Open versus thoracoscopic thymectomy for juvenile myasthenia gravis.

BACKGROUND: Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children. METHODS: A retrospective review of all patients younger than 20 years of age who underwent a thymectomy for JMG at two pediatric institutions between 2001 and 2018 was performed. Children were divided into either an open (transcervical or transsternal) or thoracoscopic group and baseline characteristics, perioperative, and postoperative outcomes were compared. RESULTS: Thirty-four thymectomies were performed during the 18-year study period; 18 via an open and 16 via a thoracoscopic approach. The operative time was shorter for open procedures compared thoracoscopic ones (108 ±â€¯49 and 145 ±â€¯43 min, respectively, p = 0.025). Thoracoscopic thymectomy was associated with less intraoperative blood loss (5.5 ±â€¯6.0 vs 55 ±â€¯67 ml, p = 0.007), decreased duration of postoperative intravenous narcotic use (5.0 ±â€¯1.5 vs 20 ±â€¯23 h, p = 0.018), and a shorter length of hospitalization (1.7 ±â€¯1.0 vs 2.7 ±â€¯1.1 days, p = 0.009). No perioperative complication occurred in either group. Clinical improvement was reported in 94% of children in both groups. CONCLUSIONS: Thoracoscopic thymectomy in children is a safe and effective surgical technique for the treatment of JMG. Increased acceptance of this minimally invasive approach by children, families, and referring neurologists may enable earlier surgical intervention. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: III.

Concomitant Fundoplication With Gastrostomy: A Two-State Comparison Showing Continued Use of Reflux Medications.

OBJECTIVES: We sought to determine whether practice differences for fundoplication exist between 2 geographically distinct states, and to determine the reflux medication use pattern associated with concomitant fundoplication. METHODS: A retrospective observational cohort study of children in Colorado (CO) and North Carolina (NC) insured by Medicaid from 2006 to 2008. Children who received a surgical gastrostomy during the study period were included, and our primary outcome measure was the performance of a concomitant gastric fundoplication. Thirty-day prescription fills for reflux medications were examined before and after gastrostomy procedure. RESULTS: We examined 969 surgical gastrostomy admission in both states over the 3-year study period (CO, n = 341 and NC, n = 628). Patients in each state had similar age (median age, 6 months, P = 0.97). Use of pH probe (CO: 15%, NC: 11%) and diagnosis of reflux (CO: 84%, NC: 72%) differed in each state. Concomitant fundoplication was performed in 60% of patients in CO and 43% in NC (P < 0.01). Age less than 6 months was associated with an increased adjusted odds of fundoplication in CO (OR 9.77, CI, 3.91, 24.43), but less so in NC (OR 2.73, CI, 1.48, 5.04). Among patients undergoing gastrostomy, the proportion of patients on reflux medication 4 to 6 months post-discharge did not differ between those receiving fundoplication and those that did not in either state. CONCLUSIONS: Rates of concomitant fundoplication varied in the 2 states despite patients having similar demographic and clinical characteristics. Antireflux surgery was not associated with a reduction in reflux medications in either state.

Failure of resolution of portal fibrosis during omega-3 fatty acid lipid emulsion therapy in two patients with irreversible intestinal failure.

Parenteral omega-3 fatty acid lipid emulsions have been evaluated for their potential role in reversing intestinal failure-associated liver disease. We report our experience using Omegaven in 2 patients with irreversible intestinal failure and intestinal failure-associated liver disease. Despite biochemical and histologic improvement in cholestasis, both patients had persisting, significant portal fibrosis on liver biopsy.