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Outcomes of Laparoscopic Nephrectomy with Renal Autotransplantation for The Loin Pain-Hematuria Syndrome
Wisit Cheungpasitporn
May 2016
Disclosure• None
Burke JR. Pediatr Nephrol. 1996 Apr;10(2):216-20
Causes of Loin Pain- Obstructing nephrolithiasis
- Autosomal dominant polycystic kidney disease
- Renal cell carcinoma
- Recurrent renal thromboembolism, as with atrial fibrillation
Less common causes include:
- Endometriosis
- Left renal vein entrapment (nutcracker syndrome)
EPIDEMIOLOGY• < 500 cases reported to date
• An extremely rare disease (prevalence ~0.012%)
• First report 3 women in 1967
• 70% were women
• Almost all Caucasian
• Common in late 20s (range 10 - 60 years)
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Signs &Symptoms in LPHS
• Location: unilateral or bilateral (less often) flank
• Radiation: abdomen, medial thigh or groin
• Characteristic: constant severe stabbing or dull aching pain
• Duration: hours
• Aggravating factor: gentle punch, exercise
• Associated findings: • Intermittent microscopic or gross hematuria• Nausea and vomitting• low-grade fever and dysuria (rare)• UTI is not present
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Hematuria in LPHS• Dysmorphic RBCs
• Microscopic, but most patients experienced at least 1 episode of gross hematuria
• Gross hematuria - almost always accompanied by worsening pain, usually last a few days, but gross hematuria and pain can persist for weeks to months
• Between episodes of gross hematuria, UA typically shows microscopic hematuria; however, sometimes the hematuria clears up but the pain persists
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Kidney function in LPHS• Mean serum creatinine 0.9 mg/dL• Protein excretion
• > 150 mg/d in 32%• > 500 mg/d in 6% • Maximum 1,635 mg/d
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Diagnosis• Diagnosis of exclusion
Weisberg LS. Am J Nephrol. 1993;13(4):229-37.
Investigation: LPHS
Kidney Biopsy• Warranted if underlying acquired glomerular disease is
suspected.
• Renal biopsy in patients with primary LPHS • LM or IF
• normal glomeruli• RBC or RBC casts are present in the tubules.
• EM• thin or thick glomerular basement membranes
are noted in approximately 60 percent of specimens.
Kidney Biopsy- LPHS
Spetie DN et al.. Am J Kidney Dis 47(3):419-427, 2006.
7.2% vs 1.6%; P< 0.0001
PATHOPHYSIOLOGY- LPHS
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Spetie DN et al.. Am J Kidney Dis 47(3):419-427, 2006.
PSYCHOLOGICAL MANIFESTATIONS• Psychogenic in nature
• Somatoform pain disorder • Drug-seeking behavior
• LPHS is a medical disorder and the psychiatric symptoms are a result of the accompanying pain rather than its cause
• A psychiatric evaluation is required to identify psychological issues potentially associated with pain to avoid improper surgical intervention
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Treatment• ACE inhibitors/ARBs:
• Reduce frequency/severity of hematuria and pain by reducing glomerular hydrostatic pressure
• Case series:• 4 of 7 patients with LPHS treated with enalapril for 7-48
months had fewer/less severe episodes
• A metabolic stone workup • Chronic and acute pain control:
• Non-opioid therapy should be attempted first
Hebert LA. Kidney Int. 1996;49(1):168Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
Blacklock AR. Br J Urol. 1989 Aug;64(2):203-4.
Surgical Renal Denervation
Sheil AG. Am J Kidney Dis. 1998 Aug;32(2):215-20.
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.
LAPAROSCOPIC RETROPERITONEAL LIVE DONOR RIGHT NEPHRECTOMY
Gill IS. J Urol. 2000 Nov;164(5):1500-4.
LAPAROSCOPIC RETROPERITONEAL LIVE DONOR RIGHT NEPHRECTOMY
Gill IS. J Urol. 2000 Nov;164(5):1500-4.
• Gill et al. was the first to report the use of laparoscopic nephrectomy for kidney autotransplantation in 2 patients with LPHS, with both experiencing successful surgical outcomes.
Eisenberg ML. J Urol. 2008 Jan;179(1):240-3.
A total of 15 patients underwent autotransplantation for complex ureteral stricture disease and 4 underwent it for renal tumors. Median follow-up was 29 months (range up to 64).
Clinical and Safety Outcomes of Laparoscopic Nephrectomy with Renal Autotransplantation for The Loin Pain-Hematuria Syndrome: A 14-year Longitudinal Study
Adeel S. Zubair, BS, Wisit Cheungpasitporn, MD,
Stephen B Erickson, MD, Mikel Prieto, MD
http://onlinelibrary.wiley.com/doi/10.1111/jebm.12199/abstractPMID: 27186938
Conclusion• Pain control and Quality of life is better after
renal autotransplantation by laparoscopic hand-assisted nephrectomy
• Despite postoperative risk, renal autotransplant seems to be safe for survival and renal outcomes
Kidney Autotransplantation• Selection criteria should include
• patients with severe pain requiring high doses of analgesics to control their pain
• those for whom extensive nonsurgical therapies have been unsuccessful
Questions & Discussion
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