Double barrel enteroplasty for the management of short bowel syndrome in children.

BACKGROUND: Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. METHODS: We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. RESULTS: All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. CONCLUSIONS: Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.

Tunnelled central venous access devices in small children: A comparison of open vs. ultrasound-guided percutaneous insertion in children weighing ten kilograms or less.

PURPOSE: Ultrasound-guided (USG) percutaneous insertion of tunnelled central venous access devices (CVADs) has been shown to be safe and effective in adults. However, there have been concerns over the safety of this technique in small children. This paper analyses the safety of USG percutaneous CVAD insertion in the pediatric population weighing ten kilograms or less. METHOD: All surgically inserted CVADs for children weighing ten kilograms or less, between January 2010 and December 2015 at the Children's Hospital at Westmead were retrospectively reviewed. Open and USG percutaneous techniques were compared with intraoperative complications as the primary outcome variable. Secondary outcome measures included conversion to open technique, postoperative complications, operating time and catheter longevity. RESULTS: 232 cases were identified: 96 (41.4%) open, 136 (58.6%) USG percutaneous. Age ranged <1-48 months; weight 0.7-10 kg. CVADs ranged 2Fr-9Fr in size. Eleven USG percutaneous cases required conversion to open. There was no significant difference in intraoperative complication rate between open (11/96, 11.5%) and USG percutaneous (19/136, 14.0%) groups (p = 0.574). There was no significant difference in overall postoperative complications, operative time or catheter longevity. Mechanical blockage was significantly higher in the open group than the USG percutaneous group (21% vs 10%, p = 0.015). CONCLUSION: USG percutaneous CVAD insertion is safe in children weighing ten kilograms or less. Open catheter insertion may be associated with higher rates of post-operative catheter blockage in small children. LEVEL OF EVIDENCE: Level III.

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.

Focused image-guided parathyroidectomy in the current management of primary hyperparathyroidism.

OBJECTIVE: Primary hyperparathyroidism (PHPT) in childhood and adolescence has been considered a different disease to that seen in adults, with predominantly familial aetiology mandating open exploration to exclude parathyroid hyperplasia in contrast to the adoption of focused image-guided parathyroidectomy (FP) in adults. STUDY DESIGN: A retrospective cohort study in a tertiary referral hospital setting of all children and adolescents (<18 years) undergoing parathyroid surgery for PHPT. Data were obtained from a dedicated endocrine surgery database and hospital medical records. RESULTS: Over the 35-year study period (1980-2014), there were 31 patients who underwent parathyroidectomy for PHPT. 3 patients were from known multiple endocrine neoplasia type 1 syndrome (MEN1) families, 3 had an isolated family history of PHPT and 25 were sporadic. In the sporadic group, 24 (96%) presented with symptomatic hypercalcaemia, affecting the gastrointestinal, musculoskeletal, genitourinary or neuropsychiatric systems. In the 25 patients with sporadic PHPT, nine (36%) had FP with a single adenoma removed with a 100% initial cure rate. Sixteen patients (64%) in the sporadic group had an open exploration: 14 had single gland disease while 2 patients required a second procedure to achieve a final cure rate of 100%. Of the three patients with MEN1, one was cured, one has persistent hyperparathyroidism after FP and the third has permanent hypoparathyroidism after open exploration. CONCLUSIONS: The majority of children and adolescents with PHPT have symptomatic disease due to a single adenoma. They can therefore be managed in a similar fashion to their adult counterparts with preoperative localisation studies aiming to permit FP in a day case setting.

Expression of HOXB2, a retinoic acid signaling target in pancreatic cancer and pancreatic intraepithelial neoplasia.

PURPOSE: Despite significant progress in understanding the molecular pathology of pancreatic cancer and its precursor lesion: pancreatic intraepithelial neoplasia (PanIN), there remain no molecules with proven clinical utility as prognostic or therapeutic markers. Here, we used oligonucleotide microarrays to interrogate mRNA expression of pancreatic cancer tissue and normal pancreas to identify novel molecular pathways dysregulated in the development and progression of pancreatic cancer. EXPERIMENTAL DESIGN: RNA was hybridized to Affymetrix Genechip HG-U133 oligonucleotide microarrays. A relational database integrating data from publicly available resources was created to identify candidate genes potentially relevant to pancreatic cancer. The protein expression of one candidate, homeobox B2 (HOXB2), in PanIN and pancreatic cancer was assessed using immunohistochemistry. RESULTS: We identified aberrant expression of several components of the retinoic acid (RA) signaling pathway (RARalpha, MUC4, Id-1, MMP9, uPAR, HB-EGF, HOXB6, and HOXB2), many of which are known to be aberrantly expressed in pancreatic cancer and PanIN. HOXB2, a downstream target of RA, was up-regulated 6.7-fold in pancreatic cancer compared with normal pancreas. Immunohistochemistry revealed ectopic expression of HOXB2 in 15% of early PanIN lesions and 48 of 128 (38%) pancreatic cancer specimens. Expression of HOXB2 was associated with nonresectable tumors and was an independent predictor of poor survival in resected tumors. CONCLUSIONS: We identified aberrant expression of RA signaling components in pancreatic cancer, including HOXB2, which was expressed in a proportion of PanIN lesions. Ectopic expression of HOXB2 was associated with a poor prognosis for all patients with pancreatic cancer and was an independent predictor of survival in patients who underwent resection.