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Dieulafoy lesions and PHACE syndrome
Lukas Kieswetter1, Thomas D Walters2, Irene Lara-Corrales3
1Department of Paediatrics, McMaster University, Hamilton, Ontario, Canada.
Pediatric Dermatology
|July 31, 2019
Summary
Dieulafoy
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Vascular Malformations
Background:
- Dieulafoy's lesions (DL) are rare causes of gastrointestinal bleeding, typically seen in adults.
- Pediatric DLs are exceptionally uncommon, presenting diagnostic and therapeutic challenges.
Observation:
- A 9-year-old girl with PHACE syndrome (posterior fossa brain malformations, hemangiomas, arterial lesions, cardiac abnormalities, eye abnormalities) presented with jejunal DLs.
- The patient experienced significant gastrointestinal bleeding attributed to these multiple jejunal lesions.
Findings:
- Multiple jejunal Dieulafoy's lesions were identified as the source of bleeding.
- Rapamycin was utilized for the treatment of pediatric DLs, marking its first described use in this context.
Implications:
- This case highlights the rare occurrence of DLs in children, particularly those with complex syndromes like PHACE.
- The successful use of rapamycin suggests a potential novel therapeutic avenue for pediatric DLs, warranting further investigation.
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