Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
Abdominal ultrasound is of some benefit, but not diagnostic. Features that suggest posterior urethral valves are bilateral hydronephrosis, a thickened bladder wall with thickened smooth muscle trabeculations, and bladder diverticula.
Voiding cystourethrogram (VCUG) is more specific for the diagnosis. Normal "plicae circularis" are variable in appearance and often not seen on normal VCUGs. PUV on voiding cystourethrogram is characterized by an abrupt tapering of urethral caliber near the verumontanum, with the specific level depending on the developmental variant. Vesicoureteral reflux is also seen in over 50% of cases. Very often the posterior urethra maybe dilated thus making the abrupt narrowing more obvious. the bladder wall may show trabeculations or sacculations or even diverticuli.
Diagnosis can also be made by cystoscopy, where a small camera is inserted into the urethra for direct visualization of the posteriorly positioned valve. A limitation of this technique is that posterior valve tissue is translucent and can be pushed against the wall of the urethra by inflowing irrigation fluid, making it difficult to visualize. Cystoscopy may also demonstrate the bladder changes.
Centers in Europe and Japan have also had excellent results with cystosonography, although it has not been approved for use in the United States yet.
The younger the patient and the lower the grade at presentation the higher the chance of spontaneous resolution. Approximately 85% of grade I & II VUR cases will resolve spontaneously. Approximately 50% of grade III cases and a lower percentage of higher grades will also resolve spontaneously.
The following procedures may be used to diagnose VUR:
- Cystography
- Fluoroscopic voiding cystourethrogram (VCUG)
- Abdominal ultrasound
- Technetium-99m Dimercaptosuccunic Acid (DMSA) Scintigraphy
An abdominal ultrasound might suggest the presence of VUR if ureteral dilatation is present; however, in many circumstances of VUR of low to moderate, even high severity, the sonogram may be completely normal, thus providing insufficient utility as a single diagnostic test in the evaluation of children suspected of having VUR, such as those presenting with prenatal hydronephrosis or urinary tract infection (UTI).
VCUG is the method of choice for grading and initial workup, while RNC is preferred for subsequent evaluations as there is less exposure to radiation. A high index of suspicion should be attached to any case where a child presents with a urinary tract infection, and anatomical causes should be excluded. A VCUG and abdominal ultrasound should be performed in these cases
DMSA scintigraphy is used for the evaluation of the paranchymal damage, which is seen as cortical scars. After the first febrile UTI, the diagnostic role of an initial scintigraphy for detecting the damage before the VCUG was investigated and it was suggested that VCUG can be omitted in children who has no cortical scars and urinary tract dilatation.
Early diagnosis in children is crucial as studies have shown that the children with VUR who present with a UTI and associated acute pyelonephritis are more likely to develop permanent renal cortical scarring than those children without VUR, with an odds ratio of 2.8. Thus VUR not only increases the frequency of UTI's, but also the risk of damage to upper urinary structures and end-stage renal disease.
In boys, history and physical exam is adequate to make the diagnosis. In girls, VCUG (voiding cystourethrogram) is usually diagnostic. Other tests may include:
- Urine analysis
- Urine culture
- CBC, basic metabolic panel
- Renal and bladder ultrasound
Urethral diverticulum is often an incidental finding. It can be diagnosed using magnetic resonance imaging and/or micturating cystourethrography. Other studies that can be used to diagnose urethral diverticulum include intravenous urography, urethroscopy, and/or ultrasound. Conditions that should be distinguished from urethral diverticulum in a differential diagnosis include overactive bladder, Gartner's duct cyst, Gartner's duct abscess, ectopic caeco-ureterocele, interstitial cystitis, pelvic inflammatory disease, endometriosis, and cancer.
Following urethroplasty, patients should be monitored for a minimum of 1 year, since the vast majority of recurrences occur within 1 year.
Because of the high rate of recurrence following dilation and other endoscopic approaches, the provider must maintain a high index of suspicion for recurrence when the patient presents with obstructive voiding symptoms or urinary tract infection.
Urethroplasty refers to any open reconstruction of the urethra. Success rates range from 85% to 95% and depend on a variety of clinical factors, such as stricture as the cause, length, location, and caliber. Urethroplasty can be performed safely on men of all ages.
In the posterior urethra, anastomotic urethroplasty (with or without preservation of bulbar arteries) is typically performed after removing scar tissue.
In the bulbar urethra, the most common types of urethroplasty are anastomotic (with or without preservation of corpus spongiosum and bulbar arteries) and substitution with buccal mucosa graft, full-thickness skin graft, or split thickness skin graft. These are nearly always done in a single setting (or stage).
In the penile urethra, anastomotic urethroplasties are rare because they can lead to chordee (penile curvature due to a shortened urethra). Instead, most penile urethroplasties are substitution procedures utilizing buccal mucosa graft, full-thickness skin graft, or split thickness skin graft. These can be done in one or more setting, depending on stricture location, severity, cause and patient or surgeon preference.
In females, meatal stenosis can usually be treated in the physician's office using local anesthesia to numb the area and dilating (widening) the urethral opening with special instruments.
In boys, it is treated by a second surgical procedure called meatotomy in which the meatus is crushed with a straight mosquito hemostat and then divided with fine-tipped scissors. Recently, home-dilatation has been shown to be a successful treatment for most boys.
If suspected antenatally, a consultation with a paediatric surgeon/ paediatric urologist maybe indicated to evaluate the risk and consider treatment options.
Treatment is by endoscopic valve ablation. Fetal surgery is a high risk procedure reserved for cases with severe oligohydramnios, to try to limit the associated lung underdevelopment, or pulmonary hypoplasia, that is seen at birth in these patients. The risks of fetal surgery are significant and include limb entrapment, abdominal injury, and fetal or maternal death. Specific procedures for "in utero" intervention include infusions of amniotic fluid, serial bladder aspiration, and creating a connection between the amniotic sac and the fetal bladder, or vesicoamniotic shunt.
There are three specific endoscopic treatments of posterior urethral valves:
- Vesicostomy followed by valve ablation - a stoma, or hole, is made in the urinary bladder, also known as "low diversion", after which the valve is ablated and the stoma is closed.
- Pyelostomy followed by valve ablation - stoma is made in the pelvis of the kidney as a slightly "high diversion", after which the valve is ablated and the stoma is closed
- Primary (transurethral) valve ablation - the valve is removed through the urethra without creation of a stoma
The standard treatment is primary (transurethral) ablation of the valves. Urinary diversion is used in selected cases, and its benefit is disputed.
Following surgery, the follow-up in patients with posterior urethral valve syndrome is long term, and often requires a multidisciplinary effort between paediatric surgeons/ paediatric urologists, pulmonologists, neonatologists, radiologists and the family of the patient. Care must be taken to promote proper bladder compliance and renal function, as well as to monitor and treat the significant lung underdevelopment that can accompany the disorder. Definitive treatment may also be indicated for the vesico-ureteral reflux.
Penile color duplex doppler ultrasound can help with preoperative planning to minimize bulbar necrosis.
The primary treatment for urethral diverticulum is surgical. The surgery is conducted transvaginally, usually when there is no acute inflammation to better aid dissection of the delicate tissues.
Diagnosis is based on results of bladder catheterization, ultrasonography, CT scan, cystourethroscopy, or pyelography, depending on the level of obstruction.
Imaging studies, such as an intravenous urogram (IVU), renal ultrasonography, CT or MRI, are also important investigations in determining the presence and/ or cause of hydronephrosis. Whilst ultrasound allows for visualisation of the ureters and kidneys (and determine the presence of hydronephrosis and / or hydroureter), an IVU is useful for assessing the anatomical location of the obstruction. Antegrade or retrograde pyelography will show similar findings to an IVU but offer a therapeutic option as well. Real-time ultrasounds and Doppler ultrasound tests in association with vascular resistance testing helps determine how a given obstruction is effecting urinary functionality in hydronephrotic patients.
In determining the cause of hydronephrosis, it is important to rule out urinary obstruction. One way to do this is to test the kidney function. This can be done by, for instance, a diuretic intravenous pyelogram, in which the urinary system is observed radiographically after administration of a diuretic, such as 5% mannitol, and an intravenous iodine contrast. The location of obstruction can be determined with a Whittaker (or pressure perfusion) test, wherein the collecting system of the kidney is accessed percutaneously, and the liquid is introduced at high pressure and constant rate of 10ml/min while measuring the pressure within the renal pelvis. A rise in pressure above 22 cm HO suggests that the urinary collection system is obstructed. When arriving at this pressure measurement, bladder pressure is subtracted from the initial reading of internal pressure. (The test was first described by Whittaker in 1973 to test the hypothesis that patients' whose hydronephrosis persists after the posterior urethral valves have been ablated usually have ureters that are not obstructed, even though they may be dilated.)
Kay recommends that a neonate born with untreated in utero hydronephrosis receive a renal ultrasound within two days of birth. A renal pelvis greater than 12mm in a neonate is considered abnormal and suggests significant dilation and possible abnormalities such as obstruction or morphological abnormalities in the urinary tract.
The choice of imaging depends on the clinical presentation (history, symptoms and examination findings). In the case of renal colic (one sided loin pain usually accompanied by a trace of blood in the urine) the initial investigation is usually a spiral or helical CT scan. This has the advantage of showing whether there is any obstruction of flow of urine causing hydronephrosis as well as demonstrating the function of the other kidney. Many stones are not visible on plain X-ray or IVU but 99% of stones are visible on CT and therefore CT is becoming a common choice of initial investigation. CT is not used however, when there is a reason to avoid radiation exposure, e.g. in pregnancy.
For incidentally detected prenatal hydronephrosis, the first study to obtain is a postnatal renal ultrasound, since as noted, many cases of prenatal hydronephrosis resolve spontaneously. This is generally done within the first few days after birth, although there is some risk that obtaining an imaging study this early may miss some cases of mild hydronephrosis due to the relative oliguria of a newborn. Thus, some experts recommend obtaining a follow up ultrasound at 4–6 weeks to reduce the false-negative rate of the initial ultrasound. A voiding cystourethrogram (VCUG) is also typically obtained to exclude the possibility of vesicoureteral reflux or anatomical abnormalities such as posterior urethral valves. Finally, if hydronephrosis is significant and obstruction is suspected, such as a ureteropelvic junction (UPJ) or ureterovesical junction (UVJ) obstruction, a nuclear imaging study such as a MAG-3 scan is warranted.
After diagnosing rectovaginal fistula, it is best to wait for around 3 months to allow the inflammation to subside. For low fistulae, a vaginal approach is best, while an abdominal repair would be necessary for a high fistula at the posterior fornix.
A circular incision is made around the fistula and vagina is separated from the underlying rectum with a sharp circumferential dissection. The entire fistulous tract, along with a small rim of rectal mucosa is incised. The rectal wall is then closed extramucosally.
Most rectovaginal fistuals will need surgery to fix. Medications such as antibiotics and Infliximab might be prescribed to help close the rectovaginal fistula or prepare for surgery.
The Society of Fetal Ultrasound has developed a grading system for hydronephrosis, initially intended for use in neonatal and infant hydronephrosis, but it is now used for grading hydronephrosis in adults as well:
- Grade 0 – No renal pelvis dilation. This means an anteroposterior diameter of less than 4 mm in fetuses up to 32 weeks of gestational age and 7 mm afterwards. In adults, cutoff values for renal pelvic dilation have been defined differently by different sources, with anteroposterior diameters ranging between 10 and 20 mm. About 13% of normal healthy adults have a transverse pelvic diameter of over 10 mm.
- Grade 1 (mild) – Mild renal pelvis dilation (anteroposterior diameter less than 10 mm in fetuses) without dilation of the calyces nor parenchymal atrophy
- Grade 2 (mild) – Moderate renal pelvis dilation (between 10 and 15 mm in fetuses), including a few calyces
- Grade 3 (moderate) – Renal pelvis dilation with all calyces uniformly dilated. Normal renal parenchyma
- Grade 4 (severe) – As grade 3 but with thinning of the renal parenchyma
Patients with erectile dysfunction (ED) and PFUDD or patients with PFUDD and traumatic disruption of the dorsal arteries are susceptible to bulbar urethral necrosis. These patients need tubularized substitution urethroplasty, which is replacement of the bulbar urethra with a various number of tubularized flaps ranging from scrotal skin to sigmoid colon (and others).
Treatment, depending on cause, may require prompt drainage of the bladder via catheterization, medical instrumentation, surgery (e.g., endoscopy, lithotripsy), hormonal therapy, or a combination of these modalities.
Treatment of the obstruction at the level of the ureter:
Surgery is usually recommended for hypospadias, with the goal to restore normal appearance and function to the penis. A urinary opening that is not surrounded by glans tissue is more likely to “spray” the urine, which can cause a man to sit to urinate because he cannot reliably stand and hit the toilet. Downward curvature of the penis can impair sexual activity as an adult.
Surgery extends the urinary channel to the end of the penis, straightens bending, and corrects the foreskin abnormality by either circumcision or by repairing it to look normal (“prepucioplasty”), depending on the desire of care-givers.
When the hypospadias is third degree (penoscrotal), or has associated birth defects such as chordee or cryptorchidism, the best management can be a more complicated decision. A karyotype and endocrine evaluation should be performed to detect intersex conditions or hormone deficiencies. If the penis is small, testosterone or human chorionic gonadotropin (hCG) injections may be given to enlarge it before surgery.
Surgical repair of severe hypospadias may require multiple procedures and mucosal grafting. Preputial skin is often used for grafting and circumcision should be avoided before repair. In a minority of patients with severe hypospadias, surgery produces unsatisfactory results, such as scarring, curvature, or formation of urethral fistulas, diverticula, or strictures. A fistula is an unwanted opening through the skin along the course of the urethra, and can result in urinary leakage or an abnormal stream. A diverticulum is an "outpocketing" of the lining of the urethra which interferes with urinary flow and may result in posturination leakage. A stricture is a narrowing of the urethra severe enough to obstruct flow. Reduced complication rates even for third-degree repair (e.g., fistula rates below 5%) have been reported in recent years from centers with the most experience, and surgical repair is now performed for the vast majority of infants with hypospadias.
Most children having hypospadias repair heal without complications. This is especially true for distal hypospadias operations, which are successful in over 90% of cases.
Problems that can arise include a small hole in the urinary channel below the meatus, called a fistula. The head of the penis, which is open at birth in children with hypospadias and is closed around the urinary channel at surgery, sometimes reopens, known as glans dehiscence. The new urinary opening can scar, resulting in meatal stenosis, or internal scarring can create a stricture, either of which cause partial blockage to urinating. If the new urinary channel balloons when urinating a child is diagnosed with a diverticulum.
Most complications are discovered within six months after surgery, although they occasionally are not found for many years. In general, when no problems are apparent after repair in childhood, new complications arising after puberty are uncommon. However, some problems that were not adequately repaired in childhood may become more pronounced when the penis grows at puberty, such as residual penile curvature or urine spraying due to glans dehiscence.
These complications are usually successfully corrected with another operation, most often delayed for at least six months after the last surgery to allow the tissues to heal sufficiently before attempting another repair. Using modern surgical techniques, a normal-appearing penis can usually be expected from hypospadias repair. Results when circumcision or foreskin reconstruction are done are the same, so care-givers can choose whichever option they wish. (Figure 4a, 4b)
Patients with incontinence should be referred to a medical practitioner specializing in this field. Urologists specialize in the urinary tract, and some urologists further specialize in the female urinary tract. A urogynecologist is a gynecologist who has special training in urological problems in women. Family physicians and internists see patients for all kinds of complaints, and are well trained to diagnose and treat this common problem. These primary care specialists can refer patients to urology specialists if needed.
A careful history taking is essential especially in the pattern of voiding and urine leakage as it suggests the type of incontinence faced. Other important points include straining and discomfort, use of drugs, recent surgery, and illness.
The physical examination will focus on looking for signs of medical conditions causing incontinence, such as tumors that block the urinary tract, stool impaction, and poor reflexes or sensations, which may be evidence of a nerve-related cause.
A test often performed is the measurement of bladder capacity and residual urine for evidence of poorly functioning bladder muscles.
Other tests include:
- Stress test – the patient relaxes, then coughs vigorously as the doctor watches for loss of urine.
- Urinalysis – urine is tested for evidence of infection, urinary stones, or other contributing causes.
- Blood tests – blood is taken, sent to a laboratory, and examined for substances related to causes of incontinence.
- Ultrasound – sound waves are used to visualize the kidneys, ureters, bladder, and urethra.
- Cystoscopy – a thin tube with a tiny camera is inserted in the urethra and used to see the inside of the urethra and bladder.
- Urodynamics – various techniques measure pressure in the bladder and the flow of urine.
Patients are often asked to keep a diary for a day or more, up to a week, to record the pattern of voiding, noting times and the amounts of urine produced.
Research projects that assess the efficacy of anti-incontinence therapies often quantify the extent of urinary incontinence. The methods include the 1-h pad test, measuring leakage volume; using a voiding diary, counting the number of incontinence episodes (leakage episodes) per day; and assessing of the strength of pelvic floor muscles, measuring the maximum vaginal squeeze pressure.
Rectovaginal fistulas are often the result of trauma during childbirth (in which case it is known as obstetric fistula) where improper medical interventions are used, such as episiotomy with forceps/vacuum extraction or in situations where there is inadequate health care, such as in some developing countries. Rectovaginal fistula is said to be known as the leading cause in maternal death in developing countries. Risk factors include prolonged labour, difficult instrumental delivery and paramedian episiotomy. Rates in Eritrea are estimated as high as 350 per 100,000 vaginal births. Fistulas can also develop as a result of physical trauma to either the vagina or anus, including from rape. Women with rectovaginal fistulae are often stigmatized in developing countries, and become outcasts.
Rectovaginal fistula can also be a symptom of various diseases, including infection by lymphogranuloma venereum, or the unintended result of surgery, such as episiotomy or sexual reassignment surgery. They may present as a complication of vaginal surgery, including vaginal hysterectomy. They are a recognized presentation of rectal carcinoma or rarely diverticular disease of the bowel or Crohn's disease. They are seen rarely after radiotherapy treatment for cervical cancer.
Globally, up to 35% of the population over the age of 60 years is estimated to be incontinent.
In 2014, urinary leakage affected between 30% and 40% of people over 65 years of age living in their own homes or apartments in the U.S. Twenty-four percent of older adults in the U.S. have moderate or severe urinary incontinence that should be treated medically.
Bladder control problems have been found to be associated with higher incidence of many other health problems such as obesity and diabetes. Difficulty with bladder control results in higher rates of depression and limited activity levels.
Incontinence is expensive both to individuals in the form of bladder control products and to the health care system and nursing home industry. Injury related to incontinence is a leading cause of admission to assisted living and nursing care facilities. More than 50% of nursing facility admissions are related to incontinence.
Botulinum A toxin is a valuable alternative for patients who do not want surgical methods.
The principal treatment of chordee is surgery in infancy, usually by a pediatric urologist. With chordees caused by circumcision, the preferred method of surgical treatment is a z-plasty. The preferred time for surgery is between the ages of 6 and 18 months and correction is usually successful.
In regards to the diagnosis of pulmonary atresia the body requires oxygenated blood for survival. pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with pulmonary atresia there is no opening on the pulmonary valve for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth.
The diagnosis of pulmonary atresia can be done via the following exams/methods: an echocardiogram, chest x-ray, EKG and an exam to measure the amount of in the body.