Cystic Diseases of the Kidney Treatment & Management

  • Author: Thomas Patrick Frye, DO; Chief Editor: Bradley Fields Schwartz, DO, FACS   more...
 
Updated: Dec 15, 2010
 

Medical Care

Effective means of prevention or modulation of disease have not yet been identified. Current treatment is aimed at symptom control. In general, therapy is reserved for pain, hypertension, infection, renal salt wasting, and nephrolithiasis.

  • Inherited cystic renal disease
    • Autosomal dominant polycystic kidney disease
      • Patients have decreased ability to concentrate urine and should be encouraged to drink 1-2 L of water daily.
      • Generally, 130/80 is considered the treatment goal for hypertension in this population. Moderate hypertension may be treated with sodium restriction (ie, < 100 mEq/d), exercise, and weight control. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are effective in controlling hypertension in autosomal dominant polycystic kidney disease (ADPKD). However, ACE inhibitors have been associated with reversible renal failure in polycystic kidney disease. Calcium channel blockers also are effective in managing hypertension in ADPKD.
      • Hypertension appears to correlate with the size of the cyst, and aspiration of renal cysts results in a reduction of blood pressure.[25]
      • Prevention of infection with appropriate precautions is important, particularly in women. Avoid urinary tract instrumentation whenever possible.
      • Treatment of infection involving cystic kidneys requires a prolonged course of antibiotics. Most cyst walls are permeable to polar antibiotics, including cephalosporins, penicillin derivatives, and aminoglycosides. Occasionally, cysts are relatively impermeable to these agents and require parenteral lipophilic antibiotics, such as ciprofloxacin, erythromycin, chloramphenicol, or a tetracycline. Clinical evaluation findings, including sterile urine, lack of fever, and no renal pain on deep palpation, should guide the route and duration of antibiotic therapy.
    • Autosomal recessive polycystic kidney disease (ARPKD): The newborn is provided supportive therapy while the degree of pulmonary insufficiency and the etiology is reviewed. Dialysis may be required for renal failure. With less severe childhood disease, edema often is a problem and is managed with sodium restriction and loop diuretics. Hypertension is controlled with salt restriction and antihypertensives, with particular emphasis on the use of ACE inhibitors and ARBs.
    • Juvenile nephronophthisis (JNPHP) and medullary cystic kidney disease: With severe salt wasting, salt supplementation may improve renal function and slow renal demise. End-stage renal insufficiency necessitates dialysis or renal transplantation.
  • Acquired cystic renal disease
    • Acquired renal cystic disease (ARCD): Mild bleeding episodes may be managed with bed rest and analgesics.
    • Medullary sponge kidney (MSK): Encourage patients with nephrolithiasis to produce 2 L of urine daily. Patients with hypercalcuria may benefit from oral thiazide diuretics. Patients may develop UTI and should be taught preventative measures.
    • Simple cyst: An infected simple cyst usually requires a combination of antimicrobial and surgical management. Pathogens encountered most frequently in infected simple cysts include Enterobacteriaceae, staphylococci, and Proteus species.

Recent research is beginning to identify biochemical targets that may allow disease-modifying therapy.

  • Inhibitors of the EGF receptor tyrosine kinase have been shown to slow cyst development and extend the life span in polycystic mice. Clinical trials with these agents are underway.[26]
  • Vasopressin receptor activation results in increased levels of cAMP. cAMP has been shown to be cystogenic and provides the rationale for vasopressin receptor blockade. Tolvaptan is a vasopressin receptor antagonist with high affinity in humans and is currently in Phase III clinical trials in patients with ADPCK.[27]
  • The identification of mTOR as a possible common pathway to cyst development makes this protein an attractive target for therapy. Rapamycin inhibits mTOR and has been shown to stop kidney growth and even allow regression in kidney size in a mouse model. Additionally, a retrospective comparison of patients treated with rapamycin to those not treated demonstrated a 25% decrease in kidney volume in the treatment group.[15, 16] These animal studies have failed to correlate with human clinical trials. In adults with ADPKD and early chronic kidney disease, 18 months of treatment with sirolimus, an mTOR inhibitor ,  did not halt polycystic kidney growth.[28]
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Surgical Care

  • Multicystic dysplastic kidney (MCDK): Previously, the involved kidney was routinely removed to prevent the subsequent development of symptoms. Today, however, surgical excision is indicated only if the dysplastic kidney interferes with respiratory or digestive function or if significant hypertension has developed. Additionally, cyst rupture, which can occur spontaneously or secondary to trauma, may require emergent surgical intervention.
  • Inherited cystic renal disease
    • ADPKD: Significant chronic pain may result from expansion of renal cysts. Needle aspiration is usually the first-line approach to symptomatic cysts. Initial resolution and then return of symptoms with reaccumulation of cyst fluid increases the chance that a laparoscopic cyst decortication will eliminate the patient's pain.[29] However, for the management of severe pain, hypertension, hematuria, or infection, surgical excision may be preferred. Complex cysts can be explored laparoscopically and treated appropriately based on intraoperative frozen sections. Laparoscopic techniques have been used with good results. New studies have reported good outcomes of laparoscopic cyst decortication using a retroperitoneal approach especially for posterior or lower pole lesions.[30, 31]
    • Percutaneous endocystolysis is another technique described for treatment of symptomatic cysts. The technique involves obtaining percutaneous access, dilating the tract, and then introducing a resectoscope with rollerball electrode to cauterize the internal surface of the cyst. A 13-year experience with this technique reported clinical improvement in 100% of the patients with minimal complications.[32]
    • Nephrectomy may be performed simultaneously with renal transplantation to create space for the transplanted kidney and to relieve symptoms associated with the native polycystic kidney. The timing of performing the nephrectomy in the transplant patient has been debated. Data suggest that open ipsilateral nephrectomy at the time of transplantation with staged contralateral native nephrectomy has fewer perioperative complications than performing a laparoscopic bilateral nephrectomy.[33] In extreme cases of liver enlargement, severe pain and wasting may result. Partial hepatectomy may alleviate these symptoms.
    • ARPKD: In patients with severe portal hypertension, sclerotherapy or portosystemic shunt placement may be necessary to control bleeding. Splenectomy may be indicated for splenomegaly with significant complications.
    • JNPHP and medullary cystic kidney disease (MCKD): If transplantation is considered, selecting an older or unrelated donor is advisable to minimize the risk of the transplanted kidney also being affected with these diseases.
  • Acquired cystic renal disease
    • ARCD: Persistent or severe hemorrhage may necessitate nephrectomy or renal embolization. If a 3-cm renal mass suggestive of renal cell carcinoma (RCC) is noted, a partial or radical nephrectomy is indicated.
    • Simple, intermediate, and suspicious cysts: Simple renal cysts rarely require surgical management to relieve pain or obstruction. Treatment options include aspiration, sclerosis, open resection, endoscopic marsupialization and fulguration, percutaneous resection, and laparoscopic resection.
    • Bosniak category III and IV renal cysts require surgical exploration. Approximately 50% of Bosniak category III cystic renal lesions are malignant. Management depends on the appearance of the lesion and varies from exploration and biopsy to nephrectomy. The current standard approach is open exploration with anticipated partial nephrectomy. However, as the experience with laparoscopic exploration and nephrectomy grows, this technique may prove equally reasonable.
  • Cystic clear cell renal cell carcinoma: Whether the patient has known pathologically diagnosed malignancy from biopsy or suspected malignancy based on Bosniak classification, a urologist can anticipate good surgical outcomes after resection. A study evaluating laparoscopic nephrectomy for cystic clear cell renal cell carcinoma revealed that all patients treated were alive after 5 years and that no patient had extrarenal disease at the time of surgery. These data suggest that patients with cystic RCC should expect to be cured after surgical resection, and furthermore should undergo nephron-sparing surgery when possible.[34]
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Contributor Information and Disclosures
Author

Thomas Patrick Frye, DO  Resident

Disclosure: Nothing to disclose.

Coauthor(s)

Alex Gorbonos, MD  Assistant Professor, Division of Urology, Director, Robotic Surgery, Southern Illinois University School of Medicine

Alex Gorbonos, MD is a member of the following medical societies: Alpha Omega Alpha, American Urological Association, Endourological Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Edmund S Sabanegh Jr, MD  Director, Center for Male Fertility, Glickman Urological and Kidney Institute, Cleveland Clinic Foundation

Edmund S Sabanegh Jr, MD is a member of the following medical societies: American Medical Association, American Society for Reproductive Medicine, American Society of Andrology, American Urological Association, Society for the Study of Male Reproduction, Society of Reproductive Surgeons, and Southwest Oncology Group

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

J Stuart Wolf Jr, MD, FACS  The David A Bloom Professor of Urology, Director, Division of Endourology and Stone Disease, Department of Urology, University of Michigan Medical School

J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology

Disclosure: Baxter Healthcare Consulting fee Consulting

Chief Editor

Bradley Fields Schwartz, DO, FACS  Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists

Disclosure: Nothing to disclose.

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Cut surface of a nephrectomy specimen from a patient with a multicystic dysplastic kidney (MCDK).
Nephrectomy specimen from a patient with a large benign simple cyst.
External surface of a nephrectomy specimen from a patient with autosomal dominant polycystic kidney disease (ADPKD).
Cut surface of the same nephrectomy specimen from a patient with autosomal dominant polycystic kidney disease (ADPKD).
Cut section of nephrectomy specimen demonstrating renal cell carcinoma (RCC), with an adjacent simple cyst.
Close-up photograph of the cut surface of the same nephrectomy specimen demonstrating a simple cyst adjacent to a renal cell carcinoma (RCC).
A prenatal sonogram of a fetus with a multicystic dysplastic kidney. The right kidney is appreciated as a large multicystic paraspinal mass. The left kidney and bladder are normal, and a normal amount of amniotic fluid is present.
CT examination of the abdomen of a 70-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) is shown. The kidneys are bilaterally enlarged with multiple cysts.
CT scan of the same patient (70-year-old woman with autosomal dominant polycystic kidney disease [ADPKD]) demonstrating multiple hepatic cysts.
This CT scan demonstrates acquired renal cystic disease (ARCD) in a 70-year-old man who is dialysis-dependent. The CT scan demonstrates bilateral atrophic kidneys with multiple renal cysts.
A CT scan of a 38-year-old man with von Hippel-Lindau syndrome (VHLS). The patient previously underwent resection of multiple bilateral renal cell carcinomas (RCCs). CT scan demonstrates multiple cysts in the kidneys and pancreas, as well as solid renal lesions suggestive of malignancy.
 
 
 
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