dance_davis intubated ureterotomy in a child_05202016

8/17/2019 Dance_Davis Intubated Ureterotomy in a Child_05202016

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DAVIS INTUBATED URETEROTOMY IN A CHILD

Fellow: Logan Dance, MD

Attendings:Robin Kaye, MD; Carrie Schaefer, MD; DaRichard Towbin, MD

Institution:Phoenix Children’s Hospital, Phoenix, AZ


2/27

CHIEF COMPLAINT & HPI

▪15 year old healthy male with left flank pain and dizziness after a collisioanother player during a basketball game.

▪No relevant past medical or surgical history.


3/27

INITIAL WORK-UP

▪Physical exam:▪Diffuse left flank tenderness withoutecchymosis.

▪BP 113/65

▪HR 66

▪Labs:

▪Hemoglobin 12.9 g/dL

▪Hematocrit 37.9

▪CT abdomen/pelvis:

▪Left perinephric and retroperitonealhemorrhage (Highlighted red).

▪Enhancing 7.5 cm retroperitoneal masswith hypodense center (Yellow arrows).


4/27

INITIAL WORK-UP

▪Staging PET scan:▪Retroperitoneal mass – SUVmax 15.6

▪FDG avid mets:

▪Skull

▪Thoracic spine

▪Right humerus

▪Differential Diagnosis:▪Lymphoma

▪Metastasis (Testicular)

▪Retroperitoneal Sarcoma

▪Paraganglioma

▪Neuroblastoma


5/27

ULTRASOUND GUIDED BIOPSY

▪Prone position, posterior approach

▪16 G coaxial, end-cut core needlebiopsy

Lt kid.

Mass

Left Retroper


6/27

PATHOLOGY

B

A - Nests of Zellballen (arrows) separated by fibrovascular septa with focal nuclear pleomorphism and hyperchroma

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, M

PA(ASCP)

A


7/27

DIAGNOSIS

▪Malignant Paraganglioma (PGL)▪Malignancy defined by presence of metastases , not histology.1

▪Genetic testing: Patient and family positive for SDH-B gene, one ofseveral familial PGL syndrome genes.

▪Usually asymptomatic (silent) and non-functional.2

▪Spontaneous hemorrhage as a presenting feature of both PHEO and PGL israre but well-described and can be life-threatening.

▪Low-grade malignancy often with extended survival.

▪Most cases of extra-adrenal PGL present with episodic adrenergicsymptoms (headache, sweating, palpitations, hypertension).2


8/27

PATHOGENESIS

▪

PGL’s can arise from anywhere in the paraganglionic system throughout the bo▪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 importaprognostic and treatment differences.

▪

PGL’s: up to 50% malignant.▪PHEO’s: 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.


9/27

QUESTION

Which of the following is FALSE regarding PHEO’s and PGL’s?

A. Use of IV contrast in the imaging and intervention of PHEO/PGL can trimalignant hypertension.

B. Biopsy can trigger malignant hypertension.

C. Pre-operative embolization should be considered to aid surgical hemo

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.


10/27

CORRECT!


A. Use of IV contrast in the imaging and intervention of PHEO/PGL can trigger malignant hypFALSE. This myth has been propagated from 1984 when a study showed 5 of 8 patients had circulating catecholamines after IV contrast 4 , however multiple recent studies have found bcatecholamines are not significantly increased and patients do not experience increased adsymptoms after non-ionic IV contrast.5,6

B. Biopsy can trigger malignant hypertension. TRUE. Although rare in head/neck paragangliommasses contain high concentrations of catecholamines that can be released into the bloodsany manipulation. If biochemical testing for urine metanephrines is positive, pre-proceduraadrenoceptor pharmaceutical blockade is paramount.7

C. Pre-operative embolization should be considered to aid surgical hemostasis and provide a vmap. 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/PGneuroendocrine malignancies can be treated with radiopharmaceuticals. This is a highly spetreatment with limited availability.

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

CONTINUE WITH CASE


11/27

SORRY, THAT’S INCORRECT


A. Use of IV contrast in the imaging and intervention of PHEO/PGL can trigger malignant hypFALSE. This myth has been propagated from 1984 when a study showed 5 of 8 patients had circulating catecholamines after IV contrast 4 , however multiple recent studies have found bcatecholamines are not significantly increased and patients do not experience increased adsymptoms after non-ionic IV contrast.5,6

B. Biopsy can trigger malignant hypertension. TRUE. Although rare in head/neck paragangliommasses contain high concentrations of catecholamines that can be released into the bloodsany manipulation. If biochemical testing for urine metanephrines is positive, pre-proceduraadrenoceptor pharmaceutical blockade is paramount.7

C. Pre-operative embolization should be considered to aid surgical hemostasis and provide a vmap. 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/PGneuroendocrine malignancies can be treated with radiopharmaceuticals. This is a highly spetreatment with limited availability.

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

CONTINUE WITH CASE


12/27

IMAGING FEATURES OF PHEO’S AND PGL

▪Solid, highly vascular tumor with varied appearances.

▪Larger tumors may calcify and develop cystic andnecrotic areas.

▪Intense, early contrast enhancement with flow voidson MRI.

▪

PET imaging now thought to be superior to MIBG fordiagnosis, staging and follow-up.


13/27

CLINICAL COURSE

▪Follow-up CT showed resolution ofhemorrhage.

▪Patient started on alpha and betablockade (doxazosin, atenolol).

▪Mass resected. Extremely difficultdissection of tumor off blood vessels

and proximal ureter.

▪Blood loss: 500 mL.

LINK TO VIDEO

https://youtu.be/Ciw-v46jlJUhttps://youtu.be/Ciw-v46jlJU


14/27

CLINICAL COURSE

▪Chemotherapy and radiation.

▪T1 corpectomy with C5-T4 fusion.

▪1-month follow-up: US showed a largewell-circumscribed infrarenalretroperitoneal fluid collection and lefthydronephrosis.

kidney


15/27

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 urin

D. Multiphase CT abdomen/pelvis.

E. Urology consultation.


16/27

SORRY, THAT’S INCORRECT




C. Percutaneous nephrostomy followed by percutaneous drainage of uri

D. Multiphase CT abdomen/pelvis. CORRECT. Prior to intervention, a dela phase CT will definitively diagnose urinoma and possibly identify the loureteral injury for treatment planning.9


CONTINUE WITH CASE


17/27

CORRECT!




C. Percutaneous nephrostomy followed by percutaneous drainage of uri

D. Multiphase CT abdomen/pelvis. CORRECT. Prior to intervention, a dela phase CT will definitively diagnose urinoma and possibly identify the loureteral injury for treatment planning.9


CONTINUE WITH CASE


18/27

CLINICAL COURSE

▪Follow-up CT:▪Moderate left hydronephrosis

▪15 cm fluid collection

▪Non-distended distal left ureter

▪On 15-min delay, IV contrast isseen excreted into collection,confirming suspected urinoma

▪Dilated proximal ureterconnects to urinoma (yellowarrow) identifying site of injury

▪Non-opacified distal ureter


19/27

INTERVENTION

▪Percutaneous nephrostomy▪Contrast injection confirms a highproximal ureteral injury and freeflowing contrast into the urinoma.

▪Drainage of urinoma

Site of

injury

Urinoma drain


20/27

URETERAL REGENERATION

▪Davis Intubated Ureterotomy (Urologist)▪1943 – published initial report of use of intubatedureterotomy for repair of UPJ obstruction in humans.

▪Stent allows reconstitution of an adequate tubularlumen and prevents leakage of urine that would incitefibrosis.10

▪

3 steps of healing

11

▪Fibrosis and urothelium fill in the gap.

▪Scar retraction brings smooth muscle edges inproximity.

▪Regeneration of smooth muscle by pluripotentfibroblasts. Figure cour


21/27

PLANNING URETERAL REPAIR

▪Retrograde ureterogram showed extravasation;no contrast seen extending proximal to the injury.

▪Urologist could not place stent across site ofinjury.


22/27

INTERVENTION

▪Urologist obtainedretrograde ureteral access.

▪0.035” angled Glidewire*advanced through proximalureteral defect.

*Glidewire,Terumo, Somerset, NJ, USA


23/27

INTERVENTION

▪Proximal wire captured bysnare* advanced through a45 cm 9-French sheath*.

▪Snare and wire pulled downureter and out urethra.

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

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


24/27

INTERVENTION

▪Double J ureteral stent placed overwire through urethra.

▪Urinoma drainage catheterremained.

▪Nephrostomy placed.

▪

Alternative approaches:▪Snare may be passed antegradethrough the kidney.

▪Nephroureteral catheter may be usedin place of the nephrostomy andureteral stent.


25/27

FOLLOW-UP

▪6-week follow-up retrograde ureterogram shows a long-segment strictusite of ureteral injury but no further extravasation and no hydronephrosi

▪Patient currently requiring monthly ureteral stent exchanges.

▪Monthly denosumab (Xgeva) chemotherapy infusions.

▪After cancer therapy is finished, urologist plans to evaluate for possible f

endoscopic ureteral stricturoplasty versus definitive surgical repair if nee


26/27

SUMMARY & TEACHING POINTS

▪There is a high risk of morbidity/mortality when an unrecognized PHEO ofunctional PGL is manipulated.

▪Alpha/beta blockade should be given for all PHEO’s and all functioning Pbefore any manipulation of the tumor.

▪Pre-operative embolization prior to surgical excision should be considerecase by case basis.

▪The unique regenerative property of the ureter allows stenting alone to many ureteral injuries.


27/27

REFERENCES

1. Kimura N. et al. Pathological grading for predicting metastases in pheochromocytoma and paraganglioma. 2014 Feb 21(3): 405-4

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

3. Tischler A. et. al. Pheochromocytoma and Extra-adrenal Paraganglioma: Updates. Archives of Pathology & Laboratory Medicine: ANo. 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.31.

6. Bessell-Browne R. et al. CT of pheochromocytoma and paraganglioma: risk of adverse events with IV administration of non-ionic

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. Ma

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

dance_davis intubated ureterotomy in a child_05202016

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