C- 04: polycystic kidney disease
B-CRF: nutritional complications
B- CRF: other complications
Intravascular
Embolization
Enlarged Polycystic Liver
Therapy
in
Patients
with
Junichi Hoshino, MD, MPH , Yoshifumi Ubara, MD, PhD, Tatsuya Suwabe, MD, Keiichi
Sumida, MD, Noriko Hayami, MD, Koki Mise, MD Rikako Hiramatsu, MD, Eiko Hasegawa,
MD Masayuki Yamanouchi, MD Naoki Sawa, MD Ryoji Takei, MD Kenmei Takaichi, MD,
PhD
American Journal of Kidney Diseases
Volume 63, Issue 6, Pages 937–944, June 2014
ABSTRACT
Background
Hepatic transcatheter arterial embolization (TAE) has become an accepted treatment option
for patients with symptomatic autosomal dominant polycystic kidney disease (ADPKD) who
also have polycystic liver disease and who are not good candidates for surgery. However,
indications for TAE and long-term outcome with it are still unclear.
Study Design
Retrospective cohort study.
Setting & Participants
Symptomatic patients with ADPKD with polycystic liver disease who underwent hepatic TAE,
June 2001 to December 2012, at Toranomon Hospital and whose liver volume data were
available were studied (N = 244; 56% on dialysis therapy, none with kidney transplants).
Mean age was 55 ± 9 (SD) years, and mean liver volumes were 8,353 ± 2,807 and 6,626 ±
2,485 cm3 in men and women, respectively. Target arteries were embolized from the
periphery using platinum microcoils.
Predictors
Sex-specific quartiles (6,433, 8,142, and 9,574 cm3 in men and 4,638, 6,078, and 8,181 cm3
in women) of total liver volume pretreatment.
Outcomes
All causes of mortality were obtained from medical records, followed up until July 31, 2013.
Measurements
Laboratory values were measured before TAE and 1, 3, 6, and 12 months after. Organ
volumes were measured pretreatment, then 6 and 12 months after, by summing the products
of the organ areas traced in each computed tomographic image.
Results
Liver/cyst volume decreased to 94.7% (95% CI, 93.5%-95.8%) at 6 months and 90.8% (95%
CI, 88.7%-92.9%) at 12 months of pretreatment volumes. Serum protein and hematocrit
values improved significantly without liver damage. Survival was significantly better for
patients with liver volume ≤ 9,574 cm3 (men) and ≤8,181 cm3 (women) than for those with
larger livers (5-year survival, 69% and 48%; P = 0.02). Infection and liver failure caused most
deaths, especially in patients with larger livers.
Limitations
Referral bias and lack of control group.
Conclusions
Hepatic TAE appears to be a safe and less invasive option for patients with symptomatic
polycystic liver, especially those contraindicated for surgical treatment (eg, with malnutrition
or on dialysis therapy), improving both hepatic volume and nutrition.
Index Words:
Autosomal dominant polycystic kidney disease (ADPKD), hepatic transcatheter arterial
embolization (hepatic TAE), polycystic liver disease (PLD), survival, cause of death
COMMENTS
In autosomal dominant polycystic kidney disease (ADPKD), renal tubular and intrahepatic
biliary epithelia develop abnormally, resulting in progressive cystic enlargement that causes
decreased kidney function, massive nephromegaly, and hepatomegaly, with effects on
volume that sometimes can cause serious complications such as malnutrition and circulatory
failure.
In this article coming from Japan, a very large series of hepatic cysts embolisation is
presented. This technique compete easily with partial cyst resection, intraperitoneal
marsupialisation and partial hepatectomy.
Hepatic TAE may be a new, safe, and less invasive treatment option for many patients with
symptomatic polycystic liver disease, serving to improve both hepatic volume and nutrition,
which may improve their quality of life. Most patients undergoing hepatic TAE have had a
good subsequent course without serious complications. The genetic defect causing ADPKD
has been identified, but the mechanisms by which liver cysts develop and enlarge remain
uncertain. Combination therapies, such as liver transplantation after hepatic TAE to improve
nutritional status before transplantation, cyst aspiration following TAE to minimize cyst
bleeding after aspiration, or medical treatment plus hepatic TAE, could be the next step in
improving patient outcome and quality of life.
Pr. Jacques CHANARD
Professor of Nephrology