Surgery in the Management of Autosomal Dominant Polycystic Kidney Disease

Abstract

A 46-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) was referred to Oregon Health Sciences University because of abdominal distention due to massive bilateral kidney enlargement. ADPKD had been diagnosed by intravenous urography at age 19, during an evaluation for abdominal pain associated with a mass. During the ensuing years, she noted a progressive increase in her abdominal girth, as a result of her enlarging kidneys, and experienced progressive physical discomfort and social embarrassment. She was the subject of jokes at her place of employment about constantly being pregnant because she wore maternity clothes to be comfortable. Multiple physicians had told her that there were no therapeutic options available, except for analgesics and repeatedly advised against cyst aspiration or cyst reduction surgery for fear of impairment of renal function. Four months before referral, serum creatinine was 88.4 mmol/L (I mg!dL) and two 24-hour endogenous creatinine clearances were 1.42 and 1.28 mL/s (85 and 77 mL/min), respectively. Abdominal ultrasound at that time showed large kidneys measuring 21 X II X 18 cm on the right, and 17 X 13 X 19 cm on the left. Both kidneys contained less than 25% normal parenchyma and more than 15 cysts per kidney. The largest single cyst in the right kidney was 12.5 cm, with most of the remaining cysts being larger than 5 cm. In the left kidney, the largest cyst measured 13 cm, with most of the remaining cysts measuring 2 to 5 cm. There were no hepatic cysts noted, and there was no evidence of ascites. On admission to our hospital, she was taking no antihypertensive or analgesic medications. Physical examination showed a well-developed woman whose weight was 62.5 kg. Blood pressure was 130/96 mm Hg. Her abdomen was markedly protuberant, due to large abdominal masses. Her waist was 100 cm in circumference, and her mid-abdominal girth measurement was 114 cm. There was I + pretibial edema bilaterally. Her admission laboratory studies showed a blood urea nitrogen (BUN) of 6.8 mmol/L (19 mg/dL), serum creatinine 79.6 /Lmol/L (0.9 mg/dL), serum albumin 38 g/L (3.8 gfdL); hematocrit 45%; and urinalysis, which showed no protein or casts. There were 4 to 5 white blood cells per highpower field, but urine culture and sensitivity showed no bacterial growth. A computerized tomography (CT) scan of her abdomen confirmed massively enlarged kidneys bilaterally due to the presence of enormous cysts, occupying most of the abdomen and distorting other intra-abdominal structures. The CT scan confirmed a right kidney greater than 23 cm in length, and a left kidney greater than 24 cm in length. After obtaining informed consent, a bilateral cyst reduction operation was performed through a midline incision extending from the xiphoid process to the pubis. The peritoneal cavity was entered and the right and left colons were reflected medially to expose the kidneys for unroofing of the cysts. Several of the larger cysts were aspirated, including 3,050 mL from a single cyst in the left kidney, as well as 540 mL, 270 mL, 210 mL, and 130 mL from individual cysts in that same kidney. A total of 7,600 mL of cyst fluid was collected from the two kidneys. Superficial cysts were unroofed with electrocautery, and through the cavities of the unroofed cysts, the domes of deeper cysts were also unroofed. Following completion of cyst decompression, bilateral suction drains were placed posterior to the kidneys in the retroperitoneum. During surgery, the patient received I U of autologous whole blood, 2 L of crystalloid solution, and 25 g of mannitol. Estimated blood loss at surgery was 250 mL. At the time of closure, her mid-waist girth was 83.8 cm and her mid-abdominal girth at its greatest point was 90.2 cm. Preoperative and postoperative appearance of the patient's abdomen and her CT scans are shown in Figs I and 2. The patient had a relatively smooth postoperative course. However, she did require 2 to 3 L of crystalloid for the first 2 to 3 days postoperatively to maintain her systolic blood pressure at 110 mm Hg. She was discharged from the hospital on the ninth postoperative day with a BUN of 2.5 mmol/L (7 mg!dL) and a serum creatinine of 70.7 /Lmol/L (0.8 mg! dL). Her discharge weight was 48.9 kg. Follow-up at I month showed an endogenous creatinine clearance of 1.75 mL/s (105 mL/min), a stable blood pressure of 120/82 mm Hg, and complete relief of symptoms of abdominal pain and discomfort. She was active, playing tennis, and without symptoms. She did require repair of an incisional hernia 9 months after surgery. A 3-year follow-up shows continued relief of pain without increase in abdominal girth and normal blood pressure