Native nephrectomy in pediatric transplantation--less is more!

OBJECTIVE: Indications for pre-transplantation native nephrectomy (PTNN) include chronic renal parenchymal infection, proteinuria, intractable hypertension, polycystic kidneys and malignancy. Our aim was to establish the frequency and reasons for PTNN in children undergoing renal transplant at our center. MATERIALS AND METHODS: Children listed for renal transplant between 1998 and 2010 who underwent PTNN were analyzed. Etiology of established renal failure, indication for nephrectomy, stage of chronic kidney disease, laterality, complications, and timing of subsequent transplant were determined. Outcome of children, and that of preserved native kidneys following transplant, was reviewed. RESULTS: 21/203 children listed for transplant (10.3%) underwent PTNN (32 nephrectomies). Indications were drug-resistant proteinuria (6 children), recurrent upper tract urosepsis (6), refractory hypertension (4), malignancy/malignant predisposition (4), concomitant procedure during ureterocystoplasty (1). Median age at nephrectomy was 3.3 years; 86% had impaired renal function at time of (first) nephrectomy. Median time until transplantation following bilateral nephrectomy was 1.7 years. 19/21 children have been transplanted; 17 reached stable graft function. Only 2 children who did not undergo PTNN required nephrectomy post-transplant. CONCLUSION: When malignancies were excluded, PTNN was performed in a minority (8.4%) of children, mainly for proteinuria. This adds great advantage by reducing morbidity. Resulting graft function seems favorable.

Synchronous laparoscopic-assisted percutaneous endoscopic gastrostomy and peritoneal dialysis catheter placement is a valid alternative to open surgery.

OBJECTIVE: Gastrostomy feeding is frequently necessary in children receiving chronic peritoneal dialysis (PD). Synchronous laparoscopic-assisted placement of percutaneous endoscopic gastrostomy (PEG) and PD catheter has many potential advantages. This study investigates whether this technique is comparable to open placement. METHODS: The notes of all patients over a 16-year time period were reviewed retrospectively. Peritonitis was defined as the presence of a white blood cell count > 100/mm(3) with at least 50% being polymorphonuclear leukocytes, and infection was defined as the presence of positive peritoneal cultures with peritonitis. RESULTS: Ten patients received primary laparoscopic-assisted PEG and PD catheter insertion (LAP) and 23 patients open gastrostomy and PD catheter (OPEN). PD catheter survival was median 12 months in the LAP group and 17 months in the OPEN group. Peritonitis and infection rates per catheter-year were 0.89 and 0.7 LAP and 0.59 and 0.5 OPEN. The risk of peritonitis and infection was not related to method of placement. CONCLUSIONS: There were no statistically significant differences in outcomes between the two groups. We conclude that laparoscopic-assisted synchronous PD and PEG catheter insertion is safe and effective.

46XY girls: the importance of careful newborn examination.

STUDY OBJECTIVE: To understand the timing and factors affecting diagnosis of phenotypically female 46XY children. DESIGN, SETTING, AND PARTICIPANTS: We studied all phenotypically female 46XY children who attended our multidisciplinary disorders of sexual differentiation (DSD) clinic in Nottingham England in a 3-year period since its inception. Case notes from a prospectively maintained database were reviewed and data were analyzed on the age at presentation, family history, findings on genital examination, and underlying endocrine abnormality. RESULTS: Eleven children were studied, all of whom were being raised as girls. The median age of presentation was 18 months (range birth-15 years). Although the newborn examination detected the possibility of DSD in only 3 cases; 10 of 11 children had at least one significant abnormality in their external genitalia at presentation. CONCLUSION: Careful neonatal genital examination can identify children with DSD. However, not all children with these conditions are identified early. Early diagnosis, when possible, is important, as it has the potential to make the management of this difficult condition more straightforward.

Recognition and treatment of genitourinary complications in paediatric Crohn's disease using Infliximab.

UNLABELLED: Although genitourinary complications of Crohn's are well recognized, available information regarding their clinical course and management is sparse especially in the paediatric population. We report a myriad of urological complications in five paediatric cases, our experience from a tertiary paediatric urological and gastroenterological centre. All children with urological complications had severe Crohn's disease which necessitated the use of immuno-suppressants including Infliximab. Three of four children healed and closed their fistulas after treatment, although failed to avoid future surgery, albeit for other reasons. CONCLUSION: We suggest Infliximab should be considered as a treatment option but in the absence of a common consensus, treatment be tailored to individual cases.

Dysplastic kidneys in children - do they grow?

OBJECTIVES: Dysplastic kidneys (DK) are a common cause of chronic kidney disease (CKD). Little is known about their growth or how their sonographic appearance changes. This study aimed to test the hypothesis that DK gain little length, and to identify radiologic trends predictive of CKD. METHODS: Ultrasound scans of children with DK born in 1980-2005 and referred to a single tertiary centre were analysed by a pediatric radiologist. Renal lengths were plotted on standard nomograms and the degree of dysplastic appearance noted. Factors related to DK - bladder outlet obstruction, vesico-ureteric reflux and renal impairment - were noted. RESULTS: Fifty-three children were studied (83 kidneys), of whom 41 were boys; 289 scans were analysed. In 33 children there was associated bladder outlet obstruction or vesico-ureteric reflux. Forty-four DK were noted to fall off their renal length 'centile'. This correlated well with the development of CKD and is statistically significant. Deterioration occurred in 53% of DK; primarily progressive reduction in corticomedullary differentiation. This also correlated well with development of CKD. CONCLUSION: More than half of the DK showed poor growth velocity. This, together with the degree of sonographic abnormality, carries a high predictive value for development of CKD. We recommend diligent serial sonography to follow renal growth and dysplastic appearance in children with DK.

Congenital small bowel diverticulosis and intestinal atresia: a rare association.

An unusual case of multiple intestinal atresias with multiple small bowel diverticulae is presented. To the best of our knowledge this is the first reported case of its kind in the literature.

Surgical correction of congenital megaprepuce.

Congenital megaprepuce (CMP) is a rare entity. Two infant boys presented with a tight congenital phimosis resulting in an excessively baggy, urine-filled prepuce and a swollen scrotum. Compression of the scrotum resulted in drainage of urine. We feel this to be a separate entity from a buried penis and recommend early surgery. The phimotic tip of the foreskin was excised and the inner layer preserved to cover the full length of the penile shaft. The outer layer of the foreskin, in reality the penoscrotal junction, was anchored to the base of the penile shaft. A V-shaped edge of ventral skin was excised and the edges approximated, giving the appearance of median raphe. The final appearance was that of a circumcised penis. A third patient awaits operation.

Asymmetric conjoined twins.

Despite recent advances in diagnosis, particularly organ-imaging, and therapeutic options, the management of conjoined twins is still very challenging. We report conjoined twins attached ()end-on" at the lumbo-sacral level and describe the anatomical findings, methods of investigation, and management.

Short colon variant.

A one day old neonate with a short colon, associated exomphalos minor; bifid scrotum and ileovesical fistula is reported.