a 73 year - old patient with mild hemophilia b ( factor ix clotting activity  12% ) was referred to our department due to a left renal tumor incidentally discovered during ultrasonography performed as follow - up two years after prostate cancer brachytherapy . on admission
abdominal and pelvic ct scans revealed a multilocular tumor in the lower left kidney pole ( 57x52x52 mm ) with a solid mass of 3.5 cm in diameter ( figure 1 ) . other abdominal and chest imaging showed no abnormalities .
twelve hours before surgery , he was transfused with 4200 units of factor ix concentrate ( octanine , octapharma ag , switzerland ) , with the same dose administered one hour prior to the procedure .
on october 3 of 2014 , the procedure was performed with the patient positioned on his right side . following urinary catheterization
, a veress needle was introduced close to the umbilicus and an intraperitoneal pressure of 14 mmhg was created .
the descending colon was dissected and the fat renal capsule subsequently cut for access to the left renal artery .
the artery was then clipped with polymeric clips ( hem - o - lok xl ( teleflex , usa ) and cut in between . the same was done for the renal vein .
the kidney was excised , placed into a hemobag ( covidien , usa ) and removed through a small incision between the two ports .
a plastic drain was placed into the kidney bed and the abdominal wall was closed with layers of sutures .
peritoneal drainage was kept in place for two days with no significant output of blood or serous fluid .
substitution therapy was continued for 9 days with the level of factor ix clotting activity at 90130% of the normal value on days 13 , 6574% on days 46 and 3040% on days 79 .
the postoperative course was uneventful and the patient was discharged on 10 day after surgery .
histopathological investigation revealed renal carcinoma , clear cell type ( g2 ) , limited to the parenchyma , without capsule infiltration or angioinvasion .
no abnormalities were revealed in ultrasonography and ct scans . computed tomography scan revealed a left kidney tumor mass .
hemophilia b ( christmas disease ) is a congenital bleeding disorder resulting from factor ix deficiency .
hemophilia b patients undergoing surgical procedures are at a high risk of severe bleeding complications unless surgery is performed after adequate correction of the diathesis .
secure hemostasis in hemophilia b patients is achieved with transfusions of factor ix coagulation factor concentrate , either recombinant or plasma - derived .
desirable preoperative activity is 6080% of the normal value and should be maintained until complete wound healing , usually through 710 days . on days 13 the recommended value is 6080% , on days 46 4060% and 3040% on days 710 .
minimally invasive procedures in patients with hemophilia , apart from well - known benefits such as reduced postoperative pain , fewer complications , better cosmetic effect , shorter hospitalization , quicker convalescence and return to professional activity , also contribute to shorter duration of substitution therapy .
the first laparoscopic nephrectomy was performed by clayman et al . in 1990 . since then
, the procedure has been performed in an increasing number of urologic centers [ 4 , 5 ] .
there are only few reports in the literature of urinary tract operations in patients with congenital coagulation disorders [ 6 , 7 ] ; nonetheless , there have been none of laparoscopic nephrectomy in a hemophilia patient .
we present the first case of laparoscopic nephrectomy in a hemophiliac performed with an adrenal - saving , transperitoneal lateral approach using four trocars .
no significant complications were observed either postoperatively or in the long - term , which can most likely be attributed to the adequate preparation of the hemophilia patient for surgery , as well as the effective cooperation between urologist and hematologist .
perioperative and postoperative bleeding episodes are the most frequent complications reported in hemophilia patients . for patients with no coagulation disorders ,
tlrn intraoperative bleeds are observed at 2.22.8% , and are frequently a reason for conversion to an open procedure .
there are no significant differences in the reported percentage of all surgical complications , hemorrhage included , between hemophilia patients with adequate substitution of deficient coagulation factor and patients with no coagulation disorders [ 8 , 9 ] .
it is therefore crucial for hemophilia patients to undergo surgery in special reference centers , which provide expert health - care services and are equipped with a modern laboratory for careful daily monitoring of deficient coagulation factor activity [ 9 , 10 ] . in conclusion ,
laparoscopic radical nephrectomy in hemophilia patients is a safe and effective procedure for renal carcinoma .
surgery should be performed by an experienced urologist in cooperation with a multidisciplinary team , providing both perioperative and postoperative care , supported by a specialized laboratory .
 surgery in patients with hemophilia is a serious challenge . 
 it requires a comprehensive approach , as well as careful postoperative monitoring . 
 we present here the first case of a transperitoneal laparoscopic radical nephrectomy ( tlrn ) for renal cell carcinoma , of the clear - cell type , performed in a hemophilia b patient . 
 the level of factor ix clotting activity before surgery and on postoperative days 16 was maintained at 65130% and at 3040% on subsequent days until healing of the post - operative wound was achieved . 
 the intraoperative and postoperative courses were uneventful . 
 tlrn can therefore be considered safe and effective for renal cell carcinoma . in hemophilia patients , 
 the tlrn procedure requires proper preparation , as well as adequate substitution therapy for the deficient coagulation factor provided by a multidisciplinary team in a comprehensive center . 
CASE PRESENTATION
DISCUSSION
CONFLICTS OF INTEREST