Simultaneous Liver Kidney Transplantation in a Primary Type 2 Hyperoxaluria With Corrected TOF and Severe Cardiomyopathy: A Case Report.

Background: Primary type 2 hyperoxaluria is a very rare genetic disorder,1,2 where in the progression to renal failure was assumed to be insidious and not very common.3 PH2 is due to deficient glyoxylate reductase/hydroxypyruvate reductase (GRHPR),1,2 which was thought to have extra-hepatic production also.4 The progression to renal failure in these patient subgroups is well documented in the Literature and the role of SLK (simultaneous liver and kidney transplantation) has not been clearly established.8. Method: We present a case report of a young girl with PH2, who successfully underwent SLK, with evidence of reduction in the urine oxalate levels post SLK. Results: PH2, though a rare genetic disease, has a proven potential to progress to chronic renal failure requiring transplantation, renal transplantation alone has not shown any benefit, these patients can be offered SLK as a primary treatment option, to improve the outcomes, this needs further validation with consensus and studies.

Laparoscopic reconstruction of ureteral strictures involving solitary renal units-1 year and 5 year outcomes.

CONTEXT: Long-term outcome following a laparoscopic reconstruction of ureteral strictures (US) involving solitary renal units (SRU) are scarcely reported. AIMS: The aim was to report short-term (1 year) and long-term (5 years) outcomes following a laparoscopic reconstruction of US in a solitary kidney. SETTINGS AND DESIGN: Retrospective. MATERIALS AND METHODS: Records of patients operated for similar scenarios between January 2004 and January 2014 were evaluated. Clinical, biochemical and radiological profile were noted. Operative and post-operative profile were recorded. Follow-ups were scheduled at regular intervals (3 months post-procedure, 6 monthly for 2 years and yearly thereafter. Imaging was repeated at yearly intervals). Outcome was assessed by comparing pre-operative and post-operative clinical, biochemical, and radiological parameters. STATISTICAL ANALYSIS USED: SAS software 9.2 version. A P < 0.05 was inferred as statistically significant. RESULTS: Seven patients underwent a laparoscopic reconstruction. Stricture location was upper ureter (n = 1), mid ureter (n = 2), lower ureter (n = 4). Surgeries performed were ureteroureterostomy, Boari flap ureteroneocystostomy and ureteroneocystostomy with psoas hitch. Four patients reported prior contralateral nephrectomy. Three patients underwent prior endoscopic correction. Four patients presented with elevated serum creatinine (>1.4 mg/dl). Till last follow-up, improvement in symptomatology and improvement or stabilisation of serum creatinine was perceived in all. Ureteral patency with resolution of hydronephrosis was observed in five patients at 1 year follow-up. Two patients revealed ureteral patency with persistence of hydronephrosis. Clinical, biochemical and radiological outcomes were maintained till long-term follow-up. CONCLUSION: Laparoscopic reconstruction of US in SRU offers impressive short- and long-term outcome.

Laparoscopic nephrectomy for autosomal dominant polycystic kidneys in patients with end-stage renal disease on maintenance hemodialysis: 10-year single surgeon experience from an Indian center.

CONTEXT: Pure laparoscopic nephrectomy in patients with ADPKD (autosomal dominant polycystic kidney disease) and ESRD (end-stage renal disease) on MHD (maintenance hemodialysis) is challenging with high incidence of complications. Limited experiences from India has been reported in these scenarios. AIMS: To present a 10-year single surgeon experience from India in laparoscopic nephrectomy in autosomal dominant polycystic kidneys (ADPKD) and end-stage renal disease (ESRD) on maintenance hemodialysis (MHD). SETTINGS AND DESIGN: Retrospective. MATERIALS AND METHODS: Retrospective analysis of records of similar subset of patients who were offered laparoscopic nephrectomy between 2003 and 2012. Preoperative, operative and postoperative parameters were recorded. Few technical modifications were adopted over the years. Patients were sub-classified into two groups (Group I: 2003-2006, Group II: 2007-2012) based on surgical technique. STATISTICAL ANALYSIS USED: SAS software 9.1 version. RESULTS: 75 patients (84 renal units, Group I: 31, Group II: 53) were included in this analysis. Unilateral procedure was performed in 66 and bilateral staged or simultaneous procedure in 9. Despite larger kidneys in Group II (mean longitudinal renal length 25.7 ± 3.4 vs 17.5 ± 2.7 centimeters, P <0.001), improved operative and postoperative profile were noted in Group II in several parameters-mean total operative time (205 ± 11.5 vs 310 ± 15.3 min, P = 0.00), time for specimen retrieval (30.5 ± 3.5 vs 45 ± 4.1 min, P = 0.02), postprocedure drop in hemoglobin (1.1 ± 0.1 vs 2.27 ± 0.03 grams/deciliter, P = 0.00). Conversion rates, intraoperative and postoperative events were also considerably less in Group II. CONCLUSIONS: Despite existence of comorbidities and technical difficulties, laparoscopic nephrectomy in patients with ADPKD with ESRD and on MHD is a feasible option. Technical modifications with increasing surgeon's experience allows successful conductance of this approach in more complex cases with better outcome.

Laparoscopic management of recurrent ureteropelvic junction obstruction following pyeloplasty.

OBJECTIVE: The aim was to analyze the operative, postoperative and functional outcome of laparoscopic management of previously failed pyeloplasty and to compare operative and postoperative outcome with laparoscopic pyeloplasty for primary ureteropelvic junction obstruction (UPJO). MATERIALS AND METHODS: All patients who underwent laparoscopic management for previously failed dismembered pyeloplasty were analyzed in this study. Detailed clinical and imaging evaluation was performed. Transperitoneal approach was followed to repair the recurrent UPJO. Operative, postoperative, and follow-up functional details were recorded. Operative and postoperative outcomes of laparoscopic redo pyeloplasty were compared with that of laparoscopic primary pyeloplasty. RESULTS: A total of 16 patients were managed with laparoscopic approach for previously failed pyeloplasty. Primary surgical approach for dismembered pyeloplasty was open in 11, laparoscopy in four patients and robotic assisted in one patient. Fifteen were treated with redo pyeloplasty and one with ureterocalicostomy. Mean operative time was 191.25 ± 24.99 min, mean duration of hospital stay was 3.2 ± 0.45 days and mean follow-up duration was 29.9 ± 18.5 months with success rate of 93.3%. Operative time was significantly prolonged with redo pyeloplasty group compared with primary pyeloplasty group (191.25 ± 24.99 vs. 145 ± 22.89, P = 0.0001). CONCLUSION: Laparoscopic redo pyeloplasty is a viable option with a satisfactory outcome and less morbidity.

Ex-vivo nephron-sparing surgery and autotransplantation for renal tumours: Revisited.

INTRODUCTION: We studied the feasibility of ex-vivo nephron-sparing surgery and autotransplantation for complex renal tumours. We also studied the role of laparoscopy in these situations. METHODS: All patients who underwent renal autotransplantation for renal tumour at our centre were included in this retrospective study. Patient profiles were recorded in detail. Operative and postoperative details were also recorded. RESULTS: Our series includes 3 patients. Two patients had complex renal cell carcinoma and 1 patient had bilateral large angiomyolipoma. In first 2 patients, laparoscopic approach was used for nephrectomy. Operative time for case 1, 2 and 3 was 5.5, 4.5, 8 (right side) and 6 (left side) hours, respectively. Cold ischemia time was 110, 90, 150 and 125 minutes, respectively. One patient required temporary postoperative hemodialysis. CONCLUSION: Ex-vivo nephron-sparing surgery and autotransplantation still remain a viable option for complex renal tumours. It offers satisfactory renal functional outcome with acceptable morbidity. The laparoscopic approach should be used whenever possible to reduce morbidity.

Laparoscopic extirpation of giant adrenal ganglioneuroma.

Laparoscopic adrenalectomy is the standard of care for management of adrenal neoplasms. However, large sized adrenal lesions are considered as relative contraindication for laparoscopic extirpation. We report laparoscopic excision of giant ganglioneuroma of adrenal gland in a 33-year-old female patient. Patient was presented with left loin pain of 2 months duration. Computed tomography (CT) scan was suggestive of non-enhancing left suprarenal mass measuring 17 × 10 cm. Preoperative endocrine evaluation ruled out functional adrenal tumor. Patient underwent transperitoneal excision of suprarenal mass. The lesion could be completely extirpated laparoscopically. Duration of surgery was 250 minutes. Estimated blood loss was 230 milliliters. Specimen was extracted through pfannenstiel incision. No significant intraoperative or postoperative happenings were recorded. Microscopic features were suggestive of ganglioneuroma of adrenal gland.

Laparoscopic reconstruction for obstructive megaureter: single institution experience with short- and intermediate-term outcomes.

PURPOSE: To narrate our experience with laparoscopic reconstruction of obstructive megaureter (MGU) and assess the intermediate-term outcome achieved. PATIENTS AND METHODS: Patients were evaluated in detail including presenting complaints, biochemical profile, and imaging (ultrasonography [USG], diuretic renography [DR], magnetic resonance urography [MRU], and voiding cystourethrography [VCUG]). All patients with a diagnosis of obstructive MGU and salvageable renal unit were offered laparoscopic reconstruction. The standard laparoscopic exercise included ureteral adhesiolysis until the pathologic segment, dismemberment, straightening of the lower ureter, excisional tapering, and a nonrefluxing ureteroneocystostomy. Operative and postoperative parameters were recorded. Patients were evaluated postprocedure on a 3-month schedule. Follow-up imaging included USG and VCUG at 6 months and 1 year postprocedure and then at yearly intervals. MRU and DR were repeated at 1 year postprocedure. RESULTS: Twelve patients (13 units-11 unilateral, and 1 bilateral) underwent laparoscopic tailoring and reimplantation for obstructive MGU. Mean age was 98.6 months. All patients were male. Mean body mass index was 17.69 kg/m(2). Presenting complaints were flank pain (n=8) and recurrent urinary infection (n=12). All procedures were completed via a laparoscopic approach. Mean operation duration was 183 minutes, and mean blood loss was 75 mL. Mean duration of hospital stay was 2.1 days. No major intraoperative or postoperative happenings were recorded. All patients were asymptomatic at follow-up with stable renal profile. Follow-up MRU revealed a decrease in ureteral and upper tract dilatation with satisfactory drainage in all. Follow-up VCUG demonstrated grade I vesicoureteral reflux in one patient. Eight patients completed 3-year follow-up with a satisfactory outcome. CONCLUSION: Laparoscopic reconstruction of obstructive MGU offers satisfactory immediate- and intermediate-term outcome without undue prolonged morbidity.

Transperitoneal laparoscopic dismembered pyeloplasty in unusual circumstances--is the outcome comparable to that achieved in familiar pathologies?

OBJECTIVE: To compare the operative outcome, morbidity profile, and functional outcome after transperitoneal laparoscopic dismembered pyeloplasty for ureteropelvic junction obstruction in unusual circumstances (intrinsic pathology in anomalous kidneys or unusual extrinsic pathologies; group 1) to the outcome after this procedure in familiar pathologies (normally located kidneys with intrinsic dysfunctional segment or extrinsic compression due to a crossing vessel; group 2). METHODS: The patients were evaluated in detail. All patients underwent transperitoneal laparoscopic dismembered pyeloplasty. The operative and postoperative parameters were recorded. Patients were followed up after the procedure on a 3-month protocol. Imaging was repeated at 1 year. No intervention during the follow-up period (ie, nephrostomy, ureteral stenting, or redo pyeloplasty) and improvement in the hydronephrosis grade and diuretic renogram parameters was interpreted as procedural success. The operative, postoperative, and follow-up parameters in the 2 groups were compared. RESULTS: Group 1 included 17 patients with intrinsic pathologic features and renal anomalies with ureteropelvic junction obstruction due to unusual extrinsic pathology. All procedures were successfully completed with the laparoscopic approach. A significant difference was noted in the mean operative duration (group 1, 196.9 ± 10.3 minutes; group 2, 125.44 minutes, P = .00). The other operative and postoperative parameters were comparable. No significant operative or postoperative events were noted. A total of 14 patients (group 1) completed the 1-year follow-up protocol. The success rate was 92.9% (13 of 14) in group 1 and 97.9% (44 of 45) in group 2 (P = .42). CONCLUSION: The procedural duration for laparoscopic dismembered pyeloplasty in unusual circumstances is longer than in familiar pathologies. However, the morbidity profile and functional outcome in these 2 scenarios were comparable.

Upper ureteric obstruction secondary to entrapment between twin segments of inferior vena cava--an unusual occurrence.

A retrocaval ureter is a rare congenital cause of upper ureteric obstruction that results from entrapment of the upper ureter by the inferior vena cava (IVC) as it courses posterior to the cava. We report an interesting scenario of upper ureteric obstruction secondary to entrapment between twin segments of IVC.

Laparoscopic reconstruction of iatrogenic-induced lower ureteric strictures: Does timing of repair influence the outcome?

CONTEXT: Influence of timing of repair on outcome following laparoscopic reconstruction of lower ureteric strictures AIMS: To assess the influence of timing of repair on outcome following laparoscopic reconstruction of lower ureteric strictures in our adult patient population. SETTINGS AND DESIGN: Single surgeon operative experience in two institutes. Retrospective analysis. MATERIALS AND METHODS: All patients were worked up in detail. All patients underwent cystoscopy and retrograde pyelography prior to laparoscopic approach. Patients were categorised into two groups: early repair (within seven days of inciting event) and delayed repair (after two weeks). Operative parameters and postoperative events were recorded. Postprocedure all patients were evaluated three monthly. Follow-up imaging was ordered at six months postoperatively. Improvement in renal function, resolution of hydronephrosis and unhindered drainage of contrast through the reconstructed unit on follow-up imaging was interpreted as a satisfactory outcome. STATISTICAL ANALYSIS USED: Mean, standard deviation, equal variance t test, Mann Whitney Z test, Aspin-Welch unequal variance t test. RESULTS: Thirty-six patients (37 units, 36 unilateral and 1 simultaneous bilateral) underwent laparoscopic ureteral reconstruction of lower ureteric stricture following iatrogenic injury - 21 early repair (Group I) and 15 delayed repair (Group II). All patients were hemodynamically stable at presentation. Early repair was more technically demanding with increased operation duration. There was no difference in blood loss, operative complications, postoperative parameters, or longterm outcome. CONCLUSIONS: In hemodynamically stable patients, laparoscopic repair of iatrogenically induced lower ureteric strictures can be conveniently undertaken without undue delay from the inciting event. Compared to delayed repairs, the procedure is technically more demanding but morbidity incurred and outcome is at par.