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Renal Abnormalities

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Shambel Negese
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8 views15 pages

Renal Abnormalities

Uploaded by

Shambel Negese
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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RENAL

ABNORMALITIES
RENAL ABNORMALITIES
• Congenital anatomic anomalies of the genitourinary tract are more common than those
of any other organ system. Urinary tract anomalies predispose patient to many
complications, including UTI, obstruction, stasis, calculus formation and impaired renal
function
• Genital anomalies may cause voiding or sexual dysfunction, impaired fertility
psychosocial difficulties or a combination. Genitourinary anomalies frequently require
surgical reconstruction.
• Many genitourinary anomalies are diagnosed in utero via routine prenatal
ultrasonography. Some congenital renal anomalies (e.g. autosomal dominant polycystic
kidney disease, medullary sponge kidney, hereditary nephritis) typically do not manifest
until adulthood
congenital genitourinary anomalies
• Bladder anomalies.
• Cryptorchidism.
• Penile and urethral anomalies.
• Prune-belly syndrome.
• Renal anomalies.
• Testicular and scrotal anomalies.
• Ureteral anomalies.
• Vaginal anomalies.
• Vesicoureteral reflux.
Urinary tract infections in neonates

• UTI in neonates (infants ≤30 days of age) is associated with bacteraemia and
congenital anomalies of the kidney and urinary tract.
• Upper tract infections (i.e. acute pyelonephritis) may result in renal
parenchymal scarring and chronic kidney disease.
• Neonates with UTI should be evaluated for associated systemic infection, and
anatomic or functional abnormalities of the kidneys and urinary tract.
HYPOSPADIAS
I. Definition. Hypospadias is characterized by altered development of the ventral urethra, ventral (downward)
penile curvature, and dorsal hooded prepuce with deficient ventral foreskin. It occurs in about 1 in 200 to 300
live births.
II. Clinical presentation/diagnosis. The urethral abnormality is usually noted on physical exam. In most cases,
the urethral meatus is on the distal third of the penile shaft.
Moderate or severe cases include cases where the meatus is more proximal on the penile
shaft, scrotum, or perineum. In general, the more proximal the location of the urethral meatus, the more severe
is the degree of curvature. Megameatus with an intact prepuce is an uncommon variant and is usually found at
the time of circumcision.
III. Management. Surgical correction of hypospadias is ideally done between 6 and 18 months of age.
Because the foreskin is sometimes used for surgical repair, a neonatal circumcision should not be done. If the
infant with hypospadias also has a UDT and in cases of severe penoscrotal hypospadias, an evaluation for DSD
is warranted
EPISPADIAS-
EXSTROPHY COMPLEX
I. Definition. Epispadias-exstrophy complex is a spectrum of
genitourinary malformations ranging from isolated epispadias
(least severe) to cloacal exstrophy (most severe).
A. Isolated epispadias ranges from a mild defect of the dorsal
urethra at the glan penis to a penopubic variant with complete
incontinence. Incidence is about 1 in 117,000 males.
B. Classic bladder exstrophy occurs in 1 in 10,000 to 50,000
live births. It is definedby incomplete formation of the anterior
abdominal wall, bladder, and dorsal phallus due to altered
development of the cloacal membrane. Male-to-female ratio is
3:1 to 6:1
C. Cloacal exstrophy includes the features of bladder exstrophy
in addition to altered
hindgut development and presence of an omphalocele.
Clinical presentation
• A. Epispadias is usually diagnosed at birth on physical exam.
– Males have an incom-plete dorsal urethra, incomplete glans fusion, exposed urethral plate, and dorsal
penile curvature.
– Females present with bifid clitoris and anteriorly positioned vagina.

B. Bladder exstrophy may be identified prenatally or at birth. In addition to


epispadias findings, there is a large abdominal wall defect and visible bladder plate.
C. Cloacal exstrophy can present on prenatal ultrasound as absence of a full bladder,
infraumbilical abdominal wall defect, or omphalocele. On exam, an open bladder
plate is divided by hindgut structures, and the omphalocele extends superiorly.
Genital malformations are similar to bladder exstrophy but more severe.
Diagnosis
A. Most abnormalities are easily visible at birth. In bladder exstrophy,
the pelvic bones are externally rotated with a wide pubic diastasis and
anterior displacement of the anus.
B. Cloacal exstrophy is associated with renal abnormalities, müllerian
fusion anomalies, intestinal anomalies, hip or limb defects, and neural
tube defects. Renal and spinal ultrasound and skeletal imaging should
be done.
Management
A. In epispadias, surgical correction of the urethra and penile curvature is done electively between 6
and 18 months of age. The bladder neck and continence mechanism repair are delayed until 4 to 5
years of age.
B. In bladder and cloacal exstrophy, the bladder plate must be protected with a thin plastic
nonadherent covering. Do not use saline-soaked gauze. Timing and method of closure in bladder
exstrophy are variable across institutions. Most children will require multiple surgeries to correct the
defects.
c. Cloacal exstrophy repair requires multiple staged procedures. First, the bladder plate and
omphalocele are repaired. Subsequent procedures address the hindgut and genital malformations.
D. Latex precautions should be instituted as there is a high incidence of latex sensitization in these
patients
Undescended TESTIS (CRYPTORCHIDISM)

• Definition. Undescended testis (UDT), or cryptorchidism, refers to the


absence of the testis within the scrotum and is the most common
congenital anomaly of the male geni- talia. The incidence is 1% to 4% in
full-term neonates and up to 45% in preterm neonates; 10% to 25% of
patients with UDT at birth have bilateral cryptorchidism.
• II. Clinical presentation.
• UDT is diagnosed by physical exam. A UDT may be nonpalpable or
palpable. Other exam findings may include ipsilateral hypoplastic
scrotum, inguinal hernia, and hydrocele. UDT can be an isolated finding
or occur as part of a syndrome (eg, disorder of sexual differentiation,
Eagle-Barrett syndrome, bladder exstrophy).
Diagnosis.
• Begin the exam with the patient in a supine position and legs abducted
Sweep a hand lateral to medial from the anterior superior iliac spine
toward the ipsilateral scrotum.
• If the UDT is palpable, note the location, size, mobility, and consistency
of the testis. The contralateral testis and penis should be examined as
well.
A. Palpable testis. A palpable testis is a testis that can be felt on exam
within the inguinal canal, upper scrotum, perineum, or an ectopic
location. Neonates withpalpable UDT should be reexamined as they
grow to ensure proper descent by age 6 months.
Diagnosis.
• B. Nonpalpable testis. If the testis cannot be palpated on exam, it
may be intra-abdom- inal, absent, or atrophic (vanishing testis or
testicular regression). If both testes are nonpalpable, urgent
evaluation for congenital adrenal hyperplasia (CAH) in a genetic
female or other disorder of sexual differentiation (DSD) is warranted
Management.
• Spontaneous descent of the testis can occur within the first 3 to 6 months of life.
• After age 6 months corrected for gestational age, surgery is recommended for a UDT. Inguinal
or scrotal orchiopexy is done for palpable testes.
• Diagnostic laparoscopy and potential laparoscopic orchiopexy are standard of care for non-
palpable testes.
• Imaging studies are not recommended to help locate nonpalpable undescended testes, as they
usually do not alter management recommendations.
• Neonates with hypospadias and UDT should undergo urgent evaluation for underly-ing genetic
or endocrine anomaly such as CAH or other DSD. CAH with salt wasting can be life
threatening.

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