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

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,...

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Autores principales: Ghanam, Ayad, Elouali, Aziza, Nour, Merouane, Rkain, Maria, Benajiba, Noufissa, Babakhouya, Abdeladim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9155935/
https://www.ncbi.nlm.nih.gov/pubmed/35656331
http://dx.doi.org/10.1155/2022/8245139
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author Ghanam, Ayad
Elouali, Aziza
Nour, Merouane
Rkain, Maria
Benajiba, Noufissa
Babakhouya, Abdeladim
author_facet Ghanam, Ayad
Elouali, Aziza
Nour, Merouane
Rkain, Maria
Benajiba, Noufissa
Babakhouya, Abdeladim
author_sort Ghanam, Ayad
collection PubMed
description 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/m(2), 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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spelling pubmed-91559352022-06-01 Bean Syndrome in a Child Treated with Sirolimus: About a Case Ghanam, Ayad Elouali, Aziza Nour, Merouane Rkain, Maria Benajiba, Noufissa Babakhouya, Abdeladim Case Rep Pediatr Case Report 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/m(2), 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. Hindawi 2022-05-24 /pmc/articles/PMC9155935/ /pubmed/35656331 http://dx.doi.org/10.1155/2022/8245139 Text en Copyright © 2022 Ayad Ghanam et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ghanam, Ayad
Elouali, Aziza
Nour, Merouane
Rkain, Maria
Benajiba, Noufissa
Babakhouya, Abdeladim
Bean Syndrome in a Child Treated with Sirolimus: About a Case
title Bean Syndrome in a Child Treated with Sirolimus: About a Case
title_full Bean Syndrome in a Child Treated with Sirolimus: About a Case
title_fullStr Bean Syndrome in a Child Treated with Sirolimus: About a Case
title_full_unstemmed Bean Syndrome in a Child Treated with Sirolimus: About a Case
title_short Bean Syndrome in a Child Treated with Sirolimus: About a Case
title_sort bean syndrome in a child treated with sirolimus: about a case
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9155935/
https://www.ncbi.nlm.nih.gov/pubmed/35656331
http://dx.doi.org/10.1155/2022/8245139
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