In line with its mission, the Editorial Board of MedTvoiLokony makes every effort to provide reliable medical content supported by the latest scientific knowledge. The additional flag “Checked Content” indicates that the article has been reviewed by or written directly by a physician. This two-step verification: a medical journalist and a doctor allows us to provide the highest quality content in line with current medical knowledge.
Our commitment in this area has been appreciated, among others, by by the Association of Journalists for Health, which awarded the Editorial Board of MedTvoiLokony with the honorary title of the Great Educator.
Seventeen percent of birth defects with which babies are born affect the urinary system. Babies are born with a double renal pelvis, hydronephrosis, and underdeveloped valves. Without prompt surgical intervention, their lives would be very short. See what types of problems parents and children with urinary tract defects face …
The detection of urinary tract defects has increased significantly since the spread of ultrasound diagnostics in both gynecological and neonatal-pediatric diagnostics. At the same time, it turns out that less than half of these deformities will require surgical treatment. It is the task of nephrologists and urologists to hatch only those who really need surgical or nephrological treatment. The basis for differentiation is the parameters of renal function, whether the disease is bilateral, and the frequency and nature of infections. Features of kidney failure, turbulent infections destroying the kidney parenchyma, and bilateral changes indicate that the patient’s life is at stake.
Urinary tract physiology
The role of the urinary tract is, on the one hand, efficient, unidirectional transport of urine from the kidneys to the bladder and storage at a sufficiently low pressure, and on the other hand, periodic complete excretion to the outside without retention. These mechanisms are still mature in newborns and infants. Their upper urinary tract is very susceptible, which results in a rapid dilatation of the calyx-pelvic system of the kidney and ureters, due to even a slight increase in pressure in the bladder. In turn, the bladder collects urine under greater pressure than in later life, the bladder muscle is prone to hyperactivity and generates higher pressures, and the lack of proper coordination leads to small portions of the bladder, which is typical for a newborn and small infant. This physiology of the urinary tract promotes abnormal ultrasound images in the first year of life, which tend to subside with functional maturation.
Birth defects
Disadvantages of numbers and positions – the most common deformity in this group is the duplication of the collective system of the kidney, erroneously called the double kidney, although it is actually one organ, and the duplication concerns only the renal pelvis and ureter. It is more common in women, often bilaterally and is usually only a harmless anatomical variant. But not always. On the basis of duplication, a whole group of diseases arises, in some cases in configurations that are difficult to diagnose and treat, especially when they affect both kidneys. Typical are hydronephrosis, abnormal development or cirrhosis of the upper double kidney system associated with its ectopic mouth or cyst of the bladder ureter, vesicoureteral outflow to the lower system, sometimes hydronephrosis of the lower system.
Other defects in the number of kidneys are unilateral underdevelopment or underdevelopment, and the very rare supernumerary kidney – indeed an extra organ. Sometimes the kidneys join together to form a horseshoe kidney (the lower poles are usually fused), a plaque or a sigmoid kidney – associated with the displacement of one of the organs to the opposite side.
The location of the kidney in an atypical place is called an ectopy. Such a kidney usually lies lower than usual, often on the plate of the iliac bone.
Hydronephrosis – this is the widening of the kidney’s collective system caused by an obstruction (or its suspicion) in the outflow of urine, by default at the site of the pyeloureteral junction. These extensions are very often innocent and are not related to impeding the outflow. Perhaps they are the remains of a temporary obstacle that existed in utero, or they are the result of the specific physiology of the urinary tract during this period. The basic diagnostic examination here is ultrasound, and the anterior-posterior dimension of the pelvis greater than 15 mm is of concern. The test qualifying for surgical treatment is renoscintigraphy.
Hydronephrosis and giant ureter – this is a situation when not only the renal pelvis but also the ureter is dilated, which suggests an obstacle in the outflow of urine at the level of the uretero-bladder junction or below, if the lesions are bilateral. Despite their sometimes spectacular appearance in ultrasound and urography, these changes have a remarkable tendency to spontaneously resolve over the course of usually several years. Then they do not show symptoms or they give very little (e.g. one infection in the first year of life), and control scintigraphic tests do not show complete block in urine outflow and progressive damage to the renal parenchyma. Urological supervision should be intensified in the case of bilateral lesions, where the presence of an anatomical obstruction in the urethra should be excluded.
Vesicoureteral outflows — it is the regurgitation of urine from the bladder into the kidneys, which is normally prevented by the specific structure of the uretero-bladder junction. It may be due to an abnormal anatomy of this site, such as in the case of vesicoureteral outflow to the inferior doubling system, or it may be secondary obstacles in the urethra, functional disorders, or simply infection. It is believed that a low degree of vesicoureteral drainage in children older than 5 years is not particularly harmful. The pressure of recurrent urine itself is dangerous for the kidneys of the fetus, newborn and infant. Later in life, it promotes infection to the kidneys and only promotes the destruction of their flesh. Vesicoureteral outflows should be treated primarily as a symptom, not the essence of the disease.
Posterior coil valve – is one of the most serious urological diseases in boys and can very often lead to kidney failure. The presence of mucosa folds within the posterior urethra during fetal life blocks the outflow of urine from the bladder, preventing or impeding the proper development of the kidney parenchyma. Babies are born with bilateral outflows or giant ureters, severely damaged kidney parenchyma, and a thickened bladder wall. The features of renal failure are found in biochemical tests. Significant damage to the renal parenchyma requires decompression with uretero-cutaneous fistulas, which allows the kidneys to complete the maturation process. If the kidneys are in good condition or only one is damaged, only the valve can be resected and closely monitored in the following months.
Strain the urinary bladder and cloaca – Bladder eversion is a rare but extremely difficult to treat and it determines the quality of life. In these patients, the symphysis pubis is split, the bladder lacks the entire anterior wall, and the urethra is also split dorsally. Surgical treatment consisting in the reconstruction of the bladder is implemented, if possible, in the first 3 days of a child’s life. Most often, over the years, further treatments are required to ensure proper urinary incontinence and to improve the cosmetology of the genital organs. Related defects are epithelium and the most severe of all: eversion of the cloaca.
Neurogenic bladder – there is quite a large group of patients suffering from urine storage and excretion disorders secondary to the damage to the nervous system. These are children with congenital meningeal hernia, cerebral palsy, hydrocephalus and other defects. They all lead to disturbances in the functions of the bladder, urethral sphincters and their mutual coordination. In the most dangerous variant, the detrusor muscle generates high pressure in the bladder, and the urethral sphincters contract, preventing urine from being excreted. This leads to the development of either vesicoureteral outflow or hydronephrosis and giant ureters. These disorders wreak particularly havoc on the urinary system in the first year of a child’s life. Therefore, all newborns and infants with nervous system injuries should also be urologically monitored.
Hypocrisy – is a common malformation affecting about 1 in 250 boys. It consists of a split foreskin, underdevelopment of the urethra, which flows out of the shaft of the penis or even within the scrotum, and sometimes the penis is bent. Surgical treatment is troublesome and sometimes multi-stage. The psychological trauma associated with leading these children is very large, sometimes enormous. They are relatively best tolerated by the youngest children, so treatment should be started at the end of the first year of life and carried out by surgeons dealing with this problem on a daily basis.
Cryptorchidism – is a common developmental defect, the treatment of which is the domain of pediatric urology. It consists in stopping the descent of the testicle on the physiological path between the retroperitoneal space and the scrotum. It requires surgery immediately if the testicle does not descend into the scrotum until the child is 1 year old. A specific problem is the so-called non-testable, which requires slightly different treatment. The migrating testicle, which is a physiological state before the age of 10, must be distinguished from these strongly surgical conditions. The reasons for the periodic wandering of the testicle include the remarkably expressed reflex from the testicular levator muscle at this age, its relatively small size and the large diameter of the inguinal canal in a child.
Phimosis – this is the inability to return the foreskin to the gastric groove. This condition is physiological in young children – the foreskin is brittle and breaks easily when forced to express. Patients with this diagnosis constitute the most numerous group in a pediatric urologist’s office. Most of them do not have phimosis but only a glued foreskin. Both are a derivative of the hormonal state of the organism at this age, which can be described as hormonal silence. It is a mistake of art to make any surgical attempts to detach and withdraw the foreskin before the age of 6. Such interventions can lead to the formation of a real scar of the foreskin, requiring surgery later, and cause unnecessary suffering to the child. Inflammation – suppuration of the mastic accumulated under the glued foreskin – is also a natural thing and most often it calms down spontaneously within three days after using rivanol napkin. Surgical detachment and “cleaning” in such a situation prolongs healing.
In times of widespread access to information, it is good for parents to get acquainted with information about their child’s illness. Reasonable and matter-of-fact dialogue places them in the position of a treatment partner and allows them to participate in it. Conscious parents interact better with doctors in the treatment process, and young patients benefit from it.
Marek Górniak, MD, PhD – Pediatric Urologist Specialist, Pediatric Surgeon Specialist CenterMed Hospital and Clinic, Kraków