Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience.

PURPOSE: Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period. METHODS: Patients with achalasia were identified from a database built during the period of January 2000-January 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described. RESULTS: Thirteen patients were studied; median age was 7 (1-14) years. Most frequent symptoms were vomiting (84.6 %) and dysphagia (69.2 %). Weight loss occurred in 46.0 % of patients and chronic cough in 46.1 %. Associated disorders were Down's syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up. CONCLUSION: Achalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.

Changing pattern of indications of endoscopic retrograde cholangiopancreatography in children and adolescents: a twelve-year experience.

BACKGROUND: There are few data regarding endoscopic retrograde cholangiopancreatography (ERCP) usefulness in children and adolescents. We reviewed the long-term experience with diagnostic and therapeutic ERCP in a tertiary single center in Southern Brazil. METHODS: A retrospective chart review of patients aged 0-18 years who had undergone ERCPs from January 2000 to June 2012 was done. Data on demographics, indications, diagnosis, treatments, and complications were collected. RESULTS: Seventy-five ERCPs were performed in 60 patients. The median age of the patients at the procedure was 13.9 years (range: 1.2-17.9). Of the 60 patients, 47 (78.3%) were girls. Of all ERCPs, 48 (64.0%) were performed in patients above 10 years and 35 (72.9%) of them were in girls. ERCP was indicated for patients with bile duct obstruction (49.3%), sclerosing cholangitis (18.7%), post-surgery complication (12%), biliary stent (10.7%), choledochal cyst (5.3%), and pancreatitis (4%). The complication rate of ERCP was 9.7% involving mild bleeding, pancreatitis and cholangitis. Patients who had therapeutic procedures were older (13.7±3.9 vs. 9.9±4.9 years; P=0.001) and had more extrahepatic biliary abnormalities (82% vs. 50%; P=0.015) than those who had diagnostic ERCPs. A marked change in the indications of ERCPs was found, i.e., from 2001 to 2004, indications were more diagnostic and from 2005 therapeutic procedures were predominant. CONCLUSIONS: Diagnostic ERCPs are being replaced by magnetic resonance cholangiopancreatography and also by endoscopic ultrasound. All these procedures are complementary and ERCP still has a role for therapeutic purposes.

Acid and Weakly Acidic Gastroesophageal Reflux and Pepsin Isoforms (A and C) in Tracheal Secretions of Critically Ill Children.

BACKGROUND: Gastroesophageal reflux (GER) and pulmonary aspiration are frequent in patients in the ICU. The presence of pepsin in airways seems to be the link between them. However, pepsin isoforms A (gastric specific) and C (pneumocyte potentially derived) need to be distinguished. This study aimed to evaluate GER patterns and to determine the presence of pepsin A and C in tracheal secretions of critically ill children receiving mechanical ventilation. METHODS: All patients underwent combined multichannel intraluminal impedance-pH (MII-pH) monitoring. Tracheal secretion samples were collected to determine the presence of pepsin. Pepsin A and C were evaluated by Western blot. MII-pH parameters analyzed were number of total GER episodes (NGER); acid, weakly acidic, and weakly alkaline GER episodes; and proximal and distal GER episodes. RESULTS: Thirty-four patients (median age, 4 months; range, 1-174 months) were included. MII-pH monitoring detected 2,172 GER episodes (77.0% were weakly acidic; 71.7% were proximal). The median NGER episodes per patient was 59.5 (25th-75th percentile, 20.3-85.3). Weakly acidic GER episodes per patient were significantly more frequent than acid GER episodes per patient (median [25th-75th percentile], 43.5 [20.3-68.3] vs 1.0 [0-13.8], respectively; P < .001). Only three patients had an altered acid reflux index (44.9%, 12.7%, and 13.6%) while not taking antacid drugs. Pepsin A was found in 100% of samples and pepsin C in 76.5%. CONCLUSIONS: The majority of GER episodes of children in the ICU were proximal and weakly acidic. All patients had aspiration of gastric contents as detected by pepsin A in tracheal fluid. A specific pepsin assay should be performed to establish gastropulmonary aspiration because pepsin C was found in > 70% of samples.