Transcript for:
Understanding Urinary Tract Birth Defects

hello and welcome to this channel my name is Victoria and in this video we will talk about birth defects in the urinary tract and kidneys in children the urinary tract consists of the kidneys the ureters the urinary bladder and the urethra and in every of these parts anomalies can occur many structural abnormalities can already be found while the baby is still inside the uterus so antenatal and those abnormalities are usually screened for this screening is important because before it was routinely done children were often diagnosed later in childhood or even adult life when the anomaly causes symptoms abnormalities in the urinary tract occur in around 1 out of 200 to 400 births if the defects are not identified early and taken care for they can damage the developing organs and cause problems for the rest of the life of the child congenital anomalies can be associated with abnormal development or abnormal function of the organs and can make the child more susceptible to infections after birth in the next part we will talk about the anomalies that can occur in each of the parts of the urinary system there are numerous but I will try to keep it as short and simple as possible let's start at the very top or the most Superior aspect with the kidneys they can have anomalies in their number size position relation to each other they are structure or their blood supply anomalies in the number of the kidneys include adenesia aplasia unilateral multicystic dysplasia and supernumerary kidneys in a Genesis there is the complete missing of one or both of the kidneys while in a pleasure one or both of the kidneys develop in their most primitive form but not further than that in unilateral multipistic dysplasia one of the kidneys develops normally while the other one develops as irregular cysts that are differently large these cysts are usually not functional in supernumerary kidneys the child has more than two kidneys they may or may not be fused together to the other ones this defect usually results from an abnormal separation of the embryonal structure that usually forms the kidney anomalies in the size of the kidney can be simple hypoplasia hypoplasia with dysplasia oligon nephronium nephron obtruses or fanconi and segmental hypoplasia in the simple hypoplasia there is one kidney that is significantly smaller than the other one it usually also has less nephrons so functional units of the kidney in hyperplasia with dysplasia there is one significantly smaller kidney than the other in which the renal tissue develops unorganized and undifferentiated so there are some normal nephrons and some that don't develop properly and are non-functional nephron of teases or fanconi is an outer summer recessive disease in which the patient usually has a chronic tubular interstitial nephritis due to inflammation and scarring of the nephrons which leads eventually to renal failure usually during the second decade of life in the juvenile form or before the age of five in the infantile form oligone nephronia is a congenital defect in which the kidney has less than normal number of nephrons but the ones that are present are usually functional segmental renal hypoplasia or ask up Mark kidney is a type of kidney hypoplasia that usually leads to severe hypertension in this defect some areas of the kidney don't develop normally they usually present with scar formation atrophy of the tubules and hyperplasia of the renal blood vessels in the affected segment often no or only few glomerally can be found anomalies in the position of the kidney include homolateral dystopia or simple ectopy as well as heterolateral dystopia or crossed ectopy the kidneys develop inside the pelvis and during the development of the baby they normally rise up to their normal position but sometimes they do not rise up together or one of the kidneys does not find its physiological position and is found somewhere else in the body in cases of homolateral ectopy the kidney is in the same side of the body but at a different level while encrust ectopy the kidney is in the other side of the body in homilateral dystopia the kidney may be found in the pelvis iliac area lumbar area or the chest in heterolateral dystopia the crossed kidney may or may not fuse with the other one that is located in its normal position the kidney can also be malrotated so that the high Loom where the blood vessels nerve and ureter go in and out of the kidney is not pointing towards the spine but towards the flank nephoptosis is another condition in which the kidney drops into the pelvis when the patient stands up and goes back to its normal position when sitting this is also called floating kidney and occurs more frequently in women than men anomalies in a relation between the kidneys usually means that the kidneys fuse this can occur in different forms and usually describes the way we see the kidney on an X-ray or in a sonography the kidney is confused to form a horseshoe kidney where the two lower poles or Isthmus of the kidney fuse they can also fuse to form an l-shape or an S shape anomalies in the structure of the kidney include usually cyst formations that occur in either one or both of the kidneys the cyst can be only one called solitary kidney cyst and in the case of only one kidney having one cyst it is called solitary unilateral kidney if one kidney has several cysts we call it multilucular unilateral cyst also included in this group is the polycystic kidney disease this is an inherited disorder and can occur either as an autosomal recessive or autosomal dominant disease the cysts occur all over the kidneys in different sizes and in the long run usually lead to chronic kidney failure the last group about the kidneys are the vascular anomalies this can include apparent renal arteries so that not only one renal artery goes into the hilum of the kidney but one or two extra arteries also a stenosis or aneurysm of the renal artery or its branches is possible in the next part we will talk about anomalies of the kalisas the pelvis and the ureters the calluses are small cup shaped spaces that collect the urine that is produced in the kidneys and give it further into the ureters anomalies of the calluses include calcium diverticulas and megacalicrosis in calistheal diverticulum is an out pouching of the calyx's scene this is a rather rare birth defect in around 50 percent of patients also a stone is found in these out pouchings and they predisposed a patient for infections of the kidney Mega Kali courses another rare congenital anomaly is that the renal calluses are dilated while the renal pelvis and ureter are usually normal this dilation can be seen in an x-ray with contrast and a calluses make up most of the space in the kidney and can impair the normal function of the glomerally now we will talk about renal pelvis and the ureters for this part it is important to know about a pyelore uriteral segment so where the renal pelvis or the urine is collected meets the ureter that leads to urine to the urinary bladder this part can be hypoplastic or hyperplastic as well as clear roast with additional valves or there can be adhesions or kinks in the segment in the case of a hypoplastic pile or uriteral segment the connection between the two structures is short and narrow in the case of a hyperplastic segment it is usually dilated and funnel shaped all the before mentioned anomalies can obstruct the outflow of urine in the kidney and can cause it to back up and lead to hydronephrosis and pressure atrophy of the renal parenchyma as well as predisposition for infections of the kidneys there can also be anomalies in the number of ureters there can be a complete uriteral duplication so that two completely formed ureters join the kidney and the pelvis and lead into the urinary bladder it is also possible that the ureter is only doubled in a specific part of the entire length it is hugely divided into duplication of the upper middle or lower third of the ureter the next part we will talk about is the ureterio vesicle segment so where the ureter goes into the urinary bladder this includes Amiga ureter of mechanical causes as well as the mega ureter of dynamic causes Mega ureter is the term for pathologically dilated ureter that is much wider in diameter than it usually should be in numbers if it is more than seven millimeters wide mechanical causes include a stenosis at some level so that the ureter is widened due to urine accumulation uriteral diverticula bladder diverticula and ectopic uriteral orifices an ectopic ureteral orifice is when the ureter connects to the bladder somewhere else than the trigone of the bladder where it usually should connect the connection point is usually lower than the trigone so that the urine flows in at the bottom of the bladder it is also possible that a ureter connects directly to the urethra which leads to incontinence Dynamic causes of Omega ureter include congenital physical ureteral renal reflux so that the urine flows from the bladder back to the kidney and distends the ureter ureteral sealer so an outcouching of the most distal part of the ureter which can block the drainage of urine from the ureter into the bladder now we will talk about anomalies of the bladder the anomalies include adonisium so that the bladder does not form at all hypoplasia so that the bladder forms but is much smaller than usually duplicated bladder so that there are two urinary bladders draining into one urethra or two urinary bladders training in each its own urethra it is also possible that one bladder is divided into two by a septum so tissue strand dividing the cavity other anomalies of the bladder include the vesicle vaginal fistula or the bladder and vagina have a connection and the urine can flow through this connection and bladder estrophy bladder estrophy is a huge Topic in itself but to break it down it is when the abdominal wall does not fully develop and the hip bones are usually malformed so that a bladder is exposed to the outside environment this usually is a combined by anomalies of the genital organs and the surgery is necessary to bring the bladder back into the abdomen and to correct the position and structure of the other organs and hip bones now we already made it quite far all that is left for now is the anomalies in the point where the bladder goes over into the urethra and the urethra itself anomalies in the point where the bladder meets the urethra so the musical urethral segment includes sclerosis of the neck of the bladder so that the outflow of the bladder is narrow and in some cases completely closed due to the sclerosis the bladder neck cannot open properly when it should to release the urine and so the urine can flow back into the kidneys other anomalies are valves in the urethra which can be either coming from the anterior or posterior aspect of the urethra these tissue flaps can prevent the urine from flowing out the urethra may also not form at all called urethral adenosia or it may be true urethrust calling duplicated urethra in case of male patients the opening of the urethra can be located at another site than the tip of the penis in hypospadias the opening is located at the underside and can be found anywhere from the perineum to the gland's penis and 20 percent of cases it occurs at a posterior aspect and 30 percent in the middle part or shaft and then 50 in the anterior aspect so either glandular or sub coronal another anomaly is ap's bodyas this one can also occur in girls in girls epispadias usually shows as the opening of the urethra being wider the man's pubis is flattened and a urethra is shorter than usually in a clitoris and symphysis pubis is split in the middle so they do not unite in the middle in both epispadias shows as the opening of the urethra on the dorsal side of the penis and a corpora carvanosa are usually split and a corpus bungalisum does not form when we examine a patient in regards of the congenital anomalies of the urinary tract we want to keep special attention to the anamnesis and ask about urinary stream prior urinary tract infections a family history of renal diseases polyurea so frequent urination polydipsia so increased thirst and Analysis so bad wetting the diagnosis is usually done untenatally so in the ultrasound before the baby is born but sometimes photo imaging techniques are done or A congenital anomaly might not be seen in the antenatal screening especially used is the ultrasound examination but also contrast X-rays and cities sometimes also chromosomal and genetic testing is done especially in some structural anomaly that is found or suspected to be familial that's it for this video thank you for watching and if you like our Channel Please Subscribe and hopefully see you again in the next video