DAVIS INTUBATED URETEROTOMY IN A CHILD

Fellow: Logan Dance, MD

Attendings: Robin Kaye, MD; Carrie Schaefer, MD; David Aria, MD;
Richard Towbin, MD
Institution: Phoenix Childrens Hospital, Phoenix, AZ

CHIEF COMPLAINT & HPI

15 year old healthy male with left flank pain and dizziness after a collision with
another player during a basketball game.
No relevant past medical or surgical history.

INITIAL WORK-UP
Physical exam:
Diffuse left flank tenderness without
ecchymosis.
BP 113/65
HR 66

Labs:
Hemoglobin 12.9 g/dL
Hematocrit 37.9

CT abdomen/pelvis:
Left perinephric and retroperitoneal
hemorrhage (Highlighted red).
Enhancing 7.5 cm retroperitoneal mass
with hypodense center (Yellow arrows).

INITIAL WORK-UP
Staging PET scan:
Retroperitoneal mass SUV
max 15.6
FDG avid mets:
Skull
Thoracic spine
Right humerus

Differential Diagnosis:
Lymphoma
Metastasis (Testicular)
Retroperitoneal Sarcoma
Paraganglioma
Neuroblastoma

ULTRASOUND GUIDED BIOPSY

Prone position, posterior approach
16 G coaxial, end-cut core needle
biopsy

Lt kid.

Mass

Left Retroperitoneum

PATHOLOGY

A - Nests of Zellballen (arrows) separated by fibrovascular septa with focal nuclear pleomorphism and hyperchromasia (arrow
heads). Focal angioinvasion present. H&E x 10
B - Diffuse hyperemia and foci of necrosis. Rind-like pseudocapsule is present (arrows).

Pathology Images and Findings courtesy of: Jeff Jacobsen, MD; Daphne de Mello, MD; Steve Taylor, MHS,
PA(ASCP)

DIAGNOSIS
Malignant Paraganglioma (PGL)
Malignancy defined by presence of metastases , not histology.1
Genetic testing: Patient and family positive for SDH-B gene, one of
several familial PGL syndrome genes.
Usually asymptomatic (silent) and non-functional.2
Spontaneous hemorrhage as a presenting feature of both PHEO and PGL is
rare but well-described and can be life-threatening.

Low-grade malignancy often with extended survival.

Most cases of extra-adrenal PGL present with episodic adrenergic

symptoms (headache, sweating, palpitations, hypertension).2

MIBG (+)

PATHOGENESIS
PGLs can arise from anywhere in the paraganglionic system throughout the body3
Adrenal medulla (uniquely named PHEO)
Chemoreceptors (carotid and aortic bodies)
Vagal body
Small thoracic, abdominal, and retroperitoneal paraganglia.

While histologically identical, PHEO and PGL deserve distinction due to important
prognostic and treatment differences.
PGLs: up to 50% malignant.
PHEOs: about 5% are malignant.
Local recurrence common in all types.

Multicentric tumor present in about 10% of cases.

Mets: bone, liver, peritoneum, lymph nodes, and lung.
30% of cases are familial: familial PGL (20%), MEN-2, NF-1, VHL.

QUESTION
Which of the following is FALSE regarding PHEOs and PGLs?
A. Use of IV contrast in the imaging and intervention of PHEO/PGL can trigger
malignant hypertension.

B. Biopsy can trigger malignant hypertension.

C. Pre-operative embolization should be considered to aid surgical hemostasis
and provide a vascular road map.
D. Metastatic disease can be treated with high-dose I-131 MIBG.
E. Local control of metastases can be achieved with RF ablation.

CORRECT!
Which of the following is FALSE regarding PHEOs and PGLs?
A.

Use of IV contrast in the imaging and intervention of PHEO/PGL can trigger malignant hypertension.
FALSE. This myth has been propagated from 1984 when a study showed 5 of 8 patients had increased
circulating catecholamines after IV contrast4, however multiple recent studies have found both that
catecholamines are not significantly increased and patients do not experience increased adrenergic
symptoms after non-ionic IV contrast.5,6

B.

Biopsy can trigger malignant hypertension. TRUE. Although rare in head/neck paragangliomas, these
masses contain high concentrations of catecholamines that can be released into the bloodstream with
any manipulation. If biochemical testing for urine metanephrines is positive, pre-procedural - and adrenoceptor pharmaceutical blockade is paramount.7

C.

Pre-operative embolization should be considered to aid surgical hemostasis and provide a vascular road
map. TRUE. This is especially true with head and neck tumors.

D.

Metastatic disease can be treated with high-dose I-131 MIBG. TRUE. In addition to PHEO/PGL, other
neuroendocrine malignancies can be treated with radiopharmaceuticals. This is a highly specialized
treatment with limited availability.

E.

Local control of metastases can be achieved with RF ablation. TRUE. Palliative local control of PHEO/PGL
metastases can be achieved with percutaneous RF ablation, however, alpha/beta blockade must be
given for functioning tumors and careful monitoring during and after the procedure is critical.8

CONTINUE WITH CASE

SORRY, THATS INCORRECT

Which of the following is FALSE regarding PHEOs and PGLs?
A.

Use of IV contrast in the imaging and intervention of PHEO/PGL can trigger malignant hypertension.
FALSE. This myth has been propagated from 1984 when a study showed 5 of 8 patients had increased
circulating catecholamines after IV contrast4, however multiple recent studies have found both that
catecholamines are not significantly increased and patients do not experience increased adrenergic
symptoms after non-ionic IV contrast.5,6

B.

Biopsy can trigger malignant hypertension. TRUE. Although rare in head/neck paragangliomas, these
masses contain high concentrations of catecholamines that can be released into the bloodstream with
any manipulation. If biochemical testing for urine metanephrines is positive, pre-procedural - and adrenoceptor pharmaceutical blockade is paramount.7

C.

Pre-operative embolization should be considered to aid surgical hemostasis and provide a vascular road
map. TRUE. This is especially true with head and neck tumors.

D.

Metastatic disease can be treated with high-dose I-131 MIBG. TRUE. In addition to PHEO/PGL, other
neuroendocrine malignancies can be treated with radiopharmaceuticals. This is a highly specialized
treatment with limited availability.

E.

Local control of metastases can be achieved with RF ablation. TRUE. Palliative local control of PHEO/PGL
metastases can be achieved with percutaneous RF ablation, however, alpha/beta blockade must be
given for functioning tumors and careful monitoring during and after the procedure is critical.8

CONTINUE WITH CASE

IMAGING FEATURES OF PHEOS AND PGLS

Solid, highly vascular tumor with varied appearances.
Larger tumors may calcify and develop cystic and
necrotic areas.

Intense, early contrast enhancement with flow voids

on MRI.
PET imaging now thought to be superior to MIBG for
diagnosis, staging and follow-up.

CLINICAL COURSE
Follow-up CT showed resolution of
hemorrhage.
Patient started on alpha and beta
blockade (doxazosin, atenolol).
Mass resected. Extremely difficult
dissection of tumor off blood vessels
and proximal ureter.

Blood loss: 500 mL.

LINK TO VIDEO

CLINICAL COURSE
Chemotherapy and radiation.
T1 corpectomy with C5-T4 fusion.
1-month follow-up: US showed a large
well-circumscribed infrarenal
retroperitoneal fluid collection and left
hydronephrosis.

kidney
fluid

QUESTION
What is the next best step in management?
A. Percutaneous nephrostomy.
B. Percutaneous urinoma drainage catheter.
C. Percutaneous nephrostomy followed by percutaneous drainage of urinoma.
D. Multiphase CT abdomen/pelvis.
E. Urology consultation.

SORRY, THATS INCORRECT

What is the next best step in management?
A. Percutaneous nephrostomy.
B. Percutaneous urinoma drainage catheter.
C. Percutaneous nephrostomy followed by percutaneous drainage of urinoma.
D. Multiphase CT abdomen/pelvis. CORRECT. Prior to intervention, a delayed
phase CT will definitively diagnose urinoma and possibly identify the location of
ureteral injury for treatment planning.9
E. Urology consultation.

CONTINUE WITH CASE

CORRECT!
What is the next best step in management?
A. Percutaneous nephrostomy.
B. Percutaneous urinoma drainage catheter.
C. Percutaneous nephrostomy followed by percutaneous drainage of urinoma.
D. Multiphase CT abdomen/pelvis. CORRECT. Prior to intervention, a delayed
phase CT will definitively diagnose urinoma and possibly identify the location of
ureteral injury for treatment planning.9
E. Urology consultation.

CONTINUE WITH CASE

CLINICAL COURSE
Follow-up CT:
Moderate left hydronephrosis
15 cm fluid collection
Non-distended distal left ureter
On 15-min delay, IV contrast is
seen excreted into collection,
confirming suspected urinoma
Dilated proximal ureter
connects to urinoma (yellow
arrow) identifying site of injury
Non-opacified distal ureter
Ureter

INTERVENTION
Percutaneous nephrostomy
Contrast injection confirms a high
proximal ureteral injury and free
flowing contrast into the urinoma.

Drainage of urinoma
Site of
injury

Urinoma drain

URETERAL REGENERATION
Davis Intubated Ureterotomy (Urologist)
1943 published initial report of use of intubated
ureterotomy for repair of UPJ obstruction in humans.
Stent allows reconstitution of an adequate tubular
lumen and prevents leakage of urine that would incite
fibrosis.10

3 steps of healing11
Fibrosis and urothelium fill in the gap.
Scar retraction brings smooth muscle edges in
proximity.
Regeneration of smooth muscle by pluripotent
fibroblasts.

Figure courtesy of the European Association of Urology

PLANNING URETERAL REPAIR

Retrograde ureterogram showed extravasation;
no contrast seen extending proximal to the injury.
Urologist could not place stent across site of
injury.

INTERVENTION
Urologist obtained
retrograde ureteral access.
0.035 angled Glidewire*
advanced through proximal
ureteral defect.

*Glidewire,Terumo, Somerset, NJ, USA

INTERVENTION
Proximal wire captured by
snare* advanced through a
45 cm 9-French sheath*.
Snare and wire pulled down
ureter and out urethra.

*Flexor Ansel 9F 45 cm guiding sheath, Cook Medical, Bloomington, IN, USA

*Ensnare, Merit Medical Systems, South Jordan, UT, USA

INTERVENTION
Double J ureteral stent placed over
wire through urethra.
Urinoma drainage catheter
remained.
Nephrostomy placed.
Alternative approaches:
Snare may be passed antegrade
through the kidney.
Nephroureteral catheter may be used
in place of the nephrostomy and
ureteral stent.

FOLLOW-UP
6-week follow-up retrograde ureterogram shows a long-segment stricture at the
site of ureteral injury but no further extravasation and no hydronephrosis.
Patient currently requiring monthly ureteral stent exchanges.

Monthly denosumab (Xgeva) chemotherapy infusions.

After cancer therapy is finished, urologist plans to evaluate for possible future
endoscopic ureteral stricturoplasty versus definitive surgical repair if needed.

SUMMARY & TEACHING POINTS

There is a high risk of morbidity/mortality when an unrecognized PHEO or
functional PGL is manipulated.
Alpha/beta blockade should be given for all PHEOs and all functioning PGLs
before any manipulation of the tumor.
Pre-operative embolization prior to surgical excision should be considered on a
case by case basis.
The unique regenerative property of the ureter allows stenting alone to suffice in
many ureteral injuries.

REFERENCES
1.

Kimura N. et al. Pathological grading for predicting metastases in pheochromocytoma and paraganglioma. 2014 Feb 21(3): 405-414.

2.

Feng N. et. al. Clinicopathological analysis of paraganglioma with literature review. World J Gastroenterol. 2009 Jun 15(24):3003-3008.

3.

Tischler A. et. al. Pheochromocytoma and Extra-adrenal Paraganglioma: Updates. Archives of Pathology & Laboratory Medicine: August 2008, Vol. 132,
No. 8, pp. 1272-1284.

4.

Raisanen J et al. Plasma catecholamines in pheochromocytoma: effect of urographic contrast media. AJR 1984; 143:43-46.

5.

Mukherjee J. et al. Pheochromocytoma: effect of nonionic contrast medium in CT on circulating catecholamine levels. Radiology. 1997 Jan: 202(1):22731.

6.

Bessell-Browne R. et al. CT of pheochromocytoma and paraganglioma: risk of adverse events with IV administration of non-ionic contrast material. AJR.
2007 Apr; 188(4):970-4.

7.

Pacak, K. Preoperative Management of the Pheochromocytoma Patient. J Clin Endocrinol Metab. 2007: 92(11): 4069-4079

8.

Venkatesan A. et al. Radiofrequency Ablation of Metastatic Pheochromocytoma. JVIR 2009 Nov; 20(11): 1483-1490.

9.

Titton R. et al. Urine Leaks and Urinomas: Diagnosis and Imaging-guided Intervention. RadioGraphics 2003; 23:1133-1147.

10.

Trautner K. et al. Histological examination of the regeneration of the ureter in dogs after intubated ureterotomy. 1954. J Urol. Mar; 71(3): 274-86.

11.

Bergman H. et al. The Ureter, 2nd Edition. 1981. Springer-Verlag, New York, NY.