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LM is a 22-year-old white female
who was referred for consultation concerning vaginal agenesis/stenosis.
LM is 5’4” and weighs 97 lbs. She is a nonsmoker, nondrinker, who
does not consume caffeine. She is significant for wearing glasses,
sinus problems, discoloration of the skin, tired/sluggish, too hot/too
cold, abdominal pain, nausea, vomiting, GERD, back pain, urinary
symptoms, and depression. She currently takes Cipro and Macrodantin
four times daily. Her past medical history dates back to infancy when
she was diagnosed with multiple congenital anomalies including sacral
agenesis, imperforated anus, duplicated reproductive organs, and
hypospadia of the urethra. During her first year of life, she
underwent multiple reconstructive surgeries including insertion of
urethra into the fornix of the uterus at birth, a urethral reimplant
into her vagina using vaginal mucosa for reconstruction, and a
pull-through operation for the imperforated anus.
An MRI of the pelvis
with and without contrast revealed the following:
- Two separate
uteri, each with its own cervix identified extending to a vagina
which has a prominent midline septation, this is consistent with a
bicollis, bi-corpus, uterine didelphyis. Both uteri have normal
functional anatomy.
- There are single
right and left ovaries that appear normal.
- The bladder is
somewhat thickened and demonstrates multiple diverticuli.
- There appears to
be two ureters extending to each kidney consistent with bilateral
ureteral duplication.
- The mid and distal
sacrum is not visualized however, both IS joints are normal in
appearance; this is consistent with partial sacral agenesis.
LM currently cannot participate in
intercourse and desires reproductive capabilities if possible. A
gynecological exam revealed normal external genitalia; however, the
urethral meatus was difficult to visualize as was the urethra and
bladder. Cervix, adnexa, and uterus could not be evaluated owing to a
shortened vaginal dimple that has about 3 cm of depth. LM does have
menstrual periods on a regular basis associated with severe cramping.
Vaginal reconstructive surgery is scheduled at this time to treat
vaginal agenesis/stenosis.
LM’s bowel symptoms are
significant for fecal incontinence involving solid and loose stool.
She has sensation that this is going to happen. She does not take any
fiber, and has not been treated with fiber in the past. She also has
bowel symptoms of constipation two or more times a month straining up
to a 100% of the time. She has incomplete emptying, splinting up to
100% of the time, and fecal smearing up to 50% of the time. She has a
history related to her multiple anomalies of imperforate anus.
Physical examination revealed an absent external anal sphincter and
markedly decreased tone. There was soft stool in the perianal area.
The sacral dermatomes were in tact, and the clitoral-anal reflex was
absent. For LM’s constipation and fecal incontinence a high fiber
diet was recommended and education was provided on this topic. LM’s
case will be further discussed with colorectal surgery and further
recommendations will be made.
LM has a history of “neurogenic”
bladder and has to perform self-catheterization. A past renogram
revealed 56% of the function from the right side and 44% from the left
side. At that time the decision was made to take her to the operating
room. An exam was performed under anesthesia and a cystogram, which
showed no reflux but a significantly trabeculated bladder. At the
same time, urethral calibration and vaginal septum takedown was
performed to increase the size of the introitus. At that time, LM was
initiated on clean intermittent catheterization and several months
later again showed hydronephrosis when the bladder was full but
decrease after catheterization of her bladder. Currently LM has urge
urinary incontinence and has tried Ditropan in the past with
significant constipation problems. Furthermore, she has a problem
with multiple urinary tract infections and was recently hospitalized
for a urinary tract infection requiring IV antibiotics. Her urinary
tract infections have been shown to grow E. coli, Pseudomonas,
Proteus, and Enterococcus.
LM was infused with 50 mL of
Omnipaque-300 to image the kidneys, ureters, and bladder to be
evaluated for hydronephrosis. Findings from the test were as follows:
- The kidneys are
significantly disparate in size, with the right kidney being much
larger than the left kidney. It is possible the left kidney is
somewhat atrophic in overall size, while the right kidney is
definitely enlarged.
- Significant
dilation and blunting of the renal collecting system is seen
bilaterally, greater on the right than the left.
- There is bilateral
cortical thinning, greater of the right than the left.
- On the initial
delayed films, the right ureter did not opacify beyond the right UPJ.
- The left ureter
demonstrated opacification down to the level of the bladder.
- After the delayed
images when the patient had spent some time upright, the right
ureter was opacified, severely tortuous in its course and
significantly dilated.
- Bladder
demonstrated incomplete emptying after the patient catheterized
herself and a contour suggestive of a thick wall.
LM had been told in the
past that she has a tortuous right ureter and right hydronephrosis.
Her BUN was 24, and her creatinine was 1.2. A renal scan with Lasix
was performed to evaluate hydronephrosis. The scan was performed
using 10.9 mCi of Tc 99m MAG-3. 40 mg of Lasix was administered at 15
minutes into the study. Findings are as follows:
Click to enlarge and magnify image
- There is prompt
and adequate flow to both kidneys.
- The right kidney
is significantly larger than the left.
- Functional imaging
shows enlarged right kidney with irregular distribution of the
tracer in the cortex, most likely due to the hydronephrosis.
- Cold defects are
noted through the cortex in the upper and lower poles, which may
indicate scarring.
- Cortical activity
is 4 to 6 minutes in both kidneys.
- No definite
evidence of obstruction is seen in the left kidney; however, the
right kidney is hydronephrotic and shows no visualization of
collecting system beyond the renal pelvis until following Lasix.
- Even with the
Lasix, the ureter is seen late and is tortuous.
- The portion of the
ureter, which crosses the pelvic inlet, and is more horizontally
oriented, has little in the way of visualization throughout the
study.
- Partial
obstructions in this area cannot be completely excluded.
- The right renal
collecting system shows increasing activity throughout the study,
even following Lasix.
- The left kidney
shows increasing activity until Lasix is administered, and then
there is normal drainage from the collecting system.
- Quantitative
information shows that 60% of the total renal function comes from
the right kidney and 40% from the left.
- The right kidney
has no calculable T –1/2 from administration of Lasix.
- The left kidney
drains with a T-1/2 of 10 minutes following injection of Lasix.
- Renogram curves
are blunted in phase 2 on the left and normal following Lasix in
Phase 3.
- Right kidney shows
blunted phases throughout.
- Right kidney is
obstructed.
- Left kidney has
significant stasis but is not obstructed.
LM was catheterized
during the first renogram and the urologist requested a second
renogram without the catheter to compare it to the first renogram.
The patient was intravenously given 10.5 mCi of Tc 99m MAG-3;
posterior flow images of the kidneys were performed, followed by
sequential static images for approximately 28 minutes. At 15 minutes
into the study, the patient received intravenous 40 mg of Lasix. Time
activity curves were generated for both kidneys. Findings from the
second renogram without catheterization are as follows:
·
Examination is stable since the prior
examination.
·
The right kidney continues to be a little
larger than the left.
·
Prompt uptake is visualized by both
kidneys with poor clearance bilaterally.
·
The left kidney demonstrates clearance
after Lasix administration; however, the right kidney does not
demonstrate significant clearance.
·
Right kidney obstruction with stasis in
the left kidney, but not obstruction.
For
LM’s urge urinary incontinence and urinary retention, she may be a
candidate for sacral-neuromodulation. However, with her
sacral-agenesis, placement on the pudendal nerve via an ischio-anal
fossa approach would have to be considered. The urologist will
accompany the surgical team during LM’s vaginal reconstructed surgery
to help localize the vaginal opening and to perform a cystostomy of
the bladder with retrogrades. After the vaginal reconstructive
surgery and cystostomy, LM’s condition will be reviewed and further
recommendations will be made at that time.
BACK
© 2004 Nuclear Education Online
- Case contributed by Joy Daniel,
PharmD candidate. Images courtesy of University
of Arkansas for Medical Sciences Dept of Nuclear Medicine.
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