Cyanosis is a physical finding that can occur at any age but presents the greatest challenge when it occurs in the newborn. The cause is multiple, and it usually represents an ominous sign, especially when it occurs in association with neonatal sepsis, cyanotic congenital heart disease, and airway abnormalities. Cyanosis caused by abnormal forms of hemoglobin can also be life-threatening, and early recognition is mandatory to prevent unnecessary investigations and delay in management. Abnormal hemoglobin, such as hemoglobin M, is traditionally discovered by electrophoresis, so the newborn screen, which is mandatory in several states, is a useful tool for the diagnosis. Although acquired methemoglobinemia, caused by environmental oxidizing agents, is common, congenital deficiency of the innate reducing enzyme is so rare that only a few cases are documented in the medical literature around the world. We present a neonate with cyanosis as a result of congenital deficiency of the reduced nicotinamide adenine dinucleotide-cytochrome b5 reductase enzyme. This infant was found to be blue at a routine newborn follow-up visit. Sepsis, structural congenital heart disease, prenatal administration, and ingestion of oxidant dyes were excluded as a cause of the cyanosis by history and appropriate tests. Chocolate discoloration of arterial blood provided a clue to the diagnosis. A normal newborn screen and hemoglobin electrophoresis made the diagnosis of hemoglobin M unlikely as the cause of the methemoglobinemia (Hb A 59.4%, A2 1.8%, and F 38.8%). Red blood cell enzyme activity and DNA analysis revealed a homozygous form of the cytochrome b5 reductase enzyme deficiency. He responded very well to daily methylene blue and ascorbic acid administration, and he has normal growth and developmental parameters, although he shows an exaggerated increase in his methemoglobin level with minor oxidant stress such as diarrhea.

Prehospital care of children is suboptimal compared with adults in areas of endotracheal intubation, establishment of peripheral intravenous access, and fluid resuscitation.

ImportanceReports of toxic leukoencephalopathy (TLE) due to opioids have been extensively documented within the adult literature. There is a paucity of literature with respect to the incidence, complications, and outcomes of TLE in the pediatric population.ObjectiveTo describe a rare complication of opioid ingestion in the pediatric population and serve as the first large review of published cases of opioid-induced leukoencephalopathy. Thirteen case reports with varying treatments are herein reviewed in addition to our own case. The range of treatment modalities, morbidity and mortality are broad and outcomes secondary to supportive care versus neurosurgical intervention is explored.Evidence reviewAll cases of pediatric opioid-induced toxic leukoencephalopathy published on pubmed and google scholar were included in this review.FindingsWe report the case of a 4-year old male surgically treated for acute oxycodone-induced TLE who initially presented with Glascow Coma Scale of 4 and a comatose state for weeks. Over the next several months he recovered with spasticity of all extremities, oral aversion, substantial vision loss, and the ability to speak in short sentences. In addition, we found thirteen other reported cases of opioid-induced leukoencephalopathy reported in the literature. The treatment approaches described range from supportive care alone, to invasive neurosurgical interventions including placement of extraventricular drains, removal of hemorrhagic tissue, and craniectomy. The outcomes of patients with opioid-induced leukoencephalopathy is also variable. Reports demonstrate a range of outcomes that include patients who died to those with no residual neurologic deficits.ConclusionsThis review of reported pediatric cases of opioid-induced leukoencephalopathy highlights the importance of early neurosurgical intervention for prevention of devastating outcomes.

The authors observed the effect of drotrecogin alfa (activated) in a case of pediatric severe sepsis. A 4-month-old male infant with Serratia marcescens septic shock, multiple organ dysfunction syndrome (MODS), and consumptive coagulopathy was admitted. The safety and efficacy of drotrecogin alfa (activated) has not yet been established for patients younger than 18 years of age. This is the first published report of the use of drotrecogin alfa (activated) in an infant with severe sepsis. Within 6 hours of starting therapy, there was a significant improvement in hemodynamics, which was not maintained after the drotrecogin alfa (activated) infusion was temporarily discontinued. No significant bleeding complications occurred during the infusion. A brain MRI on day 22 after drotrecogin alfa (activated) infusion showed bilateral small occipital hemorrhages. Drotrecogin alfa (activated) in this infant was temporally related to significant improvement. It is unknown whether the MRI brain lesions are related to severe sepsis with disseminated intravascular coagulation or drotrecogin alfa (activated) infusion. The authors believe that drotrecogin alfa (activated) should be considered in select children with life-threatening severe sepsis.

Here we describe a case of propofol-related infusion syndrome (PRIS) in a child with malignant refractory status epilepticus treated with partial-exchange blood transfusion (PEBT), an innovative method of resuscitation that has the potential to reduce the mortality rate associated with this syndrome. Our patient is a 4-year-old boy with malignant status epilepticus associated with bacterial meningitis. Propofol was used because of persistent seizure activity refractory to adequate doses of phenytoin, phenobarbital, levetiracetam, and midazolam infusion at 0.7 mg/kg per hour. Propofol was escalated from 0.6 mg/kg per hour to an electroencephalogram-burst-suppressing dose of 15.6 mg/kg per hour. Signs of PRIS were noticed after 48 hours on propofol. The severe bradycardia responded only to infusions of calcium gluconate. PEBT corrected all the cardiac abnormalities and returned enough hemodynamic stability to permit continuous veno-venous hemodialysis for renal failure and removal of toxins. PEBT is a safe and innovative option for correcting the metabolic abnormalities that result in cardiac dysfunction, which is typically the most serious and usually terminal event in PRIS. When done with small aliquots, it avoids the severe hemodynamic instability that is usually a hindrance with hemodialysis, continuous veno-venous hemodialysis, and extracorporeal membrane oxygenation, which are other methods of supporting these children during the crisis that are mentioned in the literature.

Aplasia cutis congenita (ACC) is the clinical manifestation of an uncommon group of skin disorders. One postulated etiology is disseminated intravascular coagulation from release of thrombogenic maternal arising from placental injury or fetal demise. This leads to disruption of the ectodermal blood supply responsible for the skin defects. We present a neonate with group V ACC, one of an initial triplet gestation, associated with fetal demise at 14 weeks and formation of a fetus papyraceus. The practice of selective fetal reduction as a result of multiple gestation seen with the use of fertility drugs may in theory increase the incidence of group V ACC.

Objective, To survey the pediatric trauma programs to ascertain if and how etomidate is being used for rapid sequence intubation (RSI) in pediatric trauma patients. Design, A 25 question survey was created using REDCaps. A link to the survey was emailed to each of the pediatric and adult trauma programs that care for pediatric patients. Setting, Pediatric trauma programs and adult trauma programs caring for pediatric patients. Intervention, None. Measurements and Main Results, A total of 16% of programs responded (40/247). The majority of the centers that responded are urban, academic, teaching Level 1 pediatric trauma centers that provide care for > 200 pediatric trauma patients annually.  The trauma program directors were the most likely to respond to the survey (18/40).  33/38 respondents state they use etomidate in their RSI protocol but it is not used in all pediatric trauma patients.  26/38 respondents believe that etomidate is associated with adrenal suppression and 24/37 believe it exacerbates adrenal suppression in pediatric trauma patients yet 28 of 37 respondents do not believe it is clinically relevant. Conclusions, Based on the results of the survey, the use of etomidate in pediatric trauma patients is common among urban, academic, teaching, level 1 pediatric trauma centers.  A prospective evaluation of etomidate use for RSI in pediatric trauma patients to evaluate is potential effects on adrenal suppression and hemodynamics is warranted.

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