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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 40-41

Sirolimus for multifocal infantile haemangiomas with hepatic & adrenal involvement


1 Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 029, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi 110 029, India

Date of Submission29-Sep-2019
Date of Web Publication25-May-2021

Correspondence Address:
Jhuma Sankar
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_1671_19

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How to cite this article:
Sankar J, Das P. Sirolimus for multifocal infantile haemangiomas with hepatic & adrenal involvement. Indian J Med Res 2020;152, Suppl S1:40-1

How to cite this URL:
Sankar J, Das P. Sirolimus for multifocal infantile haemangiomas with hepatic & adrenal involvement. Indian J Med Res [serial online] 2020 [cited 2021 Sep 27];152, Suppl S1:40-1. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/40/316724

Consent to publish clinical information and images obtained from patient's father.


Multifocal haemangiomas are a rare subtype of infantile haemangiomas (IHs) with multiple complications.

A 40 day old male infant with multifocal IH presented to the Emergency department at the All India Institute of Medical Sciences, New Delhi, India, in January 2019, with eye involvement, liver failure and high-output cardiac failure [Figure 1]A. The child was initially managed with propranolol to which the lesions were refractory. Angiogenesis inhibitor sirolimus was tried with good clinical response. The lesions regressed in size [Figure 1]B and liver functions improved after two weeks of therapy. Unfortunately, the child succumbed to a nosocomial infection at four weeks of intensive care unit stay. Post-mortem examination revealed multifocal haemangiomas in the liver [Figure 2]A and adrenal gland [Figure 2]B. The histopathology confirmed IHs [Figure 3]. This case highlights the challenges faced by the treating team in managing multifocal haemangiomas with hepatic involvement. Sirolimus appears to be a promising therapy in these cases.
Figure 1: (A) Lesion over the lower back before sirolimus therapy. (B) Image taken at three weeks after sirolimus therapy (notice reduced size and fading of the lesion).

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Figure 2: (A) The gross specimen of the liver showing multiple varying sized sub-capsular grey-white lesions with central dark brown areas (arrows) with areas of congestions all over the liver. (B) The gross specimen of the adrenal gland showing similar varying sized grey-white lesions (arrows).

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Figure 3: Histopathology of liver showing intra-hepatic vascular tumour (arrows), comprising cleft-like areas, lined with endothelial cells. A few entrapped benign bile ducts were seen within the lesion (A, ×100). The endothelial cells (arrows) lining the vascular spaces did not show atypia (B, ×200) and were positive for CD34 stain (arrows) (C, ×200). Similar vascular lesion (black arrows) was also identified inside the left adrenal gland (blue arrows) (D, ×40), and the lesional cells (black arrows) were positive for CD34 stain (E, ×40). In skin also, similar lesions were identified (F, ×100).

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Acknowledgment: Authors acknowledge the contributions of Drs Viswanath, Sagar Tugal and Rakesh Lodha from the department of Pediatrics and Dr Hena Khandakar from the department of Pathology in the case management and for providing images.

Conflicts of Interest: None


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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