Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation
Venous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous m...
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| Format: | Article |
| Language: | English |
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Georg Thieme Verlag KG
2020-01-01
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| Series: | European Journal of Pediatric Surgery Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0040-1716895 |
| _version_ | 1818607608926306304 |
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| author | Mohamed Aly Abdelbaky Iman Ahmed Ragab Amr AbdelHamid AbouZeid Shaimaa Abdelsattar Mohammad Mohamed Moussa Dahab Mohammed Elsherbeny Hatem Abdelkader Safaan |
| author_facet | Mohamed Aly Abdelbaky Iman Ahmed Ragab Amr AbdelHamid AbouZeid Shaimaa Abdelsattar Mohammad Mohamed Moussa Dahab Mohammed Elsherbeny Hatem Abdelkader Safaan |
| author_sort | Mohamed Aly Abdelbaky |
| collection | DOAJ |
| description | Venous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous malformation in the pelvis of a 21-month-old boy who presented with life-threatening complications. With a history dating since the day 2 of life, the patient suffered from chronic bleeding due to scrotal skin ulcerations, in addition to recurrent attacks of severe bleeding per rectum necessitating hospital admission and blood transfusion (three attacks since the age of 7 months). Pelvic magnetic resonance image showed the typical findings of extensive venous malformation involving the pelvis, perineum, scrotum, and extending to the gluteal region. The lesion was seen totally encasing the anorectum with marked thickening of their walls almost occluding their lumen. Oral sirolimus (2 mg/m2) was started with a target blood trough level of 5 to 10 ng/mL. Over a follow-up period of 5 months, there was obvious clinical improvement that included healing of skin lesions (scrotal ulcer) with complete re-epithelialization, absence of bleeding per rectum with improvement of constipation, and rise of hemoglobin level from 7.5 to 11.5 g/dL. |
| first_indexed | 2024-12-16T14:29:28Z |
| format | Article |
| id | doaj.art-3f8eade24bbf44dba08a6bc44f36c011 |
| institution | Directory Open Access Journal |
| issn | 2194-7619 2194-7627 |
| language | English |
| last_indexed | 2024-12-16T14:29:28Z |
| publishDate | 2020-01-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | European Journal of Pediatric Surgery Reports |
| spelling | doaj.art-3f8eade24bbf44dba08a6bc44f36c0112022-12-21T22:28:16ZengGeorg Thieme Verlag KGEuropean Journal of Pediatric Surgery Reports2194-76192194-76272020-01-010801e90e9410.1055/s-0040-1716895Sirolimus: A Rescue Drug to Control Complications of Extensive Venous MalformationMohamed Aly Abdelbaky0Iman Ahmed Ragab1Amr AbdelHamid AbouZeid2Shaimaa Abdelsattar Mohammad3Mohamed Moussa Dahab4Mohammed Elsherbeny5Hatem Abdelkader Safaan6Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, EgyptDepartment of Pediatrics, Ain Shams University, Faculty of Medicine, Cairo, EgyptDepartment of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, EgyptDepartment of Radiodiagnosis, Ain Shams University Faculty of Medicine, Cairo, EgyptDepartment of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, EgyptDepartment of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, EgyptDepartment of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, EgyptVenous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous malformation in the pelvis of a 21-month-old boy who presented with life-threatening complications. With a history dating since the day 2 of life, the patient suffered from chronic bleeding due to scrotal skin ulcerations, in addition to recurrent attacks of severe bleeding per rectum necessitating hospital admission and blood transfusion (three attacks since the age of 7 months). Pelvic magnetic resonance image showed the typical findings of extensive venous malformation involving the pelvis, perineum, scrotum, and extending to the gluteal region. The lesion was seen totally encasing the anorectum with marked thickening of their walls almost occluding their lumen. Oral sirolimus (2 mg/m2) was started with a target blood trough level of 5 to 10 ng/mL. Over a follow-up period of 5 months, there was obvious clinical improvement that included healing of skin lesions (scrotal ulcer) with complete re-epithelialization, absence of bleeding per rectum with improvement of constipation, and rise of hemoglobin level from 7.5 to 11.5 g/dL.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0040-1716895multidisciplinary teamvascular anomaliesvascular malformationsvenous malformations |
| spellingShingle | Mohamed Aly Abdelbaky Iman Ahmed Ragab Amr AbdelHamid AbouZeid Shaimaa Abdelsattar Mohammad Mohamed Moussa Dahab Mohammed Elsherbeny Hatem Abdelkader Safaan Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation European Journal of Pediatric Surgery Reports multidisciplinary team vascular anomalies vascular malformations venous malformations |
| title | Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation |
| title_full | Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation |
| title_fullStr | Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation |
| title_full_unstemmed | Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation |
| title_short | Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation |
| title_sort | sirolimus a rescue drug to control complications of extensive venous malformation |
| topic | multidisciplinary team vascular anomalies vascular malformations venous malformations |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0040-1716895 |
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