A Case of Colonoscopy-induced
Wunderlich’s syndrome in a hemodialysis patient: Coincidence or Link?
Authors:
Antoine Jean Zgheib: MD; Department of Nephrology, N D Maritime
Hospital, Jubail, Lebanon.
Elias Gerges Mansour: MD; Department of Urology, N D Maritime Hospital,
Jubail and St George Hospital University Medical Center, Beirut,
Lebanon.
Joe Nohra Nohra: MD; Department of Urology, N D Maritime Hospital,
Jubail and St George Hospital University Medical Center, Beirut,
Lebanon.
Abstract
Wunderlich syndrome is a rare condition encountered in patients
undergoing chronic hemodialysis (HD) usually attributed to acquired
cystic kidney disease (ACKD) among other causes. However, presentation
of renal hemorrhage after colonoscopy, to the best of our knowledge, has
not been previously documented. Nevertheless,
only splenic injury was rarely
mentioned. Management can range from conservative treatment to
angiographic embolization or exploration and nephrectomy. We report an
unusual case of a 54-year-old woman HD patient who presented with SRH
within a few days of colonoscopy.
The reason of spontaneous renal hemorrhage (SRH) was rupture of a cyst
of ACKD. We assumed that colonoscopy was a provoking factor and
elaborated hypotheses for its
etiopathogenesis. The patient underwent left successful nephrectomy. The
importance of this case lies in the fact that colonoscopy is not always
an innocent procedure in HD patients, and could be complicated by renal
cyst hemorrhage.
Keywords: Spontaneous renal hemorrhage, Hemodialysis, Acquired cystic
renal disease, Colonoscopy.
Introduction
Wunderlich syndrome, also known as SRH is a rare and potentially fatal
condition in patients undergoing hemodialysis (HD) that is attributed to
acquired cystic kidney disease (ACKD) among other plentiful causes most
commonly being angiomyolipoma and renal cell carcinoma (RCC) while
vascular entities such as polyarteritis nodosa and Wegener polyangiitis
being rare.1 Most published cases following
colonoscopy discuss splenic rupture as unusual complication, and may
present in 1-21/100000 of cases.2 To the best of our
knowledge SRH has never been described before as a complication of renal
cyst rupture in ACKD after colonoscopy. We describe a HD patient with
ACKD who developed SRH after colonoscopy along with a discussion of the
contributing factors and highlight that HD patients should be under
close surveillance after gastrointestinal endoscopy. We report this case
because of its rarity and significance with respect to being a
complication in a dialysis patient.
Case presentation
A 54-year-old Lebanese woman, on maintenance hemodialysis (HD) twice
weekly for focal segmental glomerulosclerosis (FSGS) was admitted to the
emergency department, after she had undergone her scheduled hemodialysis
the day before, for sudden onset of severe left flank pain, nausea, and
vomiting. The pain had started suddenly about 12 hours prior to
presentation and worsened rapidly. On a side note, a severe secondary
hyperparathyroidism with cutaneous calciphylaxis was treated by
cinacalcet for 3 years without response required parathyroidectomy (four
glands hyperplasia) 8 months before her current admission. Five days
prior to presentation, she was admitted to the hospital because of
severe anemia and rectorrhagia. Her renal ultrasound evaluation showed
bilaterally small kidneys with thinned parenchyma containing cysts up to
3 cm and vascular calcifications without hydronephrosis. Colonoscopy
through terminal ileum revealed a 7 mm polyp in the left descending
colon which was removed by mucosectomy. Microscopy returned as sessile
tubular adenoma with low-grade dysplasia. The patient denied fever or a
history of trauma. There was no previous history of antiplatelet use. In
the emergency department, her blood pressure and pulse rate were
112/65mmHg and 96 bpm, her temperature was 36.8∘C and
she had mildly distended abdomen with severe left costovertebral angle
tenderness. Laboratory findings showed Hemoglobin 9.0 gm/dL while
hematocrit was 29.7%, total leucocyte count 12.2 × 1000/microliter
creatinine: 6.06mg/dL); C-Reactive Protein level: 6 mg/L.. In addition,
PT was 83% and INR was 1.13. A multiphasic computed tomography (CT)
scan of the thorax, abdomen and pelvis was performed without and with
intravenous contrast. The scan revealed a large cyst and huge distorting
left perirenal intraparenchymal hematomas with a subcapsular collection
up to 1.8 cm thick with hematic collection throughout the renal
compartment reaching a diameter of more than 9x4 cm, left hydronephrosis
and edema surrounding fat throughout the flank and calcifications in the
hilum (Figure 1). For decompression of the renal pelvicalyceal system
and drainage, we chose to treat her with retrograde left double-J
ureteral stent insertion which identified a clot in the left mid-ureter
and urine culture and cytology were taken and were negative. Plain film
of abdomen showed the double J in place (Figure 2). The patient remained
hemodynamically stable but with further drop in hemoglobin (6.1 g/dL)
the same evening, mandates a transfusion of one unit of leuco-depleted
red cell concentrate for stabilization. A decision was made for surgical
exploration with presumptive diagnosis of subacute left perirenal
hematoma and for exclusion of underlying malignancy. She underwent a HD
session without heparin. On day 3, she underwent open radical left
nephrectomy with conservation of the adrenal gland. She received 2 more
units of leuco-depleted red cell concentrate packed during the
intervention. The renal parenchyma is almost entirely replaced by a
hematoma measuring 9x9x7 cm and extending into the peri-renal fat. A
small papillary mass was sent separately. The detailed histopathological
evaluation revealed a renal parenchyma with lesions of chronic nephritis
mutilated by large hemorrhagic areas which extend to the peri-renal fat.
In addition, there was a neoplastic proliferation, measuring 2 mm, with
papillary architecture covered by little atypical cubic cells and foamy
macrophages in the axis compatible with papillary adenoma. On next
operative day the patient underwent HD and received a pack of
leuco-depleted red blood cells. The post-operative period was
uneventful. Hemoglobin level remained around 10 g/dL. She was discharged
on the seventh day without any bleeding sign or symptoms. She remains
well and asymptomatic and is meanwhile on regular maintenance HD.
Discussion
Since it was first described in 1856, spontaneous renal hemorrhage (SRH)
has been reported infrequently in the literature, where dialysis
patients are often excluded. Classic symptoms, such as acute flank pain,
flank mass, and signs of internal bleeding, known as Lenk’s triad, have
limited sensitivity and specificity for detecting
SRH.3Nowadays, computed tomography (CT) is the method
of choice for the diagnosis. Most frequent etiologies are renal cysts,
benign and malignant renal tumors, vascular lesions, and antiplatelet or
anticoagulant therapy.3
ACKD is a well-known late stage complication of end-stage renal disease
(ESRD). It can develop in up to 74% of patients with end-stage renal
failure undergoing chronic HD lasting more than four
years.3 The prevalence of ACKD is directly related to
the duration of dialysis and cysts tend to grow with time. Numerous
complications have been described in relation to this entity including
lithiasis, infection, urinary tract obstruction, malignant degeneration
and intracystic bleeding that can cause a spontaneous retroperitoneal
hemorrhage (RH). Hemorrhagic renal cysts are the most frequent
complication of patients with ACKD.4 Bleeding is
usually confined within the cyst but occasionally extends into the renal
collecting system leading to hematuria or into the perinephric space
leading to perirenal hematoma, or RH. Perinephric hematomas have been
reported in up to 13% of patients with ACKD.3 Among
dialysis patients, ACKD has been recognized to be the most frequent
cause. Approximately 50% of patients with ACKD develop hemorrhagic
renal cysts.4 Malek-Marín et al. reported a single
center incidence of SRH in hemodialysis patients as 0.86 cases/100
patients.5 When SRH was of renal origin in dialysis
patients, the most common cause was cyst rupture in patients with
ACKD.5 The usual definition of ACKD requires three or
more cysts per kidney in a patient on dialysis who does not have a
hereditary cause of cystic disease. Within the first 3 years of
dialysis, approximately 10–20% of patients develop ACKD. By 5 years,
40–60% of patients have ACKD and by 10 years more than 90% of
patients exhibit ACKD.6Our patient had risk factors
for development of ACKD, including long duration of renal replacement
therapy. Moreover, our patient did not have any cysts in the kidney at
the start of the dialysis. Nephrectomy material also histologically
confirmed the diagnosis of ACKD. It is well documented that pre-existing
renal disease can predispose a kidney to injury. Another notable
observation in SRH secondary to cyst rupture is that there is a
remarkable difference in frequencies between SRH due to ACKD and
Autosomal dominant polycystic kidney disease (ADPKD). Most of the
described patients in the literature had underlying ACKD rather than
ADPKD. Some putative factors have been put forward to explain this
disparity. The more medullary location of cysts in ADPKD compared with
cortically residing cysts of ACKD and differences in the speed of cyst
growth might account for differences in observed prevalence of
SRH.5Among hemodialysis patients, perirenal or
retroperitoneal bleeding due to acquired cyst rupture tends to occur
after the dialysis session, possibly as a consequence of
heparinization.6
Although the mechanism of SRH in the present case remains elusive;
several factors might have contributed to the bleeding. First, the
histories of hyperparathyroidism, uremia, chronic HD, and atrophied
kidney with cystic change of ACKD were risks for hemorrhage. Second, the
patient received her heparin dose the day before the colonoscopy. Third,
the presence of calciphylaxis, also known as calcific uremic
arteriolopathy, should also be considered. It has been reported in 1%
to 4.5% of patients in dialysis with vascular calcifications with
calcium oxalate crystals tissues depositions.7 Our
patient had diffused small artery calcification and calcium oxalate
crystals in her left nephrectomy specimen and her skin lesions of
calciphylaxis where healed progressively after parathyroidectomy.
Fourth, it would be difficult to establish that maneuvers performed
during colonoscopy and endoscopic mucosal resection (EMR) as the cause
of SRH due to the anatomical proximity the left kidney which is
posterior to the descending colon, we believe that elevated intraluminal
pressure as well as increased intraperitoneal pressure from gas
insufflation during and external pressure on the left hypochondrium
exacerbated by ileus from deep sedation, since patients cannot report
pain associated with stretching, that also may contribute to increased
perinephric pressure. Finally, it should be pointed out that renal cysts
of ACKD are associated with fragile vessels focally calcified stretched
across their distended walls, as in our patient, with thickened
hypertrophic intima and fibrosis. When intracystic pressure rises, these
vessels may leak blood into the cyst, causing it to expand rapidly,
resulting in severe pain. If bleeding continues, then the cyst may
rupture into the collecting system or, alternatively, it may rupture
into the subcapsular compartment and eventually dissect through the
renal capsule to fill the retroperitoneal space.
Since most of the clinically overt SRH cases in dialysis patients are
secondary to rupture of acquired cysts, especially after 5 years of
renal replacement therapy, patients should be screened for the presence
of ACKD; this surveillance should be continued because of risk of cyst
enlargement and malignancy. Because of the high probability of small
clinical non-apparent underlying malignancy in HD patients who had ACKD
with SRH, nephrectomy was necessary in our anuric patient.
Conclusion
SRH is rare but important and potentially fatal complication. It is most
probably underestimated in frequency in daily practice. We described a
HD patient with ACKD who developed SRH after colonoscopy and we believe
that this procedure has not been reported before as an underlying
precipitating etiology. The reason of SRH was rupture of cysts of ACKD.
HD patients should be under close surveillance related to ACKD
development and associated bleeding risks after colonoscopy. Finally,
reporting of similar cases in medical literature seems necessary to shed
more light on this obscure entity and increase the awareness among
clinicians.
Conflict of Interest: No conflict of interest was declared by the
authors.
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