OK432 versus doxycycline for treatment of macrocystic lymphatic malformations.

OBJECTIVES: A variety of sclerotherapy agents are used to treat macrocystic lymphatic malformations (LMs). This retrospective study at a single institution was performed to compare the outcomes of pediatric macrocystic LMs of the head and neck that were treated with doxycycline or with OK432. METHODS: The outcomes measured included early response to therapy, number of treatments required, operating room time, and adverse events. RESULTS: The rates of clinical success for OK432 and doxycycline were similar (83% and 82%, respectively; p > 0.05), although OK432-treated patients required more treatments than did doxycycline-treated patients (1.9 versus 1.0 injections; p = 0.01; 95% confidence interval, 1.57 to 0.27). The average operating room time for a single OK432 injection was significantly shorter than that for doxycycline (53.2 versus 98.1 minutes; p < 0.001); however, when the total number of treatments administered was considered, the overall times in the operating room were similar. Adverse events in the early postoperative period were more common in OK432-treated patients, who experienced marked postoperative swelling compared to doxycycline-treated patients. CONCLUSIONS: OK432 and doxycycline are both effective sclerosants for the treatment of predominantly macrocystic LMs. The administration time for OK432 is shorter than that for doxycycline, but OK432 required more treatments overall to achieve clinical success. Early adverse events were more common in OK432-treated patients, but longer follow-up is necessary to determine whether rates of recurrence and adverse events are similar, particularly in light of the risk of tooth discoloration in doxycycline-treated patients.

Pediatric fasting times before surgical and radiologic procedures: benchmarking institutional practices against national standards.

Prolonged preoperative fasting can be associated with adverse outcomes, particularly in children. Our aims were to assess the time pediatric patients fasted prior to surgical or radiologic procedures and evaluate whether fasting (NPO) orders complied with national guidelines. We measured NPO start time, time of last intake, and time test or surgery was scheduled, took place, or was cancelled in 219 pediatric patients. Findings demonstrate that pediatric patients experienced prolonged fasting before procedures and that the majority of NPO orders were non-compliant with national guidelines. We have developed strategies to reduce fasting times and ensure compliance with recommended national fasting standards.

Congenital paraesophageal hernia: Contemporary results and outcomes of laparoscopic approach to repair in symptomatic infants and children.

BACKGROUND: Congenital paraesophageal hernia (CPEH) is a rare diaphragmatic anomaly for which repair has primarily been described by laparotomy, although, more recent case series describe laparoscopic repair. In reports with over five patients, the predominant approach has been with laparotomy. The purpose of our study was to review our recent institutional experience and results with exclusively laparoscopic repair of CPEH in infants and children. METHODS: An IRB approved retrospective review of all patients with CPEH who underwent laparoscopic treatment at a tertiary children's hospital from 2010 to 2017 was performed. We included only those patients from our own institution with primary CPEH, or CPEH with prior repair (s) at other centers, with recurrence presenting for operation. Data including demographics, diagnostic studies, operative details, complications, outcomes, and follow up were analyzed. Age at diagnosis was 1 day to 25 years of age (mean 2.5 years). RESULTS: A total 28 patients underwent 30 operations to treat CPEH. All operations were completed laparoscopically with no conversions to open. There were 6 Type II, 16 Type III, and 6 Type IV CPEH patients. Seventeen patients were less than one year of age (61%). Weight at time of repair was 10.3 kg (1.2-44 kg). Twelve patients were less than 5 kg (43%), eight patients (28.5%) were less than 10 kg, and 8 were more than 10 kg (28.5%). Operative time averaged 125 min (range 61-247 min). Three patients underwent initial CPEH repair (s) (open: 2 and laparoscopic: 1) at other institutions before laparoscopic revision was performed at our hospital (11%). Crural repair was performed in all patients, fundoplication in 26 (93%) and concomitant gastrostomy was performed in 14 patients (50%). Complications included two patients with recurrent hiatal hernias, which were redone laparoscopically (2/28 or 7% recurrence) and 1 capnothorax requiring pigtail drainage postoperatively. There were no deaths, no requirement for esophageal dilations, or esophageal lengthening. One patient required laparoscopic gastrostomy six weeks post initial repair for failure to thrive. Follow-up ranged from 4 months to 8 years (average 36 months). CONCLUSION: Congenital paraesophageal hernia in infants and children is uncommon. Based on our experience, the laparoscopic approach to repair is feasible, even for neonates, with excellent results, acceptably low recurrence rate, and may even be considered for revisional operations. STUDY TYPE: Clinical research paper. LEVEL OF EVIDENCE: Type IV.

Propranolol vs prednisolone for symptomatic proliferating infantile hemangiomas: a randomized clinical trial.

IMPORTANCE: While propranolol is touted as superior to prednisolone for treating infantile hemangiomas (IH), a randomized clinical trial (RCT) comparing the outcome and tolerability of these medications for symptomatic, proliferating IH has not been reported. OBJECTIVES: To determine if oral propranolol is more efficacious and better tolerated than prednisolone in treating symptomatic, proliferating IH and to determine the feasibility of conducting a multi-institutional, RCT comparing efficacy and tolerability of both medications. DESIGN, SETTING, AND PARTICIPANTS: Phase 2, investigator-blinded, multi-institutional RCT conducted in 3 academic vascular anomalies clinics on 19 of 44 eligible infants aged between 2 weeks and 6 months. All participating patients had symptomatic proliferating IH treated between September 1, 2010, and August 1, 2012. INTERVENTIONS: Treatment with oral propranolol vs prednisolone (2.0 mg/kg/d) until halted owing to toxic effects or clinical response. MAIN OUTCOMES AND MEASURES: Primary outcome was change in IH size after 4 months of therapy. Secondary outcomes were response rate and frequency and severity of adverse events (AEs). RESULTS: The primary outcome showed no difference in lesion size or affected skin area after 4 months of therapy: 41% and 1.32 mm2 for prednisolone vs 64% and 0.55 mm2 for propranolol (P = .12 for lesion size, and P = .56 for affected skin area). Longitudinal analyses showed a faster response in total lesion outer dimension with prednisolone (P = .03), but this advantage over time was not noted when central clearing and outer dimension were included in the analysis (P = .91). The overall frequency of AEs was similar (44 for prednisolone vs 32 for propranolol) (P = .84), but prednisolone-treated participants had more grade 3 severe AEs (11 vs 1) (P = .01), particularly growth retardation resulting in size and weight below the fifth percentile. Early study withdrawal owing to AEs occurred in 6 (75%) of 8 patients in the prednisolone group but 0 of 11 propranolol-treated participants. The mean duration of therapy was shorter for prednisolone (141 vs 265 days), reflecting the higher rate of early withdrawals. CONCLUSIONS AND RELEVANCE: Both medications show similar efficacy for reducing the area of symptomatic, proliferating IH. Although prednisolone showed a faster response rate, propranolol was better tolerated with significantly fewer severe AEs. Propranolol should be the first line of therapy for symptomatic IH unless contraindicated or unless future studies demonstrate severe AEs from propranolol. Recruiting participants for a phase 3 RCT would be difficult owing to safety profiles measured here and emerging trends favoring propranolol. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00967226.

Strategy for management of retroperitoneal tumors with caval tumor thrombus.

The surgical management of retroperitoneal tumors extending into the inferior vena cava (IVC) can be challenging. Although Wilms' tumor is the most common retroperitoneal tumor extending into the IVC, one must approach these tumors systematically as other diagnoses are possible. We present 4 consecutive cases of retroperitoneal tumors with IVC extension as a basis for a management strategy in approaching these patients. Despite similar presentations, these cases illustrate the nuances in surgical management and need for multidisciplinary care with the pediatric oncologists, pediatric surgeons, and pediatric cardiac surgeons.

Aortic bifurcation rupture after blunt abdominal trauma in a child: a case report.

Blunt trauma to the abdomen resulting in aortic injury is rare in children with only a few case reports in the past 40 years. We describe the diagnosis and management of a 2-year-old boy who survived an aortic bifurcation rupture after blunt trauma.

Solid pseudopapillary tumor in a pancreatic rest of the jejunum.

Solid pseudopapillary tumors of the pancreas (SPTP) are very rare, and an SPTP arising in a pancreatic rest has been reported only 4 times previously and never in association with the jejunum. We report this unusual case of a 16 year old girl who presented with 4 days of intermittent, crampy abdominal pain and was found to have an SPTP arising in a pancreatic rest of the jejunum.

Lap-belt syndrome: management of aortic intimal dissection in a 7-year-old child with a constellation of injuries.

We present a case of lap-belt motor vehicle injury in a 7-year-old male who was admitted with abdominal ecchymoses and pain with associated aortic intimal flap, bowel injury, hemoperitoneum, and retroperitoneal hematoma at initial imaging with CT. Most of these findings were confirmed at subsequent laparotomy, and the patient underwent operative repair of bowel injuries. His aortic intimal flap was followed with US and color Doppler imaging during which time he was treated conservatively until there was sonographic evidence of intimal healing. This patient illustrates a multimodality approach to imaging and conservative management.

Significance of 'blush' on computed tomography scan in children with liver injury.

BACKGROUND/PURPOSE: The aim of this study was to determine if the presence of "blush" (an indication of active bleeding) on abdominal CT in children with blunt liver injury adversely affected their clinical outcome as has been reported in adults. METHODS: The authors reviewed the records of 105 children ages 1 to 16 years with blunt liver injury seen on admission IV contrast CT seen over a 6-year period. Demographic characteristics measured were age, mechanism of injury, and injury severity score (ISS). Clinical outcomes included ICU stay, hospital length of stay (LOS), transfusion requirement (milliliters per kilogram), operations performed, and mortality rate. CT scans were evaluated retrospectively by a radiologist blinded to prior reports, for a "blush" and grade of liver injury. No patient underwent arterial embolization. The authors eliminated children with grade I-II injuries (30 patients), because only one had a blush, and analyzed the 75 patients with severe liver injuries (grades III-V). Those patients without a blush (n = 53) seen on CT were the control group, whereas patients with a blush (n = 22) were the study group. Data were analyzed using the Fisher's Exact and Mann-Whitney U test. The level of significance was set at.05. RESULTS: Patients with a blush had a significantly larger transfusion requirement (17.3 +/- 30.5 mL/kg v 5.0 +/- 10.9 mL/kg; P =.02) and mortality rate (23% v 4%; P =.02), but the ISS also was significantly greater (25.8 +/- 14.5 v 17.5 +/- 12.2; P =.019). All other data were similar between the 2 groups. CONCLUSIONS: Children with a blush seen on abdominal CT after blunt liver injury have higher transfusion requirements and greater risk of mortality than those without blush. Mortality is primarily related to the severity of their other injuries.