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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 15  |  Issue : 4  |  Page : 357-360

A rare and challenging renal transplant in a child with complex congenital defects - A case report


1 Department of Urology, Command Hospital (Southern Command), Pune, Maharashtra, India
2 Department of Internal Medicine and Nephrology, ESIC Medical College and PGIMSR, Chennai, Tamil Nadu, India
3 Department of Urology, AIIMS, Jodhpur, Rajasthan, India

Date of Submission05-Oct-2020
Date of Decision31-Jan-2021
Date of Acceptance07-Feb-2021
Date of Web Publication30-Dec-2021

Correspondence Address:
Dr. Bharath N Kumar
Department of Urology, Command Hospital (Southern Command), Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijot.ijot_126_20

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  Abstract 

Renal insufficiency is a distinct possibility in patients with congenital lower urinary tract abnormalities even though they are managed right from early childhood. Previously, such patients were not considered candidates for renal transplantation due to the fear of abnormal bladder affecting the graft kidney too. In the current era, it has been shown that the graft and patient survival rates are similar in such patients to those with normal lower urinary tract, if the urological abnormalities are managed well, before the transplantation. We present a case of successful renal transplantation in a pediatric patient with renal insufficiency due to pure urogenital sinus.

Keywords: Congenital lower urinary tract abnormalities, Mitrofanoff procedure, pediatric renal transplantation, pure urogenital sinus


How to cite this article:
Kumar BN, Baliga KV, Sandhu AS. A rare and challenging renal transplant in a child with complex congenital defects - A case report. Indian J Transplant 2021;15:357-60

How to cite this URL:
Kumar BN, Baliga KV, Sandhu AS. A rare and challenging renal transplant in a child with complex congenital defects - A case report. Indian J Transplant [serial online] 2021 [cited 2022 May 25];15:357-60. Available from: https://www.ijtonline.in/text.asp?2021/15/4/357/334418




  Introduction Top


Kidney transplantation is the optimal treatment for children with end-stage renal disease (ESRD). Success in pediatric renal transplantation is a challenging undertaking. This case report highlights the successful management of the difficulties encountered in renal transplantation, performed on a child with complex congenital defects.


  Case Report Top


A 12-year-old girl first came to medical attention at the age of 2 years with features of bladder outlet obstruction, advanced renal failure, acidosis, and urosepsis. She was the second of two siblings and the product of a nonconsanguineous marriage. Examination and genitoscopy at that time revealed a vaginal-type urogenital sinus with low confluence and a stenotic meatus. Blood urea nitrogen and serum creatinine were 120 mg/dl and 5.8 mg/dl, respectively. Ultrasound and noncontrast computerized tomography scan of the abdomen revealed a large capacity bladder with diverticulae and bilateral hydroureteronephrosis. Blocksom vesicostomy was done, and peritoneal dialysis carried out. At the age of 3 years, she underwent bilateral ureteric reimplantation for bilateral Grade V vesicoureteric reflux, excision of paraureteral diverticulae, and closure of vesicostomy elsewhere. She was on regular dilatation for the stenotic meatus. Corrective surgery for left congenital dislocation of the hip was performed at the age of 4 years. When she was 8 years old, she underwent a Mitrofanoff procedure since bladder outlet obstruction was persisting. To rule out an intersex disorder, karyotyping was done which revealed a 46 XX pattern with negative Y' fluorescence. The findings of the genitogram are shown in [Figure 1].
Figure 1: Genitogram

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She had now progressed to ESRD, the cause of which was obstructive uropathy due to urethral stenosis compounded by reflux nephropathy. She had difficulty in catheterizing the Mitrofanoff stoma. She was passing urine per-urethra in a fair stream. On examination, there were growth retardation and features of renal osteodystrophy. Mitrofanoff stoma was inferolateral to the umbilicus on the right side with multiple lower abdominal surgical scars [Figure 2]. The external genitalia and anus appeared normal. Examination of the spine was normal. Urodynamic study showed adequate bladder capacity for age (367 ml), normal sensation, and compliance during the filling phase and unobstructed voiding during the voiding phase.
Figure 2: Abdomen with previous surgical scars and Mitrofanoff stoma

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A renal transplant was planned and revision of Mitrofanoff stoma was carried out 8 weeks before transplant. Pretransplant, the patient had anemia which was corrected with parenteral iron and erythropoietin and hemoglobin was improved to 11.9 g/dl. She had mild hypertension, controlled with oral nifedipine 10 mg bd and metoprolol 25 mg bd. Her cardiac function was good with concentric left ventricular hypertrophy, ejection fraction of 55%, and no regional wall motion abnormality on echocardiography. Serum calcium was 8.8 mg/dl (corrected for serum albumin), serum phosphorus was 5.5 mg/dl, and i-PTH was 260 pg/ml. She received six sittings of hemodialysis, through a right internal jugular vein catheter. She received a live-related donor kidney with one haplomatch from her father who was 40 years of age. There was no significant size mismatch between the donor and the recipient. The surgical challenges for transplant were (a) multiple previous lower abdominal surgeries and anticipated difficulty in access and extraperitoneal placement of allograft and (b) urogenital sinus, which would pose a difficulty in intraoperative bladder catheterization. Vaginoscopy was done to locate the urethral opening in the anterior vaginal wall. A guidewire was passed into the bladder, and a 12 Fr Foleys catheter railroaded into the bladder [Figure 3]. Left iliac fossa was chosen for implantation since it was less scarred. With careful and meticulous dissection, the extraperitoneal space was entered. Vascular anastomoses were done to recipient external iliac vein and internal iliac artery [Figure 4]. Modified Lich-Gregoir ureteroneocystostomy could be performed, but with difficulty due to extensive scarring around the bladder.
Figure 3: Genitalia with per-urethral catheter in situ

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Figure 4: Vascular anastomoses

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There was immediate graft function with brisk diuresis and normalization of serum creatinine to 0.7 mg/dl within 36 h posttransplant, and it remained so on follow-up [Figure 5]. Immunosuppression protocol was tacrolimus, mycophenolate mofetil, and prednisolone. Induction was not given in view of haplomatch. Postoperative period was complicated by hypertension, recurrent focal seizures, and behavioral abnormalities. Magnetic resonance imaging (MRI) of the brain showed evidence of right fronto-parietal cerebritis. Encephalography was normal. Tacrolimus level was high (>15 ng/ml) despite reducing its dose and hence was substituted with sirolimus. There was progressive improvement in the patient's condition. Both the donor and recipient were negative for cytomegalovirus; however, the recipient received valganciclovir prophylaxis for 6 months posttransplant. She was also on cotrimoxazole prophylaxis, and there was no documented infection. Postoperative renal dynamic scan showed satisfactory allograft function. She has normal renal parameters 18 months posttransplant, along with satisfactory growth and sexual development. She performs clean intermittent catheterization (CIC) through the Mitrofanoff stoma but voids in a fair stream as well through the urethral meatus without significant postvoid residue.
Figure 5: Perfused functioning renal graft

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  Discussion Top


In urogenital sinus anomaly, there is a persistent communication of the urinary tract with the vagina, which is associated with four conditions, namely genital ambiguity states, pure urogenital sinus with normal external genitalia, cloacal anomalies, and female exstrophy. Our patient belonged to the category of pure urogenital sinus with normal external genitalia.

Hendren has reported the common association of cloacal malformations and vesicoureteral reflux, but the association of pure urogenital sinus with reflux is unusual.[1] Previously, patients with lower urinary tract anomalies were considered poor candidates for renal transplantation because the bladder contributes to the destruction of the native kidneys threatening a subsequently placed renal allograft.[2],[3] Morita et al. have stated in their study that the posttransplant clinical outcome of pediatric transplantation in patients with urological anomalies is comparable to that of the recipients with primary renal disease. Appropriate urinary tract reconstruction and management is essential to reduce the risk of graft dysfunction because of urinary problems.[4] All urological problems have to be solved before transplantation. Marchal et al. reported renal transplantations in four patients with a urogenital sinus malformation. Two patients had a bladder reconstruction with ileal augmentation, a bladder neck closure, and a continent Monti diversion. One patient had a continent ileal diversion by a Koch pouch, and one patient had an incontinent diversion. The mean age at transplantation was 21.5 years (±10.9). Initial transplantation was the case for three patients and a second for one patient. With a mean follow-up of 6.2 years, all transplants were still functioning. Mean creatinine level was 1.58 mg/dl with a mean GFR of 68.2 ml/min/1.73 m2. Even if enterocystoplasty and continent urinary diversions exposed grafts to more frequent acute pyelonephritis, patient and graft survival rates at 10 years were similar to other kidney transplantations on native bladder in their study. Attention should be drawn to postvoid residual volume and promotion of CIC.[5] Our patient did not require any surgery for the bladder since the compliance was good on urodynamic study. Hypertension posttransplant related to tacrolimus and steroids would have resulted in seizures in our patient, which later settled with switch to sirolimus. Cerebritis was probably related to cerebral edema caused by hypertension, as repeat MRI after 4 weeks was normal.

Kitta et al. reported a case of a pure urogenital sinus anomaly without hydrometrocolpos or hydroureteronephrosis.[6] The patient maintained spontaneous voiding without any long term consequences after an initial period of CIC. Our patient would require a flap vaginoplasty at a later date for widening the introitus and urogenital sinus.[6],[7]


  Conclusion Top


Congenital urological abnormalities are not a contraindication for pediatric renal transplantation though they pose unique challenges and dilemmas. These can be tackled successfully by thorough evaluation, meticulous surgical technique, and an energetic multidisciplinary team approach.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for her images and other clinical information to be reported in the journal. The patient's parents understand that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hendren WH. Cloacal malformations: Experience with 105 cases. J Pediatr Surg 1992;27:890-901.  Back to cited text no. 1
    
2.
Akoh JA, Choon TC, Akyol MA, Kyle K, Briggs JD. Outcome of renal transplantation in patients with lower urinary tract abnormality. J R Coll Surg Edinb 1999;44:78-81.  Back to cited text no. 2
    
3.
Sheldon CA, Gonzalez R, Burns MW, Gilbert A, Buson H, Mitchell ME. Renal transplantation into the dysfunctional bladder: The role of adjunctive bladder reconstruction. J Urol 1994;152:972-5.  Back to cited text no. 3
    
4.
Morita K, Iwami D, Hotta K, Shimoda N, Miura M, Watarai Y, et al. Pediatric kidney transplantation is safe and available for patients with urological anomalies as well as those with primary renal diseases. Pediatr Transplant 2009;13:200-5.  Back to cited text no. 4
    
5.
Marchal S, Kalfa N, Iborra F, Badet L, Karam G, Broudeur L, et al. Long-term outcome of renal transplantation in patients with congenital lower urinary tract malformations: A multicenter study. Transplantation 2020;104:165-71.  Back to cited text no. 5
    
6.
Kitta T, Kakizaki H, Iwami D, Tanda K. Successful bladder management for a pure urogenital sinus anomaly. Int J Urol 2004;11:340-2.  Back to cited text no. 6
    
7.
Hendren WH. Surgical management of urogenital sinus abnormalities. J Pediatr Surg 1977;12:339-57.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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