Life-Threatening Hemoptysis in a Pediatric Referral Center.

OBJECTIVES: Hemoptysis is uncommon in children, even among the critically ill, with a paucity of epidemiological data to inform clinical decision-making. We describe hemoptysis-associated ICU admissions, including those who were critically ill at hemoptysis onset or who became critically ill as a result of hemoptysis, and identify predictors of mortality. DESIGN: Retrospective cohort study. Demographics, hemoptysis location, and management were collected. Pediatric Logistic Organ Dysfunction-2 score within 24 hours of hemoptysis described illness severity. Primary outcome was inhospital mortality. SETTING: Quaternary pediatric referral center between July 1, 2010, and June 30, 2017. PATIENTS: Medical/surgical (PICU), cardiac ICU, and term neonatal ICU admissions with hemoptysis during or within 24 hours of ICU admission. INTERVENTIONS: No intervention. MEASUREMENTS AND MAIN RESULTS: There were 326 hemoptysis-associated ICU admissions in 300 patients. Most common diagnoses were cardiac (46%), infection (15%), bronchiectasis (10%), and neoplasm (7%). Demographics, interventions, and outcomes differed by diagnostic category. Overall, 79 patients (26%) died inhospital and 109 (36%) had died during follow-up (survivor mean 2.8 ± 1.9 yr). Neoplasm, bronchiectasis, renal dysfunction, inhospital hemoptysis onset, and higher Pediatric Logistic Organ Dysfunction-2 score were independent risk factors for inhospital mortality (p < 0.02). Pharmacotherapy (32%), blood products (29%), computerized tomography angiography (26%), bronchoscopy (44%), and cardiac catheterization (36%) were common. Targeted surgical interventions were rare. Of survivors, 15% were discharged with new respiratory support. Of the deaths, 93 (85%) occurred within 12 months of admission. For patients surviving 12 months, 5-year survival was 87% (95% CI, 78-92) and mortality risk remained only for those with neoplasm (log-rank p = 0.001). CONCLUSIONS: We observed high inhospital mortality from hemoptysis-associated ICU admissions. Mortality was independently associated with hemoptysis onset location, underlying diagnosis, and severity of critical illness at event. Additional mortality was observed in the 12-month posthospital discharge. Future directions include further characterization of this vulnerable population and management recommendations for life-threatening pediatric hemoptysis incorporating underlying disease pathophysiology.

Mortality and Factors Associated With Hemorrhage During Pediatric Extracorporeal Membrane Oxygenation.

OBJECTIVES: To quantify and identify factors associated with bleeding events during pediatric extracorporeal membrane oxygenation. DESIGN: Retrospective cohort study with primary outcome of bleeding days on extracorporeal membrane oxygenation. SETTING: Single tertiary care children's hospital. SUBJECTS: One-hundred twenty-two children supported with extracorporeal membrane oxygenation for greater than 12 hours during January 2015 through December 2016. INTERVENTIONS: Bleeding days were identified if mediastinal or cannula site exploration, activated factor VII administration, gastrointestinal, pulmonary, or intracranial hemorrhages occurred. Logistic regression was used to assess factors associated with bleeding days. MEASUREMENTS AND MAIN RESULTS: Study population was identified from institutional extracorporeal membrane oxygenation database. Clinical, laboratory, and survival data were obtained from medical records. Only data from patients' first extracorporeal membrane oxygenation run were used. One-hundred twenty-two patients with median age of 17 weeks (interquartile range, 1-148 wk) were analyzed. Congenital heart disease (n = 56, 46%) was the most common diagnosis. Bleeding days comprised 179 (16%) of the 1,121 observed extracorporeal membrane oxygenation-patient-days. By extracorporeal membrane oxygenation day 4, 50% of users had experienced a bleeding day. Central rather than peripheral cannulation (odds ratio, 2.58; 95% CI, 1.47-4.52; p < 0.001), older age (odds ratio, 1.31 per increased week; 95% CI, 1.14-1.52; p < 0.001), higher lactate (odds ratio, 1.08 per 1 mmol/L increase; 95% CI, 1.05-1.12; p < 0.001), and lower platelets (odds ratio, 0.87 per 25,000 cell/µL increase; 95% CI, 0.77-0.99; p = 0.005) were associated with bleeding days. Patients who experienced more frequent bleeding (> 75th percentile) had fewer ventilator-free and hospital-free days in the 60 days after cannulation (0 vs 31; p = 0.002 and 0 vs 0; p = 0.008) and higher in-hospital mortality (68 vs 34%; p < 0.001). CONCLUSIONS: Central cannulation, older age, low platelets, and high lactate are associated with bleeding days during pediatric extracorporeal membrane oxygenation. Patients who bleed more frequently during extracorporeal membrane oxygenation have higher in-hospital mortality, longer technological dependence, and reduced hospital-free days.

Extracorporeal Membrane Oxygenation Support After Heart Transplantation in Children-Outcomes of a Single Center Cohort.

OBJECTIVES: Extracorporeal membrane oxygenation is used for postcardiotomy low cardiac output but is less established following heart transplantation. We characterized outcomes for children supported with extracorporeal membrane oxygenation after heart transplantation. DESIGN: Single-center retrospective study. SETTING: Large pediatric cardiac referral center. PATIENTS: All patients who received heart transplantation and were cannulated to extracorporeal membrane oxygenation between 1995 and 2016. INTERVENTIONS: Primary outcome measure was mortality 12 months postextracorporeal membrane oxygenation. Patient characteristics were analyzed for association with outcome according to early graft failure (extracorporeal membrane oxygenation ≤ 7 d after heart transplantation), or late graft failure. MEASUREMENTS AND MAIN RESULTS: There were 246 heart transplants during the study period and 50 extracorporeal membrane oxygenation runs in 44 patients. Median time from transplant to extracorporeal membrane oxygenation was 1 day (range, 0-11.7 yr), with early graft failure in 28 patients (median 1, range 0-2 d) and 22 extracorporeal membrane oxygenation runs in 20 late graft failure patients (median, 0.8 yr; range, 8 d to 11.7 yr), including four patients with prior extracorporeal membrane oxygenation for early graft failure. Twenty-six patients (59%) survived to hospital discharge, and survival 12 months postextracorporeal membrane oxygenation was 24 patients (55%), lower in those with late graft failure (40% vs 67%; p 0.02). Independent risk factors for 12-month mortality were congenital heart disease, higher pulmonary vascular resistance indexed to body surface area (> 2.2 Woods U/m), and higher creatinine. Higher panel reactive antibody levels were associated with 12-month mortality in the late graft failure group only. CONCLUSIONS: Extracorporeal membrane oxygenation can be effectively used to rescue patients with graft dysfunction after heart transplantation but is associated with high early mortality. Factors associated with mortality within 12 months include presence of congenital heart disease, renal dysfunction, elevated pulmonary vascular resistance indexed to body surface area and in those supported with extracorporeal membrane oxygenation late after heart transplantation, significant human leukocyte antigen sensitization.

Pediatric Hemoptysis without Bronchiectasis or Cardiac Disease: Etiology, Recurrence, and Mortality.

OBJECTIVES: To describe the etiologies of hemoptysis in patients without pre-existing bronchiectasis or cardiac disease; to assess odds of recurrent hemoptysis by diagnostic category; and to assess odds of mortality by diagnostic category. STUDY DESIGN: This retrospective case series included all patients with hemoptysis documented during an admission to Boston Children's Hospital from January 1, 2007 to June 1, 2017. Patients with bronchiectasis, congenital heart disease, primary pulmonary hypertension, bleeding above the glottis, hemoptysis before 38 weeks of corrected gestational age, hematemesis, foreign body, and trauma were excluded. Patients were also characterized by coagulation status. Primary outcomes were recurrent hemoptysis and death. Univariate analysis was performed to determine ORs for recurrence and death per diagnostic category with infection as the reference category. RESULTS: In total, 257 patients met study criteria and were analyzed. The most common causes of hemoptysis were infection (n = 122), neoplasm (n = 58), and other diagnoses (n = 49). Of the patients with infection, recurrence was 28% and all-cause mortality was 12%. Neoplasm had lower odds of recurrence (OR 0.3, P = .012) but higher odds of mortality (OR 15.8, P < .001). Thrombocytopenia had lower odds of recurrence (OR 0.2, P = .005) but higher odds of mortality (OR 5.9, P < .001). Patients with a tracheostomy had higher odds of recurrence (OR 6.3, P < .001), but lower odds of death (OR 0.4, P = .042). CONCLUSIONS: This study confirms that infection is the most common cause of hemoptysis in patients without severe underlying pulmonary or cardiac disease. Hemoptysis associated with neoplasm and/or thrombocytopenia confers mortality risk. Tracheostomy confers risk of recurrence. Future prospective research on diagnoses associated with hemoptysis is warranted.

RBC Exposure in Pediatric Extracorporeal Membrane Oxygenation: Epidemiology and Factors Associated With Large Blood Transfusion Volume.

OBJECTIVES: To quantify and identify factors associated with large RBC exposure in children supported with extracorporeal membrane oxygenation. DESIGN: Retrospective cohort study. SETTING: Single tertiary care children's hospital. PATIENTS: One-hundred twenty-two children supported with extracorporeal membrane oxygenation for greater than 12 hours during January 1, 2015, to December 31, 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Clinical, laboratory, and survival data were obtained from medical records. Only data from patients' first extracorporeal membrane oxygenation run were used. The primary outcome was RBC volume exposure during extracorporeal membrane oxygenation (mL/kg/d). Patients with RBC exposure volume greater than 75th percentile were categorized as "high RBC use" patients. A "bleeding day" was identified if mediastinum or cannula sites were explored and/or Factor VIIa administration, gastrointestinal, pulmonary, or intracranial hemorrhages occurred. Median age was 0.3 years (interquartile range, 0-3 yr). Congenital heart disease (n = 56; 46%) was the most common diagnosis. Median RBC volume transfused during extracorporeal membrane oxygenation was 39 mL/kg/d (interquartile range, 21-66 mL/kg/d). High RBC use patients were more likely be supported by venoarterial extracorporeal membrane oxygenation (100 vs 76%; p = 0.006), have congenital heart disease (68 vs 39%; p = 0.02), and experience bleeding (33 vs 11% d; p < 0.001). High RBC use patients showed a trend toward higher in-hospital mortality (58 vs 37%; p = 0.07). In the multivariable analysis, younger age (-9% per year; 95% CI, -10% to -7%; p < 0.001), more blood draws per day (+8%; 95% CI, 6-11%; p < 0.001), and higher proportion of bleeding days (+22% per 10% increase; 95% CI, 16-29%; p < 0.001) were associated with larger RBC exposure (model R = 0.66). CONCLUSIONS: Bleeding during extracorporeal membrane oxygenation, frequent laboratory draws, and younger age were associated with increased RBC exposure during extracorporeal membrane oxygenation. Higher transfusion volume was associated with increased mortality.

Cauda Equina Syndrome Secondary to Diffuse Infiltration of the Cauda Equina by Acute Myeloid Leukemia: Case Report and Literature Review.

BACKGROUND: Cauda equina syndrome (CES) results from the dysfunction of the lumbar, sacral, and coccygeal rootlets composing the cauda equina. The underlying etiology is most commonly compression secondary to a large herniated lumbosacral disk; however, any pathology affecting the rootlets can result in the syndrome. METHODS: We present a rare case of CES secondary to neoplastic polyradiculitis in a patient with acute myelogenous leukemia (AML) and review the pertinent literature. A 72-year-old male with a medical history of AML presented with 2 weeks of difficulty ambulating, followed by acute-onset low back pain radiating to the buttocks bilaterally. RESULTS: Imaging of the lumbar spine demonstrated diffuse enhancement and thickening of the cauda equina rootlets. Lumbar puncture showed numerous blasts with monocytoid features consistent with AML, and the patient was diagnosed with polyradiculopathy of the cauda equina secondary to diffuse metastatic infiltration. CONCLUSIONS: Central nervous system involvement of leukemia is poorly understood, even though such lesions are not uncommon in advanced disease. As treatment has improved, many types of leukemia, such as AML, are believed to be curable, and patients with the disease are living longer. With improved survival, it is reasonable to suspect that such involvement by AML may become more common. Our patient is a classic presentation of CES secondary to diffuse infiltration by AML and serves as an example of this rare manifestation of hematologic malignancy.

Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease.

BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. METHODS: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. RESULTS: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ±â€¯0.2 °C vs 34.7 ±â€¯0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. CONCLUSION: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.