Arterial tortuosity syndrome (ATS) is an autosomal-recessive genetic connective tissue disorder that affects the aorta and medium-sized arterial vessels.1 We report a case of successful surgical management of the descending aorta in a ten-day-old patient with ATS who presented with multiorganic failure. The aortic reconstruction with a pulmonary homograft patch was performed using full-flow body perfusion.
Get full access to this article
View all access options for this article.
References
1.
Al-BlushiSBantanNAAAl-AbdullatifSTaherMM.
Arterial tortuosity syndrome in a newborn: a case report with literature review. Cureus.
2022;
14(
12):
e32899. doi:https://doi.org/10.7759/cureus.c86
2.
EkhatorCDeviMBarkerC, et al.Arterial tortuosity syndrome: unraveling a rare vascular disorder. Cureus.
2023;
15(
9):
e44906. doi:https://doi.org/10.7759/cureus.44906
3.
PichlerKRalserEReschM, et al.Newborn with arterial tortuosity syndrome: the importance of timely diagnostic work-up in patients presenting with Cutis Laxa. J Funct Morphol Kinesiol.
2016;
1(
2):
249–
253. doi:https://doi.org/10.3390/jfmk1020249
4.
WesselsMWCatsman-BerrevoetsCEManciniGM, et al.Three new families with arterial tortuosity syndrome. Am J Med. Genet. A.
2004;
131(
2):
134–
143.
5.
Dodge-KhatamiJSimpsonSADodge-KhatamiA.
A severe form of arterial tortuosity syndrome presenting with significant airway obstruction in an infant. World J Pediatr Congenit Heart Surg.
2020;
11(
2):
238–
240.
6.
KulyabinYYGorbatykhYNSoynovIANichayNRZubritskiyAVBogachev-ProkophievAV.
Double arterial cannulation in the critical management of neonatal aortic arch obstruction with closed ductus arteriosus. World J Pediatr Congenit Heart Surg.
2019;
10(
1):
105–
108.
7.
OztasDMMericMBeyazMO, et al.Off-pump ascending aorta or aortic arch to descending aorta bypass with a pericardial roll for the treatment of critically ill infants with interrupted aortic arch. Cardiol Young.
2020;
30(
8):
1095–
1102.
8.
van BeynumIMKurulSKrasemannT, et al.Reconstruction of the aortic arch in neonates and infants: the importance of patch material. World J Pediatr Congenit Heart Surg.
2021;
12(
4):
487–
491.