Haematuria
is alarming symptom (in a urinary tract disorder), and a cause of
much anxiety to a patient, even more so in autosomal dominant polycystic
kidney disease (ADPKD) patients, who are already bearing the brunt
of the burden of an inherited disorder. He grossly enlarged size of
the kidneys in ADPKD patients makes them more vulnerable to traumatic
injury, with minor trauma often going unnoticed. Haematuria and pain
is often associated with haemorrhage within these cysts, which are
seen as hyperdense cysts on non-enhanced computed tomography (NECT).
Calculus disease is also commonly encountered in ADPKD patients and
can cause haematuria, pain or both. The role of Multislice Computed
Tomography (MSCT) in the evaluation of ADPKD patients is elaborated
upon in the following discussion.
Introduction
Haematuria being an alarming symptom, is a cause of tremendous anxiety
to a patient, particularly so in ADPKD patients. ADPKD is a very prevalent
hereditary disorder, occurring with a frequency of 1:500 to 1:1000.2
This disorder typically remains silent until the third or the fourth
decade; when flank pain, haematuria, abdominal masses or hypertension,
first calls attention to the process.1 We present here two cases of
ADPKD, presenting with haematuria and flank pain, and on questioning
further, gave a h/o trivial trauma 6 months back. The role of MSCT
has been emphasized upon, in evaluating such patients, and in pinpointing
the cause of haematuria and/or pain in such patients, which can be
related to cyst bleed or associated calculus disease.
Case Reports
1. A 33 year old male, presented with haematuria and flank pain following
blunt abdominal trauma. Axial plain and post contrast CT scans of
the abdomen were performed on a MSCT scanner (Somatom Volume Zoom,
Siemens) utilizing a 5 mm slice thickness.
The CT findings were suggestive of ADPKD, with few
liver cysts. There were a blood level seen in one of the inferiorly
located cysts and haemorrhage was also noted in the left pararenal
space.
2. A 35 year old male, also presented with pain in the flanks and
haematuria. He gave a history of trivial trauma about 6 months back.
The CT findings were suggestive of ADPKD with few liver cysts, with
non enhancing hyperdense sub capsular cysts, suggestive of haemorrhage
within the cysts.
Discussion
ADPKD is the third most frequent systemic hereditary condition, ranking
behind familial hypercholesterolaemia and dominant osteosclerosis.2
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| Fig.
1 : Unenhanced CT scan showing multiple cysts in both
kidneys with few of the surface cysts being hyperdense (arrows). |
|
Fig.
2 : Unenhanced CT scan showing bilaterally enlarged kidneys
with multiple cysts; one of the cysts in left kidney reveals hyperdense
level. Few of the surface cysts are hyperdense. |
Patients frequently
present with haematuria or pain, or both, as the initial manifestation
of polycystic kidney disease.3,4 This may or may not be
preceded by trauma. Both pain and haematuria may be related to cysts
or associated calculus disease. Pain occurs in 50% to 62% of patients
at sometime in their life, being most frequent in the third and the
fourth decade.2 Common causes of cyst pain include intracystic
haemorrhage, with resultant cyst expansion causing increased intra
cystic pressure, cyst infection or clot colic. Nephrolithiasis has
been reported in upto 36% of cases of ADPKD, and can be one of the
causes of pain and haematuria in these patients.4 Some
of these calculi may be very small (even < 1 mm).
Cyst haemorrhage in ADPKD can occur because of rupture of unsupported
sclerotic vessels in the cyst wall, either randomly as a natural part
of the disease process, or as a result of trauma.1 As larger
kidneys are more prone to traumatic injuries than normal kidneys,
it is possible that minor trauma, which is often unnoticed by the
individual, may be an important cause of intracystic haemorrhage.
The sub capsular location of most high density renal cysts in ADPKD
supports a traumatic aetiology, since the renal surface is most susceptible
to direct trauma.1
Hyperdense cysts on CT in ADPKD are seen in 68% of cases.1
On unenhanced CT, they are seen as multiple, sharply circumscribed
cysts, generally sub capsular in location with attenuation values
ranging from 40 to 90 HU. They appear significantly denser than uncomplicated
cysts and the remaining renal parenchyma. The hyperdense appearance
is believed to result from cyst haemorrhage. They do not enhance and
are usually isodense/hypodense on enhanced scans.
Multislice CT (MSCT) is an advancement over conventional single slice
CT scanners due to its rapid acquisition and longer anatomic coverage;
and thus making scanning of patients, especially with a history of
trauma extremely easy. Improved spatial, temporal and contrast resolution,
thin slice collimation and retrospective reconstruction techniques,
has improved the diagnostic accuracy immensely, particularly due to
its ability to avoid respiratory misregistration artifacts and partial
volume averaging artifacts.5
Ultrasonography is usually not helpful, since such “high density
cysts” often cannot be reliably distinguished from adjacent
cysts.1,6 Differentiation between calculi and cyst calcification,
based on sonography is also often difficult. Besides, visualization
of very tiny calculi and assessing their exact location may not be
possible with ultrasound.
MRI, with its ability of higher contrast resolution and better tissue
characterization, diagnoses haemorrhagic cyst in ADPKD very easily.
However, the limitation of detecting calcification and calculi on
MRI is well documented.
The superiority of MSCT over other modalities in such ADPKD patients
is well justified due to its ability to detect even tiny calculi (upto
1 mm), and to distinguish calculi from cyst calcification from other
calcific densities. Additionally, CT fluoroscopic guidance can add
to the therapeutic intervention value while performing cyst aspiration
in haemorrhagic cyst, thus almost obviating the need of surgery. Cyst
haemorrhage may at times also cause haemorrhage of sufficient severity
so as to necessitate blood transfusion, transcatheter renal artery
embolization or even nephrectomy.1 MSCT, by giving a vascular
map through CT angiography may prove to be extremely useful in such
cases.
In both our cases; MSCT findings revealed the presence of hyperdense
cysts, which are suggestive of haemorrhage within cysts, and therefore
the cause of haematuria. MSCT was thus useful in diagnosing the cause
of haematuria and pain in these patients was cyst related and not
due to calculi, which would have necessitated a different management.
Summary
ADPKD is a commonly occurring inherited disorder, often getting noticed
following trauma, leading to haematuria and/or pain. MSCT, as a single
modality, can specify the cause for these symptoms; whether cyst related
or calculus related or both, and can even be utilized in the management
of such patients (CT guided cyst aspiration) accordingly. A CT angiography
in the same sitting can prove of great utility in those cases that
may require renal artery embolization or nephrectomy in the future.
References
1. High-density renal cysts in Autosomal Dominant Polycystic Kidney
disease demonstrated by CT. Levine Errol, Jared Grantham. Radiology
1985; 154 : 477-82.
2. Peter L Choyke. Inherited cystic diseases of kidney. Radiologic
clinics of North America 1996; 34 : 5;925-43.
3. Klein AJ, Kozar RA, Kaplan LJ. Traumatic hematuria in ADPKD. Am
Surg 1999, May; 65 (3) : 464-66.
4. Errol Levine, Jared Grantham. Calcified renal stones and cyst calcifications
in Autosomal Dominant Polycystic kidney disease : clinical and CT
study in 84 patients. AJR 1992 July; 159 : 77-81.
5. Jonas Rydberg, Kenneth A Buckwalter, Karen S Caldmeyer, et al.
Multisection CT : Scanning techniques and clinical applications. Radiographics
2000; 20 : 1781-1806.
6. Zirinsky K, Auh YH, Rubenstein WA, Williams JJ, Pasmantier MW,
Kazam E. CT of the hyperdense renal cyst : sonographic correlation.
AJR 1984; 143 : 151-6.
7. Pamela L Hippert, Arnold C Friedman, Paul D Radecki, et al. MRI
in haemorrhagic renal cysts in polycystic kidney disease. AJR 1986,
June; 146 : 1167-72.
8. Rhyner P, Federle MP, Jefferey RB. Computed tomography of trauma
to the abnormal kidney. AJR 1984, April; (142) : 747-50.
STRESS
MAY EXACERBATE MULTIPLE SCLEROSIS
Stress is related to exacerbation of multiple sclerosis, but the
size of the effect is modest. In a meta-analysis that included
14 papers, Mohr and colleagues found that non-traumatic stressful
life events significantly exacerbated multiple sclerosis. The
exact mechanism is unclear, and specific stressors could not be
identified as causing exacerbation in individual patients.
BMJ, 2004; 328 ; 731. |
