Genetically Confirmed Malignant Hyperthermia in a Six-Week-Old Infant: A Case Report.

A six-week-old 3.9-kg infant presented for microlaryngoscopy and diaphragmatic hernia repair. While positioning for laparoscopy after microlaryngoscopy, the infant developed muscle rigidity, worsening hypercarbia, tachycardia, and early hyperthermia. Sevoflurane was discontinued, and the clinical picture indicating malignant hyperthermia (MH) resolved without dantrolene. Subsequent genetic testing revealed that both the patient and his father carried a mutation in the RYR1 gene that is diagnostic for MH. This is the second youngest genetically confirmed case of MH. This case adds to a limited body of evidence regarding MH presentation and diagnosis in neonates and infants.

Packed red blood cell donor age affects overall survival in transfused patients undergoing hepatectomy for non-hepatocellular malignancy.

BACKGROUND: Patients undergoing hepatectomy often require packed red blood cell (PRBC) transfusion, which has been associated with worse oncologic outcomes. However, limited data exist regarding the impact of PRBC donor factors. We hypothesized that PRBC donor age impacts survival after hepatectomy for non-hepatocellular malignancies. METHODS: Patients who underwent hepatectomy for non-hepatocellular malignancy from 2005 to 2014 were retrospectively evaluated. Impact of clinicopathologic and PRBC factors on oncologic outcomes were assessed. RESULTS: Of 149 identified patients, 76 received a perioperative PRBC transfusion (median 2 units). Transfusion was associated with increased median length of stay (8 vs. 6 days; p < 0.01) and median operative blood loss (700 vs. 350 mL; p < 0.01) versus non-transfused, respectively. In transfused patients, receipt of PRBC from older donors compared to younger resulted in decreased RFS (0.94 vs. 2.63 years, respectively; p = 0.02) and OS (1.94 vs. 3.44 years, respectively; p = 0.6). The PRBC donor age was an independent predictor of decreased recurrence free survival on multivariate analysis (HR 2.5, p = 0.04). CONCLUSIONS: In patients undergoing hepatectomy for non-hepatocellular malignancies and receiving perioperative transfusion, PRBC donor age may impact survival and warrants further investigation.

Preoperative enteral access is not necessary prior to multimodality treatment of esophageal cancer.

BACKGROUND: Surgical enteral access prior to multimodality treatment for esophageal cancer is controversial as dysphagia is often used for feeding tube referral. We hypothesized that enteral access before neoadjuvant chemoradiation for esophageal cancer provides no benefit compared to that placed during definitive esophagectomy. METHODS: Patients undergoing esophagectomy for esophageal malignancy from 2007 - 2014 were retrospectively identified. Clinicopathologic factors were recorded including preoperative enteral access, weight change, nutritional laboratory works, and perioperative complications. RESULTS: Of 156 identified patients, 99 (63.5%) received neoadjuvant chemoradiation and comprised the study cohort. Fifty (50.5%) underwent enteral access (gastrostomy [14], jejunostomy [32], other [4]; "Access Group") prior to chemoradiation followed by esophagectomy and were compared to 49 "No-Access" patients who underwent enteral access during esophagectomy. Clinicopathologic variables were similar between cohorts. The Access and No-Access cohorts had similar reported dysphagia (86% vs 75.5%, respectively; P = .2) and mean preesophagectomy serum albumin (3.9 vs 4 gm/dL, respectively; P = .2). Weight loss ± 6-month periesophagectomy was similar between access versus No-Access cohorts (-11.2% vs -15.4%, respectively; P = .1). Weight loss during this period was likewise similar for patients with dysphagia in the Access (-11%) versus No-Access group (-15.2%, P = .1). No difference in complication rates was noted between Access (64%) and No-Access groups (51%, P = .2). CONCLUSION: Despite healthcare provider bias, there seems to be no nutritional or perioperative benefit for enteral access before neoadjuvant chemoradiation for esophageal malignancy. Patients with esophageal malignancy should therefore proceed to appropriate neoadjuvant and surgical therapy with enteral access performed during definitive resection or reserved for those with frank obstruction on endoscopy.