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[Bean's syndrome in children: about two cases]
Achraf El Bakkaly1, Fouad Ettayebi1, Houda Oubeja1
1Service des Urgences Chirurgicales Pédiatriques, CHU Ibn Sina, Faculté de Médecine Mohammed V, Rabat, Maroc.
Insights
Diffuse angiomatosis (Bean's syndrome) involves venous malformations in the skin and digestive tract, causing severe bleeding. Surgical intervention effectively controlled bleeding and reduced transfusion needs in two pediatric cases.
Area of Science:
- Vascular Malformations
- Pediatric Surgery
- Gastroenterology
Background:
- Diffuse angiomatosis (Bean's syndrome) is a rare condition characterized by venous malformations affecting the skin and gastrointestinal tract.
- These malformations can lead to significant hemorrhage, anemia, and the need for frequent blood transfusions.
Observation:
- This report details two pediatric cases of diffuse angiomatosis treated surgically.
- Diagnosis involved rectal bleeding, melena, severe anemia, and skin angiomas.
- Radiological findings revealed small intestinal angiomatosis in one patient, while the other had no abdominal lesions.
Findings:
- Surgical exploration identified actively bleeding angiomas.
- Enterotomy was performed, leading to the cessation of bleeding post-operatively.
- Surgical treatment demonstrated efficacy in managing complications.
Implications:
- Surgical intervention is a beneficial approach for controlling hemorrhage in diffuse angiomatosis.
- Effective management can reduce the frequency of blood transfusions required for patients with this rare condition.
Abstract:
Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Radiological evaluation showed the presence of multiple lesions at the level of the jejunum and ileum consistent with small intestinal angiomatosis in the child aged 9 and a half years. It didn't show abdominal lesions in the child aged 5 years. The two patients were admitted to the operating block. Angiomas were surgically individualized. Some of them were actively bleeding. Enterotomy was performed. Postoperative sequelae was marked by the stop of the bleedings. This study aims to update the current understanding of this rare pathology as well as the benefit of surgical treatment in controlling the complications caused by this pathology and in reducing the frequency of transfusions.