Posterior urethral valve

Introduction

Posterior urethral valve is the most common cause of severe obstruction uropathy in male
infants. Posterior urethral valves are congenital obstruction of posterior urethra. As this disorder
develops early in gestation, the bladder and upper urinary tract are exposed to elevated pressure
throughout development, leading to far-reaching changes to bladder function and renal injury.
There are obstructing flaps of tissue in the urethra that prevents the normal urine flow from the
bladder.
Pathology
Posterior urethral valves result from the formation of a thick, valve-like membrane from a tissue
of Wolffian duct origin (failure of regression of the mesonephric duct ) that courses obliquely
from the verumontanum to the most distal portion of the prostatic urethra. This is thought to
occur in early gestation (5-7 weeks). The valve is a diaphragm with a central pinhole, however as
it is more rigid along its line of fusion it gradually distends and becomes distended into a bilobed
sail-like or windsock-like structure
Classification
Dr. Hugh Hampton Young, M.D classified it into tree types:
Type 1:
Obstructing membrane that extends distally from each side of the verumontanum towards the
membranous urethra where they fuse anteriorly. (most common)
Type 2:
It described as folds extending cephalad from the verumontanum to the bladder neck.
Type 3:
Represent a diaphragm or ring-like membrane with a central aperture just distal to the
verumontanum. Thought to represent incomplete dissolution of the urogenital membrane.(least
commom)
Presentation
The severity of symptoms and age at presentation depend on the degree of obstruction that the
valves cause in the urinary tract. The most common typical presentation is detection of
hydronephrosis during routine prenatal ultrasound. The symptoms vary from mild cases that
present at a later age to severe cases that are detected prenatally with low levels of amniotic fluid
and problems with lung and kidney development. In severe cases a newborn child may have
difficulty balancing fluids and electrolytes and require mechanical ventilation due to difficulty
with breathing.
Diagnosis
The diagnosis of posterior urethral valves is made by radiographic imaging with ultrasound and
voiding cystourethrogram. Ultrasound will usually show a dilated urethra, bladder, and kidneys;
it is supportive of the diagnosis of posterior urethral valves, but not confirmatory. Voiding
cystourethrogram is the most definitive study for diagnosis and will show a characteristic
tapering of the urethra. Occasionally, confirmation of valves with cystoscopy (small camera is
inserted into the urethra for direct visualization of valves) is required.
Treatment
Shortly after delivery a catheter will be placed into the urethra to continuously drain the urine
from the bladder. Once your child is stable the posterior urethral valves are treated. This is done
inserting a camera into the urethra. The valves are cut from the inside using the camera
(endoscopic valve ablation). Occasionally the urethra is too small for the camera to fit. In this
case the bladder is temporarily brought up to the skin on the belly so that the urine can directly
drain into the diaper and not have to flow through the urethra. Fetal intervention is possible at
specialized centers if amniotic fluid levels are dangerously low and the fetus is between 20-32
weeks gestation.
Complication

 Incontinence
 Urinary tract infection
 Renal failure
 Vesicoureteral reflux
 Chronic kidney disease
 Vesico-urethral reflex

Introduction
Vesicoureteral reflux (VUR) is the abnormal retrograde flow of urine from the bladder into the
ureter and possibly the kidney. It can be primary in which VUR presents at birth and arises due
to defect in the development of ureter. Primary VUR is more common as compared to the
secondary VUR and it can be detected shortly after birth. In secondary VUR, it occurs when
obstruction in the bladder or the urethra forces the urine to flow back to the kidney it may occur
in children with posterior urethral valves or neurogenic bladders. Secondary reflux also occurs in
children who have complete ureteral duplication as well as due to injury, surgery or post-
infection. And it can occur at any age.
Causes
In healthy people the ureters enter the urinary bladder obliquely and run submucosally for some
distance. These phenomena as well as the ureter's muscular attachments, helps secure and
support them posteriorly. Together these features produce physiologic valve that occludes the
ureteric opening during storage and voiding of urine. In people with VUR, failure of this
mechanism occurs, with resultant backward flow of urine to the kidney,
Clinical features
Most children with vesicoureteral reflux are asymptomatic. Vesicoureteral reflux may be
diagnosed as a result of further evaluation of dilation of the kidney or ureters draining urine from
the kidney while in utero as well as when a sibling has VUR (even though it’s somehow
controversial). Reflux also increases risk of acute bladder and kidney infections, so testing for
reflux may be performed after a child has one or more infectionsVUR is associated with two
related consequences: UTI and renal scarring. In infants, the signs and symptoms of a urinary
tract infection may include only fever and lethargy, with poor appetite and sometimes foul-
smelling urine, while older children typically present with discomfort or pain with
urination and frequent urination.
Grades
International reflex study in children grades vesico-urethral reflex into five grades based on it’s
severity:
 Grade I: VUR does not reach the renal pelvis.
  Grade II: VUR extends up to the renal pelvis without dilation.
 Grade III: Mild or moderate dilation of the ureter and the renal pelvis. No or slight
blunting of the fornices.
 Grade IV: there are moderate dilation of the ureter, renal pelvis, and calyces. Complete
obliteration of the sharp angle of the fornices but maintenance of the papillary impression
in most calyces.
Grade V: there is gross dilation and tortuosity of the ureter, as well as gross dilation of the renal
pelvis and calyces. The papillary impressions are not visible in most calyces.
Diagnosis
Whenever suspected for the presence of vesico-urethral reflex through history or clinical
presentation, there are different procedures that can be used to diagnose it. these modalities
include: A urine culture, to check for a UTI, cystography, fluoroscopic voiding
cystourethrogram (VCUG), abdominal ultrasound and technetium-99m dimercaptosuccunic,
acid (DMSA) Scintigraphy. A voiding cystourethrogram (VCUG) is the standard diagnostic
approach, with the advantages of allowing grading of VUR and providing excellent
anatomicdetails.
Management
Because VUR has a high rate of spontaneous resolution, conservative nonoperative treatment of
VUR grades I–III is the standard initial approach. The goal is to prevent UTIs and consequent
renal scarring until the VUR resolves. The aim of the treatment is mostly to minimize infection by
the use of anti-biotic. This is how it’s recommended:

 Amoxicillin or ampicillin – infants younger than 6 weeks

 Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months

After 2 months the following antibiotics are suitable:

 Nitrofurantoin {5–7 mg/kg/24hrs}

 Nalidixic acid
 Bactrim
 Trimethoprim
 Cephalosporins
Endoscopic injection involves applying a sugar containing gel around the ureteral opening to
create a valve function and stop urine from flowing back up the ureter. Surgical approaches that
are indicated four grades IV & V as well as infections refractory to the drugs include ;
endoscopic (STING/HIT procedures); laparoscopic; robotic-assisted laparoscopic; and open
procedures.
Complications
Renal scaring, hypertension and kidney failure are among its complications.

Nephrolithiasis

Introduction
Nephrolithiasis also known as kidney stone, urolithiasis or renal calculi is the formation of solid
crystal minerals in the kidney. Kidney stones usually form in the kidney but pass to the outside
world if it’s less than 5cm. it’s one of the most common cause of hematuria and abdominal pain.
It’s believed to arise from the interaction of genetic (susceptible people) and the environment
(food/fluid and life style).
Pathophysiology
Even though kidney stones are common, it only arises in the presence of many factors together.
Nephrolithiasis occurs in change of urine saturation, absence of protective roles absence of
inhibitors of stone formations, change in urine ph and infection with certain bacteria. The
stimulant of the stone formation is the supersaturation of the urine which may be due to
increased mineral concentrations in the urine of dehydration. The most common mineral
compounds that causes kidney stone is calcium-oxalate (monohydrate or dihydrate), in acidic
urine, uric acid, in purine-rich food struvite (magnesium ammonium phosphate), after infection
with proteus mirabilis, Proteus vulgaris and morganella morganii, calcium phosphate, in
alkaline urine and cystein. This helped by the decrease or absence of inhibitory factors like
citrate, magnesium, calgranulin (a calcium-binding protein), glycosaminoglycans and
prothrombin F1 peptide.
Clinical features
While some kidney stones may not produce symptoms (known as "silent" stones), people who
have kidney stones often report the sudden onset of excruciating, cramping pain in their low back
and/or side, groin, or abdomen. Changes in body position do not relieve this pain. The
abdominal, groin, and/or back pain typically waxes and wanes in severity, characteristic of
colicky pain (the pain is sometimes referred to as renal colic). It may be so severe that it is often
accompanied by nausea and vomiting. The pain has been described by many as the worst pain of
their lives, even worse than the pain of childbirth or broken bones. Kidney stones also
characteristically cause bloody urine. If infection is present in the urinary tract along with the
stones, there may be fever and chills. Sometimes, symptoms such as difficulty urinating, urinary
urgency, penile pain, or testicular pain may occur due to kidney stones.
Diagnosis
The diagnosis of kidney stones is suspected when the typical pattern of symptoms is noted and
when other possible causes of the abdominal or flank pain are excluded. The different modalities
that can be used for the diagnosis include: ultrasound –high-yield test and cost-effective. Plain x-
ray- 80% yield. A noncontrast spiral computed tomography (CT) scan-high yield test. Check serum
and urine calcium. Urine analysis and urine culture (to hunt urease producing bacteria- exclude H.
pylori) ,IV pylegrram and CBC.

Management
 Analgesia, hydration and bed rest are the mainstays of treatment.
 Shockwave lithotripsy for stones <2 cm but fragments can themselves cause obstruction.
Uretroscopy
 Percutaneous removal
 Borderline-sized stones 5-7 mm can be expelled by using nifedipine and tamsulosin.