Alka Goyal

The otolaryngologic manifestations in children with eosinophilic esophagitis

OBJECTIVES (1) To describe the incidence of eosinophilic esophagitis (EoE) in the population of patients undergoing esophagoscopy with biopsy by a pediatric otolaryngology service. (2) To elucidate the demographics, presenting symptoms, and endoscopic findings in children with EoE. DESIGN Case series. PATIENTS/METHODS The reports of esophageal biopsy specimens taken over 5 years in 2429 patients were reviewed. Ninety-two patients who received their initial diagnosis of EoE by the pediatric otolaryngology service with specimens showing 15 or greater eosinophils per high power field (HPF) were included. INTERVENTIONS The demographic data, history, presenting symptoms, and endoscopic findings were reviewed retrospectively for the patients. MAIN OUTCOME MEASURE The percentage of children diagnosed with EoE of all children undergoing esophageal biopsy. RESULTS A total of 92 children were diagnosed with EoE (3.8% of total children biopsied). The mean age at biopsy was 4.4 years, much lower than previously reported in the literature (approximately 8 years); 73% were boys and 27% girls. The main presenting symptom was cough (46%) followed by hoarseness, throat clearing, burping/vomiting, and abdominal pain. Forty three percent had a history of asthma and 17% a history of GERD. Half of patients had esophageal edema, a third were normal, and only a quarter had mucosal furrowing on endoscopic examination. CONCLUSIONS EoE is increasingly diagnosed as a clinical entity with a distinct symptom profile and etiology. Increased understanding of EoE and its predisposing factors requires a multidisciplinary approach to diagnosis and management involving the pediatric otolaryngologist.

Liver Enzyme Elevations Within 3 Months of Diagnosis of Inflammatory Bowel Disease and Likelihood of Liver Disease

Background: Inflammatory bowel disease–associated liver diseases (IBD-LDs) include autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and an overlap syndrome. Prospective unbiased multicenter data regarding the frequency of IBD-LD in patients with pediatric inflammatory bowel disease (IBD) are lacking. We examined early alanine aminotransferase (ALT) and &ggr;-glutamyl transpeptidase (GGT) elevations in children diagnosed as having IBD and assessed the likelihood of IBD-LD. Methods: Data collected from the prospective observational Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry enrolling children of age <16 years within 30 days of diagnosis. AIH, PSC, and overlap syndrome were diagnosed using local institutional criteria. Results: A total of 1569 subjects had liver enzymes available. Of the total, 757 had both ALT and GGT, 800 had ALT only (no GGT), and 12 had GGT only (no ALT). Overall, 29 of 1569 patients (1.8%) had IBD-LD. IBD-LD was diagnosed in 1 of 661 (0.15%) of patients with both ALT and GGT ⩽ 50 IU/L compared with 21 of 42 (50%) of patients with both ALT and GGT > 50 (odds ratio 660, P < 0.0001). Of the 29 patients with IBD-LD, 21 had PSC, 2 had AIH, and 6 had overlap syndrome. IBD-LD was more common in patients with ulcerative colitis and IBD-unclassified (indeterminate colitis) than in those with Crohn disease (4% vs 0.8%, respectively, P < 0.001). Conclusions: Elevation of both ALT and GGT within 90 days after the diagnosis of IBD is associated with a markedly increased likelihood of IBD-LD. Both ALT and GGT levels should be measured in all of the pediatric patients newly diagnosed as having IBD.

Primary gastric plasmacytoma: a rare cause of hypertrophic gastritis in an adolescent.

BACKGROUND This report describes a 16-year-old patient with gastric rugal hypertrophy caused by a primary gastric plasmacytoma. She had a 3-month history of nausea and burning abdominal pain. Radiographic studies showed giant rugal hypertrophy. Superficial endoscopic gastric biopsies showed mild inflammation with plasma cells of polyclonal origin in the mucosa. When symptoms persisted, she underwent laparoscopic full-thickness gastric biopsy. There was monoclonal plasma cell infiltration histologically diagnostic of plasmacytoma and inconsistent with Helicobacter pylori-associated mucosa-associated lymphoid tissue (MALT) lymphoma. There was no evidence for involvement of the bone marrow or regional lymph nodes. The tumor did not respond to radiotherapy, necessitating total gastrectomy. METHODS Blood samples were analyzed for interleukin (IL)-6 by enzyme-linked immunosorbent assay. Gastric biopsy and gastrectomy specimens were subjected to immunophenotyping for kappa and lambda light chains, CD45, CD20, and LN1 and to polymerase chain reaction analysis for herpes virus HHV8. RESULTS There was no elevation in circulating IL-6 levels, militating against a pathogenesis akin to that of Castlemans disease. There was no evidence for infection with the Kaposis sarcoma-associated herpes virus HHV8, which has recently been found in patients with multiple myeloma. CONCLUSIONS This diagnosis and the characteristics of the tumor are very unusual, if not unique, for a patient of this age. The diagnostic evaluation of this patient also demonstrates the importance of deep endoscopic or full-thickness biopsies in some children with hypertrophic gastritis.

Congenital cricopharyngeal achalasia in a 4.5-year-old managed by cervical myotomy: A case report

INTRODUCTION Congenital cricopharyngeal achalasia (CCA) is a rare disorder in children characterized by inappropriate contraction of the cricopharyngeus muscle, resulting in the inability to relax the upper esophageal sphincter during deglutition. We report the diagnostic process and management of a relatively older patient who underwent cricopharyngeal myotomy at the age of 4.5 years. METHODS A retrospective review of the case and clinical follow-up was performed. RESULTS This young patient had a long history of dysphagia, choking, nasal reflux and recurrent pneumonia and croup since birth and was diagnosed with CCA at 22 months of age. She underwent balloon dilation of the cricopharyngeus muscle shortly thereafter with only transient relief of her symptoms of feeding difficulty (choking and aspiration). The parents were reluctant for her to undergo further interventions until 2 years later when they consented to cricopharyngeal myotomy. She underwent transcervical myotomy at age 4.5 years and had complete relief of her symptoms. She had no post-operative complications and has done well for nearly 12 months following myotomy. DISCUSSION Our patient is one of the oldest children reported to have undergone myotomy, recovered quickly, and had no difficulty swallowing at any time following surgery. We suggest transcervical cricopharyngeal myotomy as the preferred treatment due to its lasting effects and repeated success in relieving dysphagia in young patients with CCA.

CASE REPORT: Pancreatic Ascites in an Infant: Lack of Symptoms and Normal Amylase

A 4-month-old boy presented with 9 days of abdominal distension. The abdomen was tense, distended, and nontender, with a fluid wave. Hypoalbuminemia, hyponatremia, high lipase, normal amylase, high ascitic fluid: lipase, amylase, and serum-ascites albumin gradient < 1.1 were present. Abdominal CT showed large ascites, edema, and pancreatic cyst. No improvement was noted with bowel rest, TPN, albumin, furosemide, octreotide, and paracentesis. Endoscopic retrograde cholangiopancreatography showed disrupted pancreatic duct and a cyst. Pancreatic duct stenting was complicated by early outward migration of the stent and was thus ineffective. An exploratory laporatomy revealed a cyst. Cystogastrostomy resolved the pancreatitis and ascites. The patient was discharged off TPN and tolerating enteral nutrition. Pancreatic ascites is rare, producing few or no symptoms in infants. In conclusion, our patient may have had viral pancreatitis, complicated by a disrupted duct and/or ruptured pseudocyst with ascites formation. Medical management was ineffective. Surgery appears to have been curative.

Safety, Clinical Response, and Microbiome Findings Following Fecal Microbiota Transplant in Children With Inflammatory Bowel Disease

Background The role of fecal microbiota transplant (FMT) in the treatment of pediatric inflammatory bowel disease (IBD) is unknown. The aims of this study were to assess safety, clinical response, and gut microbiome alterations in children with Crohns disease (CD), ulcerative colitis (UC), or indeterminate colitis (IC). Methods In this open-label, single-center prospective trial, patients with IBD refractory to medical therapy underwent a single FMT by upper and lower endoscopy. Adverse events, clinical response, gut microbiome, and biomarkers were assessed at baseline, 1 week, 1 month, and 6 months following FMT. Results Twenty-one subjects were analyzed, with a median age of 12 years, of whom 57% and 28% demonstrated clinical response at 1 and 6 months post-FMT, respectively. Two CD patients were in remission at 6 months. Adverse events attributable to FMT were mild to moderate and self-limited. Patients prior to FMT showed decreased species diversity and significant microbiome compositional differences characterized by increased Enterobacteriaceae, Enterococcus, Haemophilus, and Fusobacterium compared with donors and demonstrated increased species diversity at 30 days post-FMT. At 6 months, these changes shifted toward baseline. Clinical responders had a higher relative abundance of Fusobacterium and a lower diversity at baseline, as well as a greater shift toward donor-like microbiome after FMT compared with nonresponders. Conclusions A single FMT is relatively safe and can result in a short-term response in young patients with active IBD. Responders possessed increased Fusobacterium prior to FMT and demonstrated more significant microbiome changes compared with nonresponders after FMT. Microbiome characteristics may help in predicting response.

P-198 YI Comparative Analysis of the Efficacy of Fecal Transplantation in Pediatric Inflammatory Bowel Disease Patients with and Without Clostridium Difficile Infection

Background:Current evidence suggests that inflammatory bowel disease (IBD) results from an aberrant immune reaction to gut bacterial antigens. Patients with IBD have been shown to have an altered microbial diversity resulting in dysbiosis, which could be a cause or result of gut inflammation. Oftentimes, IBD patients presenting with a flare are found to be positive for Clostridium difficile infection (CDI) that may be due to colonization versus CDI-related colitis. There is still a paucity of data on the role of fecal microbiome transplantation (FMT) in IBD therapy. Despite the abundant literature on the superiority of FMT for the treatment of primary recurrent CDI (RCDI), its efficacy in treatment of IBD flares with concurrent RCDI has not yet been established. We report a comparative analysis of the efficacy of FMT in pediatric IBD patients with and without RCDI. Methods:The aim of this study was to compare the efficacy of FMT between 2 cohorts of patients: IBD patients who were found to have RCDI while having a disease flare (CDI+) and IBD patients with a flare but were negative for CDI (CDI−). CDI+ patients who received FMT as a part of their clinical care were retrospectively reviewed for CDI resolution and IBD flare response. Response was defined as clinical resolution of IBD flare symptoms. CDI− patients were prospectively studied as a part of a clinical trial on the safety and efficacy of FMT (NCT02108821). Response was defined as an improvement in the Pediatric Ulcerative Colitis Activity Index (PUCAI) by 15 points or in the Pediatric Crohns Disease Activity Index (PCDAI) by 12.5 points and/or a normalization of elevated fecal calprotectin markers. The screening criteria for the recipient and donor as well the FMT protocol in both groups was similar. All patients were followed for a minimum of 6 months. Results:Thirty patients with IBD (age range 1–21 years; median 14) who received 33 FMTs for disease flare with and without RCDI were included. Twenty-one CDI+ patients received a total of 24 FMTs, of which 12 were by nasojejunal, 7 by colonoscopic alone, and 5 by both endoscopic and colonoscopic routes. Three CDI+ patients received 2 FMTs each. Of the 24 FMTs in CDI+ group, 18 had enduring resolution, 3 failed to resolve CDI, and 3 had relapsed CDI within a 6-month period. Nine CDI- patients received a single FMT by both endoscopy and colonoscopy except for one patient who received by colonoscopy alone. Initial response at 1 month was observed in 62% (13/21) and 67% (6/9) of CDI+ and CDI− patients, respectively. By 6 months, only 43% (9/21) of CDI+ and 22% (2/9) of CDI− patients maintained their response. Though the numbers were small, there was no significant difference in the response rates between the 2 groups (1 month P = 1; 6 month P = 0.42). Conclusions:There was a trend toward higher resolution of IBD flare in CDI+ patients than in CDI- patients, though it did not achieve statistical significance. Further prospective, randomized trials may be warranted in a larger population.