Unusual bladder mass in a 14-year-old boy: bladder paraganglioma.

A 14-year-old boy presenting headache crisis, sweating and palpitation was observed. On ultrasound scan, a 3 × 2.5 cm hypoechoic mass, highly vascularized, was observed arising from the left bladder wall. Magnetic resonance imaging confirmed the mass situated close to the ureteral orifice, with intense peripheral enhancement and a central non-enhanced portion. Left partial cystectomy was performed. Large clear cells, presenting cytoplasmatic granules positive for S100 and chromogranin, were observed at histology. Bladder paraganglioma derives from chromaffin tissue of the sympathic system and it is uncommon in children. The estimated prevalence is very low (0.06% of bladder tumors). Symptomatology is related to the catecholamine production. In pediatrics, prognosis is usually good but long-term follow-up is required.

Glanular Venous Malformation in Pediatric Age: An Uncommon Vascular Disorder.

Glanular venous malformations are uncommon in pediatric patients. The diagnosis can be easily achieved by observation, even if color Doppler ultrasound is useful for a better characterization. Abdomino-pelvic MRI is necessary to assess the extension of complex lesions and check for associated anomalies. Several therapeutic options are reported in literature. We report 3 paediatric cases successfully treated by surgery with no complications and functional sequelae. Cosmetic results were satisfactory, with minimal surgical scarring. In our opinion, surgery for small glanular venous malformations is indicated within puberty to prevent traumatic bleeding and psychological impact.

Arterial changes in children undergoing renal transplantation.

INTRODUCTION: Pathological changes of large arterial walls and the heart have been described in patients with chronic renal failure. The aim of the study was to verify the incidence of arterial changes among our series of pediatric patients with end-stage renal disease undergoing transplantation. PATIENTS AND METHODS: From January 2004 to December 2006, 26 patients (15 boys and 11 girls) of overall mean age of 13.12 years (range=3-27 years) underwent renal transplantation in our department. The pretransplant dialysis treatment was peritoneal in eight and hemodialysis in 18 cases. All patients were divided in two groups according to primary renal disease: group A were 18 patients with congenital urinary malformations; and group B, eight patients with acquired glomerular diseases. In each case, a sample of artery from both donor (aortic patch of kidney) and recipient (iliac patch of graft allocation) was obtained during renal transplantation. The donors were considered to be the control group. RESULTS: Light microscopy showed pathological changes in 12/26 recipient arteries: nine showed light fragmentation of the internal elastic lamina; two, more severe fragmentations; and one, fragmentation of the internal elastic lamina associated with mucopolysaccharide deposits. Pathological changes were more evident in group A than B, but the difference was not significant (P> .05). Among the donor group, 11 patients showed light fragmentation of the internal elastic lamina, but there was no significant difference with the recipients (P> .05). CONCLUSIONS: Among our group of patients, we observed only slight modifications of the arterial wall. These changes were nonspecific, similar to those in a control group of donors matched for age. A possible explanation of these findings may depend on the short time of dialysis before transplantation.

Renal embolization as an alternative to surgical nephrectomy in children.

PURPOSE: Complete renal embolization may be an alternative to surgical nephrectomy. The indications for renal embolization do not differ from those for surgical nephrectomy, but the less invasive nature of the technique is a major advantage. Few case reports are available in the pediatric age group. Our experience showed that complete renal embolization was feasible in pediatric patients with results comparable to those obtained in adults. MATERIALS AND METHODS: Twelve pediatric patients underwent 14 renal embolizations. The indications for embolization were as follows: (1) severe hypertension in 7 patients with end-stage renal failure; in these cases, a unilateral native nephrectomy was recommended prior to renal transplantation; (2) end-stage hydronephrosis in 3 patients with moderate hypertension or recurrent urinary infection; (3) nephrotic syndrome in 1 patient; or (4) ablation of an irreversibly rejected renal allograft in 1 patient. The embolization was performed under epidural anesthesia in 10 patients and under general anesthesia in 2 patients, by means of a polyvinyl alcohol injection with hemostatic gelatin powder and placement of coils. Postembolization course was followed. RESULTS: The embolization was successful in all 12 patients. In 1 patient, the procedure had to be repeated as a small accessory artery had revascularized the upper pole. In another patient, the procedure was bilateral in 2 separate sessions. In 10 patients, severe flank pain required narcotic analgesia. Two patients had fever. None had hypertension peaks. Median hospital stay was 4 days. At mean follow-up of 16 months, the results were stable. CONCLUSION: Renal embolization can avoid surgical nephrectomy also in pediatric patients. The advantages are less morbidity and shorter hospital stay. Our results in the short and medium term were equal to those of surgical removal. The procedure appeared to be safe and minimally invasive.

Asynchronous Bilateral Ovarian Torsion: Three Cases, Three Lessons.

BACKGROUND: Ovarian torsion (OT) is a serious condition, and delay in surgical intervention may result in loss of the ovary. Children and adolescents who have suffered from ovarian torsion may be at risk for asynchronous torsion of the contralateral ovary. STUDY OBJECTIVE: Three cases of asynchronous bilateral ovarian torsion were reported to analyse clinical history of three patients, to review the current literature, and to draw a conclusion for future treatment. DESIGN: Case reports and review of the literature. RESULT: When a prepubertal girl presents with an ovarian torsion, several considerations have to be taken in account in order to preserve her future fertility; in particular, the pediatric surgeon/gynecologist has to preserve as much as possible the twisted ovary in addition to considering the fate of the contralateral ovary. SUMMARY AND CONCLUSIONS: Pelvic pain in a young girl has always raised the clinical suspect of an ovarian torsion; the possibility of asynchronous bilateral ovarian torsion is rare, but it is described in the literature and has catastrophic consequences; this condition has to be known and treated in the proper way by pediatric surgeons as well as by gynecologists in order to maximize the future fertility of the young patients.

Hypothesis on etiopathogenesis, congenital or acquired, of an imperforate distal ureter: a case report.

INTRODUCTION: Ureteral atresia is a rare disease usually associated with a non-functioning kidney. Its association with other urinary anomalies is rare. CASE PRESENTATION: In this study we discuss the possibility of congenital or acquired etiology of a right imperforate distal ureter. Here we report the case of 11-month-old white boy with a right ureteropelvic junction obstruction. He underwent a right pyeloplasty when he was 11-months old, and 3 weeks after surgery a cystoscopy was performed. Two months after the first operation, he underwent a right ureteral meatoplasty and a new pyeloplasty. CONCLUSIONS: To the best of our knowledge, few cases of imperforate distal ureter have been described in the literature. The suspicion of a non-patent terminal ureter, occurring during upper urinary tract surgery, must be intraoperatively clarified to preserve the renal function and to avoid more complex surgical approaches.

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.

Combined heart and kidney transplantation in a child: will we need it more in the future?

A 12-year-old girl affected by idiopathic dilated cardiomyopathy and renal failure was referred to our institution for cardiac transplantation. A simultaneous heart-kidney transplantation from the same donor was decided. The immunosuppression schedule consisted of azathioprine, antithymocyte globulin, steroids, and cyclosporine. At a follow-up visit at 24 months after transplantation, no episodes of heart or kidney rejection had occurred and cardiac and renal function were good. Concomitant failure of heart and kidney is well known in the literature, but it appears to be more frequent in adult as compared with the pediatric population. This is the first case of combined heart and kidney transplantation in a child. Because of the successful outcome and good follow-up, the number of combined organ transplantations will most likely increase in the future.

Unilateral cryptorchidism corrected in prepubertal age: evaluation of sperm parameters, hormones, and antisperm antibodies in adult age.

OBJECTIVE: To evaluate whether prepubertal orchidopexy for unilateral cryptorchidism can reduce adult dysspermia. DESIGN: Patient follow-up comparison with control groups. SETTING: Surgical and endocrinologic sections of a children's hospital and a university infertility-care center. PATIENT(S): Seventy-one patients with unilateral cryptorchidism who underwent orchidopexy in prepubertal age (6.4 +/- 2.8 years) were followed up as adults (20.0 +/- 2.8 years). MAIN OUTCOME MEASURE(S): Patients underwent testicular examination and hormonal evaluation, 49 of these had semen analysis and antisperm antibody tests. Semen results were compared with those of two age-matched control groups: a group of 20 healthy, randomly selected subjects and a group of 20 patients operated on in postpubertal age for cryptorchidism. RESULT(S): Unilateral reduced testis size was found in 30.1% of patients, eight patients had a low LH level, eight had a low T level, and none had abnormal FSH values. Antisperm antibodies were found in 1 of 49 cases. Cluster analysis of sperm parameters showed that the mean values of patients were worse than those of the healthy controls but better than those of the subjects operated on in postpubertal age. CONCLUSION(S): This study indicates that prepubertal orchidopexy can given better results than postpubertal correction.

Urinary continence in Müllerian duct anomalies.

Müllerian duct congenital anomalies such as Rokitansky-Mayer syndrome, Urogenital Sinus, Vaginal Atresia and Cloacal Malformation are relatively uncommon (1-5% of born female newborns). The complexity of these malformations has taken great interest regarding mainly the surgical procedure available for correction of genital abnormalities. However, the problem of urinary incontinence is still underestimated, and continence is often a goal difficult to achieve. Authors report 11 cases of congenital anomalies of Müllerian duct differentiation which have been observed in the last decade in pediatric age with special regard to preoperatory diagnostic procedures, urinary continence valuation after surgical correction, therapy chances for continence, considering the psychological implication of this unresolved abnormality in everyday-lifetime.

Preliminary report of electrogastrography in pediatric gastroresection: can it be predictive of alteration of gastric motility?

BACKGROUND/PURPOSE: Gastric resection is an infrequent surgical procedure in childhood. However, the use of the stomach for bladder augmentation and substitution is well documented. Partial gastrectomy performed in gastrocystoplasty (GCP) involves the greater curvature of the stomach, the same area in which gastric pace-maker cells are known to be placed. The aim of this study was to assess, by electrogastrography (EGG), if subtotal gastric resection can alter gastric motility in children submitted to partial gastrectomy for GCP. METHODS: Gastric electrical activity (GEA) was evaluated in 25 children using EGG: 10 patients (4 boys, 6 girls; mean age, 11.6 years) previously submitted to GCP, and 15 normal subjects (12 boys, 3 girls; mean age, 8.62 +/- 2.77 years) as controls. All patients were submitted to cutaneous EGG; recording GEA for 30 minutes before and after a standard test meal. The percentage of 3 cycles per minute (3CPM), bradygastria, tachygastria, DFIC (dominant frequency instability coefficient), DPIC (dominant power instability coefficient), PDP (period dominant power), PDF (period dominant frequency) were recorded and analyzed using Wilcoxon matched-pair test. Data were considered statistically significant if P <.05. RESULTS: Normal subjects as well as operated patients showed a statistically significant difference in bradygastria (P =.05), PDP and PDF (P =.05) percentage, comparing pre versus postprandial period. In the normal group, 3CPM (P =.0012) and DFIC (P =.0008) were statistically different between the pre- and postprandial period. Patients who underwent GCP did not show any statistically significant difference in 3CPM and DFIC pre- and postprandial. CONCLUSIONS: In normal subjects, GEA showed a complete variation after the meal, whereas in operated patients GEA was impaired and only partially modified after the meal. This observation suggests that in patients with gastric resection, adaptation of the stomach to food ingestion is present but incomplete with respect to normal subjects; it can be caused by surgical removal of the pace-maker cells of the greater curvature. For this reason a follow-up analysis of gastric function is recommended for all patients undergoing GCP.

[Retractile testis and gliding testis. Two distinct clinical entities]. / Testicolo retrattile e gliding testis. Due entità cliniche distinte.

The gliding testis is a testicle located below the external ring, can be manipulated to the upper scrotum, but is prone to ascend to its original position. Histologic changes can be detected in these gonads by 7 years of age. We evaluated 427 consecutive prepubertal boys referred for cryptorchidism. One hundred and twenty-three had classical undescended, 71 ectopic, 55 retractile, and 178 (mean age 6 years 2 moths) gliding testes. The gliding testes were smaller than controlaterals in 24% of boys. All gliding testes were unilateral whereas bilaterality was 85% in the retractile group (p < 0.0001), 17.5% in the undescended (p < 0.001), and 10% in the ectopic group (p < 0.01). A history for one or more of the following: orchidopexy (3), hormonal treatment (5), late testicular descent (9), spermatic cord torsion (5), testicular pain (10), actual retractile testes (20) or actual gliding testis (58), was present in 93 (52.2%) of the fathers of the gliding group. Forty-seven (81%) paternal gliding testes were hypotrophic. Seventy-five boys with gliding testis underwent initial hormonal therapy with transient benefit, and 57 were operated on. Two anatomical findings are typical of the gliding testis: the absence of the gubernaculum and a processus vaginalis partially patent from the upper scrotum to the mid groin area. This feature explains the mobility of the gliding testis from the external ring to the upper scrotum. The absence of the gubernaculum may be responsible for a higher incidence of spermatic cord torsion in this population. The gliding testis is a distinct entity, representing the mildest degree of a true undescended testis. As hormonal treatment gives only transient results, orchidopexy should be considered before testicular damage occurs.

Fetal surgery for spina bifida aperta: to be or not to be?

Fetal SBA (spina bifida aperta) has been operated upon in more than 213 cases in the USA in the last 8 years. Indications and results still remain controversial. Our group reproduced successfully Meuli's model in sheep and reviewed the literature on SBA and the Internet updated sites on the theme. Then we reviewed the follow-up of all cases operated upon postnatally in 20 years at a Neurosurgery Department in Rome. We published all the data regarding the three above-mentioned steps. This paper contains reflections on the three, and our opinion on the correct approach to prenatally diagnosed SBA, as far as fetal surgery is concerned.

Early detection of bladder dysfunction following posterior urethral valves ablation.

Urinary incontinence following Posterior Urethral Valves (PUV) ablation has been attributed in the past to sphincter injury, but it is nowadays accepted that bladder dysfunction (BD) plays a determinant role. In order to assess BD evolution, we have evaluated, from 1982 to 1994, 48 boys with PUV by urodynamics (UD) studies. Age of the patients ranged from 10 months to 15 years. A total of 65 examinations were performed. We considered four groups: 0-3 years; 4-7 years; 8-12 years and > 12 years. Uninhibited detrusor contractions (instability), end filling pressure (EFP), bladder capacity and voiding detrusor pressure were evaluated in order to assess the presence of BD distinguishing it in: hypercontractility, hypocontractility and low-compliant bladder. The results collected confirm a high percentage of BD in PUV boys (71%) and the evolution of hypercontractility versus hypocontractility in 60% of patients considered.

[Urinary incontinence in children: etiopathogenesis and clinical aspects]. / L'incontinenza urinaria nelle bambine: etiopatogenesi e clinica.

Urinary incontinence can only be considered a disease after a child has completely acquired spinal mechanisms of vesico-sphincteric nervous control. This normally happens for the majority of children between two and two-and-a-half years of age regarding voluntary daytime control, while complete night-time control is obtained in 90% of cases within the fourth year. By urinary incontinence we mean an involuntary leak of urine through the bladder neck. This can vary from an occasional leakage consisting of a few drops to a continuous and uncontrollable stream of urine. From 1981 to 1986 we evaluated, in the Pediatric Urological Service at Bambino Gesù Hospital, Rome, 426 children suffering from urinary incontinence. According to the etiology we divided the patients into two large groups: 1) patients with incontinence due to anatomic causes 56 (13%); 2) patients with incontinence due to dysfunctional causes 370 (87%).

[Tumors of Meckel's diverticulum]. / Tumori del diverticolo di Meckel.

The presentation of a rare case of leiomyoma of Meckel's diverticulum in a boy of 5 yr forms the starting point for a discussion of such cancers. In adult age, the complication is not so rare as the sporadic publications would suggest, incidence of tumoral forms being around 1 : 50 M.D. with a high malignancy frequency. In paediatric age, on the other hand, the tumoral degeneration of a M.D. is an exceptional occurrence: a review of more than 1,200 reported cases and personal experience (48 cases of M.D. in 15 yrs) confirm the rarity of the observation. The need to seek M.D. on each occasion and remove it to prevent the risk of tumoral degeneration in adult age is stressed. M.D. surgical removal tecniques are commented on and emphasis laid on the desirability of ample intestinal resection in cases of cancer of the diverticulum.

[Hepatic resection in the fetal rabbit. Histologic comparison of tissue regeneration in the fetus versus the adult]. / Resezione epatica fetale nel coniglio. Confronto istologico della rigenerazione tissutale del feto e dell'adulto.

Fetal tissues present peculiar features of repair after injury. Although the development of fetal hepatocytes have already been studied in vitro and in transplant models, an in vivo study of fetal liver regeneration is still missed in the literature, to the best of our knowledge. Eight time-dated pregnant California rabbits (23, 24, 25, 30 days of gestational age) and 2 adult male California rabbits were anesthetized following a standardized i.v. protocol (ketamine 50 mg/kg; xilazine 5 mg/kg; propiopromazine 0.75 mg/kg; spontaneous breathing; no anesthetic gas). All the pregnant does underwent a midline laparotomy and a minimal hysterotomy to approach a fetus per each animal. In 2 cases, 1 fetus was delivered and prior to sacrifice the fetal liver was sampled in toto (30 days of gestational age). These pregnancies were allowed to continue to term and were uneventful with a full-term spontaneous delivery of the remaining fetuses. In the other 6 pregnancies, after the hysterotomy, the fetal abdomen was entered through a right-sided longitudinal incision and the liver was partially resected by thermocauterization. Fetal abdomen was closed in 1 layer (non absorbable suture 7-0). The fetus was then returned in the uterus and, after amniotic fluid restoration with warmed saline, the hysterotomy was sutured in double layer (polyglycolic 5-0). Maternal abdomen was closed in 1 layer (polyglycolic 4-0) and the skin in a continuous overlying fashion (silk 3-0). The abdominal cavity of the 2 adult male rabbits was entered through a right subcostal incision. Partial liver resection was performed, and abdominal and skin closure followed the same techniques used for the pregnant does. The treated livers were then sampled in toto at 24, 48, 72 hrs and 4 days after surgery from the fetuses, and at 7 days from the adult rabbits. Histological stains were: H & E; Van Gieson; Masson; Alcian Bleu; PAS. Fetal histology showed a low inflammatory reaction poor in PMN cells with minimal deposition of collagen and a high amount of glycogen in the hepatocytes. The inflammatory response to resection was much more evident in the adult samples as much as the abundant intra and extra-cellular deposition of collagen associated to a minor amount of intracellular glycogen. The peculiar features of liver regeneration in the fetus, deserve further experimental studies.

Bladder instability and enuresis treated by acupuncture and electro-therapeutics: early urodynamic observations.

The authors report the results of a study on 20 children suffering from a particular type of enuresis, associated with bladder instability, characterized by uninhibited contractions of the detrusor muscle. The children selected showed symptoms of enuresis, frequency, urinary urgency and a positive urodynamic test. This test was performed on 11 patients before and after acupuncture. In 16 out of 20 children, the authors observed a net increase in the intensity and frequency of uninhibited bladder contractions 30 minutes after acupuncture. At 60 minutes the contractions decreased and at 24 hours they had practically disappeared. Clinically, a gradual elimination of enuresis has been observed in 11 cases and an improvement in the other 7. From these early observations, the efficiency of acupuncture in suppressing uninhibited bladder contractions seems to be proved, even though the therapeutic mechanism has yet to be clarified.

[Intrapancreatic lipoma: a case report]. / Lipoma intrapancreatico: descrizione di un caso.

A rare case of asymptomatic pancreatic lipoma in an eleven-month-old child is presented. The abdominal mass was discovered occasionally during a grip episode. Preoperative tests proved the presence of a neoformation arising from the pancreas. It was multilobed, capsulated and located between colon transversus, spleen and stomach without infiltration of surrounding structures. The histological picture was that of a mature lipoma. The authors discuss the differential diagnosis versus other fat tumors.

Gait analysis in bladder exstrophy patients with and without pelvic osteotomy: a controlled experimental study.

BACKGROUND: The role of bony pelvic anomalies in bladder exstrophy is long established and has generated many papers addressing walking problems. Biomechanical studies and kinematic gait analysis were performed on very young children. AIM: A direct kinetic gait evaluation has never been performed, nor has the effect of pelvis dimorphism on the upper body been studied. DESIGN: Controlled experimental study. SETTING: Outpatients were studied at the time of periodic follow up. POPULATION: Nineteen patients with bladder exstrophy, age 14±8 years, and twenty-five healthy control participants, age 15±8 years, were enrolled in the present gait analysis study. METHODS: Clinical evaluation and standard gait analysis were performed. RESULTS: Gait analysis deviations between exstrophy patients and controls and between patients that received pelvic osteotomy (OT--6 patients) and those that did not (no-OT--13 patients) were analyzed. Bladder exstrophy significantly affects kinematics and kinetics of trunk, spine, pelvis, knee and foot; in particular: in OT, trunk retroversion, pelvic retroversion and rotation, hip adduction angle and moment, knee flexion and its maximum power during loading response increased, whereas in no-OT, spine angle, pelvic posterior tilt, hip extension, and the external rotation of the foot progression angle increased. All the kinetics parameters analyzed in the study showed lower values in the patient group than in controls. CONCLUSION: . Walking in patients with bladder exstrophy is accomplished by retroversion of the pelvis and deviations mainly in the spine angle in no-OT and in knee flexion in OT. CLINICAL REHABILITATION IMPACT: Gait analysis was shown to be an effective tool for the detection of walking deviations that should be identified early, prompting rehabilitative treatment in order to prevent spine and knee diseases.