Studer orthotopic ileal bladder substitute construction - surgical technique and complication management: one-center and 12-year experience.

PURPOSE: We have performed Studer neobladder creation in 61 patients (53 male and 8 female). The aims of this study were to evaluate the clinical outcomes, to review the surgical technique modification, postoperative complications management and metabolic disturbances. MATERIAL AND METHODS: All patients were retrospectively studied and followed-up. The follow-up: 12 years to 2 months. 44 patients (41 male and 3 female) returned for a control visit. All completed IIQ-7 questionnaire. Continence was analysed. Kidney ultrasound, post void residual and uroflowmetry, blood tests (electrolytes, kidney markers, acid-base balance) were performed. All patients were divided into two groups: I (with Zuber mineral water intake) and group II (without Zuber mineral water intake) for acid-base balance analysis. RESULTS: Early complications occurred in 13.1% (enterocolitis, neobladder-urethral anastomosis leakage, pyelonephritis, and lymphorrhoea). Late complications occurred in 14.0% (stricture of the neobladder-urethra anastomosis, urosepsis secondarily to bilateral hydronephrosis, stone formation, and pyonephrosis). In the follow-up 88.6% of patients revealed normal continence. The nocturnal incontinence, nocturia, and external or indwelling catheter were reported in 9, 6 and 5 patients, respectively). In IIQ-7 the mean negative impact of continence level on patients quality of life was 10.08% ± 14.47%. The mean Qmax., Qave., post void residual were 15.8 ± 4.9 ml/s, 7.9 ± 3.0 ml/s, and 151.2 ± 139.2 ml, respectively. Patients who regularly intake the Zuber present significant decrease of BE deficiency as compared to patient without Zuber usage. CONCLUSIONS: The Studer neobladder is the alternative urinary diversion. This is the difficult, skill demanding procedure, nevertheless gaining experience with self modifications resulted in decrease of complications. The Zuber mineral water intake ameliorates the base excess deficiency after Studer creation.

Kidney transplantation in children with bladder augmentation or ileal conduit diversion.

INTRODUCTION: Various congenital and acquired diseases of the lower urinary tract can lead to chronic renal failure requiring renal replacement therapy. AIM: The aim of the study was to assess problems and results of kidney transplantation in children with significant lower urinary tract dysfunction. MATERIALS AND METHODS: Between 1984 and 2007, there were 33 kidney transplantations in children with end-stage renal disease and severe lower tract dysfunction out of 539 kidney transplantations performed in our department. The patients were 23 males and 10 females. Thirty patients received a kidney from a deceased donor, the remaining 3 from a living related donor. The age at transplantation ranged from 2.25 years to 19 years. In 26 patients an ileal conduit modo Bricker was created (in 21 patients at transplant operation). Bladder augmentation was performed in 6 patients and a continent urinary reservoir was created in 1. RESULTS: Post-transplant follow-up ranged from 7 to 88 months (mean 32 months). Overall patient survival is 100% and graft survival is 97%. Creatinine concentrations ranged from 0.3 to 3.4 mg% (mean 0.92 mg%). Surgical complications were diagnosed in 16 patients. All surgical complications were treated successfully and none of them caused graft loss. Urinary tract infections (UTI) were the most commonly observed complication, occurring in 26/33 (78%) patients, but the majority of these UTI were asymptomatic and had no influence on graft function. CONCLUSIONS: Kidney transplantation in children with lower urinary tract dysfunction and end-stage renal failure offers excellent medium term results in our experience, despite the creation of non-standard urinary drainage. Recurrent urinary tract infections are the most common complications in these patients, but in the majority of cases this does not lead to impairment of graft function.

Vascular complications after pediatric liver transplantation from the living donors.

Early arterial or portal vein thrombosis is a complications that can lead to graft loss and patient death or need of immediate retransplantation. The aim of the study was to assess the incidence, causes, treatment, and outcome of vascular thrombosis after living related donor liver transplantation (LRdLTx). Between 1999 and 2004 71 LRdLTx were performed in children aged from 6 months to 10 years. Vascular thrombosis was found in 12 recipients. Hepatic artery thrombosis (HAT) occurred in 4 (5.6%), portal vein thrombosis (PVT) in 8 (11.2%) cases. HAT occurred 5 to 8 days, PVT 1 to 22 days after LTx. Diagnosis of vascular thrombosis was confirmed by routine Doppler ultrasound examination. Thrombectomy was successful in one patient with HAT and in three patients with PVT. Venous conduit was performed in one patient with PVT after second thrombosis. Two children developed biliary strictures as a late complication of HAT and required additional surgical interventions. Two children with PVT developed portal hypertension with esophageal bleeding, which required surgical intervention; one another underwent endoscopic variceal ligation for grade III varices. Follow-up ranged from 7 to 60 months. One patient died as a result of HAT after retransplantation due to multiple intrahepatic abscesses 2 months after first transplant. Any risk factors of vascular thrombosis that can be controlled should be avoided after transplantation. Routine posttransplant Doppler examination should be performed at least twice a day within 7 to 14 posttransplant days. Immediate thrombectomy should be always carried out to avoid late complications and even mortality.

Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass.

AIMS: Progressive familial intrahepatic cholestasis (PFIC, Byler's disease) is an autosomal recessive disorder resulting in liver fibrosis/cirrhosis and liver insufficiency. Before the 1990s, liver transplantation was the only effective therapy for these children. During the last 12 years, two alternative methods of surgical treatment have been proposed: partial external biliary diversion (PEBD) and ileal bypass procedure (IB), which allow for effective elimination of bile acids accumulated in the body. In this study, we compare the efficacy of these surgical techniques for PFIC. METHODS: During the last 20 years, we have treated 52 children with PFIC. PEBD was done in 21 patients (since 1995), and IB in 5 patients (since 1998), transplantation was performed in 9 patients (since 1990). The efficacy of non-transplantation surgical treatment was assessed by patients' clinical outcome, liver biochemistry, and survival without transplantation during a follow-up period of 12 to 48 months. RESULTS: In 15 out of 21 patients clinical symptoms improved after PEBD and liver function tests normalised (blood bile acids), 1 patient had to be converted to IB due to too high output biliary fistula, 2 patients were transplanted and 3 are considered for transplantation. Out of the 5 children after IB, 4 improved clinically and biochemically, but, after 12 months, symptoms recurred in 3 patients, one patient was converted successfully to PEBD. No significant influence on growth was observed, irrespective of the type of treatment in this group of patients. CONCLUSIONS: PEBD is more effective than IB for the permanent improvement of symptoms of PFIC. Ileal bypass procedure, although initially effective, does not ensure good long-term results in more than 50 % of patients, probably due to intestinal re-absorption of bile acids increasing over time.

Liver retransplantation in children in Poland.

An average of 15% of patients require retransplantation due to irreversible liver graft failure due to primary graft nonfunction, chronic rejection, vascular and biliary complications, or infections. The survival of patients and grafts after retransplantation is inferior to that after primary transplantation. The purpose of the present study was to examine the incidence, indications, and outcome of retransplantation in children. In our center 169 liver transplantations had been performed in 154 patients, and 14 patients (9%) required 15 retransplantations: nine in the early postoperative period, five late after primary transplantation, and one late after the second transplantation. One-year patient survival after primary transplantation was 82%, but after early retransplantation it was 55%.

Liver transplantation across ABO blood groups in children.

Late results after ABOI LTx are inferior to ABO compatible organs. We report seven patients who received LTx across ABO group for emergency indications. The blood type combinations were: A to O in three, B to O in two, and B to A in two. Episodes of acute and chronic rejection, immunosuppression, and biochemical and functional tests after transplantation as well as patient and graft survival were compared between ABOI group and patients with compatible ABO group transplanted due to FLF (group I) or in an elective setting (group II). Four children are alive. Two children died of sepsis and CNS damage or MOF, and one patient died during transplantation because of cardiac failure. All recipients of ABOI grafts received immunosuppression with cyclosporine or tacrolimus and steroids. MMF was added in two subjects, and induction with antilymphocyte globulins used in five patients. An acute rejection episode was diagnosed in two recipients between 7 and 11 days after LTx. All four living patients with ABOI grafts are doing well with follow-up time between 11 months and 5 years. In one patient PTLD occurred at 1 year after ABOI LTx but was cured by discontinuation of immunosuppression and administration of rituximab. Graft survival in the ABOI group was 57.1% versus 71% in group I and 73% in group II. Respective patients survival was 57.1% 71%, and 82.0% respectively. In conclusion, in urgent cases ABOI transplantation is justified in pediatric patients when compatible grafts are not available.

Switching cyclosporine blood concentration monitoring from C0 to C2 in children late after liver transplantation.

BACKGROUND: Measurement of cyclospoprine (CsA) blood levels at 2 hours after oral administration (C(2)) has been proposed as a better measurement of trough level (C(0)) due to reduced intrapatient variability, acute rejection rate and renal toxicity. The aim of the present study was to assess whether there was any advantage to conversion from C(0) to C(2) CsA blood level monitoring in children late after liver transplantation. We reviewed the data from 44 children more than 1 year after liver transplantation. We measured the daily dose of CsA and the C(0) level before switching versus the daily dose and C(2) level at 6 months after conversion, in addition to the alanine aminotransferase (ALT) activity, creatinine blood concentration, and episodes of acute rejection. RESULTS: Conversion from C(0) to C(2) monitoring was not associated with a significant change in mean daily dose of CsA, mean concentration of creatinine, ALT activity or occurrence of rejection episodes. CONCLUSION: Switching from C(0) to C(2) monitoring did not seem to proffer any benefits for children late after liver transplantation.

Acute coagulopathy after reperfusion of the liver graft in children correction with recombinant activated factor VII.

BACKGROUND: Several studies have proven that massive blood loss increases postoperative morbidity and mortality in liver graft recipients. Since we have successfully corrected coagulopathy preoperatively using an intravenous (IV) bolus of recombinant activated factor VII (rFVIIa) in 2 patients with fulminant liver failure, we observed that there was rapid reversal of preexisting advanced coagulopathy in another 40 patients with high risk for intraoperative bleeding by this treatment immediately before transplantation. Recently to control hemostasis we have administered rFVIIa also to patients presenting with acute coagulopathy and nonsurgical bleeding after graft reperfusion as described herein. MATERIALS AND METHODS: We have used rFVIIa in 7 children presenting with severe coagulopathy and nonsurgical bleeding after liver graft reperfusion. The dosage of rFVIIa ranged between 37 and 148 mcg/kg. An antifibrinolytic agent (aprotinin, tranexamic acid) was administered simultaneously. RESULTS: APTT before rFVIIa was 86.10 to 183 seconds, (mean, 132.1 +/- 39.88), after the bolus of rFVIIa 49.4 to 206.1 (mean, 112.7 +/- 58.53), and at the end of surgery 71.70 to 180 (mean, 110.3 +/- 40.98). INR after reperfusion was 1.82 to 3.91 (mean, 2.56 +/- 0.67), 1.03 to 1.92 (mean, 1.54 +/- 0.35) after rFVIIa, and 1.74 to 5.58 (mean, 2.64 +/- 1.35) at the end of surgery. Before rFVIIa administration intraoperative blood transfusions after graft reperfusion were 900 to 4200 mL of red blood cells (RBC) (0.82-5.4 total blood volume) and after reperfusion 0 to 1800 mL of RBC (0-2.5 TBV). No postoperative vascular complications were observed. CONCLUSIONS: A single dose of rFVIIa effectively reverses the severe coagulopathy developing after graft reperfusion, establishing effective hemostasis in liver transplant recipients without an increased risk of thrombotic complications.

Renal and ureteric trauma: diagnosis and management in Poland.

OBJECTIVE: To analyse retrospectively kidney and ureteric injuries (the former often associated with multiple-organ trauma) and thus optimize diagnostic and treatment methods. PATIENTS AND METHODS: The records and details of kidney and ureteric injures treated between 1995 and 1999 in 61 urological departments in Poland were analysed. RESULTS: In all, 887 kidney injuries were analysed; blunt trauma comprised 97%, with most injuries classified as renal contusion and minor parenchymal damage (687 cases). Intravenous urography was used in 80% of the patients and computed tomography in only 20%. In all, 234 patients (26%) underwent surgery; nephrectomy was the most common surgical treatment, in 170 patients (73% of those undergoing surgery). Complications occurred in 9% of patients after conservative treatment and in 5% after surgery. Of the 452 ureteric injuries, 340 (75%) were iatrogenic, 81 (18%) blunt injuries and 31 (7%) open injuries. Of the iatrogenic injuries 73% occurred during gynaecological procedures, 14% in general surgery and 14% in urological procedures. The most frequent diagnostic method was intravenous urography (244 cases), with retrograde pyelography (98) and ureteric catheterization in 125. The diagnosis was established immediately during surgery in 104 patients. The most frequent surgical treatment was uretero-neocystostomy (213, 47%), the others being a Boari flap (113, 25%), end-to-end anastomosis (92, 20%), reconstruction with an ileal loop (30, 7%) and autotransplantation (four, 1%). CONCLUSION: In Poland, patients with blunt kidney injuries often undergo surgery, with nephrectomy the most frequent procedure. Computed tomography with the intravenous administration of contrast medium should be considered the diagnostic method of choice for kidney injures. Catheterization of the ureters before surgery and an indigocarmine intravenous infusion (to stain the urine) when a ureteric injury is suspected may reduce the rate of iatrogenic injury and improve the rate of intraoperative diagnosis. We suggest catheterizing the ureters in any doubtful case to avoid injury, because prevention is better than treatment.

External and iatrogenic trauma of the urinary bladder: a survey in Poland.

OBJECTIVE: To assess incidence of urinary bladder injuries (frequently associated with pelvic trauma and often iatrogenic) in Poland. MATERIALS AND METHODS: The records and details of urinary bladder injuries treated between 1995 and 1999 were analysed for 61 urological departments in Poland. RESULTS: During the 5-year period 512 patients had urinary bladder injuries; in 210 (41%) the injury was caused by a road traffic accident, in eight (2%) by compression (crushing injury) within the limits of the pelvic bones, in 40 (8%) by a fall from a height, in three by a gunshot wound and in the remaining 251 (49%) the injury was iatrogenic. Among the 261 bladder injuries that were not iatrogenic, 41 (16%) were associated with pelvic bone trauma. In 36 patients there was simultaneous injury of the urinary bladder and posterior urethra, constituting 14% of such injuries and 7% of all trauma cases. The iatrogenic injuries were in 98 patients (39%) in urological departments, in 130 (52%) women in gynaecological departments and in 23 (9%) on surgical wards. The injury was open in 102 patients (20%) and closed in 372 (73%); there was bladder contusion in 38 patients (7%). The injuries were intraperitoneal in 225 patients (44%) and extraperitoneal in 287 (56%). For diagnosis, abdominal ultrasonography was used in 455 (89%) patients, intravenous pyelography in 266 (52%), cystography in 388 (76%) and computed tomography in 15 (3%). The delay between trauma and diagnosis was 0.5-124 h. Surgical treatment of the injury comprised a monolayer suture of the bladder wall in 51 patients (10%), a two-layered suture in 461 (90%), perivesical drainage in 468 (91%) and inspection of the peritoneal cavity in 232 (45%). The mean (range) interval between diagnosis and treatment was 14 (7-70) days. Seven patients died after the treatment failed. CONCLUSIONS: Almost half the patients had iatrogenic injuries, of which over half occurred in gynaecological and maternity wards. Thus it is important teach the basic range of urological operations to trainee doctors. The commonest diagnostic method was not ascending cystography but ultrasonography; we recommend ascending cystography be used with at least two views after filling the urinary bladder with approximately 300 mL of contrast medium, with an additional film after emptying the contrast medium. In patients with pelvic bone trauma it is reasonable to use spiral computed tomography with virtual analysis before surgery. A two-layered suture of the bladder wall with perivesical drainage should be used.

Assessing the vascular-stromal coefficient in patients with benign prostatic hyperplasia or prostate cancer using transrectal ultrasonography and power Doppler analysis.

OBJECTIVES: To assess a coefficient of the prostatic blood supply in patients with prostate cancer or benign prostatic hyperplasia (BPH), and thus improve the diagnostic and prognostic variables needed to evaluate patients with prostate cancer. PATIENTS AND METHODS: The vascular-stromal coefficient was defined as the ratio of the blood vessel surface area and the surface area of the prostate, using power Doppler functions and transrectal ultrasonography. The index was calculated for the plane of the prostatic surface from the apex to the widest points of the peripheral zone, and for the plane across the highest density of blood vessels in the transition zone. A micobubble contrast agent was used to intensify the acoustic signals from prostatic vessels. The images were reconstructed in three dimensions. In all, 61 patients with BPH and 32 with confirmed prostate cancer were examined, and compared with a control group of young men. RESULTS: In the horizontal plane crossing the apex of the prostate, the vascular-stromal coefficient was 0.0054 for the controls, 0.0072 for BPH and 0.0067 for cancer. At the level of the highest density of blood vessels the respective values were 0.0083, 0.038 and 0.029. The respective values for the first plane after using the contrast agent were 0.025, 0.092 and 0.0365, and in the second level 0.04, 0.15 and 0.14. CONCLUSION: The vascular-stromal coefficient measured at both levels was higher in patients with BPH than in those with cancer or than in the controls. The prostatic vascular system can be computer-reconstructed in three dimensions, allowing an evaluation of asymmetry and other vascular abnormalities. The contrast agent effectively increases the vascular acoustic signals from the prostate, enhancing by a factor of five the visualization of blood vessels when using the power Doppler option.

Vascular angiographic asymmetry on three-dimensional transrectal power Doppler ultrasonography in patients with organ-confined prostate cancer.

OBJECTIVE: To assess whether organ-confined prostate cancer not detected on routine transrectal ultrasonography (TRUS) can be detected using three-dimensional (3-D) power Doppler methods. PATIENTS AND METHODS: A preliminary trial of the use of TRUS with 3-D power Doppler image reconstruction was conducted in 116 patients being evaluated for lower urinary tract symptoms. Using a microbubble enhancing agent, an adequate horizontal plane allowed the simultaneous visualization and assessment of the symmetry of echogenicity and vascularization of the peripheral area of both prostate lobes, which is not possible in standard TRUS. The peripheral zone vascular asymmetry in the horizontal plane was also assessed retrospectively in those patients with pT2a,b prostate carcinoma. RESULTS: Stage pT2a,b prostate carcinoma was confirmed in 26 patients; in this group, two independent investigators confirmed the vascular asymmetry between the left and right peripheral zone, in the horizontal plane, in 22 patients, giving a sensitivity of 85%. CONCLUSIONS: Vascular asymmetry allows guided biopsy in lesions that are invisible using standard TRUS. Microbubble agents are an effective method of increasing the acoustic signals from small vessels of the prostate.

Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion.

Progressive intrahepatic familial cholestasis (PFIC), previously called Byler's disease, is a syndrome in which children develop severe cholestasis progressing to biliary cirrhosis and chronic liver failure, usually during the first decade of life. Clinical features include jaundice, hepatomegaly, splenomegaly, growth retardation and severe pruritus. Laboratory tests demonstrate elevated bilirubin and bile acids, without an increase in serum gamma-glutamyl-transpeptidase or cholesterol. This study was performed to evaluate our experience with medical therapy as well as two types of surgical treatment used in children with PFIC, particularly partial external biliary diversion (PEBD) as an alternative method of therapy to liver transplantation (OLTx). Between 1979 and 1998 we have treated 46 children with PFIC (27 boys and 19 girls), aged 10 months to 19 yr (at the time of this study). Medical treatment with ursodeoxycholic (UDCA) was used in 39 patients for the period between 6 and 82 months. PEBD (cholecysto-jejuno-cutaneostomy) was performed in 16 patients, OLTx in eight children (including one after unsuccessful PEBD). Retrospective analysis of the clinical course and selected laboratory tests (bilirubin, ASPAT, ALAT, bile acids), and histopathological examinations were performed. Results of treatment were assessed by means of influence of the type of treatment on clinical symptoms, laboratory tests, progress of liver cirrhosis and hepatic failure, as well as physical development and survival. Medical therapy was effective in the long term in four (10%) of the patients resulting in clinical and biochemical normalization. Both surgical methods of therapy of PFIC, PEBD and OLTx, resulted in an 80% success rate and therefore should be used as complementary therapies. In patients before established liver cirrhosis, PEBD should be the first choice of treatment. Patients presenting with cirrhosis or after ineffective PEBD should qualify for OLTx. With this strategy most children with PIFC can be cured.

Primary vascular thrombosis after renal transplantation in children.

The aim of the study was to assess the incidence, causes, diagnostic and treatment modalities, and outcome of vascular thrombosis after kidney transplantation in children. Between 1984 and 1995 we performed 176 kidney transplants in pediatric recipients aged 1 to 18 years. Vascular thrombosis followed 7 transplants, 4 were renal vein and 3 arterial thromboses. Venous thromboses occurred 2 to 12 days after transplantation. All of the patients with a renal vein thrombosis lost their grafts. Arterial thrombosis developed in 2 cases of double renal arteries which were separately anastomosed into the recipient vessels. One graft was lost, but the other was saved by thrombolytic therapy (streptokinase). One child experienced intrarenal segmental artery thrombosis during acute vascular rejection, which resolved following combined anti-rejection and thrombolytic (intra-arterial streptokinase) treatment with full recovery of graft function. In all, vascular thrombosis complicated 7 out of 176 transplants (4.0%), and was the cause of 5 graft losses (2.8%). The incidence of vascular thrombosis was not increased in grafts with vascular anomalies (3/34 v. 4/142; p>0.05, chi sq.). We conclude that acute tubular necrosis, rejection and unstable volemia may predispose to vascular thrombosis. In selected cases, early diagnosis of vascular thrombosis may enable graft salvage by surgical or thrombolytic treatment.

Esophageal manometric studies in children with achalasia before and after operative treatment.

This study manometrically assessed and compared esophageal function in 16 children with achalasia before and after surgical treatment (anterior esophagomyotomy with antireflux partial fundoplication). Manometric examinations were done in 10 children preoperatively and in 12, 3 months to 8 years postoperatively. Both pre- and postoperative examinations were done in 6 patients. The following parameters were measured: lower esophageal sphincter (LES) pressure and length, spontaneous motility of the esophageal body, and motility provoked by swallowing of fluids. Preoperative examinations confirmed disturbances typical for achalasia: increased LES pressure (mean 39.4 mmHg), lack of relaxation upon swallowing, and various types of anomalous esophageal motility (lack of propulsive waves, segmental waves, breaks in propagation of contractions, tonic contractions, etc.). Postoperative examinations showed normalization of LES pressure; however, relaxation did not appear in any patient. Esophageal motility improved after surgery in most patients and was already noticeable 3-6 months postoperatively, but motility never returned to normal. Clinically, all but 1 patient with reflux esophagitis were doing well despite persistent motility disturbances. Our study confirms that achalasia is a complex motor disorder of the entire esophagus. The improvement of esophageal contractility after esophagomyotomy suggests both primary and significant secondary damage to motility of the esophageal body in most patients. It appears that secondary disturbances are reversible to some extent in children after surgical treatment.

Diagnosis and monitoring of cytomegalovirus infection after liver transplantation in children.

Cytomegalovirus infection (CMV) complicated the posttransplant course in 9 of 24 children after liver transplantation. We found specific antibodies (IgG and IgM) to be of very low value in diagnosis and monitoring of CMV infection after liver transplantation. Detection of CMV-DNA by PCR method in the blood or urine was very useful for diagnosis, but less for monitoring of the course of disease and its treatment. Measurements of early immediate CMV antigen (IEA), in peripheral blood leucocytes allowed for very early diagnosis of CMV infection and correlated well with the course of disease and response to treatment of the patient.