comprehensive review on kidney liver cystic diseases

1. Renal-liver cysts
DR SAJID ALI
PG TRAINEE KTH

2. introduction
 Cyst is closed cavity lined by membrane and division compared to nearby
tissue
 Pseudocyst when there is no definite membrane
 Abscess when it is filled with pus

3. Prevalence
 27 -35% greater than 50 year have a simple renal cyst
 Prevalence increases by increasing age and almost every person by the age
of 70 years has a simple renal cyst

5. Bosnaik Classification
In 1986 Morton Bosniak published a review article in
which he suggested a classification and further
management of cystic lesions of the kidneys based on
findings on contrast- enhanced computed tomography.
The classification was gradually adopted by imaging
specialists and urologists,
. The classification underwent a small change in 2005,
reaching its current format

6. Bosnaik Classification
 Category 1
 category I correspond to simple cysts without septa or vegetation's, with
thin and smooth walls, and no contrast enhancement after the
administration of intravenous contrast agents
 Category 2
 Category II includes cysts with thin septations, minimally thick walls and
fine parietal calcifications, and no contrast enhancement after intravenous
contrast agent injection

9. Bosnaik classification
 Category 3
 Homogeneous hyperdense cysts ≤ 3.0 cm are included in this category.
Lesions with irregular and/or thick septa, with course calcifications, and
clear enhancement after intravenous contrast injection are described as
category
 Category 4
 Category IV is reserved for lesions with septa or walls with well-defined
solid components that demonstrate contrast-enhancement after
intravenous contrast injection

12. Bosnaik continued…..
 Category 2f
 Category II-F corresponds to indeterminate lesions with findings in
between 2 and 3 which, although not sufficient to indicate surgical
exploration, suggest a slight risk of malignancy
 Absent or hair like enhancement

13. Category Non contrast features Contrast features
1 Water density (0–20 HU), thin margins,
sharp delineation with the renal
parenchyma, thin and smooth walls,
homogeneou
No contrast enhancement
2 Presence of one or few thin septations,
small and fine calcifications; hyperdense
cysts measuring up to 3.0 cm (60–70 HU)
No contast enhancement
2F More complex lesions which cannot be
included in category II or III. Multiple
septa. Walls or septa with nodular or
irregular calcifications
Absent,hair like
enhancement
3 Thick-walled cystic lesion, septum
irregularity and heterogeneous septum
and wall and/or contents. Gross and
irregular calcifications with measurable
enhancement
Wall or septum
enhancement
4 Lesions with all the findings of category
and solid component, soft parts,
dent of finding of wall or septa
Enchancement of wall
solid component

14. Treatment category wise
Category treatment
1 None
2 Ct/us follow up
2F Follow up
3 Partial nephrectomy
4 nephrectomy

15. Polycystic liver diseases

16. Polycystic liver diseases
 Polycystic liver diseases (PLD) represent a group of genetic
disorders in which cysts occur in the liver (autosomal
dominant polycystic liver disease) or in combination with cysts
in the kidneys (autosomal dominant polycystic kidney disease)
 Regardless of the genetic mutations, the natural history of
these disorders is alike.

17. Natural history
 The natural history of PLD is characterized by a
continuous increase in the volume and the number of
cysts. Both genders are affected; however, women
have a higher prevalence.
 The association between polycystic liver disease (PLD)
and autosomal dominant polycystic kidney disease
(AD- PKD) was described for the fist time by Bristowe
in 1856

18. Incidence and genetics
 ADPKD affects up to 0.2% of the general population
 isolated PLD has prevalence of less than 0.01% Both
 ADPKD and PLD are autosomal dominant and 75%-90% of patients with
ADPKD have associated PLD

19. Genetics
 In humans, PLD has been linked to mutations of four genes.
Two genes (PKD1, locus 16p13.3, encoding polycystin-1 and
PKD2, locus 4q21, and encoding polycystin-2) are
predominantly associated with renal disease and less
frequently with PLD.
 PKD1 mutations are more common and account for 85%-90%
of the cases, whereas mutations in PKD2 affect approximately
10%-15% of patients

20. Genetics….
 The remaining two mutations (PRKCSH, locus 19p13.2, encoding the
protein kinase C substrate 80K-H or hepatocystin and SEC63, locus 6q21,
encoding the Sec63 protein) are linked only to the development of PLD.
 these mutations explain just 25% to 40% of cases of PLD

21. Pathophysiology
 Malformation of the hepatic ductal plate and cilia of cholangiocytes is the
main characteristic linked to the pathophysiology of PLD
 Ductal plate malformation
 Abnormal primary cilia

22. Pathophysiology
 The ductal plate is the anatomical template for the de-velopment of the
intra-hepatic bile ducts
 Normal bile ducts arise from the ductal plate through a complex sequence
of growth and apoptosis. Complexes of dis- connected intralobular bile
ductules (von Meyenburg complexes) are retained because they do not
undergo apoptosis in PLD As a consequence, multiple cysts arise from
progressive dilatation of these abnormal ductules that display the same
epithelium and structures of functioning cholangiocytes

23. Pathophysiology…
 Cholangiocytes are the only ciliated cells in the liver. Cilia have
mechanosensory capacity and modulate the intracelular levels of cAMP
and Ca2+ when bent by the flow of bile. They also detect changes in
osmolarity and composition of the bile Ciliary defects result in a decreased
cytoplasmic level of Ca2+ and an increased cytoplasmic level of cAMP.
These changes are responsible for the hyperproliferation of cholangiocytes
and for the cystogenesis that is a consequence of the altered balance
between fluid secretion and absorption in the lumen of the biliary ducts

24. Natural history and risk factors
 The natural history of PLD is characterized by a continuous increase in the volume
and the number of cysts
 The annual growth of affected livers is in the range of 0.9%-3.2% of the initial
hepatic volume. Both genders are affected; however, women have a higher
prevalence.
 Exposure to estrogen during pregnancies, the use of oral contraceptive pills or
estrogen replacement therapy seems to accelerate the progression of the disease
 Other risk factors are the severity of renal dysfunction that is dependent on the
volume of the cysts in the kidneys


25. Clinical presentation
 Abdominal distention
 Early satiety
 Postprandial fullness
 Gastro-oesophageal reflux
 Malnutrition
 Dyspnoea
 Hepatic venous-outflow obstruction (Budd-Chiari syndrome)
 Inferior vena cava syndrome
 Portal-vein compression Bile-duct compression
 Infection
 Rupture
 Torsion
 Haemorrhage

26. Diagnosis
 The most common methods for the diagnosis of PLD are cross
sectional imaging studies. Abdominal ultrasound (US) and
computerized tomography (CT) are the two most frequent
investigations
 For hepatic cysts, MRI is more sensitive and specific, and it is
a valuable test for patients with intravenous contrast allergies
or renal dysfunction or when other studies are unable to
satisfy the diagnostic needs

27. Infected cysts
 INFECTED CYSTS Hepatic cysts may become infected, and cause
life- threatening sepsis. Often, infected hepatic cysts are responsible
for recurrent episodes of fever without any other signs or
symptoms. In these circumstances, the diagnosis can be quite
difficult as the accuracy of imaging tests remain low due to the
altered anatomy of the liver parenchyma. A promising investigation
technique for suspected infected hepatic cysts is In-111 WBC scan.

28. Gigots classification
 Gigot’s classification relies on imaging findings and was designed to
identify the best candidates for fenestration of symptomatic cysts
 Type Ⅰ: presence of less than 10 large hepatic cysts measuring more than
10 cm in maximum diameter.
 Type Ⅱ: diffuse involvement of liver parenchyma by multiple cysts with
remaining large areas of non-cystic liver parenchyma.
 Type Ⅲ: pres-ence of diffuse involvement of liver parenchyma by small
and medium-sized liver cysts with only a few areas of

29. Gigots…

30. Which gigot???

31. Which gigot?

32. Which gigot?

33. Treatment
 Most patients with PLD are asymptomatic and do not require
any intervention
 Symptomatic PLD patients might require treatment when
they experience severe dysfunction of organs around the liver
due to the increased hepatic volume or when one or more
cysts get torted, infected or develop intra-cystic hemorrhages

34. Treatment
 Nonsurgical/ Medical
 Somatostatin analogues
 mTOR inhibitors
 Interventional radiology:
 Arterial embolization
 Percutaneous sclerotherapy
 Surgical
 Fenestration
 Hepatic resection with fenestration
 Liver transplantation

35. Treatment
 AVOIDANCE OF EXPOSURE TO ESTROGENS Observational and
experimental studies have shown that PLD may worsen under the influence
of estrogen during pregnancy or when patients are prescribed estrogen
replacement therapy
 Estrogen can increase both the number of liver cysts and their volume,
therefore, hormonal therapy should be stopped in most symptomatic
patients when appropriate

36. Treatment
 NON-SURGICAL TREATMENTS Medical management may be valuable in
symptomatic patients with Gigot’s type Ⅱ/Ⅲ.
 SOMATOSTATIN ANALOGUES
 Somatostatin analogues are inhibitors of cAMP and they reduce the
secretion of fluid and the proliferation of many cell types, including
cholangiocytes

37. Arterial embolization
 Trans-catheter arterial embolization has been used since the early 2000s
 Hepatic artery branches supplying the hepatic segments replaced by the
cysts are targeted by using microcoils or polyvinyl alcohol particles
measuring 150-250 μm in diameter

38. Percutanous sclerotherapy
 This technique requires radiologically guided percuta- neous
aspiration of the content of the cysts followed by the injection of a
sclerosing agent that inhibit the reaccumulation of fluid by
damaging the epithelial lining the cyst
 Symptomatic patients with one to five large dominant cysts
(Gigot’s type Ⅰ) are suitable for percutaneous sclerotherapy. Most
commonly, cysts with a diameter larger than 5 cm are candidates
for this treatment. Puncturing of the cyst can be done with a 5 or 7
French catheter and sclerosing agents commonly used include
ethanol, ethanolamine oleate, minocycline and tetracycline.
Although a single session is often sufficient, some patients require
more than one.

39. Role of surgery
 Patient and treatment selection remain a clinical challenge. There is no
consensus on selection criteria for surgery, the optimal timing, and technique.
Current surgical options include fenestration, partial liver resection and OLT.
Fenestration and partial liver resection are options for Gigot’s type Ⅰ and Ⅱ
patients. For Gigot’s type Ⅲ disease, fenestration and partial liver resection are
often ineffective, and OLT should be considered as it is the only curative
treatment. In general, several factors have to be considered before any surgical
intervention is recommended
 The degree of cystic burden;The distribution of the cysts; and The proximity of
the cysts to the main biliary ducts and portal and hepatic vein branches.

40. Fenestration
 FENESTRATION Fenestration is a surgical technique that combines aspira- tion and
surgical unroofing of the cyst. It has the advan- tage that multiple cysts can be treated in
one session[48,82]. Fenestration is effective in symptomatic patients with Gigot’s
type Ⅰ and Ⅱ disease[83]. Patients with superficial and a limited number of large cysts
are the best candi- dates for this procedure[48]. Fenestration may be achieved by
laparotomy or laparoscopy[48]. Patients with the major- ity of their cysts located in the
right posterior segments (Ⅵ, Ⅶ), or at the dome of the liver (segment Ⅷ) may be better
candidates for open fenestration because these cysts are difficult to be visualized and
fenestrated by lapa- roscopic approach[48]. Published series describing open and
laparoscopic fenestration are summarized in Table 7. Immediate symptom relief is
achieved in 92% of the patients, whereas up to 25% experience recurrence of the cysts
or symptoms[10]. Complication rate after fenestra- tion is in the range of 23% while
mortality is about 2%[10]. Complications include ascites, pleural effusion, hemor- rhage
and bile leakage[84]. Factors that predict failure of fenestration are previous abdominal
procedures, deep- seated cysts, incomplete unroofing, cysts in segments Ⅶ- Ⅷ, and the
presence of diffuse PLD[10].

41. Hepatic resection with fenestration
 Hepatic resection is usually reserved for highly symptomatic
patients who are incapacitated by their disease due to the massive
expansion of their livers (Gigot’s type Ⅱ and Ⅲ)
 In these circumstances fenestration alone is rarely successful
because the liver parenchyma is rigid and it does not collapse
 Symptom relief is achieved in 86% of cases

42. Liver transplantation
 OLT is the only curative treatment for patients with severe PLD. It is
indicated in those patients with disabling symptoms that lead to decreased
performance status and quality of life.
 Patients with PLD usually have normal liver function and the current organ
allocation system based on the Model for End-Stage Liver Disease (MELD)
is often unable to assist this group of patients. For these patients, MELD
exception criteria are needed
 Because of the shortness of available grafts, the need for life-long
immunosuppression and the perioperative risks, OLT is indicated only for
symptomatic patients

43. Autosomal dominant polycystic kidney
disease
 Autosomal dominant polycystic kidney disease (ADPKD) is a multisystemic
and progressive disorder characterized by cyst formation and enlargement
in the kidney and other organs (eg, liver, pancreas, spleen).
 Up to 50% of patients with ADPKD require renal replacement therapy by
60 years of age.

44. Symptoms
 Pain in flanks----caused by
 Enlargement of one or more cysts
 Bleeding: May be confined inside the cyst or lead to gross hematuria with
passage of clots or a perinephric hematoma
 UTI (eg, acute pyelonephritis, infected cysts, perinephric abscess)
 Nephrolithiasis and renal colic
 Rarely, a coincidental hypernephroma

45. Diagnosis
Examination
 Hypertension: One of the most common early manifestations of ADPKD,in
which increased diastolic BP is the rule; clinical course in ADPKD is usually
more severe early on, then becomes less problematic as the renal insufficiency
progresses
 Palpable, bilateral flank masses: In advanced ADPKD
 Nodular hepatomegaly: In severe polycystic liver disease
 Ultrasonography is the procedure of choice in the workup of patients with
autosomal dominant polycystic kidney disease (ADPKD). It is also ideal for
screening patients' family members. Computed tomography (CT), magnetic
resonance imaging (MRI),

46. Cerebral aneurysms
 Cerebral aneurysms are among the most serious complications of ADPKD;
they occur in 4-10% of patients with ADPKD. In the study by Rahman et al,
the mortality rate from cerebrovascular events in ADPKD was
approximately 7%.
 Rupture usually occurs in patients younger than 50 years who have
uncontrolled hypertension; however, a stroke from hypertension and
intracerebral hemorrhage is more common. There is no relationship
between the risk of rupture and the severity of renal disease.

47. Liver cysts
 Frequent extrarenal manifestation of ADPKD
 Liver size in massive polycystic liver disease tends to stabilize after
menopause. Hepatic cysts occur in almost 50% of affected patients.
Cysts occur in approximately 20% of patients during the third
decade of life and in 75% during the seventh decade of life. They
are rare in children, and the frequency increases with age.

48. Approach consideration
 In patients with autosomal dominant polycystic kidney disease (ADPKD),
pharmacologic therapy is necessary to accomplish the following:
 Control blood pressure; rigorous blood pressure control is recommended
in early ADPKD
 Control abnormalities related to renal failure
 Treat urinary tract infections
 Treat hematuria
 Reduce abdominal pain produced by enlarged kidneys

49. Hypertension
 In patients with renal disease, the goal is a blood pressure of less than
130/88 mm Hg. If more than 1 g/day of urinary protein is present, the
target blood pressure is less than 125/75 mm Hg.
 The drugs of choice for this condition are angiotensin-converting enzyme
(ACE) inhibitors (eg, captopril, enalapril, lisinopril) or angiotensin II receptor
blockers (ARBs) such as telmisartan, losartan, irbesartan, and candesartan

50. Pain control
 Avoid nonsteroidal anti-inflammatory drugs (NSAIDs), because they can
worsen renal function and potentiate hyperkalemia. Treatment involves
surgical cyst decompression, which is effective for pain relief in 60-80% of
patients.

51. Surgery
 Surgical Drainage
 If infected renal or hepatic cysts do not respond to conventional antibiotic
therapy, surgical drainage may be necessary. This procedure is usually
performed with image guided puncture
 Cysts may become large enough to cause abdominal discomfort or pain.
Typically, acute pain is from cyst hemorrhage or an obstruction by a clot,
stone, or infection. When one or more cysts can be identified as causing
the pain, the symptoms can often be abated by open- or fiberoptic–guided
surgery to excise the outer walls and to drain them.
 Approximately 25% of patients with the most severe pain do not gain relief
from surgery or pharmacologic therapy with narcotics. These individuals
usually have inaccessible cysts in the medullary portions of the kidneys.
Nephrectomy is used as a last resort to control the pain in these patients.
Nephrectomy is often necessary when there is not enough room for a
kidney graft.

53. Thank you