More than Meets the Eye: Orbital Swelling in an Adolescent with Sickle Cell Disease.

Periorbital swelling is a clinical presentation with a broad differential and potentially deleterious consequence. Causes range from benign, including allergic reaction, to vision- and life-threatening, including orbital cellulitis and orbital infarction. The recent climate of SARS-CoV-2 has further complicated this differential, as the virus poses broad clinical presentations with new manifestations reported frequently. Rapid identification of the underlying etiology is crucial, as treatment approaches diverge greatly. Here, we report the case of an African American adolescent male with a history of homozygous sickle cell anemia presenting to an inner city hospital with bilateral periorbital swelling amid the coronavirus pandemic. Differentials, including orbital cellulitis, COVID-MIS-C, orbital inflammatory syndrome, Hoagland sign, and orbital infarction secondary to sickle cell crisis are contrasted. We contrast our case with 12 case reports of orbital infarction in the setting of sickle cell crisis within the past 10 years, highlighting how these presentations, along with commonly reported findings of orbital infarction, compare with our patient.

Complementary and alternative medicine for children with sickle cell disease: A systematic review.

BACKGROUND: Complementary and alternative medicine (CAM) is a popular alternative to opioid and other analgesics in sickle cell disease (SCD). We review the effectiveness, prevalence, and factors associated with CAM use in the pediatric SCD population. METHODS: The review protocol was created based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A literature search was conducted in MEDLINE, Embase, Cochrane Library, PubMed, and Web of Science. RESULTS: Twenty-four studies were examined. The prevalence of CAM use in pediatric patients with SCD ranged from 36 to 84.5%. Common inpatient CAM interventions were yoga, virtual reality, and acupuncture, which decreased pain scale scores. Outpatient CAMs were consisted of cognitive behavioral therapy, massage therapy, and guided-imagery, which increased pain tolerability and decreased pain scale scores. CONCLUSIONS: CAM modalities can decrease pain scale scores. However, the impact of specific CAM modalities on emergency department visits, hospitalizations, and school absences were inconclusive.

Congenital Methemoglobinemia and Unstable Hemoglobin Variant in a Child With Cyanosis.

Methemoglobinemia (Meth) is a rare hemoglobin (Hb) disorder with distinguished clinical features and complex pathophysiology. We present a three-year-old female who was diagnosed with congenital methemoglobinemia when she presented with peri-oral cyanosis and profound oxygen desaturation in the 20-30% range. This patient also had elevated deoxyhemoglobin (HHb) not explained by methemoglobinemia alone; the low pulse oximetry (SpO2) reading suggested a rightward-shift oxyhemoglobin (O2Hb) dissociation curve, which is the opposite of that expected in methemoglobinemia. This, along with evidence of hemolysis, raised the possibility of a concomitant low-oxygen affinity hemoglobinopathy, which could explain elevated HHb. Hemoglobin electrophoresis identified an abnormal hemoglobin variant which was categorized as heterozygous for unstable beta globin variant. The patient responded well to one dose of methylene blue, vitamin C, supportive oxygen (O2) therapy, and IV hydration and was discharged with a baseline of 50-60% SpO2 on room air. We are reporting this case along with a brief review of the medical literature.