A rare condition called midaortic syndrome can sometimes be associated with Alagille syndrome, according to a new review published in Seminars in Pediatric Surgery.

The condition is very rare, usually idiopathic, and is characterized by abdominal aorta stenosis. Hypertension is a symptom due to decreased renal perfusion. The condition can also cause abdominal pain, kidney insufficiency, heart failure, claudication, and stroke.

Diagnosis can be reached via duplex ultrasound, angiography computed tomography angiography, and magnetic resonance angiography.


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Treatment initially involves antihypertensives. This may need to be followed by invasive intervention in case of organ damage or dysfunction, poor kidney growth, stroke, left ventricular hypertrophy, or heart failure.

These interventions include renal artery bypass, autotransplantation, or renal artery reconstruction to achieve renal revascularization, and patch angioplasty or aortoaortic bypass to treat aortic lesions.

More novel techniques include using tissue expanders to lengthen the aorta to allow for primary aortic reconstruction or using the meandering mesenteric artery as an autologous aortic bypass graft.

Read more about Alagille syndrome comorbidities

“Due to the complexity of care required for treatment of these patients, an experienced multidisciplinary team of specialists that includes interventional radiologists, cardiologists, surgeons, and nephrologists is often required for optimal outcomes,” the authors wrote.

“Regardless, endovascular and surgical innovation will continue to push the field forward and expand our armamentarium for the treatment of [midaortic syndrome].”

The exact link between midaortic syndrome and Alagille syndrome is not well established but the 2 conditions have been reported concomitantly. 

Other conditions that are associated with midaortic syndrome are granulomatous vasculitis, neurofibromatosis-1, fibromuscular dysplasia, and Williams syndrome. 

Reference

Durgin JM, Slatnick BL, Vakili K, Kim HB, Cuenca AG. Midaortic syndrome and renovascular hypertension. Semin Pediatr Surg. Published online October 30, 2021. doi:10.1016/j.sempedsurg.2021.151124