Page kidney after pediatric kidney transplantation: a case report. / Riñón de Page tras trasplante renal pediátrico: informe de un caso.

INTRODUCTION: Page kidney (PK) is a rare condition caused by parenchymal compression due to a subcapsular hematoma. Irreversible damage of the graft may occur if this condition is not recognized and treated properly. CLINICAL CASE: We describe the case of a 16-year-old man with chronic renal failure secondary to corticosteroid-resistant nephrotic syndrome (CRNS) caused by NPHS2 mutations. The patient underwent a 5th fifth living-related KT. The graft was placed intraperitoneally and reperfused well without complications. On the 4th postoperative day his labs demonstrated raising creatinine associated with refractory hypertension, gross hematuria and anemia. Urgent ultrasound revealed a subcapsular hematoma with signs of parenchymal compression. PK phenomenon was suspected and urgent surgical intervention decided. COMMENTS: PK is a rare but an emergence potentially treatable and reversible complication after pediatric KT. Early diagnosis based on clinical suspicion and suggestive imaging are the key points for a favorable outcome.

INTRODUCCION: El riñón de Page (RP) es una enfermedad rara provocada por compresión parenquimatosa debido a un hematoma subcapsular. El injerto puede sufrir daños irreversibles si la enfermedad no se reconoce y se trata de forma adecuada. CASO CLINICO: Describimos el caso de un varón de 16 años con fallo renal crónico secundario a síndrome nefrótico corticorresistente (SNCR) provocado por mutaciones del gen NPHS2. El paciente se somete a un quinto TR de donante vivo. El injerto se coloca en posición intraperitoneal, con una adecuada reperfusión, sin complicaciones. Al cuarto día postoperatorio, sus análisis revelan una subida de la creatinina asociada a hipertensión refractaria, hematuria macroscópica y anemia. La ecografía de urgencia revela hematoma subcapsular con signos de compresión parenquimatosa. Se sospecha RP y se decide intervención quirúrgica de urgencia. COMENTARIOS: El RP es una complicación rara y urgente, aunque a su vez tratable y reversible, tras TR pediátrico. El diagnóstico precoz basado en la sospecha clínica y las imágenes son claves a la hora de conseguir un resultado favorable.

Riñón de Page tras trasplante renal pediátrico: informe de un caso / Page kidney after pediatric kidney transplantation: a case report

Introducción: El riñón de Page (RP) es una enfermedad rara provocada por compresión parenquimatosa debido a un hematoma subcapsular. El injerto puede sufrir daños irreversibles si la enfermedad no se reconoce y se trata de forma adecuada.Caso clínico: Describimos el caso de un varón de 16 años con fallo renal crónico secundario a síndrome nefrótico corticorresistente (SNCR) provocado por mutaciones del gen NPHS2. El paciente se somete a un quinto TR de donante vivo. El injerto se coloca en posición intraperitoneal, con una adecuada reperfusión, sin complicaciones. Alcuarto día postoperatorio, sus análisis revelan una subida de la creatininaasociada a hipertensión refractaria, hematuria macroscópica y anemia.La ecografía de urgencia revela hematoma subcapsular con signos de compresión parenquimatosa. Se sospecha RP y se decide intervenciónquirúrgica de urgencia. Comentarios: El RP es una complicación rara y urgente, aunque a su vez tratable y reversible, tras TR pediátrico. El diagnóstico precoz basado en la sospecha clínica y las imágenes son claves a la hora de conseguir un resultado favorable.

Introduction: Page kidney (PK) is a rare condition caused by parenchymal compression due to a subcapsular hematoma. Irreversible damage of the graft may occur if this condition is not recognized and treated properly.Clinical case: We describe the case of a 16-year-old man withchronic renal failure secondary to corticosteroid-resistant nephroticsyndrome (CRNS) caused by NPHS2 mutations. The patient underwent a 5th fifth living-related KT. The graft was placed intraperitoneallyand reperfused well without complications. On the 4 th postoperativeday his labs demonstrated raising creatinine associated with refractoryhypertension, gross hematuria and anemia. Urgent ultrasound revealeda subcapsular hematoma with signs of parenchymal compression. PKphenomenon was suspected and urgent surgical intervention decided.Comments: PK is a rare but an emergence potentially treatableand reversible complication after pediatric KT. Early diagnosis basedon clinical suspicion and suggestive imaging are the key points for afavorable outcome.

Outcome of kidney transplantation from young pediatric donors (aged less than 6 years) to young size-matched recipients.

INTRODUCTION: Pediatric donation is underutilized because of presumed increased risk of vascular thrombosis (VT) and graft loss. Using young pediatric donors (YPDs) for young pediatric recipients (YPRs) is suggested to be even at greater risk and therefore precluded in many centers. The aim of this study was to analyze the outcome of kidney transplantation (KT) from YPD to age-matched YPR. PATIENT AND METHODS: A retrospective study of 118 pediatric KT performed between January 2007-July 2017. The authors identified KT with YPD (considered as those aged <6 years) and age-matched YPR. Organ allocation was performed based on the best paired size (YPR for YPR). Data were collected regarding donor and recipient characteristics, surgical and urological complications, graft loss, and outcomes. RESULTS: Forty cases of YPD to age-matched YPR were identified (33.89% of the cohort). Mean recipient and donor age were 2.9 years (SD 1.68) and 2.24 years (SD 1.5), respectively. Mean recipient and donor weight were 12.7 kg (SD 4.1) and 13.7 kg (SD 4.15), respectively. Thirty of those young recipients (75%) weighed <15 kg. The most frequent primary renal disease was the congenital nephrotic syndrome. Nine out of 40 patients (22.5%) had received a previous KT before. Three received a combined liver-KT. Eight (20%) were classified as high immunological risk and 19 (47.5%) as high thrombotic risk. All allografts were implanted extraperitoneally and anastomosed to the iliac vessels. Major complications requiring reintervention occurred in seven patients (17.5%): three VT, three bleeding episodes, and one ureteral necrosis. Remarkably, only one surgical complication (VT) resulted in graft loss. Regarding long-term urological complications, four patients (10%) all with obstructive uropathy-developed vesicoureteral reflux to the graft. Actuarial graft survival at 1,5, and 10 years in the YPD to age-matched YPR cohort was 83% -78% -78%, respectively. Mean follow-up was 3.6 years (SD 3.2) (r = 7-10). Over time, eight patients lost their graft, not related to surgical factors in seven out of eight cases. CONCLUSION: The authors suggest that KT using YPD for age-match YPR yields good results in expert centers, even in high-risk patients and is associated with good graft survival. In this series, surgical complications were rarely related to graft loss.

[Is the Mathieu urethroplasty a useful technique for urethrocutaneous fistula repair after hypospadias surgery?] / ¿Es la uretroplastia Mathieu una técnica útil para la reparación de fístulas uretrocutáneas en pacientes operados de hipospadias?

AIM: There are multiple surgical techniques for distal urethrocutaneous fistulas (DUCF) repair after hypospadias surgery. Our aim was to analyze our results of simple closure (SC) and salvage mathieu urethroplasty (MU). MATERIAL AND METHODS: Retrospective study (2014-2017) of pediatric patients with DUCF who underwent SC and MU. Only subcoronal to distal fistulas were collected, excluding the proximal ones non-tributary of MU. Demographics, clinical data, treatment and results were registered. MAIN RESULTS: Over 4 years, 177 hypospadias and 28 UCFs were repaired. 15/28 met the inclusion criteria. Hypospadias type: anterior (10, 66.7%), midshaft (3, 20%) and posterior (2, 13.3%). Mean age at primary urethroplasty was 31.9 months (SD: 16.2). The most common techniques in primary surgery were Thiersch-Duplay (6) and Snodgrass (4). Median time from primary surgery to apparition of the fistula was 5 months (rank: 0-14). The surgical technique used for fistula repair was: SC (7) and MU (8). DUCF succeeded in all patients treated with MU, while among those treated with SC, 71.4% recurred, and only 4 patients underwent finally successful repair with a median of 1.5 interventions. Median follow-up was 25 months (rank: 12-55). CONCLUSIONS: MU is a useful, effective and safe technique for DUCF repair. Most importantly, it is easily reproducible. The results of MU seem to be superior to CS and could be considered as a first choice.

OBJETIVO: Existen múltiples técnicas quirúrgicas para el cierre de fístulas uretrocutáneas distales (FUCD) secundarias a la corrección de hipospadias. Nuestro objetivo fue analizar nuestros resultados mediante el cierre simple (CS) y cierre con nueva uretroplastia por técnica de Mathieu (CUM). MATERIALES Y METODOS: Estudio retrospectivo (2014-2017) de pacientes pediátricos con FUCD tratadas mediante CS y CUM. Se incluyeron solo fístulas desde la región subcoronal a distal, excluyendo las fístulas proximales no candidatas de cierre por CUM. Se recogieron datos demográficos, clínicos, tratamiento y resultados. RESULTADOS: Durante 4 años se intervinieron 177 hipospadias y 28 FUC. 15/28 cumplieron los criterios de inclusión. Tipo hipospadias: distal (10; 66,7%), mediopeneano (3; 20%) y proximal (2; 13,3%). La media de edad de la cirugía primaria fue de 31,9 meses (DS:16,2). Las técnicas primarias más utilizadas fueron el Thiersch-Duplay (6) y el Snodgrass (4). La mediana de tiempo desde la cirugía primaria a la aparición de la fístula fue de 5 meses (rango: 0-14). La técnica utilizada para el cierre fue: CS (7) y CUM (8). Todos los pacientes tratados con CUM resolvieron la fístula en un solo tiempo; de los tratados con CS, recidivaron el 71,4%, resolviéndose finalmente 4 con una mediana de 1,5 intervenciones. La mediana de seguimiento fue de 25 meses (rango: 12-55). CONCLUSIONES: El CUM es una técnica útil, eficaz y segura para el tratamiento de la FUCD y, además, fácilmente reproducible. Los resultados del cierre por CUM parecen ser superiores al CS y podría considerarse la técnica de primera elección.

Is donor age 6 years or less related to increased risk of surgical complications in pediatric kidney transplantation?

INTRODUCTION: Despite the widespread organ shortage dilemma, there is hesitancy regarding utilization of young donors (aged ≤6 years) because previous reports have suggested that this is associated with an increased risk of surgical complications and graft loss. OBJECTIVE: The aim of this study was to determine if donor age ≤6 years is related to increased risk of surgical complications or allograft loss in pediatric kidney transplantation (KT). STUDY DESIGN: A retrospective study of pediatric kidney transplants (KT) undertaken between January 2000 and July 2015. The incidence of surgical and urological complications, and allograft loss were analyzed and compared between donors aged ≤6 years (Group 1) and donors aged >6 years (Group 2). RESULTS: A total of 171 pediatric KTs were performed at the current center during the study period. Twenty-eight patients were excluded; as a result, the study comprised 143 patients: 60 (Group 1) and 83 (Group 2). Mean recipient weight was 17 kg (SD 9.7; range 3.2-47) in Group 1 and 38.2 kg (SD 15.3; range 7.8-73) in Group 2. Despite a significantly higher proportion of risk factors in Group 1, no significant between-group differences were observed in terms of: surgical complications (OR 0.4; range 0.1-1.2), early urological complications (OR 2.2; range 0.4-11), late urological complications (OR 0.3; range 0.8-1.4), lymphoceles (OR 6.2; range 0.7-51.7) and allograft loss (OR 1.5; range 0.7-3.1, summary Table). Graft survival at 1 and 5 years was: 81% and 70% (Group 1) and 92% and 79% (Group 2), respectively (P = 0.093). Mean follow-up was 90.13 ± 49.7 months. DISCUSSION: The main finding of this retrospective study was that pediatric donor kidneys from donors aged ≤6 years could safely be used in pediatric recipients without an increased risk of surgical and urological complications or graft loss. Nevertheless, KT with small donor kidneys is challenging and should be performed at experienced pediatric centers. CONCLUSION: In line with these results, the outcomes of KT using donors aged ≤6 years were encouraging and similar to those obtained with older donors. Thus, this study supported using kidney grafts from young donors, given the organ shortage and potential high mortality risk while awaiting KT.

[Rehbein's procedure versus De la Torre in Hirschsprung's disease]. / Rehbein versus De la Torre en la enfermedad de Hirschsprung.

The authors perform a retrospective study with a 4 years follow up of 46 patients operated of Hirschsprung's disease (HD). In 36 cases by with staplers Rehbein technique (TR) and in 10 others with De la Torre endorectal pull-through (TEPT). In all them diagnosis was achieved by mean of radiology, manometry, and hystochemical procedures. During the surgical procedure was performed in all cases biopsies to confirm the neuronal integrity of the colon descended. In the TR group, 16.6% of patients presented rectal achalasia with constipation due to 3 cms. aganglionic rectum remnant, while in the TEPT group this circumstance are not presented because all aganglionic rectum was eliminated. Otherwise TEPT technique permits an earlier application, diminuend the hospitalization time,shortening the start of feeding and with a good cosmetic result. None of this patients presented infection, stenosis, bleeding or incontinence.

Rehbein versus De la Torre en la enfermedad de Hirschsprung / Rehbein’s procedure versus de la torre in hirschsprung's disease

Se realiza un estudio retrospectivo con un seguimiento de cuatro años, de 46 pacientes intervenidos de enfermedad de Hirschsprung (EH).Mediante técnica de Rehbein (TR) con anastomosis mecánica lo fueron 36, y los otros 10 por descenso endoanal De la Torre (TEPT). Todos fueron diagnosticados por radiología, manometría e histoquímica. En el acto quirúrgico se realizó una biopsia peroperatoria de la zona intestinal afectada, y comprobación de la normalidad ganglionar de la zona descendida. El 16,6% de los pacientes intervenidos mediante TR presentaron acalasia, por la presencia de tres centímetros agangliónicos restantes, mientras que los pacientes intervenidos mediante TEPT no presentaron esta complicación, al ser eliminado por completo el aganglionismo. Asimismo, la TEPT ha permitido ser realizada más precozmente, disminuyendo la morbilidad, menor estancia hospitalaria, rapidez en la alimentación postoperatoria, y buen resultado cosmético. Ninguno presentó infección, estenosis, sangrado o incontinencia (AU)

The authors perform a retrospective study with a 4 years follow up of 46 patients operated of Hirschsprung’s disease (HD). In 36 cases by with staplers Rehbe in technique (TR) and in 10 others with De la Torre endorectal pull-through (TEPT). In all them diagnosis was achieved by mean of radiology, manometry, and hystochemical procedures. During the surgical procedure was performed in all cases biopsies to confirm the neuronal integrity of the colon descended. In the TR group, 16.6% of patients presented rectal achalasia with constipation due to 3 cms. aganglionic rectum remnant, while in the TEPT group this circumstance are not presented because all aganglionic rectum was eliminated. Otherwise TEPT technique permits an earlier application, diminuend the hospitalization time, shortening the start of feeding and with a good cosmetic result. None of this patients presented infection, stenosis, bleeding or incontinence (AU)