Barium Studies Research Articles

The diagnosis of gastro-esophageal reflux disease cannot be made with barium esophagograms.

For over 50years, barium studies have been used to diagnose gastro-esophageal reflux disease (GERD), but the value of this test is controversial. Our study aimed to determine if barium esophagograms can be used to diagnose GERD. Barium esophagograms and pH-impedance measurement were performed in 20 subjects with reflux symptoms. pH-impedance measurements were used as gold standard for the diagnosis of GERD. Gastro-esophageal reflux measured with the barium study was defined as a positive outcome. 50% of patients presented gastro-esophageal reflux on the barium esophagogram. No significant differences were observed in acid exposure time between subjects with (median: 7.4%; interquartile range, IQR: 8.4%) or without reflux at barium esophagography (median: 5.95%; IQR: 13.05%; p>0.05). Nor did we find differences in median proximal extent of reflux measured with impedance monitoring between patients with a positive (median: 6.7%; IQR: 1.95%) and negative barium study (median: 7.1%; IQR: 0.68%; p>0.05). Patients with reflux on barium esophagogram did not have a positive symptom association probability more often than those who did not have reflux at barium esophagography. Lastly, there were no differences in numbers of acid, weakly acidic or total reflux episodes between those with positive or negative barium esophagogram (p>0.05). No correlations were found between the maximum proximal extent of gastro-esophageal reflux during esophagography and pH-impedance parameters. Presence or absence of gastro-esophageal reflux during barium esophagography does not correlate with incidence or extent of reflux observed during 24-h pH-impedance monitoring and is not of value for the diagnosis of GERD.

Weak or Frequent Failed Peristalsis: No Difference in Outcome After Paraesophageal Hernia Repair

Introduction: Prior to the high resolution esophageal manometry Chicago classification criteria (2012), patients with “weak peristalsis” and “frequent failed peristalsis” may have been categorized as having long transition zones, ineffective esophageal motility, hypotensive peristalsis, or even as normal. Even though weak and frequent failed peristalsis are thought to be minor peristaltic abnormalities, there is little documentation of the potential effect of these findings during the performance of a traditional fundoplication. We hypothesize that patients who are categorized as having weak or frequent failed peristalsis and undergo standard paraesophageal hernia repair will experience no more dyspagia or peristaltic abnormalities on barium swallow than those patients with normal peristalsis on manometry. Methods: We performed a retrospective chart review of all patients undergoing paraesophageal hernia repair between January 2009 and December 2011. Patients were included if they had preoperative manometry and pre- and post-operative (at least 6 weeks after surgery) clinical visits and barium swallow studies. Manometry studies were re-analyzed using Chicago classification criteria and were classified as normal, weak peristalsis, frequent failed peristalsis, or other. Patients with decreased distal latency but with propagating peristalsis and esophageal shortening (n=4) were not classified as esophageal spasm, as this finding is likely due to the short esophageal length. The data were re-analyzed excluding these patients and the results were similar. Dysphagia was classified as present or absent. Barium swallow studies were classified as normal or abnormal. Barium studies with esophageal motility reported as near normal, minimally abnormal, or mildly abnormal (per the radiologist at the time of the study) were counted as normal. Results: All patients underwent primary open or laparoscopic paraesophageal hernia repair without Collis procedure or hiatal mesh mesh reinforcement. Preoperative manometry showed normal peristalsis (n=31), weak peristalsis (n=10), frequent failed peristalsis (n=9), or other. Postoperative dysphagia was noted in 2/31 (6%) of the normal group, 1/10 (10%) of the weak group, and 1/9 (11%) of the frequent failed group (p=0.87 by Chi Square analysis, p=0.61 by logistic regression). Abnormal esophageal motility seen on barium swallow occurred in 10/31 (32%) of the normal group, 1/10 (10%) of the weak group, and 3/9 (33%) of the frequent failed group (p=0.37 by Chi Square analysis, p=0.73 by logistic regression). Conclusion: Patients with weak or frequent failed peristalsis on high resolution manometry do not have any different outcome after primary paraesophageal hernia repair than those with normal manometry.

A Rare Case of Gastrointestinal Bleeding Due to Primary Intestinal Melanoma

Introduction: We present a rare case of primary small intestine melanoma. Case Report: A 69-year-old male with a history of colon polyps presents with dark stool and fatigue. His hemoglobin dropped from 16 to 11. EGD showed possible Barrett’s esophagus. Biopsy was negative for metaplasia. He had a nodule of the second portion of the duodenum. Biopsy showed normal mucosa. Colonoscopy showed hemorrhoids, diverticulosis, and normal terminal ileum. A 3-mm tubular adenoma was removed from the colon. Small bowel (SB) capsule showed a moderate-sized semi-circumferential mass in the jejunum with areas of ulceration and bleeding. A CT abdomen showed small bowel mass in the distal jejunum. Adjacent enlarged lymph nodes (LN) are suspicious for metastatic disease. The patient underwent exploratory laparotomy and partial SB resection. Pathology reveals malignant melanoma. LN was negative for metastasis. Skin evaluation by dermatology did not find any lesions that were concerning for dysplastic nevi. Ophthalmology evaluation was normal. MRI of the brain revealed no metastatic process. PET-CT revealed intense hypermetabolism in the small bowel at the area of the surgical site without any evidence of metastatic disease. He followed up with oncology and his repeat PET scan and capsule endoscopy remained negative for recurrence of cancer. Discussion: Intestinal melanomas can be primary tumors or metastases of cutaneous, ocular, or anal melanomas. Primary intestinal melanoma is extremely rare. Most cases of SB melanoma are metastases from cutaneous melanoma. Metastases to the gastrointestinal tract are found at autopsy in 60% of patients who die with malignant melanoma. The clinical picture of SB melanoma is similar to the clinical presentation of other tumors involving the SB. Thus, patients with a history of cutaneous melanoma who have intermittent abdominal pain or anemia should be investigated to rule out intestinal metastases. Diagnosis can be made by ultrasound, barium studies, endoscopy, CT, or PET. A combination of imaging techniques is recommended to improve the sensitivity and specificity of radiological imaging. No standard therapy exists for the treatment of intestinal melanoma, although surgical removal is the treatment of choice in all patients with resectable melanoma. Accurate preoperative diagnosis and assessment of the extent of intestinal metastases is essential when selecting patients for surgery. Systemic chemotherapy regimens show no benefit to overall survival. The prognosis is poor, with an overall median survival of 6-9 months and a 5-year survival rate of less than 10%. Conclusion: Primary intestinal melanoma is very rare condition with poor prognosis. Early detection and surgical resection are vital to disease survival.

A Case of Barium Inspissation Resulting in Acute Constipation and Colectomy

Introduction: Barium sulfate is an insoluble salt commonly used in radiologic studies. It is generally considered a low-risk contrast medium with the most common complications including abdominal cramping, nausea, vomiting, and constipation. However, rarely, severe complications can occur including viscus perforation, barium inspissation, embolization, aspiration, and poisoning secondary to barium intravasation. These complications are important to recognize and often require drastic endoscopic and surgical measures. Case Report: An 86-year-old female with history of refractory peptic ulcer disease requiring previous Bilroth II surgery later complicated by gastric outlet stenosis resulting in placements of gastrostomy and jejunostomy tubes was admitted for intractable bilious vomiting and septic pneumonia. Patient reported diffuse abdominal pain and was uncertain of the timing of her last bowel movement. Symptoms started after a barium swallow study 1 week prior to presentation. Physical exam was notable for tachycardia, hypotension, and distended tender abdomen with minimal bowel sounds throughout. Her labs revealed a hemoglobin 8.2 g/dL, WBC 12.2 x 1000, creatinine 1.6 mg/dL, and lactate 2.6 mmol/L. CT of her abdomen showed diffuse colonic and distal small bowel dilatation with excessive amounts of intraluminal stool and inspissated barium solution. Patient required support with vasopressors and was treated with antibiotics. Polyethylene glycol, sodium polystyrene sulfonate, lactulose enemas, tap water enemas, mineral oil, manual disimpaction, and sigmoidoscopy were unsuccessful at disimpacting the colon. The patient subsequently underwent total colectomy 6 days into hospital stay with end-ileostomy and was discharged in stable condition. Discussion: Barium impaction is a rare complication of barium studies with 1 review reporting 31 reported cases between 1950 and 2006. However, it is associated with significant morbidity as nearly half of the reported cases required surgical intervention. Risk factors for barium inspissation are any changes to intestinal anatomy or intraluminal diameter and any medical conditions or drugs affecting colon motility. Prevention with proper hydration and administration of laxatives following barium studies is recommended in those at increased risk.

Successful Combined Intralesional Corticosteroid Therapy and Wire-Guided Dilation of Severe Esophageal Graft-Versus-Host Disease

Introduction: This 63-year-old woman first presented in 2009 with a 4 year history of severe dysphagia following allogenic hematopoetic transplantation for aplastic anemia/leukemia in 2004, which was complicated by severe acute and chronic GVH. This was managed with tracrolimus. Barium studies showed severe proximal 2 cm and 9cm mid-distal esophageal strictures which progressed over time despite oral GVH therapy. She had been on a liquid only diet for 2 years, following an unsuccessful attempt at dilation in 2007 at NIH. Since then symptoms progressed and esophagectomy or gastrostomy were recommended. Initial EGD showed typical esophageal GVH changes, with areas of mucosal slough, friability and severe structuring precluding passage past the proximal esophagus. Biopsies confirmed GVH. Fluoroscopic wire guided Savary dilation to 7mm resulted in severe proximal slough and deep ulceration, with post procedural odynophagia, but no perforation. This was treated with oral liquid sucralfate. Patient remained severely symptomatic with difficulty maintaining hydration and taking her oral medications for GVH which had to be given in elixir form. 9 weeks later at repeat EGD, the stricture had recurred. This time we injected triamcinolone 40mg/2cc in 0.25-0.5cc/injection via sclerotherapy needle in the reachable strictured areas, followed by fluoroscopic wire-guided dilation to 8mm. Over the subsequent 3 years a total of 15 endoscopic sessions, with 4 to 8 0.25-0.5ml endoscopic injections of 40 mg of triamcinolone/session, followed by progressive fluoroscopic wire-guided Savary dilation. There was gradual improvement in the endoscopic appearance. After the 5th session we could dilate the entire esophagus the the point where the endoscope could be advanced into the stomach. By 2012 the esophagus was dilated to a 14 mm diameter, the mucosal GVH changes were much improved/resolved. This allowed the patient to eat normally, and take all her po medications. Only complication was recurrent candida esophagitis, which was treated with fluconazole. Since then patient has been dilated every 6-9 months and the stricture has remained stable. Chronic GI tract GVH is less frequent with advances in hematopoetic transplantation but remains disabling. Submucosal corticosteroid injections combined with oral immunotherapy have been used to treat oral GVH, but we could not find a case of similar case of combined endoscopic therapy for esophageal GVH. This approach had minimal morbidity and resulted in a marked symptomatic and endoscopic response.

High resolution manometry sub-classification of Achalasia: does it really matter?

Three variants of Achalasia have been described using high-resolution esophageal manometry (HRM). While manometrically distinct, their clinical significance has yet to be established. Our objective was to compare the outcome after myotomy in patients with these Achalasia subtypes. A retrospective chart review was performed to identify patients with Achalasia who had HRM and who underwent Heller myotomy or Per oral endoscopic myotomy (POEM). Symptoms and esophageal clearance by timed barium study were compared before and after treatment. We identified 49 patients, 21 males and 28 females, with a median age of 52 years. The primary symptom in all patients was dysphagia, with a median duration of 4 years (range 4 months-50 years). By HRM, ten patients (20 %) were classified as Type I, 30 (61 %) as Type II, and 9 (18 %) as Type III. At a median follow-up of 16 months after myotomy (range 1-63 months), the median Eckardt score was zero and was similar across subtypes. Relief of dysphagia was also similar across subtypes (80 % of Type I, 93 % of Type II and 89 % of Type III). On pre-treatment timed barium study, no patient had complete emptying at 1 or 5 min. After myotomy, complete emptying occurred within 1 min in 50 % (20/40) and within 5 min in 60 % (24/40) and was similar across groups. Myotomy for Achalasia results in excellent symptomatic outcome and improvement in esophageal clearance. There was no difference among the described HRM Achalasia variants. This calls into question the clinical utility of Achalasia sub-classification and affirms the benefit of myotomy for this disease.

Acquired constricting and restricting lesions of the descending duodenum.

The descending duodenum is a structure with distinct pathologic processes and anatomic relationships that requires a systematic approach to the differential diagnosis. Because of its tubular shape and fixed position in the retroperitoneum, both intrinsic duodenal and juxtaduodenal diseases are capable of producing luminal narrowing and obstruction. Duodenal lesions may be located in the mucosa or submucosa. Extraduodenal lesions may originate in adjacent structures--such as the pancreas, liver, gallbladder, colon, and lymph nodes--or from other retroperitoneal structures. Causes of duodenal obstruction include intraluminal masses, such as bezoars; duodenal inflammation, such as as peptic ulcers and Crohn disease; hematomas; and benign or malignant mucosal and intramural tumors. Pancreatic inflammation; tumors; and extrinsic compression caused by gallbladder processes, hepatic masses, retroperitoneal fluid collections, and tumors, including lymphoma, may produce duodenal obstruction. Abdominal radiography, barium studies, multidetector computed tomography, magnetic resonance imaging, and positron emission tomography may be used to depict and characterize duodenal strictures. Integration of imaging, clinical, laboratory, and endoscopic findings plays a major role in establishing a diagnosis of obstructive duodenal strictures.

Imaging and clinicopathologic features of esophageal gastrointestinal stromal tumors.

The purpose of this article is to describe the imaging and clinicopathologic characteristics of esophageal gastrointestinal stromal tumors (GISTs) and to emphasize the features that differentiate esophageal GISTs from esophageal leiomyomas. A pathology database search identified all surgically resected or biopsied esophageal GISTs, esophageal leiomyomas, and esophageal leiomyosarcomas from 1994 to 2012. Esophageal GISTs were included only if imaging studies (including CT, fluoroscopic, or (18)F-FDG PET/CT scans) and clinical data were available. Nineteen esophageal mesenchymal tumors were identified, including eight esophageal GISTs (42%), 10 esophageal leiomyomas (53%), and one esophageal leiomyosarcoma (5%). Four patients (50%) with esophageal GIST had symptoms, including dysphagia in three (38%), cough in one (13%), and chest pain in one (13%). One esophageal GIST appeared on barium study as a smooth submucosal mass. All esophageal GISTs appeared on CT as well-marginated predominantly distal lesions, isoattenuating to muscle, that moderately enhanced after IV contrast agent administration. Compared with esophageal leiomyomas, esophageal GISTs tended to be more distal, larger, and more heterogeneous and showed greater IV enhancement on CT. All esophageal GISTs showed marked avidity (mean maximum standardized uptake value, 16) on PET scans. All esophageal GISTs were positive for c-KIT (a cell-surface transmembrane tyrosine kinase also known as CD117) and CD34. On histopathology, six esophageal GISTs (75%) were of the spindle pattern and two (25%) were of a mixed spindle and epithelioid pattern. Five esophageal GISTs had exon 11 mutations (with imatinib sensitivity). Clinical outcome correlated with treatment strategy (resection plus adjuvant therapy or resection alone) rather than risk stratification. Esophageal GISTs are unusual but clinically important mesenchymal neoplasms. Although esophageal GISTs and esophageal leiomyomas had overlapping imaging features, esophageal GISTs tended to be more distal, larger, more heterogeneous, and more enhancing on CT and were markedly FDG avid on PET. Given their malignant potential, esophageal GISTs should be included in the differential diagnosis of intramural esophageal neoplasms.

PWE-103 Terminal Ileitis At Endoscopy In Clinical Practice: Is It Always Due To Crohn’s Disease?

IntroductionTerminal ileitis (TI) when identified at endoscopy is generally assumed to be secondary to Crohn’s disease (CD). However, TI may be due to other causes including infection, malignancy, radiation, vasculitis...

G446(P) An Unusual cause of Chronic Cough in a 15 year old girl

AimsCoughing is one of the most common paediatric presentations both in primary and secondary care. We present an unusual cause of chronic cough in a teenager.MethodA 15 year old girl...

English

A 70 year old woman developed gastropleural fistula following gastric perforation after consumption of formic acid. The diagnosis required multiple imaging studies including chest radiographs barium studies of upper gastrointestinal tract. The patient was not willing for surgical excision of the fistulous track and is currently under follow up for significant developments.

How Useful is Ultrasound in the Imaging Workup of Malrotation?

Background: The position of third portion of duodenum (D3) is always intramesenteric in malrotation. Displaying normal retromesenteric- retroperitoneal position of D3 by ultrasound (US) can help to rule out malrotation. Objectives: The aim of this study was to evaluate the feasibility of US in demonstration of retroperitoneal D3. Patients and Methods: Abdominal US study was performed for various indications in sixty newborns and infants [Mean age: 33 days (range: 4-100 days); 56.7% male] by an expert paediatric radiologist. D3 position and its adjacent structures were evaluated in axial and longitudinal planes by linear and curve transducers. Results: Normal retromesenteric-retroperitoneal D3 between superior mesenteric artery and aorta was seen by US in all patients, including those with severe bowel gas. Mean time for displaying D3 was 47.8 s (10-180 s). Ultrasound was also capable to demonstrate D3 structure, diameter, content, its adjacent structures, relative position of superior mesenteric artery and vein. Conclusions: Ultrasound is a simple, fast and highly accurate modality for confirming retroperitoneal position of D3. Ultrasound can be used as the screening method for malrotation and obviate unnecessary barium studies.

Response to “Gastric Stenosis After Laparoscopic Sleeve Gastrectomy in Morbidly Obese Patients”

1. All of the patients who had been subjected to a laparoscopic sleeve gastrectomy participated in a protocol that included barium X-ray studies performed on the third postoperative day. In addition to the latter, an endoscopic control was done 1 month after surgery, once the patient had been discharged. In patients who suffered from symptoms of stenosis, this upper digestive endoscopy was performed for diagnostic purposes while the symptoms were occurring. If stenosis was suspected, the patient was not discharged or was readmitted to the hospital. An upper digestive endoscopy and dilations were performed in the operating room under general anesthesia using simultaneous fluoroscopy in order to reduce risk to the patient. The procedure for the dilatation was performed according to the previously published technique [2]. 2. We agree with these comments. However, in our experience, and having performed a great number of dilatations for esophageal strictures (we even have classifications for this complication) [2, 3], we have found that direct endoscopic vision of balloon dilatation provides safer treatment. Furthermore, a radiological control is performed immediately after endoscopic dilatation, in order to exclude the possibility of eventual perforation. 3. Based on our study, we firmly believe that an endoscopic evaluation of all bariatric patients is essential before surgery, as included in the preoperative work up of SAGES [4]. We always perform histological studies of the stomach in order to detect beforehand Helicobacter pylori, intestinal metaplasia, or chronic atrophic gastritis which remains in the excluded stomach after the gastric bypass procedure. We have also diagnosed carcinoid tumors or early gastric cancer before surgery. We firmly believe that an upper endoscopy is mandatory before surgery. 4. We completely disagree with these comments, and we believe that they are wrong:

Heterotaxy syndrome: a case report

Heterotaxy syndrome is defined as an abnormal arrangement of some organs and vessels in association with dysmorphism. The authors describe the case of a patient with heterotaxy syndrome with poliesplenia incidentally diagnosed during imaging evaluation (computed tomography and small bowel barium study) of unrelated pathological condition.

Evaluation of bowel wall thickening by computed tomography to differentiate benign from malignant lesions.

Computed tomography(CT) is an excellent non-invasive modality to evaluate bowel wall thickening.The aim of our study was to evaluate CT appearance of bowel wall thickening due to various benign and malignant conditions taking into consideration pattern of attenuation, bowel wall thickness, extent of lesion, symmetry of lesion and other associated CT findings. The prospective study was carried out on 50 patients who underwent computed tomographic evaluation of abdomen for suspicion of bowel pathology based on ultrasonography, barium studies and/or clinical grounds. The studies were conducted on Siemens ART and GE High speed CT scanners. The examination was performed as is done routinely for an abdominal scan with imaging done from diaphragm to pubic symphysis in supine position with the right lateral decubitus scans in selected cases for better characterization of gastric antral and duodenal lesions. Oral, rectal and intravenous (IV) contrast agents were administered. The diagnosis was confirmed by cytology or histopathology of any biopsy or surgical specimen. However, in cases where surgery was not done, diagnosis was confirmed by clinical response to medical treatment. Based on the various CT characteristics of abnormal bowel wall thickening, sensitivity and specificity of classifying a lesion as benign or malignant were calculated. Majority of the malignant bowel lesions were showing the following characteristics i.e. heterogeneous pattern of enhancement, marked bowel wall thickening, asymmetry of the lesion and focal/segmental bowel involvement. Overall, CT showed a sensitivity of 97% and specificity of 93% in differentiating between benign and malignant etiology of abnormal bowel wall thickening. Due to its high sensitivity and specificity, CT is an ideal imaging modality for differentiating between benign and malignant etiology of abnormal bowel wall thickening. Radiologists should be aware of the usefulness of specific CT criteria of bowel wall thickening to better differentiate benign lesions from malignant or potentially malignant lesions that warrant further diagnostic evaluation.

Ultrasound as a feasible method for the assessment of malrotation.

SummaryBackgroundIn malrotation the position of third portion of duodenum (D3) is always intramesenteric. Demonstration of normal retromesenteric-retroperitoneal position of D3 on ultrasound (US) can rule out malrotation. The aim of this study was to evaluate the feasibility of US in demonstrating the retroperitoneal D3.Material/MethodsAbdominal US study was done for various indications in 60 newborns and infants (mean age: 33 days [range: 4–100 days]; 56.7% male) by an expert pediatric radiologist. The position of D3 and its adjacent structures was evaluated in axial and longitudinal planes by linear and curved transducers.ResultsA normal retromesenteric-retroperitoneal D3 located between the superior mesenteric artery and the aorta was seen on US in all patients, including those with extensive gas in the bowel. The mean time of D3 observation was 47.8 s (10–180 s). Ultrasound was also capable of demonstrating D3 structure, diameter, content, adjacent structures, relative position of the superior mesenteric artery and vein.ConclusionsUltrasound is a simple, fast and highly accurate tool to confirm the retroperitoneal position of D3. Ultrasound can be used as a screening method for malrotation eliminating the need for unnecessary barium studies.

Nodular lymphoid hyperplasia in the gastrointestinal tract in adult patients: A review.

Nodular lymphoid hyperplasia of the gastrointestinal tract is characterized by the presence of multiple small nodules, normally between between 2 and 10 mm in diameter, distributed along the small intestine (more often), stomach, large intestine, or rectum. The pathogenesis is largely unknown. It can occur in all age groups, but primarily in children and can affect adults with or without immunodeficiency. Some patients have an associated disease, namely, common variable immunodeficiency, selective IgA deficiency, Giardia infection, or, more rarely, human immunodeficiency virus infection, celiac disease, or Helicobacter pylori infection. Nodular lymphoid hyperplasia generally presents as an asymptomatic disease, but it may cause gastrointestinal symptoms like abdominal pain, chronic diarrhea, bleeding or intestinal obstruction. A diagnosis is made at endoscopy or contrast barium studies and should be confirmed by histology. Its histological characteristics include markedly hyperplasic, mitotically active germinal centers and well-defined lymphocyte mantles found in the lamina propria and/or in the superficial submucosa, distributed in a diffuse or focal form. Treatment is directed towards associated conditions because the disorder itself generally requires no intervention. Nodular lymphoid hyperplasia is a risk factor for both intestinal and, very rarely, extraintestinal lymphoma. Some authors recommend surveillance, however, the duration and intervals are undefined.

Evaluation of radiation dose to pediatric patients during certain special procedures

This study was intended to measure pediatric entrance surface air kerma (ESAK) and effective dose during micturating cystourethrography (MCU), intravenous urography (IVU) and barium studies (barium meal, enema, and swallow) and to propose a local diagnostic reference level (DRL). ESAK was measured for patients using calibrated thermoluminescent dosimeters (TLDs, GR200A). Effective doses (E) were calculated using the National Radiological Protection Board (NRPB) software. A total of 236 special pediatric procedures were investigated. 21.7% of the sample comprised barium procedures, 18.6% were MCU procedures while 59.5% of the sample were IVU procedures. The mean ESAK measurements (mGy) were 2.1±0.8, 3.0±23 and 1.2±0.2 for barium meal, enema and swallow in the same order. The mean patient dose for IVU procedures was 12.4±8.7mGy per procedure and the mean patient dose per MCU procedure was 5.8±7mGy. Local DRLs were proposed for all procedures. The patient doses in this study are within the reported values, suggesting that pediatric patients are adequately protected.

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study

BackgroundCongenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.MethodsMedical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.ResultsOver 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).ConclusionsCS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

Clinical Characteristics and Management of Benign Transient Non-Organic Ileus of Neonates: A Single-Center Experience

PurposeThe term benign transient non-organic ileus of neonates (BTNIN) is applied to neonates who present symptoms and plain radiographic findings of Hirschsprung's disease, but do not have aganglionic bowel and are managed well by conservative treatment. It can often be difficult to diagnose BTNIN because its initial symptoms are similar to those of Hirschsprung's disease. The aim of this study is to evaluate the clinical characteristics and proper treatment of BTNIN.Materials and MethodsA retrospective review was made on the clinical data of 19 neonates who were treated for BTNIN between January 2008 and December 2011 at a single facility.ResultsAbdominal distension occurred in every patient (19/19). Other common symptoms included emesis (5/19), explosive defecation (5/19), and constipation (4/19). The vast majority of patients (15/19) experienced the onset of symptoms between 2 and 4 weeks of age. Radiograph findings from all of the patients were similar to Hirschsprung's disease. A barium study showed a transition zone in 33.4% (6/18) of the patients. However, rectal biopsy revealed ganglion cells in the distal rectum in 88.2% (15/17) of the patients, and anorectal manometry showed a normal rectoanal inhibitory reflex in 90% (9/10). All patients responded well to conservative treatment. Symptoms disappeared at the mean age of 4.9±1.0 months, and the abdominal radiographs normalized.ConclusionBTNIN had an excellent outcome with conservative treatment, and must be differentiated from Hirschsprung's disease. A rectal biopsy and anorectal manometry were useful diagnostic tools in the differential diagnosis.