Review for 10.1186/s12876-021-01703-x (2021)

Reviewer’s report
Title: 
Liver Transplantation in Adult Polycystic Liver Disease: The Ontario Experience.
Version: 
0 
Date: 
04 Jan 2021
Reviewer's report:
This paper entitled "Liver transplantation in adult polycystic liver disease: The Ontario experience"
describes the outcome of 51 patients (43 females and 8 males) who underwent liver transplantation (LT).
Indications for LT were mass effect symptoms in 36 patients and portal hypertension in 8 (and what about
the 7 remaining patients ?). Twenty nine patients received LT alone, 7 had simultaneous liver kidney
transplant and 15 received a kidney transplant prior LT. Survival was excellent (96% at 5 and 10 years). 
Nonetheless, some questions arise and there are several missing relevant informations.
- It seems that MELD score was significantly higher in males probably due to higher kidney failure in this
group. Could the authors explain this difference ?
- Precise radiological assessment do not appear at all in the text (extend of cysts by segments and sectors,
vascular compressions) which should as a matter of fact lead to PCLD classifications (Gigot, Qian, Mayo
clinic) and surgical decision making. 
- Histology of non cystic parenchyma is lacking.
- What was the cause of portal hypertension ? Vascular compression and/or fibrotic parenchyma ?
- Do some patients could have been selected for partial hepatectomies with cyst fenestration instead of LT
(for exemple those with PCLD classified C according to the Mayo clinic classification) ?
- Operative data, transfusion requirements, post-operative course of kidney function and therapeutic
protocols and there evolution over time are missing.
- LT in PCLD patients is mainly performed in the aim to improve patient quality of life. Whether or not it
is ethically justified remains an issue. As it has been showed in several relevant articles (Chebib 2016,
Aussilhou 2010, Schnelldorfer 2009, Barbier 2018), partial hepatectomy with cyst fenestration could be
an alternative to LT when there is at least one liver sector preserved from cysts and absence of portal
hypertension; long term results are excellent and it avoids potentially deleterious immunosuppression and
taking a liver from the limited donor pool. the authors should discuss this point in the light of the
radiological evaluation of their patients. Finally, massive hepatomegaly (class D of the Mayo clinic
classification) do not only impair quality of life but becomes life-threatening over time and LT represents
the only therapeutic option.
This article could deserve to be published in reference to the rather large number of patients upon major
modifications required in this text.
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