Treatment of highly symptomatic polycystic liver disease: Preliminary experience with a combined hepatic resection-fenestration procedure

Keith D. Newman, Vicente Torres, Jorge Rakela, David M. Nagorney

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Abstract

Polycystic liver disease (PLD) associated with autosomal dominant polycystic kidney disease is usually well tolerated. However there is a small subset of patients who become incapacitated by massive liver enlargement and for whom effective nonsurgical therapy is limited. Recent surgical advances in the treatment of PLD have raised uncertainties regarding proper management of these highly symptomatic patients. We have reviewed our recent experience with a combined hepatic resection-fenestration procedure to assess its efficacy in nine patients. All patients underwent resection of two or more liver segments and extensive fenestration of residual cysts in the remnant liver. Symptomatic relief and reduction in abdominal girth were obtained in eight surviving patients, persisting for an average follow-up period of 17 months. No progression of cystic disease has been observed clinically or by computed tomography and hepatic function was preserved. Three patients had no complications. Five patients had complications including transient right pleural effusion (3) and thrombosis of an arteriovenous fistula (2). One patient who had a previous hepatic cyst fenestration and a cadaveric renal transplantation died after operation of an intracerebral hemorrhage after experiencing coagulopathy, hyperbilirubinemia, and sepsis. Our results suggest (1) some highly symptomatic patients with massive PLD may benefit from combined hepatic resection and fenestration with acceptable risk, and (2) previous liver surgery and immunosuppressive therapy may increase the risk of such surgery. Longer follow-up is needed in a larger number of patients to determine the duration of benefit from the combined resection-fenestration procedure for highly symptomatic PLD.

Original languageEnglish (US)
Pages (from-to)30-37
Number of pages8
JournalAnnals of Surgery
Volume212
Issue number1
StatePublished - Jul 1990

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Liver
Therapeutics
Cysts
Polycystic liver disease
Autosomal Dominant Polycystic Kidney
Hyperbilirubinemia
Arteriovenous Fistula
Cerebral Hemorrhage
Pleural Effusion
Immunosuppressive Agents
Kidney Transplantation
Uncertainty
Disease Progression
Sepsis
Thrombosis
Tomography

ASJC Scopus subject areas

  • Surgery

Cite this

Treatment of highly symptomatic polycystic liver disease : Preliminary experience with a combined hepatic resection-fenestration procedure. / Newman, Keith D.; Torres, Vicente; Rakela, Jorge; Nagorney, David M.

In: Annals of Surgery, Vol. 212, No. 1, 07.1990, p. 30-37.

Research output: Contribution to journalArticle

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