Case Report

Massive Hydronephrosis from Ureteropelvic Junction Obstruction

Masquerading as a Paratubal Cyst in an 11-Year-Old Girl
Mariel A. Focseneanu MD *, Diane F. Merritt MD **
Department of Obstetrics and Gynecology, Division of Pediatric and Adolescent Gynecology, Washington University, St. Louis, MO

a b s t r a c t
Background: The differential diagnosis of a cystic pelvic mass in an adolescent girl is broad, and includes gastrointestinal, urologic, and
gynecologic conditions.
Case: A premenarchal 11-year-old girl presented with abdominal pain. On transabdominal ultrasonography, abutting the lateral side of the
left ovary was a large mass with an appearance compatible with a large paratubal cyst measuring 16.7  11.9 cm. On exploratory lapa-
rotomy, the patient's uterus, tubes and ovaries were normal and a massively dilated and displaced left kidney due to an ureteropelvic
junction obstruction was ultimately diagnosed.
Summary and Conclusion: A cystic pelvic mass in an adolescent girl may not always be of ovarian or mu € llerian origin. Urinary tract
obstruction is often silent; an incidental finding of hydronephrosis on ultrasonography may be the first clue of the possibility of ureter-
opelvic junction obstruction as the underlying diagnosis. It is always best to know which organ system is involved prior to surgery, so that
the correct surgical team is present and the correct surgical approach is utilized.
Key Words: Hydronephrosis, Ureteropelvic junction obstruction, Pelvic mass, Adolescent

Introduction determined that the bleeding and cramping was due to

The differential diagnosis of a cystic pelvic mass in chil-
dren and adolescents is broad and should not be limited to
reproductive or gastrointestinal structures. We report an
interesting case of massive hydronephrosis in an adolescent
girl who presented with a left lower quadrant cystic pelvic
mass, which was initially thought to be a paratubal cyst
both clinically and on ultrasonographic imaging.
Case Presentation
A previously healthy premenarchal 11-year-old girl pre-
sented to the emergency room of a local hospital due to
abdominal pain and vaginal bleeding. She had no prior
medical history. A transabdominal ultrasonography of her
pelvis was performed which demonstrated a normal uterus,
and a right ovary measuring 2.2 cm in diameter which
contained 2 tiny follicles. The left ovary measured 2.3 cm in
diameter and a large cystic mass measuring 16.7  11.9 cm
abutted the lateral side of the left ovary. The patient was
referred to a tertiary care center for evaluation. She had
Tanner 4 breast development and a non-tender abdominal
mass palpable to the level of the umbilicus. It was
The authors indicate no conflicts of interest.
These findings were presented in a poster at the 26th Annual Meeting of the North
American Society for Pediatric and Adolescent Gynecology, Miami, FL, April
15, 2012.
* Address correspondence to: Mariel A. Focseneanu, MD, 503 Grasslands Rd, Suite
200, Valhalla, NY 10595; Phone: (646) 709-7350; fax: (914) 345-1755
** Address correspondence to: Diane F. Merritt, MD, 660 S. Euclid Ave, St. Louis, MO
63110-1010
E-mail addresses: merrittd@wudosis.wustl.edu (M.A. Focseneanu), marieldoc@
gmail.com (D.F. Merritt).
1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.
http://dx.doi.org/10.1016/j.jpag.2014.08.004
menarche, and the left lower quadrant cyst was likely a
paratubal cyst since it appeared simple and distinct from
the ovaries. Management options were discussed with the
family, who elected to have repeat imaging and laboratory
testing. Serum tumor markers also were performed,
including inhibin, bhCG, estradiol, alpha-fetoprotein, and
CA-125; these were all normal.
Six days after her initial office visit, a repeat ultraso-
nography was performed at our institution to evaluate for
any possible resolution of this mass (see Fig. 1). The uterus
and right ovary appeared normal. The left ovary was not
visible on this examination. Once again, a large cystic mass
was seen arising from the left adnexa, which was described
as having a small septum in the superior aspect of the
mass. It appeared to have enlarged, now measuring
17.7  13.6 cm. The wall appeared smooth and there was no
blood flow to the mass. At this point, the decision was made
to proceed to surgery to obtain a diagnosis and treat the
patient's pain.
The patient was taken to the operating room and placed
under general anesthesia where a rectoabdominal exami-
nation demonstrated a mass filling the entire lower pelvis
and extending above the umbilicus. At this time a decision
was made to forego a diagnostic laparoscopy, and in the
belief that the lesion was a benign cyst, a 5-cm Pfannenstiel
incision was made. Once the peritoneal cavity was entered,
pelvic washings were obtained for cytology. On surveying
the pelvis, the patient's uterus, right and left tube, and
ovaries were visualized and appeared normal. The cystic
lesion was covered by omentum and adherent to bowel.
At this time, it was felt that this cyst was likely arising
from the bowel, mesentery, or a retroperitoneal structure,
e92 M.A. Focseneanu, D.F. Merritt / J Pediatr Adolesc Gynecol 28 (2015) e91ee93
Fig. 1. Ultrasound documenting simple cyst.Ă
and a pediatric surgeon was called for an intraoperative
consultation. After extensive dissection, the pediatric sur-
geon was able to identify that the mass was connected to
the left ureter (see Fig. 2). Inspection by a pediatric urologist
revealed a massively dilated left kidney with high insertion
of a ureter that had rotated and was occupying the left
lower quadrant. The parenchyma of the kidney was thin-
ned. An ultrasonography was done intra-operatively
showing that the normal right kidney measured 10.9 cm,
which is consistent with mild compensatory hypertrophy.
The diagnosis of left ureteropelvic junction obstruction was
determined and the family was informed of this diagnosis.
Since the left kidney appeared to have some functioning
parenchyma and its level of function was unknown, a
dismembered pyeloplasty with a nephrostomy placement
was successfully performed to relieve the obstruction.
Summary and Conclusion
Urinary tract obstruction is often silent. In the newborn
infant, for example, a palpable abdominal mass most
commonly is a hydronephrotic or multicystic dysplastic
kidney. The presence of a dilated urinary tract is the most
common characteristic of obstruction. Hydronephrosis is a
Fig. 2. Pfannenstiel incision demonstrating a portion of the massively dilated kidney
and ureter (arrow).
common ultrasonographic finding. Dilation can result
from vesicoureteral reflux, or it may be a manifestation of
abnormal development of the urinary tract, even when
there is no obstruction.1 Ureteropelvic junction (UPJ)
obstruction is a partial or total blockage of the flow of urine
that occurs where the ureter enters the kidney. The etiology
of UPJ obstruction includes both congenital and acquired
conditions. Congenital UPJ obstruction is caused by
anatomic lesions or functional disturbances that restrict
urinary flow across the UPJ, resulting in hydronephrosis.
Clinical manifestations in older children include intermit-
tent flank pain or abdominal pain. The pain may worsen
during brisk diuresis (for example, after consumption of
caffeine or alcohol). These symptoms may be accompanied
by nausea and vomiting. Children may also present with
renal injury after experiencing minor trauma,2 hematuria,
renal calculi,3 or hypertension.4 In these cases, an incidental
finding of hydronephrosis on ultrasonography may be the
first clue of the possibility of UPJ obstruction as the under-
lying diagnosis. Other urinary anomalies which may
present as cystic pelvic pathology include ectopic ureters or
ureteral duplication.
In symptomatic patients, surgical intervention is rec-
ommended to relieve the obstruction and resolve symp-
toms. While open pyeloplasty is considered the criterion
standard for UPJ obstruction in infants, laparoscopic pye-
loplasty, with or without robotic assistance, is the treatment
of choice in older children and in most adults.5 In asymp-
tomatic patients with unilateral UPJ obstruction and a split
renal function greater than 40% of the affected kidney,
observation and monitoring with serial ultrasonography
and diuretic renography is appropriate. Indications for
surgical intervention include increasing hydronephrosis
and decrease in split renal function below 40% or a serial
loss greater than 10% in subsequent studies, the develop-
ment of symptoms, massive hydronephrosis with a renal
pelvic diameter greater than 50 mm, and/or parental and
patient preference.6
Our patient had a preoperative diagnosis of a simple
anechoic paratubal cyst based on examination and clinical
interpretation of 2 preoperative sonographic studies. Para-
ovarian and paratubal cysts are likely congenital and arise
from remnants of the wolffian and mu € llerian ducts. Para-
ovarian cysts are located within the mesosalpinx between
the ovary and the fallopian tube distinguishing them from
paratubal cysts, which are located near the distal end of the
tube.7 Small paraovarian and paratubal cysts are usually
asymptomatic and do not require surgical intervention.
However, these cysts can also become quite large and cause
increased abdominal girth and/or pain. In some cases, they
can lead to adnexal torsion. Rarely, torsion of a hydatid of
Morgagni (a type of paratubal cyst) around its pedicle may
produce acute abdominal pain.8 Very large simple cysts
reported in pubertal girls are typically paraovarian or par-
atubal in origin rather than functional cysts. This enlarge-
ment of paraovarian cysts presents more frequently in the
early menarchal female due to secretory activity of the tubal
epithelium, which is subject to hormonal influence after the
postpubertal years.9 If the patient is symptomatic, surgery is
indicated and these cysts can be removed laparoscopically
 M.A. Focseneanu, D.F. Merritt / J Pediatr Adolesc Gynecol 28 (2015) e91ee93
Fig. 3. Suggested algorithm for diagnosis and management of an abdominal-pelvic cystic mass.Ă
or by laparotomy, depending on the size of the mass and the
skill of the surgeon.
As gynecologists, we are inclined to focus on repro-
ductive structures in the pelvis and we are quite familiar
with adnexal and uterine masses. However, it is important
to have a basic knowledge of the full differential diagnosis
of a cystic pelvic mass in an adolescent girl, which may
involve the gastrointestinal and urinary systems (see
Fig. 3). A complete history and review of systems is critical
to screen for any urinary or gastrointestinal problems that
may assist in forming a diagnosis. In this case, our patient
did not present with any complaints that would suggest a
urinary etiology. Ultrasonography is the imaging modality
of choice for studying the pelvis in children and adoles-
cents. Renal ultrasonography should be obtained with the
presence of a large pelvic mass to assess for the presence of
hydronephrosis due to mass effect onto the ureters. In
hindsight, imaging of the upper urinary tract would have
noted the compensatory enlargement of the right kidney
and ectopic location of the left kidney in the left lower
quadrant. Preoperative evaluation of the function of the
left kidney could have been evaluated by nuclear medicine
with a renal mercaptuacetyltriglycine scan. Based on the
added risk of radiation, MRI would be the preferred im-
aging modality after ultrasonography. CT scan with oral
contrast could be used to better delineate the extent
of a neoplasm beyond the field of view of the pelvic US
(ie, if a gastrointestinal etiology is suspected). Laboratory
e93
evaluation should be based on the patient's symptoms and
suspected etiology of the mass. Although our patient ulti-
mately received the correct diagnosis and intervention, it is
better for a patient to begin with the correct preoperative
diagnosis, surgical team and incision. Preoperative inves-
tigation of a pelvic mass should include evaluation of
adjacent structures and other organ systems to best
delineate the etiology of the lesion and thus provide the
best care for the patient.
References
1. Elder JS: Congenital anomalies and dysgenesis of the kidneys. In: Kliegman RM,
Stanton BM, St Geme J, et al, editors. Nelson Textbook of Pediatrics, (19th ed.).
Philadelphia, Elsevier Saunders, 2011, pp 1827
2. McAleer IM, Kaplan GW, LoSasso BE: Congenital urinary tract anomalies in
pediatric renal trauma patients. J Urol 2002; 168:1808
3. Tekin A, Tekgul S, Atsu N, et al: Ureteropelvic junction obstruction and coexisting
renal calculi in children: role of metabolic abnormalities. Urology 2001; 57:542
4. Gonzalez R, Schimke CM: Ureteropelvic junction obstruction in infants and
children. Pediatr Clin North Am 2001; 48:1505
5. Vemulakonda VM, Cowan CA, Lendvay TS, et al: Surgical management of
congenital ureteropelvic junction obstruction: a Pediatric Health Information
System database study. J Urol 2008; 180:1689
6. Heinlen JE, Manatt CS, Bright BC, et al: Operative versus nonoperative
management of ureteropelvic junction obstruction in children. Urology 2009;
73:521
7. Damle L: Diagnosis and management of pelvic masses in children and
adolescents. Postgrad Obstet Gynecol 2011; 31:1
8. Pansky M, Smorgick N, Lotan G, et al: Adnexal torsion involving hydatids of
Morgagni: a rare cause of acute abdominal pain in adolescents. Obstet Gynecol
2006; 108:100
9. Perlman S, Hertweck P, Fallat ME: Paratubal and tubal abnormalities. Semin
Pediatr Surg 2005; 14:124