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CC BY 4.0 · European J Pediatr Surg Rep. 2020; 08(01): e90-e94
DOI: 10.1055/s-0040-1716895
Case Report

Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation

Mohamed Aly Abdelbaky
1   Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt
,
Iman Ahmed Ragab
2   Department of Pediatrics, Ain Shams University, Faculty of Medicine, Cairo, Egypt
,
Amr AbdelHamid AbouZeid
1   Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt
,
Shaimaa Abdelsattar Mohammad
3   Department of Radiodiagnosis, Ain Shams University Faculty of Medicine, Cairo, Egypt
,
Mohamed Moussa Dahab
1   Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt
,
Mohammed Elsherbeny
1   Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt
,
Hatem Abdelkader Safaan
1   Department of Pediatric Surgery, Ain Shams University Faculty of Medicine, Cairo, Egypt
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Abstract

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.

Keywords

multidisciplinary team - vascular anomalies - vascular malformations - venous malformations


Publication History

Received: 28 April 2020

Accepted: 18 August 2020

Article published online:
15 December 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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