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Case Reports
. 2022 May 24:2022:8245139.
doi: 10.1155/2022/8245139. eCollection 2022.

Bean Syndrome in a Child Treated with Sirolimus: About a Case

Ayad Ghanam  1   2 , Aziza Elouali  1   2 , Merouane Nour  2   3 , Maria Rkain  1   2 , Noufissa Benajiba  1   2 , Abdeladim Babakhouya  1   2
Affiliations
Case Reports

Bean Syndrome in a Child Treated with Sirolimus: About a Case

Ayad Ghanam et al. Case Rep Pediatr. .

Abstract

Bean syndrome (BS) or blue rubber bleb nevus syndrome is a rare clinical entity characterized by venous malformations mainly in the skin and digestive tract, whose hemorrhagic complications can be life threatening. We report a case of Bean syndrome in a 3-year-old child of nonconsanguineous parents, in whom the diagnosis of miliary hemangiomatosis was initially made in view of a huge mass on the left thigh, taking the knee, and then the progressive appearance of a skin disorder with bluish swellings of variable sizes spread over the whole body. The patient was put on beta-blockers but without improvement. The evolution was marked by an increase in the volume of the thigh mass. Ultrasound exploration coupled with Doppler imaging revealed the presence of angiomas in the thigh, requiring emergency surgery following a large hemorrhage. The patient underwent sclerotherapy. At the age of 18 months, the child returned with severe anemia and melena. The abdominal CT scan showed gallbladder intussusception secondary to an angioma requiring intestinal resection for hemostasis. At the age of three years, the angiomas worsened with an increase in volume, particularly on the face. The association of the cutaneous and digestive involvement of these venous malformations made us rectify the diagnosis. The patient was put on sirolimus (rapamycin), 2 mg/m2, with good evolution with a delay of 18 months; the patient presents no more episodes of bleeding with regression of the size of cutaneous angiomas. This observation underlines that BS is difficult to diagnose because of its low frequency, that sirolimus was effective and well tolerated in our patient, and that it can be suggested as a good and safe therapeutic option.

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Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
Huge cutaneous angioma of the left thigh taking the left knee at the age of 6 months.
Figure 2
Figure 2
Diffuse cutaneous angiomas on the whole body.
Figure 3
Figure 3
Diffuse cutaneous angiomas on the whole body (a) and cutaneous angiomas on the face (b), on the upper limbs (c), and on the lower limbs (d).
See this image and copyright information in PMC

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