A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors’ knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition

Emergent presentation of Langerhans cell histiocytosis in a pediatric patient: Acute cerebellar involvement causing obstructive hydrocephalus requiring posterior fossa decompression

Langerhans cell histiocytosis (LCH) is a disorder of the monocyte-macrophage system that can be unifocal or systemic. Here, we present a pediatric case who initially presented with osseous LCH but again presented 6 years later emergently with cerebellar symptoms, cerebellar mass and obstructive hydrocephalus. Patient underwent biopsy of the cerebellum which was path proven intracranial LCH

Against the overgrowth hypothesis: Shorter costal cartilage lengths in pectus excavatum

Background: Pectus excavatum is a common chest wall deformity with no known cause. A common hypothesis is that in patients with pectus excavatum, there is an overgrowth of costal cartilage relative to healthy individuals.Materials and methods: We obtained radiological curvilinear three-dimensional measurements of the fourth to eighth costal cartilage and associated ribs in 16 patients with pectus excavatum and 16 age- and gender-matched controls between the ages of 6 and 32 y. An analysis of variance was used to compare bone length, cartilage length, and their ratios between patients and controls.Results: Relative to bone length, patients with pectus excavatum overall had shorter costal cartilage lengths (P \u3c 0.001), especially on the left side (P \u3c 0.05). We were unable to localize this observation to specific ribs during post hoc analysis.Conclusions: This is the first study to empirically test the overgrowth hypothesis of pectus excavatum for ribs 4 through 8. Although we and others have found no evidence to support this hypothesis, we surprisingly found the alternate hypothesis to be true: patients with pectus excavatum tend to have shorter costal cartilages. Future studies should expand on these results with larger sample sizes and consider volumetric measurements longitudinally during thoracic development