Psychosexual development management of bladder exstrophy epispadias in complex patients.

INTRODUCTION: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. OBJECTIVE: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality. STUDY DESIGN: 13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO). RESULTS: The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas (Summary Table). DISCUSSION: This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this research showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program. CONCLUSION: This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people.

Bladder urothelial neoplasms in pediatric age: experience at three tertiary centers.

INTRODUCTION: Urothelial bladder neoplasms (UBN) typically occur in patients in their sixth or seventh decade of life while they are infrequent in children and young adults. They occur in 0.1-0.4% of the population in the first two decades of life. Their management is controversial and paediatric guidelines are currently unavailable. OBJECTIVE: To further expound the available data on the outcome of patients younger than 18 year old diagnosed with UBN. STUDY DESIGN: We retrospectively reviewed the files of all the consecutive paediatric patients with UBN treated in three tertiary paediatric urology units from January 1999 to July 2013. Lesions were classified according to the 2004 WHO/ISUP criteria as urothelial papillomas (UP), papillary urothelial neoplasm of low malignant potential (PUNLMP), low-grade urothelial carcinoma (LGUC), and high-grade urothelial carcinoma (HGUC). RESULTS: The table shows the results. Management after TURB varied among centres. One centre recommended only follow-up US at increasing intervals whereas another follow-up US plus urine cytologies and endoscopies, every three months in the first year, and at increasing intervals thereafter. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed disease recurrence or progression. DISCUSSION: UBN is an uncommon condition in children and adolescents and, unlike in adults, its incidence, follow-up and outcome still controversial. Paediatric guidelines are currently lacking and management varies among centres. Gross painless haematuria is the most common presenting symptom. Therefore, this symptom should never be underestimated. US is generally the first investigation and additional imaging seems unnecessary. TURB often allows for complete resection. Lesions are generally solitary, non-muscle invasive, and low-grade (mainly UP and PUNLMP). Ideal follow-up protocol is the most controversial point. Reportedly, recurrence or progression during follow-up is uncommon in patients under 20 years, recurrence rate 7% and a single case of progression reported so far. Accordingly, a follow-up mainly based on serial US might be considered in this age group compared to adults where also serial endoscopies and urine cytologies are recommended. In the selection of the follow-up investigations, it should also be taken into consideration that urine cytology has a low sensibility in the detection of low-grade lesions while cystoscopy in young patients requires a general anaesthesia and hospitalization, and carries an increased risk of urethral manipulation. CONCLUSION: UBN is a rare condition in children. Ultrasound is generally accurate in order to visualize the lesion, and TURB can treat the condition. Lesions are generally low-grade and non-muscle invasive, but high-grade lesions can also be detected. In present series, after TURB, follow-up US monitoring at increasing intervals was used at all centres, follow-up cystoscopies were added in two centres, but with different schedules. Urine cytologies were considered only at one centre. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed recurrence or progression of the disease.

Molecular biology and nuclear medicine in pediatric hydronephrosis.

Pediatric hydronephrosis may correspond to very different clinical situations, ranging from fully benign reversible dilatation to severe obstructive nephropathy. The genetic research is difficult, mainly because the condition is probably polygenic and the embryology of the urinary system is very complex and depends on a multifaceted interaction of genetic and environmental factors. Molecular biology has gained new insights in the complicated urinary system and in the mechanisms of obstructive nephropathy. Some mediators (tumor growth factor, tumor necrosis factor, renin angiotensin system, etc.) could be considered molecular markers of obstruction and it has been proposed to introduce them in clinical decision making, in order to make an accurate selection of patients needing surgical correction. Scintigraphy has been a standard procedure in the management of pediatric hydronephrosis for decades and has been used in many clinical studies designed to evaluate the role of selected molecular markers in clinical settings. The relationships between scintigraphic parameters and molecular mediators seems promising, in particular for the evaluation of the Reanin Angiotensin System, which plays many roles in the natural history of pediatric hydronephrosis. Angiotensin up-regulation is a turning point in many pediatric hydronephrosis and can be unveiled by captopril scintigraphy, which allows a timely diagnosis of obstruction, before irreversible parenchymal injury and loss of renal function.

Two-stage repair in hypospadias.

We provide the reader with a nonsystematic review concerning the use of the two-stage approach in hypospadias repairs. A one-stage approach using the tubularized incised plate urethroplasty is a well-standardized approach for the most cases of hypospadias. Nevertheless, in some primary severe cases, in most hypospadias failures and in selected patients with balanitis xerotica obliterans a two-stage approach is preferable. During the first stage the penis is straightened, if necessary and the urethral plate is substituted with a graft of either genital (prepuce) or extragenital origin (oral mucosa or postauricular skin). During the second stage, performed around 6 months later, urethroplasty is accomplished by graft tubulization. Graft take is generally excellent, with only few cases requiring an additional inlay patch at second stage due to graft contracture. A staged approach allows for both excellent cosmetic results and a low morbidity including an overall 6% fistula rate and 2% stricture rate. Complications usually occur in the first year after the second stage and are higher in secondary repairs. Complications tend to decrease as experience increases and use of additional waterproofing layers contributes to reduce the fistula rate significantly. Long-term cosmetic results are excellent, but voiding and ejaculatory problems may occur in as much as 40% of cases if a long urethral tube is constructed. The procedure has a step learning curve but because of its technical simplicity does not require to be confined only to highly specialized centers.

Clinical and molecular markers of chronic interstitial nephropathy in congenital unilateral ureteropelvic junction obstruction.

PURPOSE: We evaluated clinical and biological variables, and their meaning as reliable markers of chronic interstitial nephropathy in a selected group of children with prenatally detected hydronephrosis who underwent pyeloplasty because of congenital unilateral ureteropelvic junction obstruction. MATERIALS AND METHODS: We reviewed the clinical, prenatal and postnatal ultrasonographic, and scintigraphic records of children for whom intraoperative biopsy records were available. We performed histological analysis, and evaluated tubulointerstitial immunostaining for vimentin and alpha-smooth muscle actin, and the immunohistochemical and mRNA expression of the renin-angiotensin system peptides and transforming growth factor-beta1. RESULTS: The children were divided in 2 groups according to the absence (group 1) or presence (group 2) of chronic interstitial nephropathy in the biopsy. Patients in group 2 were significantly younger at prenatal diagnosis (p = 0.031), and had decreased split renal function (p = 0.005) and worse drainage (p = 0.035) on preoperative diuretic renography. No differences were found in terms of degree of hydronephrosis, or its prenatal and postnatal variation. Group 2 biopsies exhibited greater immunostaining for alpha-smooth muscle actin and vimentin (p = 0.004 and p = 0.047, respectively), and transforming growth factor-beta1 mRNA levels (p = 0.06). Vimentin and alpha-smooth muscle actin positivity correlated with renin, angiotensin II receptors 1 and 2, and transforming growth factor-beta1 mRNA levels, and all correlated with preoperative split renal function and post-void washout. CONCLUSIONS: In congenital unilateral ureteropelvic junction obstruction chronic interstitial nephropathy and poor postoperative recovery seem to be associated with an earlier diagnosis of hydronephrosis, functional loss greater than 10% and worse scintigraphic drainage. Moreover, there is a strong correlation between molecular fibrogenic markers and histologically and scintigraphically demonstrated renal damage.

Kidney transplantation in children with reconstructed bladder.

There is some controversy about the safety of renal transplantation in patients with an augmentation cystoplasty because of the possibility of urinary tract infection in immunosuppressed patients leading to pyelonephritis and graft loss. Nevertheless, it is now well known that in patients with a small volume and poorly compliant bladder, reconstructive bladder surgery (augmentation cystoplasty or continent reservoir) creates a low-pressure and compliant reservoir, which protects the upper urinary tract and restores a functional lower urinary tract. Graft survival is not adversely affected when a kidney transplant is drained into a reconstructed bladder. When bowel segments are used for augmentation, a voiding modality with clean intermittent self-catheterization does not increase the risk of urinary tract infections, even in immunosuppressed patients.

Angiotensin converting enzyme inhibition worsens the excretory phase of diuretic renography for obstructive hydronephrosis.

PURPOSE: A growing body of evidence identifies the renin-angiotensin system as a key factor in the onset and progression of renal damage in chronic partial obstruction, which often represents a complex diagnostic challenge. A prospective study was undertaken to evaluate the role of captopril mercaptoacetyltriglycine-3 (MAG-3) renography as an early diagnostic test of obstruction. We report the results in a subgroup of children who underwent surgical correction for pyeloureteral obstruction. MATERIALS AND METHODS: Pyeloplasty was performed in 12 patients, including 10 males, 2 to 72 months old (median age 7) with unilateral hydronephrosis, including normal renal function and blood pressure. Basal and captopril enhanced diuretic renography with 99mtechnetium MAG-3 was performed within 24 hours using the same hydration and diuretic stimulus (0.75 mg./kg. furosemide), and 0.75 mg./kg. captopril was administered orally 60 to 90 minutes before scintigraphy. RESULTS: No adverse effects or modifications of the blood pressure were observed after captopril administration. The diuretic response was deeply worsened by angiotensin converting enzyme inhibition in each hydronephrotic kidney even when the basal study was only slightly abnormal (15-minute washout basal -27 +/- 16%, after captopril -9 +/- 13, p <0.005). After surgical correction the diuretic washout during angiotensin inhibition appeared normal in all patients (15-minute washout -56 +/- 14%). Separate renal function and parenchymal transit of MAG-3 were not modified by angiotensin converting enzyme inhibition, preoperatively or postoperatively. CONCLUSIONS: Our data confirm the influence of angiotensin on the kidney excretory system in human hydronephrosis and suggest a role for captopril enhanced diuretic renography in the early diagnosis of pyeloureteral obstruction. Further work is needed to evaluate angiotensin converting enzyme inhibition as a protective agent in obstructive nephropathy.

Food entrapped in papilla of Vater: uncommon cause of vomiting.

CASE REPORT: A 20 month old girl was admitted for intractable vomiting over several days, with no other symptoms. Family and personal history were not contributive. Clinical and neurological examination, and routine blood tests and investigations (plain abdominal x ray, upper gastrointestinal tract contrast study, abdominal ultrasonography) were normal. The upper gastrointestinal endoscopy showed a mild antral gastritis and the second portion of duodenum was occupied by a tough, fibrous mass partially embedded into the papilla of Vater. The foreign body was removed and proved to be vegetable fibre (pineapple). Symptoms subsided immediately and the child was discharged with gastroprotective therapy. After two months, on endoscopic examination, the signs of gastropathy had cleared; the papilla of Vater was undamaged, but unchomped food debris was again found in the duodenum. DISCUSSION: There are sporadic reports of foreign bodies retained into the papilla of Vater, all of them in adults. This child, though her papilla was tiny, had no jaundice or pancreatitis, unlike most of the reported cases. This is the first report of this finding in a child. The cause of the vomiting was not shown on abdominal ultrasonography or contrast study. It should be added to the list of unusual causes of vomiting.

Congenital absence of portal vein with nodular regenerative hyperplasia of the liver.

Congenital absence of the portal vein is a very rare anomaly. The intestinal and splenic venous drainage bypasses the liver and drain into the inferior vena cava (IVC). Two cases of such anomaly are described. Both cases were investigated by US coupled with echo-colour Doppler examination, CT and MR imaging, followed by digital subtraction angiography (DSA) and liver biopsy. In the first case the splenic and superior mesenteric vein formed a venous trunk which emptied directly into the IVC; in the second case, the splanchnic blood flowed into a dilated hepatofugal inferior mesenteric vein which connected to the left internal iliac vein. In both cases nodular regenerative hyperplasia of the liver was present, presumably due to an abnormal hepatic cell response to the absent portal flow. The particular contribution of MR imaging to the diagnosis of both vascular abnormalities and liver parenchyma derangement and its advantages over the other diagnostic techniques is emphasized. The clinical and radiological features of 17 previously reported cases are reviewed.

Preserved vascular homograft for revascularization of pediatric liver transplant: a clinical, histological, and bacteriological study.

BACKGROUND: Arterial or venous homografts are frequently implanted for vascular reconstruction in orthotopic liver transplantation (OLT). When fresh vascular homografts (VH) from the same donor were not available, VH from another donor preserved at 4 degrees C in Terasaki (Ter) solution (modified lymphocyte culture medium) were used. METHODS: The clinical results after implantation of Ter-stored VH versus fresh VH in the revascularization of pediatric OLT were studied retrospectively. Short- and long-term follow-up of vascular patency was carried out by doppler ultrasonography in each case. A histological and bacteriological study of nonimplanted VH stored at 4 degrees C in saline (Sal), Ter and University of Wisconsin (UW) solutions for various time periods (days 0-28) was also undertaken. RESULTS: Between 1989 and 1996, 21 iliac arteries and 21 iliac veins preserved in Ter solution (mean preservation time: 8 days; range 1-26) and 100 fresh VH (68 arteries and 32 veins) (preservation time: 8 hr, range 4-21) were used in pediatric OLT. Thrombosis rates were 0 of 21 for stored arteries vs. 7 of 68 (10%) for fresh arteries (NS) and 3 of 21 (14%) for stored veins vs. 3 of 32 (9%) for fresh veins (NS). Actuarial graft survival rates were similar in both groups. Histological analysis of stored, nonimplanted VH invariably showed endothelial destruction within 24-48 hr after procurement. The bacteriological study showed contamination rates of 14 of 25 (56%) for Sal-stored VH, 5 of 25 (20%) for UW, and 1 of 19 (5%) for Ter (Sal vs. UW and Sal vs. Ter: P<0.01; UW vs. Ter: NS). CONCLUSIONS: Ter-preserved cadaveric VH could be safely used in OLT despite early destruction of endothelium.

Treatment of extrahepatic portal hypertension in children by mesenteric-to-left portal vein bypass: a new physiological procedure.

OBJECTIVE: To achieve hepatic portal revascularisation and decompression of extrahepatic portal hypertension in children with cavernoma and obstruction caused by idiopathic portal vein thrombosis. DESIGN: Selected cases. SETTING: Teaching hospitals. Belgium and Italy. SUBJECTS: 11 children who weighed between 5.9 and 54 kg (2 emergencies) with symptomatic extrahepatic portal hypertension. INTERVENTION: Interposition of venous autograft between the superior mesenteric vein and the distal (umbilical) portion of the left portal vein. MAIN OUTCOME MEASURES: Improvements in symptoms and endoscopic appearance after operation. RESULTS: 2 bypasses had to be redone because they stenosed; all 11 were patent at the time of writing (median follow-up 6 months, range 1-32 months). CONCLUSION: The bypass effectively relieved symptoms of extrahepatic portal hypertension by restoring normal hepatic portal blood flow.

Adenomyomatosis of the gallbladder in childhood.

Adenomyomatosis of the gallbladder (ADMG) is defined as an acquired disease characterized by localized or diffuse hyperplastic extensions of the mucosa into, and often beyond, the thickened gallbladder muscular layer (Rokitansky-Aschoff's sinuses). In recent years, attention has been drawn to its malignant potential. The occurrence of ADMG has never been reported in children. The authors report the case of a 5-year-old boy with symptomatic ADMG, who was successfully treated by laparoscopic cholecystectomy.

Microemulsion formulation of cyclosporine in pediatric liver transplantation.

The oral bioavailability of Sandimmun can be impaired by cholestasis, external biliary diversion, and diarrhea. We report two cases of pediatric liver transplant recipients who experienced chronic rejection and diarrhea secondary to proximal bowel resection. These conditions resulted in poor oral absorption of Sandimmun; the children were converted to the new oral microemulsion formulation Neoral, which significantly improved oral absorption, allowing intravenous cyclosporine weaning and patient discharge. Comparative pharmacokinetic studies were performed in both cases, and the relative Neoral/Sandimmun bioavailabilities were 32.9 and 5.4, respectively. Accordingly, Neoral may constitute to good alternative to ensure the effectiveness of oral cyclosporine administration, particularly in liver-transplanted children with severe cholestasis or shortened small bowel.

[The laparoscopic treatment of Hirschsprung's disease]. / Trattamento laparoscopico della malattia di Hirschsprung.

Swenson's procedure, first described in 1948 for Hirschsprung's disease, consists in resection of aganglionic intestine and distal colo-anal anastomosis provided a precise dissection of extra peritoneal rectum. Potential jeopardy of pelvic vessels and nerves stimulated alternative surgical techniques to prevent complications on bladder and genital function. We performed in laparoscopy Swenson's procedure after Toupet, taking advantage from closer view and magnification of this technique, in a 15 months girl. Laparoscopy simplified and made safer pelvic dissection and resulted in a better postoperative period and cosmetic outcome.

Autologous buccal mucosa graft for hypospadias repair: an initial report.

Autologous buccal mucosa alone or combined with bladder mucosa was used as a free graft for urethral reconstruction in 8 patients with hypospadias. Of these patients 3 with medium penile hypospadias and chordee received a buccal mucosa graft alone and 5 with severe hypospadias received a combined buccal/bladder mucosa graft. Followup at 6 to 18 months showed no complications apart from a fistula in 1 patient with a buccal mucosa graft alone. Our initial results are encouraging. Buccal mucosa seems to be a good material for hypospadias repair.