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AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (PCKD)

Background1

  • Two genes, PKD1 and PKD2

  • Ten percent of cases of end-stage renal disease (ESRD)

  • No gender or race preference

  • Most frequent etiology of inherited renal failure.

  • Up to 50% of patients progress to ESRD by age 70

Systemic findings1

  • Dysregulation of the renin–angiotensin–aldosterone system

  • Severe hypertension

  • Compression of surrounding viscera

  • Pain

  • Hemorrhage

  • Infections

  • Urosepsis

  • Potential risk of renal malignancies

Treatment Options

  • Bilateral nephrectomy (in instances of severe and/or recurrent systemic findings)

    • Before transplantation

      • Not recommended in preemptive candidates

      • Additional surgery

      • Can increase Panel Reactive Antibody (PRA) values (due to blood transfusions)

      • Increased incidence of wound complications at the time of transplantation1

      • Anephric state

    • During transplantation

      • Single surgical intervention

      • Increased operative time and complexity of surgery

      • Risk to transplanted kidney

      • Transfusion of blood products

      • Possible complications:

        • Splenic injury (potentially requiring splenectomy)

        • Vascular injury and bleeding

        • Diaphragmatic injury

        • Postoperative intraabdominal collections/infections

        • Inability to remove both kidneys due to hemodynamic instability

    • After transplantation

      • Risk to transplanted kidney

      • Additional surgery

  • Observation

    • Persistence of systemic findings (if present)

Indications1–4

  • Intractable pain

  • Recurrent infections

  • Severe hemorrhage

  • Early satiety

  • Abdominal size

  • Inability to implant a renal allograft (very rare)

Technique1–4

  • Midline laparotomy to access the native kidneys and iliac vessels

  • Native kidneys removed first

  • Transplant kidney implanted intraperitoneally after removal of native kidneys

  • Alternatively, the peritoneum at the midline incision can be separated from the abdominal wall laterally and dorsally up to the iliac vessels allowing for a retro-peritoneal implantation of the kidney

  • Allograft fixation is recommended to avoid graft torsion, given the intraperitoneal position and the vast amount of space after native nephrectomy

  • Usually performed in instances of live donor transplants

Outcomes

  • Recipients who underwent bilateral nephrectomies at the time of transplantation showed

    • Increased incidence of renal vascular thromboses than those who underwent transplantation alone (4.4% vs. 1.3%)1

    • Similar short-, intermediate-, and long term posttransplant graft and patient survival than recipients who underwent transplantation alone or who had bilateral nephrectomies before transplantation1–4

    • No increased incidence of Delayed Graft Function (DGF), deep venous thromboses (DVTs), hernias, ileus, small bowel obstruction, or urinary strictures/leaks1–3

    • No overall increased morbidity or mortality2,3,5

    • Potentially increased operative time, transfusion requirements, length of hospital stay, intensive care unit (ICU) admission

    • Lower incidence of lymphoceles1

    • Improved quality of life2–4

    • Relief of symptoms1–4

    • Patient satisfaction

    • Sixteen percent of patients with polycystic kidney disease who underwent only transplantation required native unilateral or bilateral nephrectomies within 10 years1

CONCLUSION

With appropriate indications and informed consent, simultaneous bilateral nephrectomy and kidney transplantation is associated with resolution of symptoms and patient satisfaction in the setting of graft and recipient survival equivalent to ...

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