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

Arch Dis Child. 2020 Oct;105(10):921-926. doi: 10.1136/archdischild-2018-315994. Epub 2020 Jun 12.

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.

Keywords: Commonwealth of Independent States; overdiagnosis; overmedicalisation; perinatal encephalopathy; syndrome of intracranial hypertension.

Publication types

  • Systematic Review

MeSH terms

  • Anticonvulsants / therapeutic use
  • Brain / diagnostic imaging
  • Brain Diseases / diagnosis*
  • Brain Diseases / therapy*
  • Commonwealth of Independent States
  • Dietary Supplements
  • Diuretics / therapeutic use
  • Humans
  • Infant
  • Intracranial Hypertension / diagnosis
  • Intracranial Hypertension / therapy
  • Nootropic Agents / therapeutic use
  • Vasoconstrictor Agents / therapeutic use

Substances

  • Anticonvulsants
  • Diuretics
  • Nootropic Agents
  • Vasoconstrictor Agents