{"paper_id":"1cedb46f-ac8e-4651-a055-1751809f9097","body_text":"Loin\npain hematuria syndrome (LPHS) describes a constellation of clinical features\nincluding recurrent flank pain and intermittent hematuria without a readily\nidentifiable cause [ 1 ]. Historically, LPHS has been associated\nwith psychiatric illness and is now widely considered a type of somatoform pain\ndisorder [ 2 ,  3 ]. \nMultidisciplinary pain management strategies include analgesics, nerve\nblockade, renal denervation, and/or nephrectomy with autotransplantation [ 4 ]. Though some studies suggest\nthat long-term prognosis is usually excellent [ 4 ], many urologists and patients would contest this.\n\nTwo patients with severe chronic flank pain punctuated by\nintermittent gross hematuria were evaluated after laboratory and radiographic\nevaluation failed to delineate a recognizable pathology, raising the suspicion\nof a diagnosis of LPHS. Patients\nunderwent ureteroscopic evaluation using a no-touch technique. Ureteroscopic\nevaluation was performed on the side where the patient reported pain, and/or\nbloody efflux was noted from the ureteral orifice.\nAfter informed consent and induction of general\nanesthesia, visual inspection of the bladder was performed using a 19 Fr Rigid\ncystoscope. Subsequently, a 6/7.5 Fr Wolf semirigid ureteroscope was inserted\nand the ureteral orifice of the symptomatic side cannulated without the use of\na guidewire. The ureteroscope was\nadvanced under direct vision with the use of minimal hand irrigation of saline,\nutilizing a Boston Scientific single-action pump. Semirigid ureteroscopy was utilized to the\nlevel of the proximal ureter. A Boston\nScientific sensor guidewire (0.035”) was advanced through the working channel\nto the tip of the ureteroscope and the ureteroscope was then withdrawn, using\nintermittent fluoroscopy to confirm that the guidewire did not advance beyond\nthe point of ureteroscopic inspection. \nThe Olympus URF-P3 flexible ureteroscope was then backloaded over the\nfloppy-tip of the sensor wire and advanced under fluoroscopic guidance to the\npoint of last inspection, following which the guidewire was removed. Systematic inspection of the entire\ncollecting system was then performed with the flexible ureteroscope, taking\ncare to utilize minimal irrigation throughout the procedure. A Holmium laser was kept on stand-by in the\nevent that pathology was to be identified and could be successfully ablated. Following completion of the procedure, the\nureter was inspected as the ureteroscope was withdrawn to evaluate for injury,\nand as none was identified a ureteral stent was not left in place.\n\nPatient\n1 is a 26-year-old female with a 3-year history of severe right flank pain and\ngross hematuria beginning in 2003. Her\npain was described as stabbing and located in the lower back and right flank\nwith radiation to the lower abdomen. She\nreported episodes of intense pain every three to four months that lasted 2–4 weeks, with a\nnotable basal level of pain in between exacerbations. The patient underwent ureteral stent\nplacement and ureteroscopic extraction of a small stone following a bout of\npain in 2004, and following this, had CT scans with and without contrast that\ndid not demonstrate any stones, masses, or hydronephrosis. The patient presented in early 2005 with a\nrecurrence of symptoms including pain, fever, and microscopic hematuria. A CT scan with and without IV contrast\nobtained at this time did not demonstrate any evidence of renal calculi,\nhydronephrosis, or renal mass. With\nnormal imaging studies, the patient was treated with IV antibiotics with\neventual relief of symptoms. In late\n2005, the patient was admitted to the hospital with severe right flank pain,\nfever, and gross hematuria. Her urine\nspecimen contained large blood without organisms, signs of infection, or\npositive culture or cytology. A\nnephrology consult was obtained for a nephritis workup revealing a normal serum\ncomplement and ANCA screen, a negative Hepatitis C antibody, and negative\nantiglomerular basement antibody test. \nFurther studies included an unremarkable CT angiogram of the kidney to\nevaluate for AV malformation. The\ndecision then made to perform bilateral diagnostic ureteroscopy during this episode\nof pain and gross hematuria. Right rigid\nand flexible ureteroscopy was performed, using a wireless, no-touch technique\nwith minimal irrigation fluid. Diffuse\nbleeding in a pattern of ring-like clots was noted from each fornix surrounding\nall calyces of the collecting system, without any identifiable masses or\nneoplasia (see Supplementary Video in Supplementary \nMaterial available online at doi:10.1155/2009/721850). The patient received an\ninpatient pain management consultation and was maintained on Neurontin 300 mg\nthree times daily and Dilaudid 4–8 mg every four to\nsix hours. The patient is currently\nseeking counseling regarding more aggressive treatments such as nephrectomy or\nautotransplantation.\nPatient 2 is an 18-year-old female with a 2-year history\nof severe left flank pain with intermittent gross hematuria. The patient presented in 2005 to an outside\ninstitution and underwent ureteroscopic evaluation for her flank pain. A small hemangioma was reportedly identified\nand cauterized during this evaluation. \nAdditionally, the patient was noted to have low-grade vesicoureteral\nreflux, and a deflux procedure was performed in 2006 with alleviation of pain\nassociated with voiding. However, pain\nnot associated with voiding persisted, and the patient underwent laparoscopic\nevaluation by gynecology in 2006. This\nshowed the presence of a Meckel's diverticulum and several small spots of\nendometriosis, and the patient underwent diverticulectomy, interval\nappendectomy, and fulguration of the endometriosis. The patient continued to have intermittent\nbouts of severe left flank pain, often requiring ER visits and significant\namounts of IV narcotics. The patient\nunderwent several CT scans with and without IV contrast that did not\ndemonstrate any evidence of renal calculi, masses, or hydronephrosis.\nAn outpatient renal consult was requested, and a full\nunremarkable work up for her hematuria was performed, including urine\nmicroscopy and cytology, CT angiogram, 24-hour urine collection studies, and\nnegative nephritis work up. She then\nunderwent left ureteroscopic evaluation utilizing the wireless, no-touch\ntechnique, which again demonstrated a similar pattern of diffuse bleeding in a\npattern of ring-like clots from each fornix surrounding all calyces of the\ncollecting system of the left kidney. \nThe patient received an inpatient pain management consultation and was\nmaintained on Levsin 0.25 mg every 24 hours, a 50 mcg Fentanyl patch every 72\nhours, Dilaudid 8 mg every four hours, Neurontin 300 mg three times daily, and\nFlexeril 10 mg three times daily. The\npatient eventually elected to undergo left-sided nephrectomy with\nautotransplantation into the right lower quadrant of her abdomen with\nresolution of her symptoms. The patient\nhas since developed right flank pain suggestive of LPHS in her contralateral\nkidney, and has undergone intrathecal catheter placement, epidural catheter\nplacement, and most recently a right lower quandrant fascial pain block.\n\nThough some studies suggest that long-term prognosis for\nLPHS is excellent [ 4 ], many urologists and LPHS patients would contest\nthis. Rates of spontaneous resolution of pain symptoms in LPHS have been\nreported to typically occur over a 2–5-year period [ 5 ]. \nFor those without pain resolution, prognosis can be poor and treatment may involve\na multidisciplinary approach with both pain management services (chronic analgesics,\nnerve blockade, open or laparoscopic renal denervation, and/or nephrectomy with\nautotransplantation [ 4 ]) and psychiatric evaluation (LPHS is considered\nby some to be a type of somatoform pain disorder [ 2 ,  3 ]).\nThe\npathogenesis of flank pain associated with LPHS is thought to be due to glomerular\ncapillary hemorrhage resulting in tubular obstruction and interstitial edema [ 6 ]. \nOver time, chronic edema is thought to result in capsular distention, pain, and\nfurther tubular obstruction and hemorrhage. To our knowledge, we are the first\nto report the endoscopic findings of LPHS. Both LPHS patients underwent\ndiagnostic ureteroscopy using minimal irrigation fluid (to avoid distension of\nthe renal pelvis) and a wireless, no-touch technique (to rule out iatrogenic,\ntraumatic cause of bleeding). In both patients, ring-like clots consistent with\nrecent hemorrhage were identified from each fornix surrounding all calyces of\nthe collecting system (Figures  1(a)  and  1(b) ). \nBecause diagnosis is the first step in understanding pathophysiology, we\nhypothesize that these endoscopic findings may represent a clinical, endoscopic\nmanifestation of glomerular hemorrhage and obstruction that has not been\npreviously reported.\nIt is\nimportant to realize that both LPHS patients underwent unremarkable full\nwork-up, with CT urogram, CT angiography, nephrology consultation, and\nlaboratory investigation. All other treatable causes of pain and hematuria must\nbe ruled out in these cases. Once excluded, however, upper tract endoscopy\ndemonstrating hematuria and concentric calyceal clots may strengthen the\ndiagnosis of LPHS and confirm the presence of a physiological disorder in this\npoorly understood disease. This\nobjective confirmation of disease may result in more definitive diagnoses of\nLPHS, though a larger series is needed to confirm the results. Such supportive evidence is critical as more\naggressive pain management and/or surgical approaches are considered by the\npatient and family.\n\nThe Supplementary Video shows diffuse bleeding in a pattern of ring-like clots that was noted from each fornix surrounding all calyces of the collecting system, without any identifiable masses or neoplasia.\nClick here for additional data file.","source_license":"CC-BY-4.0","license_restricted":false}