Novel Use of a Social-Media-Based Survey to Detect Regional Differences in Management of Monochorionic-Diamniotic Twins.

OBJECTIVE: This study aims to evaluate the utility of social media to distribute a patient survey on differences in management and outcomes of monochorionic-diamniotic (MCDA) pregnancies. STUDY DESIGN: A cross-sectional survey was posted to an English-language MCDA twins patient-centered support group within the social media site, Facebook from April 2, 2018 to June 26, 2018. Subjects were recruited through a technique called "snowballing," whereby individuals shared the survey to assist with recruiting. Patient reported data were analyzed using Chi-square and Kruskal-Wallis's tests to explore characteristics associated with surveillance and outcomes as related to region and provider type. RESULTS: Over 3 months, the post "reached" 14,288 Facebook users, among which 5,653 (40%) clicked on the post. A total of 2,357 respondents with MCDA pregnancies completed the survey. Total 1,928 (82%) were from the United States (US) and 419 (18%) from other countries. Total 85% of patients had co-management with maternal-fetal medicine (MFM), more in the US compared with the rest of the world (87 vs. 74%, p < 0.01). MFM involvement led to increased adherence to biweekly ultrasounds (91 vs. 65%, p < 0.01), diagnosis of monochorionicity by 12 weeks (74 vs. 69%, p < 0.01) and better education about twin-twin transfusion syndrome (90 vs. 66%, p < 0.01). Pregnancies with MFM involvement had a higher take-home baby rate for both babies (92 vs. 89%, p < 0.01) or for at least one baby (98 vs. 93%, p < 0.01) compared with those without MFM involvement. CONCLUSION: A survey distributed via social media can be effective in evaluating real-life management and outcomes of an uncommon obstetrical diagnosis. This survey elucidates wide international variation in adherence to guidelines, management, and outcomes.

Extended Sedation With Continuous Midazolam or Dexmedetomidine Infusion for Young Children Receiving 131 I-MIBG Radiopharmaceutical Therapy for Advanced Neuroblastoma.

BACKGROUND: (131) I-MIBG is increasingly used for treating neuroblastoma; however, administration requires careful adherence to radiation safety guidelines. We describe our experience using continuous sedation to facilitate safe (131) I-MIBG therapy for young children. PROCEDURE: Patients were included in this case series if they received continuous midazolam or dexmedetomidine infusion for sedation during (131) I-MIBG therapy from November 1, 2012, to October 1, 2014. Key outcomes included adequacy of sedation for both (131) I-MIBG infusion and the duration of radioactive isolation, as well as sedative-related toxicities. Additionally, nuclear medicine scans before and after (131) I-MIBG therapy were assessed using the Curie score. These scores were compared qualitatively between midazolam, dexmedetomidine, and control (no sedative infusion) groups. RESULTS: Of the 13 patients receiving continuous sedation for (131) I-MIBG therapy, seven achieved adequate sedation with midazolam, five achieved adequate sedation with dexmedetomidine, one patient (1.6 years old) failed to achieve adequate sedation with either medication and did not receive (131) I-MIBG therapy. Sedation was generally well tolerated. Common side effects for dexmedetomidine infusion included hypotension and relative bradycardia. Both treatment and control groups had multiple patients with increased Curie scores post-(131) I-MIBG therapy. However, one patient in the midazolam group and two in the dexmedetomidine group had decreased Curie scores after (131) I-MIBG therapy, while none decreased in the control group. CONCLUSIONS: Although we cannot exclude the possibility of some inhibition of (131) I-MIBG uptake by midazolam or dexmedetomidine, this case series suggests that continuous infusions of either agent can provide effective sedation to allow safe administration of (131) I-MIBG to young patients.

Clinical features and outcomes of infants with Ewing sarcoma under 12 months of age.

BACKGROUND: Ewing sarcoma peaks in incidence in adolescence. Infants <12 months old have rarely been reported. We aimed to compare clinical features, treatment, and survival of infants <12 months to those of older pediatric patients with Ewing sarcoma. PROCEDURE: We utilized the SEER database to identify patients <12 months of age diagnosed with Ewing sarcoma between 1973 and 2011. We used Fisher exact tests to compare clinical features and treatment modalities between these patients and patients aged 1-19 years. We used Kaplan-Meier methods to describe overall survival in these two groups. RESULTS: Of 1,957 patients in the cohort, 39 (2.0%) were diagnosed at <12 months of age. Infants had a different distribution of primary tumor sites, with lower extremity tumors under represented. Compared to older patients, infants were more likely to have soft tissue tumors (81.6% vs. 27.1%; P < 0.001); have primitive neuroectodermal tumor/Askin tumor (61.5% vs. 19.9%; P < 0.001); and have tumors <8 cm (81.0% vs. 53.2%; P < 0.014). Infants were less likely to receive radiation therapy (13.2% vs. 53.3%; P < 0.001). Infants were at increased risk for early death (P < 0.013 by Wilcoxon), though long-term overall survival was not different between age groups (P < 0.25 by log rank). CONCLUSIONS: Ewing sarcoma is rare in infants, with different clinical presentations and treatment approaches. These patients appear to be at higher risk for early death, but long-term survival is similar to older pediatric patients.

Acute changes in blood pressure in patients with neuroblastoma treated with ¹³¹I-metaiodobenzylguanidine (MIBG).

BACKGROUND: Iodine-131-metaiodobenzylguanidine ((131)I-MIBG) provides targeted radiotherapy for children with neuroblastoma. The aim of our study was to evaluate systematically the acute effects of (131)I-MIBG on blood pressure in patients with neuroblastoma and to identify possible predictors of hypertension. PROCEDURE: We conducted a retrospective chart review of neuroblastoma patients who were treated with (131)I-MIBG between January 1, 1999 and June 1, 2012 at the University of California, San Francisco. Clinical data for 172 patients with neuroblastoma, receiving 218 administrations of (131)I-MIBG, were collected. The primary endpoint was development of systolic blood pressure above the 95th percentile for age. Logistic regression with generalized estimating equations to account for multiple administrations in some subjects was used to identify bivariate and multivariate predictors of hypertension. RESULTS: Of the 218 administrations of (131)I-MIBG, 112 (51.3%) were associated with at least one episode of systolic hypertension during or after the (131)I-MIBG infusion. The majority of these acute elevations in blood pressure resolved within 48 hours of the infusion. Only six administrations in five patients required nifedipine administration to lower blood pressure. Younger age (P = 0.012), lower eGFR (P = 0.047), and elevated blood pressure measurements immediately before infusion began (P = 0.010) were all independently associated with risk of treatment-associated hypertension. CONCLUSIONS: Acute elevations in blood pressure are common after therapeutic doses of (131) I-MIBG. Elevations in blood pressure typically occur only within the first 48 hours after (131)I-MIBG administration. Blood pressure monitoring during this period of risk is recommended.

Lateral spurring (overgrowth) after pediatric lateral condyle fractures.

BACKGROUND: Bony overgrowth over the lateral condyle, or "lateral spurring," is commonly identified after lateral condyle fractures of the humerus in children. Despite its frequent recognition, no prior study has defined the phenomenon, established an incidence rate, explored a correlation with any fracture or treatment characteristics, nor assessed whether it is of functional significance. METHODS: We retrospectively analyzed information on 212 consecutive lateral condyle fractures in children. Spurring was defined as an overgrowth of bone over the lateral aspect of the lateral condyle resulting in an irregularity of the metaphyseal flare. The magnitude of the spurring was classified by measuring the increase in maximum interepicondylar width of the distal humerus on the latest follow-up radiograph. RESULTS: Of the 212 fractures, 55% were treated with cast immobilization, 11% with closed reduction and percutaneous pinning, and 34% with open reduction and internal fixation. Of all fractures, 73% developed a lateral spur. Of those, 43% had a mild spur, 38% a moderate spur, and 19% a severe spur. Fractures that developed a spur had a mean initial displacement of 3.3 mm, as compared with 1.1 mm in those that did not develop spurring (P<0.0001). The amount of initial displacement was higher for fractures that developed mild (2.4 mm, P=0004), moderate (3.6 mm, P<0.00001), and severe (4.9 mm, P<0.00001) spurs, as compared with fractures with no spur. At the latest follow-up, patients that developed lateral spurring had a mean relative arc of motion of 93.7% of the normal contralateral elbow, whereas patients without a spur had a relative range of motion of 94.3% (P=0.4). CONCLUSIONS: Lateral spurring is an extremely common sequela of lateral humeral condyle fractures in children. The development of a spur correlates with initial displacement and surgical treatment. The size of the spur is associated with the amount of initial fracture displacement. Despite concerns from patients, families, and physicians alike, neither the presence nor the size of the lateral spur seems to influence the final outcome. LEVEL OF EVIDENCE: Level II--retrospective study.

Outcomes of reduction more than 7 days after injury in supracondylar humeral fractures in children.

BACKGROUND: Some slightly extended type II fractures initially treated with closed reduction and casting can displace during the first 2 weeks of follow-up. Although closed reduction and percutaneous pinning are desirable for displaced supracondylar humeral fractures treated acutely, there is little or no available information regarding the surgeon's ability to obtain a satisfactory reduction when such a procedure is performed more than a week after the original injury, or the clinical outcome of it. METHODS: We reviewed the information on 143 type II pediatric supracondylar humeral fractures that were treated by closed reduction and percutaneous pinning. To determine the effect of late treatment, we compared a group of fractures that was treated within the first 7 days (group 1, n=101) with a group that was treated >7 days after the injury (group 2, n=42). RESULTS: Mean time from presentation to surgery was 2.1 days (range, 0 to 5) and 9.8 days (range, 7 to 15) for fractures in groups 1 and 2, respectively. There was no need for an open reduction in either group. An anatomic reduction was obtained in all fractures. There were no iatrogenic nerve injuries, vascular complications, or compartment syndromes in either group. Length of surgery was similar in both groups (P=0.3). There were no significant differences in final carrying angle (P=0.2) or range of motion of the treated elbow (P=0.21). Avascular necrosis of the humeral trochlea was identified in 2 fractures that were treated surgically 8 days after the original injury (group 2). CONCLUSIONS: The results of this study suggest that it is possible to obtain an anatomic reduction of a type II pediatric supracondylar humeral fracture even after 7 days from the injury. Such a delay in surgery does not appear to lead to longer surgeries, a higher incidence of open reduction, or to alter the final alignment or range of motion of the elbow. However, the risk of developing an avascular necrosis of the humeral trochlea must be considered. LEVEL OF EVIDENCE: II.