Apnea is a known complication of the Chiari II malformation presenting in infancy. Obstructive apnea secondary to bilateral abductor palsy or laryngomalacia and centrally mediated expiratory apnea with cyanosis can occur. Observations of 9 patients suggest that these forms of apnea may represent stages in a continuum of brain stem dysfunction due to the combined effects of the hindbrain malformation and its compression, hydrocephalus and progressive arachnoiditis. Obstructive apnea in some patients may be reversed by optimal control of hydrocephalus with or without cervical decompression. These patients may also develop episodes of cyanotic expiratory apnea of central origin (PEAC). This form of apnea does not respond to surgical or medical treatment and may show progressive worsening over time. Five of 6 patients with this form of apneic spell died suddenly, 2 of these died despite full recuscitative efforts. It is recommended that reports of treatment address results for both forms of apnea.

ERCP can be safely and successfully performed in a provincial centre.

Background and study aims Refractory variceal bleeding is associated with high mortality in patients with chronic liver disease. A fully-covered self-expanding metal stent (SEMS) has been reported to have excellent rates of technical success and initial bleeding control; however, studies to date are small and limited to Europe and Asia. Our aim was to evaluate the efficacy and safety of this SEMS for control of refractory variceal bleeding (VB). Patients and methods A retrospective analysis was undertaken of all patients who received the SX-ELLA Danis SEMS for management of VB at 9 tertiary centers across Australia and New Zealand. A total of 32 SEMS had been deployed in 30 patients (median age 53.3). Results Technical success of SEMS placement was achieved in 100 % of cases, resulting in immediate control of bleeding across 31 of 32 cases (96.9 %). Re-bleeding with SEMS in situ occurred in three of 32 cases (9.4 %). Mean SEMS in-dwelling time was 6.4 days. Delayed SEMS migration occurred in 6.3 % of cases. Interventional radiological therapy for management of varices within 6 weeks was performed in 12 of 30 patients (40 %). Death with SEMS in situ occurred in seven of 30 patients (23.3 %). Seven-day bleeding-related mortality was 16.7 %, 14-day mortality 23.3 %, and 6-week mortality 33.3 %. Three of 30 patients (10 %) received orthotopic liver transplantation following SEMS insertion, including two patients within 6 weeks. Conclusions SX-Danis Ella SEMS is highly effective for immediate control of refractory VB and bridging to definitive therapy because it has excellent technical success rates, appears to be relatively easy to use, and has low rates of serious adverse events.

the IV deprived group (20 min) vs IV administered group (16 min). The IV rescue group had the longest procedure time at 28 min. Drug delivery was more efficient and convenient in pts who were administered IV fluids. Conclusions: Routine administration of IV fluids does not reduce the incidence of hypotension or other complications during endoscopic procedures. On demand IV fluids can safely and quickly correct any hemodynamic abnormalities without any adverse outcomes. Universally giving IV fluids to all pts undergoing endoscopic procedures under moderate sedation may not be necessary. Even though no risk factors predicted hypotensive episodes, one should decide on the value of administering empiric IV fluids based on clinical judgement.