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Management and follow-up of harlequin siblings
R S Prasad1, R K Pejaver, A Hassan
1Department of Paediatrics, North West Armed Forces Hospital, Tabuk, Saudi Arabia.
Insights
Harlequin fetus is a rare condition where infants rarely survive past one year. Intensive multidisciplinary care can extend survival beyond the neonatal period for affected siblings.
Area of Science:
- Dermatology
- Neonatology
- Genetics
Background:
- Harlequin ichthyosis is a severe congenital ichthyosis with extremely low survival rates.
- Affected infants present with generalized thickening of the stratum corneum, leading to fissuring and ectropion.
Observation:
- Two successive siblings diagnosed with harlequin fetus were managed.
- The management involved intensive skin and eye care, fluid and electrolyte monitoring, parental support, and infection surveillance.
Findings:
- A multidisciplinary approach was crucial for managing the condition.
- The case highlights the possibility of prolonged survival beyond the neonatal period with appropriate interventions.
Implications:
- This case report suggests that intensive, coordinated care can improve outcomes for harlequin fetus.
- Further research into the genetic and management aspects of harlequin ichthyosis is warranted.
Abstract:
Harlequin fetus is a rare clinical entity, and survival of affected infants beyond the first year of life is uncommon. Management involves intensive care of the skin and eyes, close monitoring of fluid and electrolyte status, constant support and counselling of parents, and surveillance against infection and side-effects of medication. A well-coordinated multidisciplinary approach can prolong survival beyond the neonatal period. We report our experiences in the management and follow-up of two successive harlequin siblings.