O-169 - PHENOTYPIC EXPRESSION OF MEDIAL DYSPLASIA AS ARTERIAL ANEURYSMS OF INFANCY

TOPIC:
Case Reports
AUTHORS:
Gupta P. (Care Hospital ~ Hyderabad ~ India) , Kota P. (Care Hospital ~ Hyderabad ~ India) , Atturu G. (Care Hospital ~ Hyderabad ~ India) , Agarwal R. (Care Hospital ~ Hyderabad ~ India) , Atreyapurapu V. (Care Hospital ~ Hyderabad ~ India) , Sharma P. (Care Hospital ~ Hyderabad ~ India)
Introduction:
Pediatric arterial aneurysms are extremely rare, with even fewer presentations in infancy. Natural history, management guidelines and follow up protocols of these distinctive pathologies remain ill-defined even today. Risk of expansion, rupture, thrombosis, distal embolization and local soft tissue compression dictate the need for intervention. Needless to say, parents are anxious and need further answers besides the treatment for the current problem. We present our experience with two infants, diagnosed to have solitary peripheral arterial aneurysms. We discuss presentation, evaluation, treatment, pathological findings and follow up.
Methods:
We saw two male infants (2 months old and 1 year old) with asymptomatic solitary aneurysms of the right facial artery (2 x 1.5 cms) and the right radial artery (1.5 x1.5 cms) respectively. They presented as progressively increasing pulsatile swellings with no other local or systemic symptoms and signs. Both patients underwent clinical evaluation and duplex scanning. CT angiography was done for the infant with facial artery aneurysm. Intracranial aneurysms were also ruled out. Anticipating further expansion and future complications, they were offered surgical intervention. Both aneurysms were excised. Radial artery was primarily reconstructed with end-to-end anastomosis.
Results:
Both infants recovered well after the surgery. There was no post-operative morbidity. No predisposing factors such as, family history, prior trauma or history of intervention could be elicited. There was no elevation in inflammatory markers on biochemical studies. Histopathological examination showed medial dysplasia in both specimens. Post-operative follow up over the course of two years showed preserved patency of radial artery with no recurrence or evidence of novel aneurysm development in either child.
Conclusion:
Our report demonstrates the successful management of a challenging arterial pathology in infancy. We emphasize the role of Medial Dysplasia (Type VII) as a contender for non-renal pediatric arterial aneurysms. Lifelong follow up will add to the understanding of the natural history of this uncommon affliction in infants and children.
References:
1. Sarkar R, Coran AG, Cilley RE, Lindenauer SM, Stanley JC. Arterial aneurysms in children: clinicopathologic classification. J Vasc Surg. 1991;13(1):47-57. 2. Davis FM, Eliason JL, Ganesh SK, Blatt NB, Stanley JC, Coleman DM. Pediatric nonaortic arterial aneurysms. J Vasc Surg. 2016;63(2):466-76.e1.