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Encephaloceles: a review 1971-1990.
J G Docherty1, J C Daly, R Carachi
1University Department of Paediatric Surgery, Royal Hospital for Sick Children, Yorkhill, Glasgow, U.K.
Summary
Occipital encephalocele management improved significantly over two decades. Early intervention and treatment reduced mortality and improved outcomes for affected infants, highlighting advancements in pediatric neurosurgery.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neurology
Background:
- Occipital encephalocele is a severe congenital neural tube defect.
- Management strategies and outcomes for this condition have evolved over time.
Purpose of the Study:
- To review the management and outcomes of occipital encephalocele patients treated between 1971 and 1990.
- To identify trends in hydrocephalus development and mortality.
Main Methods:
- Retrospective case note review of 51 patients with occipital encephalocele.
- Analysis of patient demographics, hydrocephalus development, surgical interventions (shunting), and mortality rates.
- Follow-up via postal questionnaire to general practitioners for neurological deficits.
Main Results:
- Hydrocephalus developed in 57% of patients, with 18 requiring shunting.
- The overall one-year mortality rate was 23%, with a notable decrease in the second decade (1 death).
- Of 23 patients followed up, 14 had no obvious deficit, and 9 had combined deficits; only one death occurred after one year in a severely handicapped child.
Conclusions:
- Management of occipital encephalocele has seen significant improvements, particularly in reducing mortality.
- Early identification and intervention, including hydrocephalus management, are crucial for better patient outcomes.
- Long-term neurological deficits can vary, but many patients achieve good functional recovery.