Anomalies associated with the development of the urinary tract account for more than 35% of all congenital malformations in humans. At the same time, there are cases when such pathologies are asymptomatic and are determined only during puberty or pregnancy. Frehley's syndrome is an anomaly of the kidneys of a congenital nature, in which a decussation of the anterior and posterior branches of the superior renal artery is formed. As a result, the normal functions of the organ are disrupted.
The syndrome was named after the American urologist who first described it. The article will talk about the causes, symptoms and treatment of the disease.
What is this?
As already mentioned, with Frehley's syndrome, the posterior and anterior branches of the renal artery intersect. In this case, partial compression of the upper parts of the pelvis or pelvis-ureteral segment is possible. As a result, kidney function may be impaired or even lost. There is a possibility of formation of stones, the appearance of arterial hypertension. There may be traces of blood in the urine.
Such an anomaly occurs during the embryogenesis of the vascular system of the kidneys, whenit is possible to stop their development, but the structures are preserved.
Frehley's syndrome is localized on the right and left, that is, it usually affects one kidney. In extreme cases, both organs may be affected. In this case, the syndrome may be accompanied by a defect in the filling of the upper group of cups and right-sided or left-sided pyelectasis due to obstruction of the ureter.
Symptoms of disease
There are some signs that indirectly indicate that the patient may have Frehley's syndrome. Symptoms are manifested by palpable pain in the lumbar region, renal colic, which are present in connection with secondary nephrolithiasis. In addition, there is a slight arterial hypertension, as well as macro- and microhematuria.
Diagnosis of the syndrome
The most difficult clinical diagnosis of Frehley's syndrome is in small children, especially in infants. To make a correct diagnosis, the method of dopplerography of the kidney vessels is used, and multislice computed tomography is also used.
Children are difficult to perform angiography, so they are hardly used at present.
In addition to the above methods, patients are prescribed urine and blood tests, including sensitivity to drugs and flora. In addition, ultrasound examinations of the urinary system are carried out.
Treatment methods
Treatment of this disease is prescribed only after a thorough comprehensiveexamination and confirmation of the diagnosis. A conservative method - antihypertensive therapy - is used in rare cases. It is aimed, as a rule, at reducing pressure in the event that it is increased. They also carry out a set of measures to eliminate secondary pyelonephritis and are engaged in the prevention of urolithiasis.
However, the most reliable way to completely eliminate Frehley's syndrome is not a conservative treatment, but a surgical one. During the operation, the doctor removes the crossing of the arteries in the kidneys and the pressure on them stops.
In any case, patients (and especially babies) should register with a nephrologist and regularly receive his consultations, take the necessary tests, undergo ultrasound and radiographic studies. In later life, Frehley's syndrome may not manifest itself at all, the degree of discomfort depends on how severely the renal pelvis is pinched. With a he althy lifestyle and no bad habits, patients may not feel any symptoms at all.
Pregnancy
Pregnancy and the birth of a child with Frehley's syndrome in the mother take place only under the supervision of a specialist. As a rule, women with congenital malformations of the kidneys, which include this syndrome, are allowed to carry the fetus only after surgery. The fact is that Frehley's syndrome is often accompanied by an increase in blood pressure, pregnancy in this case is more difficult, sometimes it has to be interrupted after 22 weeks.
But even after a successful operation and restoration of kidney function, a pregnant womanYou should notify your obstetrician/gynecologist about this. The entire period of bearing a child, the patient must be under the supervision of a nephrologist, periodically take tests, undergo research and, if necessary, be hospitalized.
Very often exacerbation of kidney disease can occur at 15-16 or 26-30 weeks. Signs are urinary retention, severe swelling of the arms and legs, pain and discomfort during urination. At a later date, complications are possible due to the rapidly growing uterus, which presses on the ureters. In the event of the appearance of such signs, a pregnant woman with Frehley's syndrome requires urgent hospitalization.
Birth with Frehley's syndrome
Very often kidney defects are an indication for caesarean section. However, the threat to the child in this case is negligible.
For women in labor with Frehley's syndrome and other anomalies in the development of the kidneys, there are specialized maternity hospitals, which always have urologists and nephrologists on staff, and a newborn baby is given a comprehensive examination immediately after birth.
So, the article considered such kidney disease as Frehley's syndrome. Despite the fact that the anomaly is congenital, it is currently successfully treated, and patients after surgery can return to a normal lifestyle.
