Fetal congenital midaortic syndrome with unilateral renal artery stenosis prenatally presenting with polyhydramnios and postpartum as hyponatremic hypertensive syndrome.

The midaortic syndrome (MAS) is a rare anomaly, characterised by narrowing of the distal aorta and its major branches. The most common symptom is severe arterial hypertension. The combination of hyponatremia, polyuria and renovascular hypertension caused by a unilateral renal artery stenosis is described as hyponatremic hypertensive syndrome. We report a case of MAS with unilateral renal artery stenosis in a preterm female neonate. A pregnant woman at 34 weeks of gestation was referred with fast growing abdominal circumference and pain. The ultrasound revealed severe polyhydramnios and fetal myocardial hypertrophy. Within the first 48 hours of the neonatal period, the diagnosis of MAS was made. We conclude that symptomatic MAS, caused by unilateral renal artery stenosis, resulting in increased renin-angiotensin-aldosterone system activity and subsequent polyuria of the non-stenotic kidney, lead to clinically significant polyhydramnios.

Mid-aortic syndrome in a 3-year-old girl successfully treated by aorto-aortic grafting and renal artery implantation into the graft.

Mid-aortic syndrome, an uncommon acquired or congenital condition characterized by segmental narrowing of the abdominal or distal descending thoracic aorta, is frequently accompanied by ostial stenosis of the aorta's branches. If left untreated, it can result in life-threatening complications secondary to severe hypertension.We report the case of a 3-year-old girl with congenital mid-aortic syndrome, who was diagnosed by chance in the course of a viral illness, and whose high blood pressure values were first dismissed as inaccurate. Attempts to achieve medical or endovascular control of her hypertension were unsuccessful. She was thereafter successfully treated by aorto-aortic bypass grafting, resection of the stenotic segments of both renal arteries, and implantation of the patent arterial segments into the graft.

Extensive congenital abdominal aortic aneurysm and renovascular disease in the neonate.

Primary congenital abdominal aortic aneurysm is an extremely rare entity, with only 15 patients reported in the literature. Options for repair are often limited secondary to branch vessel size and other anatomic limitations. We present a neonate diagnosed with an abdominal aortic aneurysm on prenatal ultrasound. A postpartum computed tomography angiogram revealed an extensive type IV thoracoabdominal aortic aneurysm extending to the aortic bifurcation and resulting in bilateral renal artery stenosis. The unique features of this patient and challenges in management are discussed.

An unusual case of severe therapy-resistant hypertension in a newborn.

Hypertension can occur in up to 2% of neonates, and the spectrum of potential causes is broad. Prompt and thorough evaluation with a main focus on kidney disease is key for appropriate therapy. Here we describe a 2-day-old neonate with feeding intolerance and elevated blood pressure readings. Within 24 hours after birth, the infant's blood pressure increased significantly, with sustained mean arterial pressure >85. Renal Doppler ultrasound showed decreased venous blood flow in the right kidney with an abnormal Doppler wave form suggestive of unilateral renal venous thrombosis. Despite aggressive antihypertensive therapy including hydralazine and enalaprilat, hypertension remained sustained. On day-of-life 4, the infant developed clinical signs of hypertensive encephalopathy and significant cardiac dysfunction. A renal angiography showed complete, likely thrombotic occlusion of the right renal artery. Renal MAG3 imaging showed minimal function of the affected kidney, and a nephrectomy secondary to medically uncontrollable hypertension and worsening cardiac dysfunction was performed. The child is developing normally in all aspects on follow-up evaluations at 6 months and 1 year of age. Reevaluation of the working diagnosis in neonates with hypertension can be necessary to optimize the outcome. The overall prognosis can be excellent even in newborns with profound cardiac and neurologic involvement.

Severe hypertension and massive proteinuria in a newborn with renal artery stenosis.

Renal vein thrombosis and the congenital nephrotic syndrome have been associated with nephrotic-range proteinuria/nephrotic syndrome and hypertension in the newborn period. We describe a newborn with severe hypertension and proteinuria secondary to unilateral renal artery stenosis. Proteinuria completely disappeared with blood pressure control (with sodium nitroprusside and an angiotensin-converting enzyme inhibitor). Although renin was not measured, we speculate that proteinuria might have been induced by a high renin state, and was controlled by the angiotensin-converting enzyme inhibitor.

[Radio-anatomic study of the angle of origin of dysplastic renal arteries]. / Etude radio-anatomique de l'angle d'origine des artères rénales dysplasiques.

PURPOSE: To compare the angulation at the origin of dysplastic renal arteries compared to atherosclerotic renal arteries, in order to improve the technique for percutaneous transluminal angioplasty of FMD. MATERIALS AND METHODS: Retrospective analysis of 40 aortograms in patients who underwent renal angioplasty for dysplastic stenosis, with comparison with 45 control aortograms (with or without atherosclerotic lesions of the renal arteries). The angle of implantation of the renal arteries was measured relative to the aortic axis in the frontal plane, taking into account only the angulation of its proximal segment. We identified three types of kidneys with regard to their position relative to the renal artery ostium. RESULTS: The angle of implantation of dysplastic renal arteries is significantly sharper compared with the control group (63.8 degrees vs 80.9 degrees, p = 0.0001), irrespective of the side. The angulation did not correlate with the position of the kidney or the direction of the renal artery, suggesting a congenital origin. CONCLUSION: The angle of implantation of the dysplastic renal arteries relative to the aortic axis in the frontal plane is sharper than the angle measured in non dysplastic renal arteries.

Neonatal renovascular hypertension: haematoma within the renal arterial wall. A case report.

A two-hour-old girl with renovascular hypertension is presented. After aggressive antihypertensive medication, nephrectomy was carried out, but she expired soon after operation. Autopsy revealed intramural haematomas between the media and the adventitia at the centre of both renal arteries where the luminal occlusions were observed.

Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries.

Developmental occlusive disease of the abdominal aorta and the renal and splanchnic arteries represent an unusual vascular condition. When unrecognized or untreated this disease is associated with premature death, usually from severe secondary hypertension as a consequence of renovascular stenotic lesions. Strong circumstantial evidence indicates that developmental abnormalities occurring during the fetal union of the two dorsal aortae account for most of the occlusive lesions affecting the abdominal aorta and its visceral branches in these patients. Complete arteriographic studies are necessary to confirm and accurately delineate the disease process. Surgical treatment, which often encompasses complex vascular reconstructive efforts, affords excellent results when carefully planned and executed.

[Thrombosis of the renal artery in a newborn (author's transl)]. / Nierenarterienthrombose bei einem Neugeborenen.

Very rare informations about thrombosis of the renal artery in newborns in the literature could be found. In a six years old girl in the course of a perinatal asphyxia complicated by shock a renal artery thrombosis was observed. Profuse bleeding, anemia, thrombopenia, prolonged bleeding time and coagulation time and a low percentage of the thrombotest suggested an intravascular coagulation as a possible factor of this disease. The treatment of the coagulopathia was effective. The physical and psychical development of the girl is normal. The inhibition of the renal function, however, is in a compensated state.

Renovascular hypertension from renal artery compression by congenital bands.

Renal artery compression by fibromuscular bands containing sympathetic nerves and ganglia was encountered in 3 of 75 patients with renovascular hypertension. The hypertension was successfully managed by resection of the bands. The absence of mortality and morbidity dictates that the "stenotic" area of the renal artery be explored, especially in children and adults with minimal angiographic evidence of visceral atherosclerosis, before proceeding with a bypass graft to the renal artery.