Hypospadias is the most common congenital abnormality of the penis. The opening of the urethra is located underneath the penis, below the glans (mild cases) or all the down in the scrotum or even in the perineum (severe cases). Hypospadias can be associated with a ventral curvature (bending) of the penis in approximately one third of the patients. If mild or no curvature is present the treatment can be done with a single surgery. However a staged repair is necessary for patients with moderate or severe curvature. Successful outcomes are expected for patients with mild hypospadias. One quarter of patients with severe hypospadias develop complications that will require additional procedure for correction. 75% to 80% of patients with severe hypospadias have successful outcomes with planned surgeries.
During development of the fetus the testis gradually comes down to the scrotum (sac). When descend is incomplete the testis may located in the groin (most common); high scrotal or even inside the abdomen. It is important to bring the testis to a normal position in the scrotum so it can develop healthier. An orchidopexy is the name of the procedure to bring the testis down. In cases where the testis cannot be palpated a laparoscopy (examination of the inside of the abdomen with a lighted scope) is necessary. Most orchidopexies can be performed as a day surgery, allowing the patient to go home the same day of the procedure.
Hydronephrosis occurs when the kidney collecting system gets dilated (full of urine). Most patients with hydronephrosis have an antenatal diagnosis (during the pregnancy) and it can be associated with an obstruction (blockage) in the union between the kidney and the ureter (UPJO). Sometimes the ureter can also be dilated due to an obstruction just above the bladder (megaureter). In cases of antenatal diagnosis a thorough investigation should be done after birth that includes ultrasound (US), occasionally an X-ray of the bladder (VCUG) and a nuclear scintigraphy to assess how affected the kidneys are. Surgery is necessary whenever an obstruction is confirmed by the investigation. A pyeloplasty is done for UPJO and this procedure is successful in the great majority of the cases. When obstruction is found below, in the ureter, an opening of the dilated megaureter is placed in the skin for few months and later reconnected to the bladder (reimplantation).
The ureter is the tube that transport urine from the kidney to the bladder. Normally, a one-way valve allows the urine to enter the bladder to the kidneys. If this valve is incompletely formed it can allow reflux of urine to the kidneys. In most cases this valve gets competent with time and VUR resolves. During this period of valve development it is important to avoid infections (UTIs) because they can cause pyelonephritis (infections in the kidney and sometimes renal scarring). For this reason many patients are given a small dose of antibiotic to prevent UTI (antibiotic prophylaxis). If despite these preventive measures a UTI develops, a procedure to stop reflux maybe necessary. This can either be performed by injecting endoscopically a bulking material below the valve or by open surgery. These procedures are highly successful.
Most children develop continence (ability to control urine) around the age of 2 to 3 years old. Some patients continue wetting during the day and night. When wetting persists the child need to be evaluated to assess the reason of the incontinence. There are many reasons for incontinence. The most common cause is due to bladder dysfunction (bladder that contracts involuntarily) and is also accompanied by constipation. In other cases there is abnormalities in the nerves that come from the spinal cord to the bladder (neurogenic bladder). In such cases the bladder and the sphincter (muscle in the bladder outlet the controls urine) do not coordinate properly and the patient wets. It is important to investigate these patients properly as they can have high pressure inside the bladder that can decompensate the kidneys and cause recurrent UTIs.
Patients that wet only at night (nocturnal enuresis) usually have a benign evolution and eventually get dry after the age of 7. These groups of patients generally need support while developing continence. Nocturnal enuresis can be disturbing in older patients that may develop low self-esteem and benefit from a bladder retraining program in specialized clinic.
Posterior urethral valves are caused by the presence of small membranous leaflets at the beginning of the urethra that can cause variable degrees of obstruction for urinary flow. In severe cases the whole urinary tract gets dilated, leading to mal development of the kidneys and renal failure. Most cases are diagnosed antenatally and warrant immediate investigation and treatment after birth. Once confirmed the bladder is drained with a catheter and the valves are incised (cut), using a small light scope, when the patient is stable. Patients with PUV should be monitored carefully for their entire life as a significant number can develop chronic renal failure and need support from specialized pediatric nephrologists.
The bladder is a reservoir that accumulates urine received constantly by kidneys. In rare cases the bladder and urethra do not develop properly, remaining open and completely exposed at the skin level. Because the sphincter of the bladder is not formed properly most patients remain incontinent despite having the bladder closed after birth. Boys with exstrophy also have problems in the penis that can be bended upwards and is relatively short. Repair of bladder exstrophy is challenging and should be performed only by specialized surgeons with adequate experience and training to treat this condition. Outcomes after surgery will depend upon the anatomic findings but also on the quality of the repair, reason why it should be performed only in specialized centers.
During fetal development there is a time where the urethra, vagina and anus drain in a single orifice called cloaca. These structures gradually separate in 3 different channels: urethra, vagina and anus. If they do not separate at all it results in a cloacal malformation. If only the rectum and anus separate a persistence of the urogenital sinus will result. The treatment of these malformations is complex and should be done by a multi-disciplinary, highly specialized team since birth.
The Cloacal malformation need to have a colostomy soon after birth. Later on, the cloaca is repaired by separating the structures adequately. The bowel is placed in the normal site; the urinary tract and the vagina are then reconstructed.
Urogenital Sinus can be associated with large vaginal dilatation (called hydrocolpos) that may need to be decompress early on and later separated.
Some cloacas cases have other associated malformations, especially in the spine cord, that are important to be diagnosed as they impact on the long term outcomes.
The expectancy of a future normal life depends principally in an adequate support because of the high prevalence of functional bladder dysfunctions (not normal bladder emptying) that may impact continence and renal function.
Duplications of the urinary tract (double ureters) are not uncommon but, fortunately, the vast majority are simply normal variants that do not cause problems for the children.
Some patients, however, have one of the ureters partially blocked at the junction of the bladder. This results in ballooning of the end (ureterocele) or the entire ureter (megaureter). In addition, Ureteroceles can block the outlet of the bladder, not allowing complete emptying with consequent recurrent UTIs. Therefore, some ureteroceles need to be endoscopically decompressed early after diagnosis. Some others need complete reconstruction of the urinary tract.
Occasionally, ureters (duplicated or not) can be Ectopic; that means that ureters insert in an abnormal position, sometimes even below the sphincter, leading to constant leakage of urine (incontinence).
Finally, in duplicated urinary tracts, one of the ureters can have a malfunctioning valve mechanism on its insertion in the bladder resulting in vesicoureteral reflux (please see above). Treatment of ureteroceles, ectopic and refluxing duplicated ureters should be individualized and done only in specialized pediatric urology centers. The whole endoscopic, open or laparoscopic armamentarium is available at Sidra and will be chosen accordingly with the particular case.
In the normal boy the foreskin covers the glans that gets exposed only when retracted. In uncircumcised boys a tight ring the end of foreskin can make impossible to expose the glans. This condition is called phimosis.
A physiologic phimosis is normal during the first years of life also because the presence of congenital adhesions between the glans and the internal surface of the prepuce. With time, the phimotic ring improves and the adhesions spontaneously resolve allowing a normal glans exposure. Surgical treatment is warranted in cases of persistent phimosis, especially the ones that complicated by foreskin infection (balanitis) or para-phimosis (the ring traps the exposed glans causing pain and swelling). Usually a circumcision is performed to treat phimosis but, in selected cases, if the family does not desire to have the boy circumcised, a preputioplasty can be done.
In some cases a conservative treatment with steroid cream can be offered for patients that do not desire to have circumcision.
Renal transplant is the optimal treatment for patients with end stage renal failure. It can be a living donated kidney, usually by a family member, or by a deceased donor.
Before transplant a thorough protocol by a multidisciplinary team of nephrologists, urologists, nurses, nutritionist, social workers, dentists, etc. The aim is to prepare the patient for the transplant (search for compatibility and assessment of factors that can alter the survival of the transplanted organ) and for the postoperative time when the anti-reject therapy is started.
The surgery itself can be scheduled (if the kidney comes from living donor) or at the time a compatible organ is available. In case of living donor, the explant is done the same day of the transplant.
In the early postoperative time, the patient will be followed by the whole team until the patient is stable for discharge.
Some patients develop stones within the urinary tract. The stones are formed by minerals and they can be secondary to infection or to conditions that lead to high levels of the minerals in the urine. When the stone is identified, the physician needs to understand if is causing obstruction in the flow of the urine and the main cause of the formation of the stone itself.
The obstruction of the flow is the main cause of symptoms (colic pain). If left untreated can lead to renal damage. The goal of the treatment is the expulsion (facilitation by administration of drugs) or the surgical removal of the stone. The surgery is most of the times endoscopic (cystoscopy, ureteroscopy, nephroscopy) but other approaches can be utilized as well (laparoscopy, robotic, open).
The diagnosis of the cause of the stone formation is role of the Nephrology team in conjunction with Urology. It is crucial to retrieve a fragment of the stone for its analysis to achieve this diagnosis. The correct diagnosis will allow the physicians to prevent the recurrence of the stone formation.
The minimally invasive surgery is an approach utilized for the surgical treatment of many conditions of intraabdominal organs. It utilizes small incisions (3-5 mm) to introduce in the abdomen a camera and 1 to 3 instruments to perform the operation. It practically reproduces the surgical intervention like in open traditional access but minimizing the trauma of the wound in terms of pain (and thus, providing a faster recovery) and improving cosmesis.
The procedures suitable for minimally invasive surgery can be divided in those where an organ (or part of it) has to be removed (nephrectomy, heminephrectomy) or those where reconstruction and suturing is indicated (orchiopexy, pyeloplasty, ureteric reimplant, construction of catheterizable channels).
The robotic approach is an advanced laparoscopy where, thanks to the robotic device, the movements of the instruments are more precise and the surgeon has tridimensional vision from the camera. When suturing is needed, the robotic approach founds its principal utilization.
Urological Conditions & Suggested Treatments
There are many conditions that affect a child’s urological area. Below you will find a brief overview of some of the conditions we commonly see, along with possible treatment options. If your child has been diagnosed with any kind of urological condition, our team will take the time to educate you on the issue and what can be done to help your child recover.