Endoscopic magnetic compression stricturoplasty for congenital esophageal stenosis:A case report

BACKGROUND Congenital esophageal stenosis(CES)is a rare malformation of the digestive tract.Endoscopic dilation and thoracotomy have been the main treatments for CES.However,there is no well-defined management protocol.Magnetic compression stricturoplasty(MCS)has been used in refractory esophageal stricture in children after esophageal atresia.CASE SUMMARY We describe the first case of MCS for CES in one female child patient.The child(aged 3 years and 1 mo)was admitted due to frequent vomiting and choking after eating complementary food since 7 mo old.Esophagography and gastroendoscopy showed that there was stenosis in the lower esophagus,suggesting a diagnosis of CES.The patient did not receive any treatment for esophageal stricture including surgery or endoscopic dilation procedures before MCS.MCS procedure was smoothly conducted without complications.At 24 mo after MCS,durable esophageal patency without dysphagia was achieved.CONCLUSION MCS may serve as an alternative and efficient method for patients with CES.

Endoscopic management for congenital esophageal stenosis: A systematic review

Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscularthickening, tracheobronchial remnants(TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography(EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.

Endoscopic treatment of extreme esophageal stenosis complicated with esophagotracheal fistula: A case report

BACKGROUND At present,there is no unified and effective treatment for extreme corrosive esophageal stenosis(CES)with esophagotracheal fistula(ETF).This case had extreme and severe esophageal stenosis(ES)and ETF after ingesting an enzyme-based chemical detergent,resulting in a serious pulmonary infection and severe malnutrition.Upper gastrointestinal imaging showed that he had an ETF,and endoscopy showed that he had extreme and severe esophageal stricture.This case was complex and difficult to treat.According to the domestic and foreign lite-rature,there is no universal treatment that is low-risk.CASE SUMMARY A patient came to our hospital with extreme ES,an ETF,and severe malnutrition complicated with pulmonary tuberculosis 1 mo after the consumption of an enzy-me-based detergent.The ES was serious,and the endoscope was unable to pass through the esophagus.We treated him by endoscopic incision method(EIM),esophageal stent placement(ESP),and endoscopic balloon dilation(EBD)by using the bronchoscope and gastroscope.This treatment not only closed the ETF,but also expanded the esophagus,with minimal trauma,greatly reducing the pain of the patient.According to the literature,there are no similar reported cases.CONCLUSION We report,for the first time,a patient with extreme CES complicated with ETF,where the endoscope could not be passed through his esophagus but he could be examined by bronchoscopy and treated by EIM,ESP,and EBD.

Simultaneous type III congenital esophageal atresia and patent ductus arteriosus in a low-weight patient: A case report

BACKGROUND We report a low-birth-weight child(1.8 kg)with neonatal type III congenital esophageal atresia(CEA)combined with symptomatic patent ductus arteriosus(PDA).After comprehensive evaluation,esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications,and arterial catheter ligation was performed at the same time.Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.CASE SUMMARY We report a 6-day-old female child with type III CEA and PDA.The patient presented with foam at the mouth after birth,cough and shortness of breath after feeding.At another hospital,she was considered to have neonatal pneumonia,neonatal jaundice and congenital heart disease and transferred to our hospital.After iodine oil radiography of the esophagus and echocardiography we con-firmed diagnosis of CEA and PDA.The diameter of the PDA was 8 mm,with obvious left to right shunting.We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis,and ligation of PDA via left axilla straight incision after 5 d of hospitalization.The operations were successful,and the incision healed after 12 d,and the patient was discharged.We re-examined the patient 1 mo after surgery.She did not vomit when she ate rice flour.Esophageal angiography showed no stricture of the anastomotic stoma.The patient weighed 3.2 kg.CONCLUSION For CEA patients with multiple risk factors,comprehensive,timely and accurate diagnosis and evaluation,and early treatment may improve prognosis.

Superior mesenteric artery syndrome in a patient with esophageal stenosis: A case report

BACKGROUND Superior mesenteric artery syndrome(SMAS)is a rare condition,characterized by duodenal obstruction caused by compression of its third part by the superior mesenteric artery(SMA).Most cases of SMAS are associated with weight loss,and the most frequent clinical manifestations are nausea,vomiting,postprandial fullness,and abdominal pain.Treatment of SMAS is usually conservative,consisting mainly of adequate nutritional support,but in refractory cases surgery may be necessary,with gastrojejunostomy and duodenojejunostomy being the most commonly performed procedures.CASE SUMMARY We describe the case of a man in his forties with a pre-existing diagnosis of esophageal stricture due to sodium hydroxide ingestion,who suffered significant weight loss after replacement of his jejunostomy tube.He was admitted to the hospital due to pain and abdominal distension.A computerized tomography scan showed significant distension of the stomach and duodenum with narrowing of the duodenum at the point at which it is crossed by the superior mesenteric artery,thus establishing the diagnosis of SMAS.Due to the presence of the esophageal stricture,the patient was incapable of emesis;however,passage of a nasogastric tube for decompression was not possible.Considering the risk of gastric perforation due to distention,we opted for surgical treatment in the form of a surgical gastrojejunostomy after which he showed complete resolution of all symptoms and was discharged from the hospital 5 d after the procedure.CONCLUSION Diagnosis of SMAS can be challenging in patients with esophageal stenosis,and risk of gastric perforation may preclude conservative treatment.

Endoscopic management of esophageal stenosis in children:New and traditional treatments

Post-esophageal atresia anastomotic strictures and postcorrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures(ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the stricture's etiology, the availability of different tools and the operator's experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids(either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse,even if such relapses occur following the execution of well-conducted conservative strategies, surgical stricture resection and anastomosis or esophageal substitution are the only remaining options.

Gastroesophageal reflux and congenital gastrointestinal malformations

Although the outcome of newborns with surgical congenital diseases(e.g.,diaphragmatic hernia;esophageal atresia;omphalocele;gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery,infant survivors often require intensive treatment after birth,have prolonged hospitalizations,and,after discharge,may have longterm sequelae including gastro-intestinal comorbidities,above all,gastroesophageal reflux(GER).This condition involves the involuntary retrograde passage of gastric contents into the esophagus,with or without regurgitation or vomiting.It is a well-recognized condition,typical of infants,with an incidence of 85%,which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus,in the first few months after birth.Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood,it has been hypothesized that common(increased intra-abdominal pressure after closure of the abdominal defect) and/or specific(e.g.,motility disturbance of the upper gastrointestinal tract,damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects.Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases.The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations.

Recurrent cervical esophageal stenosis after colon conduit failure:Use of myocutaneous flap

A 53-year-old male developed cervical esophageal stenosis after esophageal bypass surgery using a right colon conduit.The esophageal bypass surgery was performed to treat multiple esophageal strictures resulting from corrosive ingestion three years prior to presentation.Although the patient underwent several endoscopic stricture dilatations after surgery,he continued to suffer from recurrent esophageal stenosis.We planned cervical patch esophagoplasty with a pedicled skin flap of sternocleidomastoid(SCM) muscle.Postoperative recovery was successful,and the patient could eat a solid meal without difficulty and has been well for 18 mo.SCM flap esophagoplasty is an easier and safer method of managing complicated and recurrent cervical esophageal strictures than other operations.

Esophageal stenosis with sloughing esophagitis:A curious manifestation of graft-vs-host disease

We report a case of a 56-year-old woman with a history of allogenic bone marrow transplantation for two years,complaining with dysphagia and weight loss. Upper endoscopy revealed esophageal stenosis and extensive mucosa sloughing. Biopsies confirmed the diagnosis of graft-vs-host disease(GVHD). Balloon dilation,corticosteroids and cyclosporin resulted in marked clinical improvement. Gastrointestinal tract is involved in the majority of patients with chronic GVHD. Esophageal manifestations are rare and include vesiculobullous disease,ulceration,esophageal webs,casts or strictures. Sloughing esophagitis along with severe stenosis requiring endoscopic dilation has never been reported in this context.

Spectrum of magnetic resonance imaging findings in congenital lumbar spinal stenosis

AIM: To investigate whether congenital lumbar spinal stenosis(CLSS) is associated with a specific degenerative changes of the lumbar spine. METHODS: The lumbar spine magnetic resonance imaging studies of 52 subjects with CLSS and 48 control subjects were retrospectively evaluated. In each examination, the five lumbar levels were assessed for the presence or absence of circumferential or shallow annular bulges, annular tears, anterior or posterior disc herniations, epidural lipomatosis, Schmorl's nodes,spondylolisthesis, pars defects, and stress reactions of the posterior vertebral elements. RESULTS: Compared to control individuals, subjects with CLSS exhibited increased incidence of circumferential and shallow annular bulges, annular tears, discherniations and spondylolisthesis(P < 0.05). CONCLUSION: CLSS is associated with increased incidence of degenerative changes in specific osseous and soft-tissue elements of the lumbar spine.

Use of tunnel endoscopy for diagnosis of obscure submucosal esophageal adenocarcinoma:A case report and review of the literature with emphasis on causes of esophageal stenosis

BACKGROUND The tunnel endoscopic technique is the treatment of choice for submucosal tumors. However, the use of tunnel endoscopy to diagnose adenocarcinoma of the esophagus originating from the submucosa has not been well studied.CASE SUMMARY A 74-year-old man who presented with dysphagia for half a year underwent a series of checks, such as gastroendoscopy, X-ray contrast examination of the upper digestive tract, endoscopic ultrasonography, high-resolution esophageal manometry, and positron emission computed tomography. It should be noted that the stenosis of the esophagus was too narrow for endoscopic ultrasoundguided fine needle aspiration. The cause remained undiagnosed. Eventually, the tunnel endoscopic technique was perform for the pathological examination in the submucosa and the final diagnosis was adenocarcinoma of the esophagus. The patient and family members chose expectant treatment due to the patient's age and the high costs of surgical treatment.CONCLUSION Tunnel endoscopy could be used to diagnose tumors. Moreover, we review the literature to provide guidance regarding the causes of esophagostenosis.

Costello Syndrome with Congenital Pulmonary Valve Stenosis and Ventriculomegaly—A Case Report

Costello syndrome is an extremely rare genetic disorder with growth delay after birth and typically results in short stature during childhood. It is one of the RASopathy of Ras/MAPK pathway syndromes. It affects the transforming protein p21, an enzyme that in humans is encoded by the HRAS gene. H-Ras is a small G protein and once bound to Guanosine triphosphate, it will activate a Raf kinase like C-Raf, the next step in the MAPK/ERK pathway (mitogen-actvated protein kinase/extracellular signal-regulated kinase) i.e., MEK (mitogen-actvated ERK kinase), a protein that phosphorylate ERK which can directly and indirectly activate many transcription factors. This pathway is also known as Ras-Raf, MEK-ERK pathway, which is a chain of proteins on the cell that communicate a signal from a receptor on the surface of the cell to the DNA in the nucleus of the cell. Activation of ERK 1/2 is involved in signal transduction pathways associated with cardiac hypertrophy. The developmental syndromes caused by germline mutations in genes that alter the RAS components of the MAP/ERK signal transduction pathway are called “RASopathies”. Cardiovascular abnormalities are important features of Costello syndrome and other RASopathies such as Noonan syndrome. Background of this case report described the congenital valvular pulmonic stenosis and ventriculomegaly associated with Costello syndrome by transthoracic echocardiographic imaging in a 9-year-old male boy.

The Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

Aims:To assess the prognostic value of left ventricular(LV)global longitudinal strain(GLS)and global longitudinal early diastolic strain rate(GLSre)with regard to cardiovascular events,as congenital aortic stenosis(AoS)is associated with significant mortality and morbidity but predictors for clinical outcome are scarce.Strain analysis provides a robust and reproducible method for early detection of LV dysfunction,which might be of prognostic value.Methods:This prospective study,included clinically stable patients with congenital AoS between 2011–2013.LV GLS and GLSre was performed in the apical 4,3 and 2-chamber views using Tomtec software.The endpoint was a composite of death,heart failure,hospitalization,arrhythmia,thrombo-embolic events and re-intervention.Results:In total 138 patients were included(33[26–43]years,86(62%)male),NYHA class I:134(97%).Mean LV GLS was–15.3±3.2%,GLSre 0.66±0.18 s–1.Both correlated with NT-proBNP,LV volumes and ejection fraction(strongest LV GLS with LV EF:r–0.539,p<0.001,strongest LV GLSre with age:r–0.376,p<0.001).During median follow-up of 5.9[5.5–6.2]years,the endpoint occurred in 53(38%)patients:4 patients died,9 developed heart failure,22 arrhythmias,8 thrombo-embolic events and 35 re-interventions.Both LV GLS(standardized HR(sHR 0.62(95%CI 0.47–0.81)and GLSre(sHR 0.62(95%CI 0.47–0.83)were associated with the endpoint.Additional multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume,NT-proBNP and prior re-interventions.Conclusion:Left ventricular GLS and GLSre are reduced in adult patients with congenital AoS.Both markers are associated with adverse cardiac events and have clear clinical relevance.

Rapid right ventricular pacing for balloon valvuloplasty in congenital aortic stenosis:A systematic review

BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during BAV on aortic regurgitation(AR)in pediatric patients.METHODS A systematic review of the MEDLINE,Cochrane Library,and Scopus databases was conducted according to the PRISMA guidelines(end-of-search date:July 8,2020).The National Heart,Lung,and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment.RESULTS Five studies reporting on 72 patients were included.The studies investigated the use of RRVP-assisted BAV in infants(>1 mo)and older children,but not in neonates.Ten(13.9%)patients had a history of some type of aortic valve surgical or catheterization procedure.Before BAV,58(84.0%),7(10.1%),4(5.9%)patients had AR grade 0(none),1(trivial),2(mild),respectively.After BAV,34(49.3%),6(8.7%),26(37.7%),3(4.3%),patients had AR grade 0,1,2,and 3(moderate),respectively.No patient developed severe AR after RRVP.One(1.4%)developed ventricular fibrillation and was defibrillated successfully.No additional arrhythmias or complications occurred during RRVP.CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV,which could potentially decrease AR rates.

Surgical repair of congenital tracheal stenosis associated with complex congenital heart disease in infants

Objective To evaluate results of one stage repair of congenital tracheal stenosis associated with complex congenital heart disease in infants. Methods Two infants with congenital stenosis associated with Tetralogy of Fallot were operated on at the age of 1. 5 and 3 years respectively. In the younger child, the 1. 2 cm long of tracheal stenosis was excised and anastomosed. The other case with 3 cm in length of stenosis at the mid-segment of trachea was excised and repaired with a patch. Results The postoperative progress was uneventful. The patients were followed-up for 6 months to 1 year without any complications. Conclusion One stage repair of congenital tracheal stenosis associated with complex congenital heart disease in infants has got satisfactory results. With cardiopulmonary bypass, the operation can be carried out safely. 6 refs,2 figs.

Surgical treatment of cicatricial esophageal stenosis in infants

Objective To review the results of surgical management of cicatricial esophageal stenosis secondary to chemical burn. Methods Retrospective study was carried out on 30 children ( male 20, female 10 ) with esophagageal cicatricial stenosis. The mean age was 29 months (17 to 60 months) and body weight ranged from 5 to 20 kilograms. One child ingested pesticide whereas the remaining ingested acidic of caustic solutions. There children had complete obliteration of cervical esophagus, 5 had near-complete obliteration and the remaining showed stringy esophageal outline on barium meal study. Five children developed stridor and inspiratory dyspnea. Six children had previous gastrostomy. All patients underwent retrosternal colonic replacement based on left ascending colonic artery. Results Post-operatively, one patient developed anastomotic leakage and one anastomotic stenosis. One patient underwent a post-operative tracheostomy. There was no mortality. All patients were followed-up for 4-20 years with normal

Use of transabdominal (external) ultrasound during balloon dilatation to diagnose tracheobronchial remnant in congenital esophageal stenosis

Preoperativedifferential diagnosis between tracheobronchial remnant(TBR)and other typesof congenital esophageal stenosis(CES)is challenging,even when usingendoscopic ultrasonography(EUS).This report is the first to demonstrate theuse of transabdominal(external)ultrasound during balloon dilatation todiagnose TBR in CES.This simple technique is a promising diagnostic tool forTBR as an alternative to EUS.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula

Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate diagnostic and treatment strategies for this rare condition.Methods:We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.Results:Among 386 patients with EA/TEF,14(3.6%)had type D EA/TEF.Only two patients were diagnosed with proximal TEF preoperatively.Seven patients were diagnosed intraoperatively.Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy.During the neonatal period,seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy.Due to missed diagnosis and other reasons,the other 7 patients underwent two-stage surgery for repair of the proximal TEF,including cervical incision and thoracoscopy.Ten of the 14 patients experienced postoperative complications including anastomotic leakage,pneumothorax,esophageal stricture,and recurrence.Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak(4/7).In contrast,only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation:Type D EA/TEF is a rare condition,and proximal TEFs are easily missed.Bronchoscopy may aim to diagnose and determine the correct surgical approach.A cervical approach may be more suitable for repairing the proximal TEF.

Transparent cap adjusted the stent placed for stenosis after endoscopic injection of esophageal varices:A case report

BACKGROUND The stent embedded in the esophageal mucosa is one of the complications after stenting for esophageal stricture.We present a case of stent adjustment with the aid of a transparent cap after endoscopic injection of an esophageal varices stent.CASE SUMMARY A 61-year-old male patient came to the hospital with discomfort of the chest after the stent implanted for the stenosis because of endoscopic injection of esophageal varices.The gastroscopy was performed,and the stent embedded into the esophageal mucosa.At first,we pulled the recycling line for shrinking the stent,however,the mucosa could not be removed from the stent.Then a forceps was performed to remove the mucosa in the stent,nevertheless,the bleeding form the mucosa was obvious.And then,we used a transparent cap to scrape the mucosa along the stent,and the mucosa were removed successfully without bleeding.CONCLUSION A transparent cap helps gastroscopy to remove the mucosa embedded in the stent after endoscopic injection of the esophageal varices stent.

Comparison of outcomes between complete and incomplete congenital duodenal obstruction

BACKGROUND Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. AIM To quantify and compare the association between CCDO and ICDO with outcome parameters. METHODS We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO (n = 27;atresia type 1-3, annular pancreas) and ICDO (n=23;annular pancreas, web, Ladd′s bands). RESULTS In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%;CCDO vs ICDO, P<0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all P≤0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group (P<0.01). CONCLUSION CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.