Perinatal encephalopathy, the syndrome of intracranial hypertension and associated diagnostic labels in the Commonwealth of Independent States: A systematic review

Abstract

Background The WHO reports excessive rates of ill-defined neurological diagnoses and ineffective and potentially harmful drug treatments in children in the Commonwealth of Independent States (CIS). Collectively termed perinatal encephalopathy and the syndrome of intracranial hypertension (PE-SIH), these diagnoses are important contributors to perceived childhood morbidity and disability in the CIS. A systematic compilation of information on PE-SIH is lacking. Methods We systematically reviewed publications between 1970 and 2020 on PE-SIH in Azerbaijani, English, Russian and Ukrainian languages and summarised information on PE-SIH. Results We identified 30 publications (70% in Russian) published 1976-2017. The diagnosis of PE-SIH was either based on unreported criteria (67% of reports), non-specific clinical features of typically developing children or those with common developmental disorders (20% of reports) or cranial ultrasound (13% of reports). The reported proportion of children with PE-SIH in the study samples ranged from 31% to 99%. There were few published studies on reassessments of children diagnosed with PE-SIH, and these did not confirm neurological disease in the majority of children. Treatments included multiple unlicenced drugs without established effectiveness and with potential unwanted effects. Conclusion This review suggests that PE-SIH is a medical diagnostic label that is used in numerous children without substantive associated disease. The diagnosis and treatment of PE-SIH is a multidimensional, iatrogenic, clinical and public health problem in the CIS. With increasing use of evidence-based medicine guidelines in the region, it is hoped that PE-SIH will gradually disappear, but actions to accelerate this change are nevertheless needed.