. CONCLUSIONS: We suggest that congenital esophageal stenosis does occur in adults and may be underrecognized. Its endosonographic appearance is described.     

磁共振脑灌注成像对先天性血管狭窄脑组织血供评估与临床治疗相关性研究

目的探讨磁共振灌注加权成像(PWI)对先天性血管狭窄脑组织血供评估与临床治疗价值的研究。 方法选取2016年6月至2019年6月就诊牡丹江医学院附属红旗医院,除外其他病变通过DSA或CTA已确诊的先天性血管狭窄患者64例,选择感兴趣区通过后处理软件处理后得到局部脑血容量(rCBV)、局部脑血流量(rCBF)、相对平均通过时间(rMTT),达峰时间(rTTP)等相关参数的数值。比较存在临床症状患者与无临床症状患者灌注上差异及存在临床症状患者不同区域的灌注情况。 结果比较存在临床症状患者与无临床症状患者灌注rCBV、rCBF、rMTT及rTTP差异,rCBF数值减低、而rMTT和rTTP数值呈现出延长(P<0.05),而两组间rCBV值无统计学差异;先天性血管狭窄患者狭窄区供血rCBF及rCBV值的减低、rMTT及rTTP值出现延长,有统计学意义(P<0.05)。 结论磁共振脑灌注功能成像技术可以有效评估先天性脑血管狭窄脑组织血供情况,对临床脑血供情况评估及治疗方案的选择具有重要意义。     

小儿食道化学性碱烧伤狭窄的内镜下气囊扩张治疗分析

目的探讨新疆地区小儿食道化学性烧伤狭窄的特点和内窥镜下气囊扩张治疗的安全性及临床价值。 方法收集2009年1月至2017年10月,新疆维吾尔自治区人民医院20例食道化学碱烧伤狭窄的患儿术前通过消化道造影及胃镜检查诊断的临床资料,本组患者均采用胃镜下气囊扩张食道术。 结果本组患者均胃镜下气囊扩张手术成功,食道穿孔0例,死亡0例,17例（85%）扩张术后再次出现进食困难及呕吐等食道狭窄症状,扩张治疗2~12次,平均3.7次／例,3例（15%）第1次扩张术后治愈。 结论化学腐蚀剂灼伤粘膜及肌层,碱性液可渗透食道全层,肌层受损,出现瘢痕狭窄,严重者出现完全梗阻。本组食道狭窄的患儿均选用胃镜下气囊扩张术,直视下进行操作是安全,无辐射性,治疗有效满意。     

内镜下黏膜切除术后食管狭窄的危险因素分析

目的 分析食管病变内镜下黏膜切除术后食管狭窄的可能的危险因素.方法 2008年至2009年间,共对219例食管早期癌或癌前病变进行内镜下黏膜切除术治疗,其中9例出现食管狭窄,对这9例患者（狭窄组）的病变性质、范围、治疗情况等进行回顾性分析,并与其它术后无狭窄患者（对照组,n=202,有8例术后病理证实有黏膜下层浸润,转外科手术,未纳入比较）进行比较.结果 两组患者在性别、年龄、病变位置、病变长度及术后病理方面无显著差别,但狭窄组创面范围＞3/4食管周径的比例（8/9,88.9%）明显高于对照组（9/202,4.5%,P＜0.01）.结论 对内镜治疗后范围超过食管周径3/4的早期食管癌或癌前病变,内镜下黏膜切除术后发生食管狭窄的风险明显增加.     

Usefulness of biodegradable stents constructed of poly-l-lactic acid monofilaments in patients with benign esophageal stenosis

AIM： To report 13 patients with benign esophagea stenosis treated with the biodegradable stent.
METHODS： We developed a Ultraflex-type stent by knitting poly-/-lactic acid rnonofilaments.
RESULTS： Two cases were esophageal stenosis caused by drinking of caustic liquid, 4 cases were due to surgical resection of esophageal cancers, and 7 cases were patients with esophageal cancer who received the preventive placement of biodegradable stents for postendoscopic mucosal dissection （ESD） stenosis. The preventive placement was performed within 2 to 3 d after ESD. In 10 of the 13 cases, spontaneous migration of the stents occurred between 10 to 21 d after placement. In these cases, the migrated stents were excreted with the feces, and no obstructive complications were experienced. In 3 cases, the stents remained at the proper location on d 21 after placement. No symptoms of re-stenosis were observed within the follow-up period of 7 mo to 2 years. Further treatment with balloon dilatation or replacement of the biodegradable stent was not required.
CONCLUSION： Biodegradable stents were useful for the treatment of benign esophageal stenosis, particularly for the prevention of post-ESD stenosis.     

Usefulness of biodegradable stents constructed of poly-l-lactic acid monofilaments in patients with benign esophageal stenosis

AIM: To report 13 patients with benign esophageal stenosis treated with the biodegradable stent. METHODS: We developed a Ultraflex-type stent by knitting poly-l-lactic acid monofilaments. RESULTS: Two cases were esophageal stenosis caused by drinking of caustic liquid, 4 cases were due to surgical resection of esophageal cancers, and 7 cases were patients with esophageal cancer who received the preventive placement of biodegradable stents for post- endoscopic mucosal dissection (ESD) stenosis. The preventive placement was performed within 2 to 3 d after ESD. In 10 of the 13 cases, spontaneous migration of the stents occurred between 10 to 21 d after placement. In these cases, the migrated stents were excreted with the feces, and no obstructive complications were experienced. In 3 cases, the stents remained at the proper location on d 21 after placement. No symptoms of re-stenosis were observed within the follow-up period of 7 mo to 2 years. Further treatment with balloon dilatation or replacement of the biodegradable stent was not required. CONCLUSION: Biodegradable stents were useful for the treatment of benign esophageal stenosis, particularly for the prevention of post-ESD stenosis.     

Abnormal esophageal motility in children with congenital central hypoventilation syndrome

BACKGROUND & AIMS: Congenital central hypoventilation syndrome, an unexplained disorder of the central control of breathing that may reflect widespread dysfunction of brainstem structures, is regarded as a form of neuro cristopathy. Because swallowing-induced peristalsis is centrally controlled and depends on neural crest-derived esophageal innervation, we looked for esophageal dysmotility in patients with congenital central hypoventilation syndrome. METHODS: Seven patients without dysphagia or any other upper gastrointestinal tract symptoms were studied prospectively (5 girls and 2 boys; median age, 14 years; range, 11-18 years). They were compared with 7 age- and sex-matched controls. Esophageal manometry was performed using a low-compliance infusion system and the station pull-through technique. At least 10 wet swallows were analyzed in each subject. RESULTS: Pressure wave propagation was abnormal in all 7 patients (median percentage of swallows propagated, 18%, and range, 0-66; controls, 90% and 80-100; P < 0.001). Lower esophageal sphincter relaxation was abnormal in 5 patients (patients, 73% and 53-100; controls, 95% and 90-100; P = 0.01). In 2 patients, lower esophageal sphincter pressure was above the 95th percentile of control values. CONCLUSIONS: These abnormalities are strong evidence of lower esophageal dysfunction in congenital central hypoventilation syndrome. We speculate that the underlying mechanism may be dysfunction of the central structures that control swallowing.     

超声内镜检查在儿童上消化道狭窄诊断与治疗中的应用价值

目的探讨超声内镜检查在儿童上消化道狭窄诊断与治疗中的应用价值。 方法回顾性分析2015年5月至2020年5月山东大学附属儿童医院收治的上消化道狭窄患儿48例,包括食管狭窄27例（其中食管闭锁术后吻合口狭窄18例,化学腐蚀性食管狭窄3例,异物所致食管狭窄1例,病因不明食管狭窄5例）,贲门失弛缓症4例,胃输出端狭窄12例,十二指肠狭窄5例。所有患儿均在麻醉状态下行超声内镜检查进行术前评估,根据上消化道狭窄部位、狭窄口大小及超声内镜下改变,选择不同的治疗方案。 结果18例食管闭锁术后吻合口狭窄患儿超声内镜显示食管管壁层次分界不清,环狭窄口周围管壁厚度不均;3例腐蚀性食管损伤患儿超声内镜显示食管损伤程度不一;异物所致食管狭窄1例患儿超声内镜显示异物回声;病因不明食管狭窄5例患儿超声内镜示管壁层次分界不清。贲门失弛缓症4例患儿超声内镜显示贲门固有肌层厚度为0.9~4.0 mm。胃输出端狭窄12例患儿超声内镜显示狭窄部位层次不清、厚薄不均。十二指肠狭窄5例患儿中,4例为先天性发育异常,超声内镜显示狭窄部位局部组织呈均匀回声;1例为消化性溃疡所致十二指肠狭窄,超声内镜显示幽门黏膜隆起处管壁层次模糊。 结论超声内镜检查可以明确儿童上消化道狭窄病变的起源及层次,有助于诊断和术前风险的评估,并为治疗方案的选择提供重要依据。     

Esophagus-enteric anastomosis ulceration caused by alendronate]

BACKGROUND: Alendronate sodium is an aminobisphosphonate indicated for the treatment of osteoporosis in post-menopausal women and has been associated with esophagitis in many reports. Esophageal stenosis, gastrointestinal symptoms as dyspepsia, nausea, vomiting and abdominal pain could be present. OBJECTIVE: Report a case of a patient who underwent total gastrectomy with Y-en-Roux anastomosis for a gastric carcinoid tumor and developed an esophagus-enteric anastomosis ulceration after the use of alendronate. PATIENT AND METHOD: A 63-year-old woman started medical therapy with alendronate in a dose of 10 mg daily. After a period of one month of medical treatment with this drug she began to complain of dysphagic symptoms and abdominal pain. She was submitted to endoscopic examination that showed an esophageal ulceration, an enteric ulceration of the anastomosis and an esophageal stenosis. RESULTS: Medical treatment with alendronate was discontinued and the symptom of abdominal pain disappeared. The intensity of dysphagia has decreased. The ulcerated lesion remitted although esophageal stenosis did not. The patient was subsequently treated with esophagus-enteric anastomosis dilation. She improved in her general state and nowadays she is free of symptoms. CONCLUSION: Alendronate sodium could cause lesions of the inferior esophageal portion or in distal segments of the gastrointestinal tube, in patients with a fast gastrointestinal transit. Special attention must be given to gastrectomized patients that use this drug because of the possibility to develop mucosal lesions in the enteric anastomosed part and its fearful complications as stenosis.     

Esophageal motility in an adult with a congenital H-type tracheoesophageal fistula

Congenital H-type tracheoesophageal fistulas (TEF) are rare. Long-standing respiratory symptoms are the most common presenting complaints. Patients with these fistulas have a congenital esophageal motor abnormality characterized by uncoordinated, low-amplitude peristalsis of the esophagal body; both low and normal lower esophageal sphincter pressures have been described. These findings persist despite fistula repair. A case history of an adult patient with congenital TEF is presented and the literature is reviewed. This patient is unusual in that esophageal symptoms (dysphagia) were more prominent than the usual respiratory symptoms.     

上消化道吻合口狭窄原因及内镜球囊扩张疗效分析

目的　探讨上消化道吻合口狭窄内镜球囊扩张治疗的近、远期疗效及其影响疗效的因素 ,分析引起吻合口狭窄的原因。方法　应用内镜球囊扩张术治疗食管癌、胃癌切除术引起的吻合口狭窄 1 94例 ,术后用内镜、钡餐造影定期随访并观察梗阻症状、生活质量及生存期等。结果　经内镜下球囊扩张治疗后吻合口狭窄的近期症状缓解率为 96 4 % (1 87/ 1 94 )。治疗后吻合口平均直径由0 4 4cm增加到 2 36cm(P <0 0 0 1 )。该方法的主要并发症为黑便 (2 1 % )和穿孔 (1 0 % )。治疗后随访 0 5、1、2和 3年梗阻症状缓解率依次为 88 1 %、92 7%、91 6 %和 90 1 % ;生存率分别为98 4 %、89 8%、2 5 8%和 2 2 3%。绝大多数患者死于肿瘤复发和转移 (92 6 % ) ,极少数死于反复狭窄 (2 1 % )。该方法的疗效与狭窄部位、狭窄程度、球囊扩张参数有关。吻合口狭窄的发生可能与吻合口部位 (如食管上段、食管 胃吻合口狭窄 )、吻合口留置过小、双合钉使用不当、放射治疗等有关。结论　应用内镜下球囊扩张治疗上消化道吻合口狭窄安全可靠 ,近期和远期均有很高的疗效。     

食管金属支架治疗125例食管狭窄和食管气管瘘临床分析

背景：食管狭窄和食管气管瘘是临床常见疾病，近年来食管金属支架逐渐成为其治疗的重要手段，并取得了良好的疗效。目的：探讨食管金属支架治疗食管狭窄和食管气管瘘的临床疗效。方法：选择125例良恶性食管狭窄和食管气管瘘患者，行内镜和x线检查以明确狭窄或瘘口的部位和性质。在x线引导下置入食管金属支架，术后密切随访观察临床疗效和并发症。结果：支架术后2周内，所有患者的吞咽困难均显著改善；食管气管瘘患者的呛咳均显著缓解，仪5例患者进流质时仍有轻微呛咳：86例(68．8％)患者术后出现胸痛，8例(6.4％)发生消化道出血，4例(3.2％)出现支架移位。术后半均随访时间为18个月。吞咽困难的平均缓解时间为7.4个月，27例恶性食管狭窄患者平均于术后6.4个月因肿瘤组织增生而导致再狭窄，8例良性食管狭窄患者平均于术后12．8个月因肉芽组织增生而导致再狭窄。38例患者有胃(或肠)食管反流表现，大多为吻合口支架或食管下段支架。7例患者出现支架移位。恶性食管狭窄患者支架术后平均存活时间为11．8个月。结论：食管金属支架能有效治疗良恶性食管狭窄和食管气管瘘，提高患者的生活质量。但支架术后再狭窄、食管功能紊乱和支架移位等并发症目前仍无法避免，且可能对支架的临床应用产生一定影响。     

Symptom priority ranking in the care of gastroesophageal reflux: a review of 1,850 cases

BACKGROUND: Clinical history remains an important part of the medical evaluation of patients with gastroesophageal reflux disease (GERD). Heartburn, regurgitation, and dysphagia are considered typical symptoms of GERD. Priority rankings of these symptoms can be determined with a standardized questionnaire. OBJECTIVE: To determine whether symptom priority ranking and symptom severity grading can provide useful information in the evaluation of patients with GERD. METHODS: From 1,850 patients that were analyzed retrospectively, patients with dysphagia unrelated to GERD were excluded. A standardized questionnaire was applied before each patient underwent any esophageal diagnostic study. Priority of symptoms was determined to be primary, secondary, tertiary, or none based on the patient response to the questionnaire. Presence of a stricture was determined either by endoscopy, esophagraphy, or both studies. Stationary esophageal manometry and 24-hour pH monitoring were performed on all patients. Through bivariate and multivariate analysis, the relationships among typical GERD symptoms, esophageal reflux-related stenosis, lower esophageal sphincter pressure, and composite score were established. RESULTS: High priority ranking of the symptom dysphagia is predictive of the presence of an esophageal stricture, but has a negative association with abnormal manometric and pH studies. In contrast, high priority ranking of the symptom heartburn and regurgitation are positively associated with abnormal manometric and pH results. CONCLUSIONS: Priority ranking can be a valuable adjunct to objective testing in the evaluation of GERD. In certain clinical situations it can obviate the need for 24-hour pH monitoring.     

Predictive factors for esophageal stenosis after endoscopic submucosal dissection for superficial esophageal cancer

Endoscopic submucosal dissection (ESD) has been utilized as an alternative treatment to endoscopic mucosal resection for superficial esophageal cancer. We aimed to evaluate the complications associated with esophageal ESD and elucidate predictive factors for post‐ESD stenosis. The study enrolled a total of 42 lesions of superficial esophageal cancer in 33 consecutive patients who underwent ESD in our department. We retrospectively reviewed ESD‐associated complications and comparatively analyzed regional and technical factors between cases with and without post‐ESD stenosis. The regional factors included location, endoscopic appearance, longitudinal and circumferential tumor sizes, depth of invasion, and lymphatic and vessel invasion. The technical factors included longitudinal and circumferential sizes of mucosal defects, muscle disclosure and cleavage, perforation, and en bloc resection. Esophageal stenosis was defined when a standard endoscope (9.8 mm in diameter) failed to pass through the stenosis. The results showed no cases of delayed bleeding, three cases of insidious perforation (7.1%), two cases of endoscopically confirmed perforation followed by mediastinitis (4.8%), and seven cases of esophageal stenosis (16.7%). Monovalent analysis indicated that the longitudinal and circumferential sizes of the tumor and mucosal defect were significant predictive factors for post‐ESD stenosis (P < 0.005). Receiver operating characteristic analysis showed the highest sensitivity and specificity for a circumferential mucosal defect size of more than 71% (100 and 97.1%, respectively), followed by a circumferential tumor size of more than 59% (85.7 and 97.1%, respectively). It is of note that the success rate of en bloc resection was 95.2%, and balloon dilatation was effective for clinical symptoms in all seven patients with post‐ESD stenosis. In conclusion, the most frequent complication with ESD was esophageal stenosis, for which the sizes of the tumor and mucosal defect were significant predictive factors. Although ESD enables large en bloc resection of esophageal cancer, practically, in cases with a lesion more than half of the circumference, great care must be taken because of the high risk of post‐ESD stenosis.     

Percutaneous treatment of multiple heart defects

We describe our experience in 6 cases with multiple congenital heart defects treated by percutaneous intervention. Their age ranged from 2.3 to 10 years (mean 6.1), with follow-up from 1 to 84 months (mean 28.8). Two cases had coarctation of the aorta (AC) and persistent ductus arteriosus (PDA). Two patients had pulmonary valve stenosis (PVS) and PDA, one case with aortic stenosis (AE) and PDA and one case with AC, mitral stenosis and subaortic stenosis (Shone's Syndrome). Ductus arteriosus was occluded in all patients with Gianturco coils or Rashkind occluder. The valvular gradient post balloon decreased in cases with AC from 46 to 9 mmHg, with PVS from 110 to 10 mmHg and with AE from 40 to 14 mmHg. In a 8 year old boy with Shone's syndrome, we performed angioplasty of aortic coarctation, mitral valvuloplasty with Inoue catheter. He was referred to surgery for subaortic repair stenosis. All are asymptomatic at follow-up. In conclusion; percutaneous intervention is possible in patients with multiple congenital heart defects.     

Esophageal stenosis in eosinophilic esophagitis

Eosinophilic esophagitis is a rare, recently discovered disease, characterized by esophageal symptoms, such as dysphagia and food impaction, associated with dense eosinophilia on endoscopic biopsy of the esophagus. Other entities such as gastroesophageal reflux disease are absent and there is a lack of response to proton pump inhibitor therapy. This disease mainly affects the pediatric population but is becoming more prevalent in adults. There are several theories on the etiopathogenesis of this entity, which may involve allergies and atopy. In advanced disease, complications such as esophageal stenosis can appear. Treatment is based on dietary elimination, corticosteroids and endoscopic dilatation. We report a case of eosinophilic esophagitis with esophageal stenosis.