RESUMO
OBJECTIVE: To develop consensus on diagnostic criteria for LUMBAR syndrome, the association of segmental infantile hemangiomas that affect the Lower body with Urogenital anomalies, Ulceration, spinal cord Malformations, Bony defects, Anorectal malformations, Arterial anomalies and/or Renal anomalies. STUDY DESIGN: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists. RESULTS: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement. 98% of panelists agreed with the diagnostic criteria, and 100% agreed the criteria would be useful in clinical practice. The diagnosis of LUMBAR requires the presence of a segmental, or patterned, infantile hemangioma of the lumbosacral, sacrococcygeal, or pelvic cutaneous regions plus one additional criterion of the urogenital, spinal, bony, anorectal, arterial, or renal organ systems. CONCLUSIONS: These diagnostic criteria will enhance clinical care by improving screening, detection, and overall awareness of this poorly understood neurocutaneous disorder. The criteria can be utilized by a wide variety of pediatric subspecialists. In addition, formal criteria will improve phenotypic uniformity among LUMBAR syndrome cohorts and a patient registry, allowing investigators to assess clinical features, long-term outcomes, and results of genetic sequencing in a standardized manner. Finally, these criteria will serve as a starting point for prospective studies to establish formal screening and management guidelines.
RESUMO
Importance: The maternal risk of strokes in the United States is approximately 30/100,000 pregnancies, and strokes are the eighth leading cause of maternal death. Because of the relationship between stroke and significant neurological disability/maternal death, obstetrical health care providers must be able to identify, evaluate, diagnosis, and treat these women. Evidence Acquisition: PubMed was searched using the search terms "stroke" OR "cerebrovascular accident" OR "intracranial hemorrhage" AND "pregnancy complications" OR "risk factors" OR "management" OR "outcome." The search was limited to the English language and was restricted to articles from 2000 to 2020. Results: There were 319 abstracts identified, and 90 of the articles were ultimately used as the basis of this review. Presenting stroke signs and symptoms include headache, composite neurologic defects, seizures, and/or visual changes. Diagnosis is typically made with computed tomography scan using abdominal shielding or magnetic resonance imaging without contrast. Management options for an ischemic stroke include reperfusion therapy with intravenous recombinant tissue plasminogen activator catheter-based thrombolysis and/or mechanical thrombectomy. Hemorrhagic strokes are treated similarly to strokes outside of pregnancy, and that treatment is based on the severity and location of the hemorrhage. Conclusions and Relevance: Early recognition and management are integral in decreasing the morbidity and mortality associated with a stroke in pregnancy. Relevance Statement: This study was an evidence-based review of stroke in pregnancy and how to diagnose and mange a pregnancy complicated by a stroke.