# Endoscopic Findings in Loin Pain Hematuria Syndrome: Concentric Clot in Calyceal Fornices

Authors: Benjamin K. Canales; Andrew Windsperger; Stephen Lukasewycz; Manoj Monga
Journal: Diagnostic and Therapeutic Endoscopy
 (2008)

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## Abstract

The loin pain hematuria syndrome (LPHS) creates a considerable burden, both for patients afflicted with the disease and for those involved in medical management and diagnosis.  To date, the diagnosis of LPHS remains one of exclusion, with some speculation regarding the extent of actual pathology.  We report ureteroscopic findings in 2 cases of LPHS.  These findings provide objective confirmation of underlying pathology in a difficult-to-manage disease process.


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## Body

## 1. Introduction

Loin
pain hematuria syndrome (LPHS) describes  a constellation of clinical features
including recurrent flank pain  and intermittent hematuria without a readily
identifiable cause [1]. Historically, LPHS has been associated
with psychiatric illness and is now widely considered a type of somatoform pain
disorder [2, 3].
Multidisciplinary pain management strategies include analgesics, nerve
blockade, renal denervation, and/or nephrectomy with autotransplantation [4]. Though some studies suggest
that long-term prognosis is usually excellent [4], many urologists and patients would contest this.


## 2. Methods

Two patients with severe chronic flank pain punctuated by
intermittent gross hematuria were evaluated after laboratory and radiographic
evaluation failed to delineate a recognizable pathology, raising the suspicion
of a diagnosis of LPHS.  Patients
underwent ureteroscopic evaluation using a no-touch technique. Ureteroscopic
evaluation was performed on the side where the patient reported pain, and/or
bloody efflux was noted from the ureteral orifice.After informed consent and induction of general
anesthesia, visual inspection of the bladder was performed using a 19 Fr Rigid
cystoscope. Subsequently, a 6/7.5 Fr Wolf semirigid ureteroscope was inserted
and the ureteral orifice of the symptomatic side cannulated without the use of
a guidewire.  The ureteroscope was
advanced under direct vision with the use of minimal hand irrigation of saline,
utilizing a Boston Scientific single-action pump.  Semirigid ureteroscopy was utilized to the
level of the proximal ureter.  A Boston
Scientific sensor guidewire (0.035'') was advanced through the working channel
to the tip of the ureteroscope and the ureteroscope was then withdrawn, using
intermittent fluoroscopy to confirm that the guidewire did not advance beyond
the point of ureteroscopic inspection.
The Olympus URF-P3 flexible ureteroscope was then backloaded over the
floppy-tip of the sensor wire and advanced under fluoroscopic guidance to the
point of last inspection, following which the guidewire was removed.  Systematic inspection of the entire
collecting system was then performed with the flexible ureteroscope, taking
care to utilize minimal irrigation throughout the procedure.  A Holmium laser was kept on stand-by in the
event that pathology was to be identified and could be successfully ablated.  Following completion of the procedure, the
ureter was inspected as the ureteroscope was withdrawn to evaluate for injury,
and as none was identified a ureteral stent was not left in place.


## 3. Results

Patient
1 is a 26-year-old female with a 3-year history of severe right flank pain and
gross hematuria beginning in 2003.  Her
pain was described as stabbing and located in the lower back and right flank
with radiation to the lower abdomen.  She
reported episodes of intense pain every three to four months that lasted 2–4 weeks, with a
notable basal level of pain in between exacerbations.  The patient underwent ureteral stent
placement and ureteroscopic extraction of a small stone following a bout of
pain in 2004, and following this, had CT scans with and without contrast that
did not demonstrate any stones, masses, or hydronephrosis.   The patient presented in early 2005 with a
recurrence of symptoms including pain, fever, and microscopic hematuria.  A CT scan with and without IV contrast
obtained at this time did not demonstrate any evidence of renal calculi,
hydronephrosis, or renal mass.  With
normal imaging studies, the patient was treated with IV antibiotics with
eventual relief of symptoms.  In late
2005, the patient was admitted to the hospital with severe right flank pain,
fever, and gross hematuria.  Her urine
specimen contained large blood without organisms, signs of infection, or
positive culture or cytology.  A
nephrology consult was obtained for a nephritis workup revealing a normal serum
complement and ANCA screen, a negative Hepatitis C antibody, and negative
antiglomerular basement antibody test.
Further studies included an unremarkable CT angiogram of the kidney to
evaluate for AV malformation.  The
decision then made to perform bilateral diagnostic ureteroscopy during this episode
of pain and gross hematuria.  Right rigid
and flexible ureteroscopy was performed, using a wireless, no-touch technique
with minimal irrigation fluid.  Diffuse
bleeding in a pattern of ring-like clots was noted from each fornix surrounding
all calyces of the collecting system, without any identifiable masses or
neoplasia (see Supplementary Video in Supplementary
Material available online at doi:10.1155/2008/721850).  The patient received an
inpatient pain management consultation and was maintained on Neurontin 300 mg
three times daily and Dilaudid 4–8 mg every four to
six hours.  The patient is currently
seeking counseling regarding more aggressive treatments such as nephrectomy or
autotransplantation.Patient 2 is an 18-year-old female with a 2-year history
of severe left flank pain with intermittent gross hematuria.  The patient presented in 2005 to an outside
institution and underwent ureteroscopic evaluation for her flank pain.  A small hemangioma was reportedly identified
and cauterized during this evaluation.
Additionally, the patient was noted to have low-grade vesicoureteral
reflux, and a deflux procedure was performed in 2006 with alleviation of pain
associated with voiding.  However, pain
not associated with voiding persisted, and the patient underwent laparoscopic
evaluation by gynecology in 2006.  This
showed the presence of a Meckel’s diverticulum and several small spots of
endometriosis, and the patient underwent diverticulectomy, interval
appendectomy, and fulguration of the endometriosis.  The patient continued to have intermittent
bouts of severe left flank pain, often requiring ER visits and significant
amounts of IV narcotics.  The patient
underwent several CT scans with and without IV contrast that did not
demonstrate any evidence of renal calculi, masses, or hydronephrosis.An outpatient renal consult was requested, and a full
unremarkable work up for her hematuria was performed, including urine
microscopy and cytology, CT angiogram, 24-hour urine collection studies, and
negative nephritis work up.  She then
underwent left ureteroscopic evaluation utilizing the wireless, no-touch
technique, which again demonstrated a similar pattern of diffuse bleeding in a
pattern of ring-like clots from each fornix surrounding all calyces of the
collecting system of the left kidney.
The patient received an inpatient pain management consultation and was
maintained on Levsin 0.25 mg every 24 hours, a 50 mcg Fentanyl patch every 72
hours, Dilaudid 8 mg every four hours, Neurontin 300 mg three times daily, and
Flexeril 10 mg three times daily.  The
patient eventually elected to undergo left-sided nephrectomy with
autotransplantation into the right lower quadrant of her abdomen with
resolution of her symptoms.  The patient
has since developed right flank pain suggestive of LPHS in her contralateral
kidney, and has undergone intrathecal catheter placement, epidural catheter
placement, and most recently a right lower quandrant fascial pain block.


## 4. Discussion

Though some studies suggest that long-term prognosis for
LPHS is excellent [4], many urologists and LPHS patients would contest
this. Rates of spontaneous resolution of pain symptoms in LPHS have been
reported to typically occur over a 2–5-year period [5].
For those without pain resolution, prognosis can be poor and treatment may involve
a multidisciplinary approach with both pain management services (chronic analgesics,
nerve blockade, open or laparoscopic renal denervation, and/or nephrectomy with
autotransplantation [4]) and psychiatric evaluation (LPHS is considered
by some to be a type of somatoform pain disorder [2, 3]).The
pathogenesis of flank pain associated with LPHS is thought to be due to glomerular
capillary hemorrhage resulting in tubular obstruction and interstitial edema [6].
Over time, chronic edema is thought to result in capsular distention, pain, and
further tubular obstruction and hemorrhage. To our knowledge, we are the first
to report the endoscopic findings of LPHS. Both LPHS patients underwent
diagnostic ureteroscopy using minimal irrigation fluid (to avoid distension of
the renal pelvis) and a wireless, no-touch technique (to rule out iatrogenic,
traumatic cause of bleeding). In both patients, ring-like clots consistent with
recent hemorrhage were identified from each fornix surrounding all calyces of
the collecting system (Figures 1(a) and 1(b)).
Because diagnosis is the first step in understanding pathophysiology, we
hypothesize that these endoscopic findings may represent a clinical, endoscopic
manifestation of glomerular hemorrhage and obstruction that has not been
previously reported.Ureteroscopic images demonstrating ring-like clots surrounding each calyx in the collecting system.
(a)(b)It is
important to realize that both LPHS patients underwent unremarkable full
work-up, with CT urogram, CT angiography, nephrology consultation, and
laboratory investigation. All other treatable causes of pain and hematuria must
be ruled out in these cases. Once excluded, however, upper tract endoscopy
demonstrating hematuria and concentric calyceal clots may strengthen the
diagnosis of LPHS and confirm the presence of a physiological disorder in this
poorly understood disease.  This
objective confirmation of disease may result in more definitive diagnoses of
LPHS, though a larger series is needed to confirm the results.  Such supportive evidence is critical as more
aggressive pain management and/or surgical approaches are considered by the
patient and family.



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