Does Combined Multichannel Intraluminal Impedance and pH (MII-pH) Testing Improve Clinical Outcomes in Children With Gastroesophageal Reflux Disease?

OBJECTIVES: The aim of the study was to investigate the role of combined multichannel intraluminal impedance and pH (MII-pH) testing in clinical management of children with gastroesophageal reflux disease (GERD) by exploring the impact of treatment changes made based on MII-pH testing results on symptoms and quality of life outcomes. METHODS: All patients (<18 years) referred to the Sydney Children's Hospital for MII-pH testing were recruited. Patients were classified by acid suppression therapy (AST) status (on AST and off AST) and changes in medical and surgical management were evaluated. Validated questionnaires (Pediatric Gastroesophageal Symptom and Quality of Life Questionnaire and Infant Gastroesophageal Reflux Questionnaire Revised) were administered at baseline at the time of MII-pH testing, and 4 weeks after treatment changes were made and questionnaire scores were compared. RESULTS: Of the 45 patients recruited, 24 patients (53.3%) were off AST and 21 patients (46.7%) were on AST. MII-pH testing led to medication changes in 30 patients (66.7%). This included 15 of 24 (62.5%) in those off AST and 15 of 21 (71.4%) in those on AST. More than 98% of patients who had treatment changes showed a significant improvement in both symptoms and quality of life scores. CONCLUSIONS: Our study is one of the first pediatric studies to evaluate the clinical validity of MII-pH testing in the pediatric population referred for suspected GERD, and its ability in guiding clinical management. Our study has shown that treatment decisions guided by and based on results of MII-pH testing led to a significant improvement in symptoms and quality of life in infants and children with GERD.

The utility of multichannel intraluminal impedance-pH testing in tailoring the management of paediatric gastro-oesophageal reflux disease.

AIM: To determine whether combined multichannel intraluminal impedance and pH (MII-pH) testing led to a change in management of children with gastro-oesophageal reflux disease (GORD). METHODS: Retrospective chart review was done in all patients who underwent MII-pH testing for GORD symptoms at Sydney Children's Hospital between 2008 and 2016. Changes to anti-reflux medications and referral for anti-reflux surgery were evaluated. RESULTS: There were 365 patients, 260 (71.2%) of whom were on acid-suppressing therapy. The median age was 4.1 ± 4.8 years, 205 patients (56%) were males, 83 (22.7%) were infants (<1 year of age) and 145 (39.7%%) had comorbid conditions. We found 72.1% had abnormal MII-pH results, of which 17.5% had abnormal acid reflux, 8.2% had abnormal number of retrograde bolus movements and 46.3% had hypersensitive oesophagus (positive symptom association only). Infants were significantly more likely to have abnormal MII-pH results compared to older children (P = .04). Results of MII-pH testing led to medication changes in 44.7% and referral for anti-reflux surgery in 6.8% of patients. CONCLUSION: Combined multichannel intraluminal impedance and pH testing is clinically useful in the management of children with symptoms of GORD and over half the patients had changes to their medical treatment or referral for anti-reflux surgery based on the results of MII-pH testing. It resulted in a treatment change in an additional 32% of patients over traditional pH-metry.

Birt-Hogg-Dubé syndrome presenting with macroscopic pulmonary cyst formation in a 15-year-old.

Birt-Hogg-Dubé (BHD) syndrome is a rare, autosomal dominant disorder caused by a germline mutation in the folliculin gene (17p11.2). It is characterized by benign skin lesions, renal tumours, and pulmonary cysts, with pneumothoraces seen exceptionally rarely in patients younger than 40 years. We report the case of a 15-year-old boy who presented with sudden onset left-sided chest pain and acute dyspnoea secondary to a large left-sided pneumothorax. This failed to resolve despite chest drain insertion and he required video-assisted thoracoscopic surgical pleurodesis, which revealed macroscopic pulmonary cyst formation. Following this, he made a good recovery and a further high-resolution computerized tomography (CT) scan of his chest identified multiple, small, subpleural parenchymal lung cysts that were not initially visible on prior imaging. Further questioning revealed a strong family history of spontaneous pneumothoraces and additional genomic sequencing, and confirmed a diagnosis of BHD syndrome. We highlight the diagnostic, management, and surveillance challenges for this rare syndrome.

Is fundoplication required after the Foker procedure for long gap esophageal atresia?

BACKGROUND: Fundoplication has been performed almost universally in children treated with the Foker procedure (FP) for long gap esophageal atresia (LGEA). We report our experience with pharmacological management and endoscopic surveillance rather than early routine fundoplication in infants treated with the FP. METHODS: A retrospective chart review was performed of all children treated with the Foker procedure at our institution. RESULTS: Nine children have undergone the FP since 2007. Median time between FP and definitive anastomoses was 22days. All nine children kept their native esophagus. There were three minor anastomotic leaks, all treated nonoperatively. All children required dilatation of anastomotic strictures (range 2-15). All have been treated with proton pump inhibitors. Three children had eosinophilic esophagitis and one had Barrett's esophagus. Only two children in this series have undergone fundoplication, which was performed for symptomatic and persistent erosive esophagitis. CONCLUSION: The question of early versus delayed fundoplication in LGEA patients managed with the FP remains unanswered. Our series demonstrates that it is possible to achieve good long-term outcomes when the operation is reserved for children with gastroesophageal reflux disease resistant to maximal medical therapy. LEVEL OF EVIDENCE: IV.

Omental infarction: risk factors in children.

PURPOSE: A series of 4 cases of omental infarction are analyzed. It is shown that obesity, as well as idiosyncratic anatomy, are factors in the development of this rare clinicopathologic entity. METHODS: The authors reviewed the preoperative and operative records of all patients. RESULTS: All of the cases of omental infarction were seen in obese children. Other than persistent right-sided abdominal pain, there were no other signs or symptoms. None of the patients had an elevated white blood cell count, and none had fever. CONCLUSIONS: Computed tomography scanning is noted to be helpful in making the diagnosis. Laparoscopy is noted to be the procedure of choice, because standard incisions may preclude one from a proper exploration.