ABSTRACT
Malignant infantile osteopetrosis is a rare inherited disorder with neurological complications and a shortened life expectancy. Vision loss is typically attributed to osseous compression of the optic nerves at the level of the optic canal. Fundus imaging is reported, as well as the first optical coherence tomography and optical coherence tomography angiography in this rare condition. Imaging revealed optic nerve pallor, subfoveal ellipsoid zone disruption, and an enlarged foveal avascular zone. These results provide insight regarding other potential mechanisms of vision loss in these patients. [Ophthalmic Surg Lasers Imaging Retina 2022; 53:398-402.].
Subject(s)
Osteopetrosis , Tomography, Optical Coherence , Fluorescein Angiography/methods , Fovea Centralis/pathology , Fundus Oculi , Humans , Osteopetrosis/diagnosis , Osteopetrosis/pathology , Tomography, Optical Coherence/methods , Vision Disorders/pathologySubject(s)
Hypertrichosis/chemically induced , Morpholines/adverse effects , Pyrimidines/adverse effects , Pyrroles/adverse effects , Receptor, Fibroblast Growth Factor, Type 2/antagonists & inhibitors , Trichiasis/chemically induced , Adult , Bile Duct Neoplasms/drug therapy , Cholangiocarcinoma/drug therapy , Female , Hair Removal , Humans , Hypertrichosis/therapy , Trichiasis/therapyABSTRACT
Hemorrhage is the main cause of preventable death in both military and civilian trauma, and many of these patients die from non-compressible torso injuries. Resuscitative endovascular balloon occlusion of the aorta (REBOA) is a minimally invasive method used for hemodynamic control of the hemorrhaging patient and has been compared to resuscitative thoracotomy (RT) with cross clamping of the aorta. REBOA has received a great deal of attention in recent years for its applicability and promise in adult trauma and non-trauma settings, but its utility in children is mostly unknown. The purpose of this review article is to summarize and consolidate what is currently known about the use of REBOA in children. Some of the challenges in implementing REBOA in children include small vascular anatomy and lack of outcomes data. Although the evidence is limited, there are established instances in the literature of children and adolescents who have undergone endovascular occlusion of the aorta for hemorrhage control with positive outcomes and survival rates equivalent to their adult counterparts. There is a need for further formal evaluation of REBOA in pediatric patients with prospective studies to look at the safety, feasibility and efficacy of the technique. STUDY TYPE: Narrative Literature Review LEVEL OF EVIDENCE: IV.
Subject(s)
Aorta/surgery , Balloon Occlusion , Hemorrhage/surgery , Resuscitation , Child , Evidence-Based Medicine , HumansABSTRACT
PURPOSE: Management of children with adhesive small bowel obstruction (ASBO) is often based on abdominal radiographs (AXR). Our purpose was to determine the significance of paucity of gas on initial AXR. METHODS: Retrospective, single center review of children with ASBO between 2011 and 2015. Analysis included chi-square, non-parametric tests and multivariate regression. RESULTS: Of 207 cases, 99 were operative. Initial AXR showed paucity of gas in 41% and gaseous loops in 59%. Paucity was more common in operative patients (49% vs. 32%, pâ¯=â¯0.01). At operation, 71% of patients with paucity had closed loop or high-grade obstruction, compared to 29% of patients with gaseous loops (p = <0.001). CONCLUSION: For children with ASBO with paucity of gas on AXR, complicated obstruction (closed loop or high-grade) should be considered. In children with high clinical suspicion of complicated obstruction, additional imaging with CT or SBFT may clarify the clinical picture.