Lucas Krauel

Transitional cell carcinoma of the bladder in children and adolescents: Six-case series and review of the literature *

OBJECTIVE Lower urinary tract tumours are uncommon in paediatrics. Transitional cell carcinoma of the bladder (TCCB) is rarely found in the first two decades of life and is exceptional under 10 years of age. The present series aimed to expand the number of reported cases in the literature. PATIENTS AND METHODS In 1984-2007, six patients (four male, two female), aged 6, 9, 12, 13, 14 and 17 years, were treated at our centre. Clinical presentation was macroscopic haematuria in five and pyelonephritis in one. Physical examination, laboratory analysis, ultrasound and cystoscopy were performed before surgical treatment in all patients. Follow up was by clinical and ultrasound assessment. RESULTS Neither physical examination nor laboratory analysis revealed any significant abnormalities, but ultrasound showed exophytic intravesical lesions. Surgical resection was performed endoscopically. Histological studies showed grade I TCCB in all cases. The immediate postoperative period was uneventful and long-term follow up did not reveal recurrence. CONCLUSION Despite its low incidence in children, TCCB must be suspected in the event of macroscopic haematuria. Ultrasound followed by cystoscopy are the ideal diagnostic tools for visualization of these tumours. Endoscopic resection proved effective in all the present cases. Follow up must be clinical with periodic ultrasound evaluation. Urine cytologic examination is ineffective. Periodic cystoscopy is indicated only in cases of clinical or ultrasonographic suspicion of recurrence.

High Pressure Balloon Dilation of the Ureterovesical Junction—First Line Approach to Treat Primary Obstructive Megaureter?

PURPOSE We describe the efficacy of dilation of the ureterovesical junction to treat primary obstructive megaureter. MATERIALS AND METHODS A total of 13 patients with primary obstructive megaureter were treated from May 2008 to December 2010. Of these patients 8 were diagnosed prenatally and the others were diagnosed after a urinary tract infection. Preoperative studies included ultrasonography, voiding cystourethrography despite vesicoureteral reflux and diuretic isotopic renogram (mercaptoacetyltriglycine). With the patient under general anesthesia, high pressure balloon dilation of the ureterovesical junction was performed under direct and fluoroscopic vision until the disappearance of the narrowed ring. A Double-J(®) catheter was positioned, and 2 months later it was withdrawn and the ureterovesical junction was reviewed. A secondary treatment was performed in those in whom the ureterovesical junction was still narrow. Followup was performed with ultrasonography, cystourethrography and isotopic diuretic renography. RESULTS A total of 18 procedures were performed in 13 patients (median age 7 months, range 4 to 24). Median diameter of the distal ureter was 14 mm (range 10 to 26), and median diameter of the renal pelvis and calyx was 27 mm (range 10 to 47) and 12 mm (range 9 to 26), respectively. Significant postoperative improvement of hydroureteronephrosis was observed in 11 of 13 patients and vesicoureteral reflux was found in 2. Only 3 patients needed ureteral reimplantation after endoscopic treatment due to hydroureteronephrosis in 2 and high grade vesicoureteral reflux in 1. CONCLUSIONS High pressure balloon dilation of the ureterovesical junction is effective in treating primary obstructive megaureter, but long-term followup is needed.

Median raphe cysts of the perineum in children.

Median raphe cysts of the perineum are uncommon congenital lesions of the male genitalia. They can be found all the way from the distal penis and scrotum toward the perineum in a midline position. They are considered as congenital alterations in embryologic development. A case of a 6 year-old boy is presented. Review of the literature relevant to children was made regarding the embryologic, diagnostic, and treatment aspects. We believe it is important that adult and pediatric urologists recognize these lesions and their management to provide the appropriate information to the parents.

Heminephroureterectomy for duplex kidney: Laparoscopy versus open surgery

OBJECTIVE To report our experience of laparoscopic heminephroureterectomy (Hnu) in pediatric patients with duplex anomalies, in comparison to open surgery. PATIENTS AND METHODS Retrospective review of data from patients who underwent Hnu from 2005 to 2008 was performed. The patients were divided into two groups: laparoscopic (LHnu) and open surgery (OHnu). Laparoscopic surgery was performed by transperitoneal approach in majority of cases. Open surgery was performed by retroperitoneal approach in all cases. RESULTS Group LHnu: nine patients (8 females, 1 male) with median age of 14 months (range 3-205). Transperitoneal approach was performed in eight patients. Mean operative time was 182 min (CI 95% 146-217). No conversion to open surgery was necessary and there were no complications. Mean hospital stay was 2.44 days (CI 95% 1.37-3.52). Group OHnu: eight patients (3 females, 5 males) underwent nine heminephrectomies at median age of 6.9 months (range 1-12). Mean operating time was 152 min (CI 95% 121-183). There were no complications and mean hospital stay was 4.38 (CI 95% 2.59-6.16) days. Statistical analysis showed no statistically significant difference (P>0.05) in operating time between groups while mean hospital stay was significant (P=0.021). CONCLUSION The laparoscopic approach is feasible, safe, reduces hospital stay, does not increase operating time and has better cosmetic results. We believe this should be the first option for heminephrectomy.

Lung Tissue Blood Perfusion Changes Induced by in utero Tracheal Occlusion in a Rabbit Model of Congenital Diaphragmatic Hernia

Objective: To analyze the impact of in utero tracheal occlusion (TO) on lung tissue blood perfusion, as measured by fractional moving blood volume (FMBV) and conventional spectral Doppler, in a rabbit model of congenital diaphragmatic hernia (CDH). Methods: In 50 fetal rabbits, a left CDH was surgically created at 23 days of gestational age (GA). At 28 days of GA, the surviving CDH fetuses were randomly assigned to undergo either TO (CDH+TO group) or a sham operation (CDH group). Twenty littermates, which were not operated on, served as internal normal controls. At 30 days of GA, lung perfusion estimated by FMBV and spectral Doppler of the proximal intrapulmonary artery were evaluated in the right lung during cesarean section. Doppler waveform analysis included the pulsatility index (PI), peak early diastolic reverse flow and peak systolic velocity. Results: Eleven CDH fetuses, 9 CDH+TO and 20 controls were suitable for the study. CDH fetuses showed a significantly higher PI [8.0 (SD 1.8) vs. 5.22 (SD 1.1), p < 0.001] and lower FMBV [13.5% (SD 4.6) vs. 23.0% (SD 2.1), p < 0.001] than the controls. In contrast, CDH+TO fetuses had a significantly lower PI [5.8 (SD 2.3) vs. 8.0 (SD 1.8), p = 0.015] and higher FMBV [27.6% (SD 7.1) vs. 13.5% (SD 4.6), p < 0.001] than CDH fetuses, with values similar to the controls. Peak early diastolic reverse flow and peak systolic velocity showed nonsignificant differences among the study groups. The lung to body weight ratio at necropsy correlated positively with lung FMBV (r = 0.60, p < 0.001) and negatively with the pulmonary artery PI (r = –0.48, p < 0.01). Conclusion: Tracheal occlusion is consistently associated with increased lung tissue perfusion and decreased intrapulmonary impedance in a rabbit model of CDH.

Use of angioembolization as an effective technique for the management of pediatric solid tumors

PURPOSE In oncology practice, angioembolization has been reported for tumor reduction before surgery, treatment of life-threatening conditions, and for palliative care. Nevertheless, the overall experience with angioembolization for the treatment of tumors is limited. We report our experience in 7 nonvascular solid pediatric tumors. MATERIALS AND METHODS A retrospective review was carried out of medical records from pediatric patients (0-18 years) with solid nonvascular tumors who underwent angioembolization in the last 5 years at our institution. RESULTS Seven patients underwent embolization: 2 neuroblastomas, 1 metastatic paraganglioma, 1 hepatoblastoma, 1 myofibroblastic tumor, 1 osteosarcoma, and 1 undifferentiated sarcoma. The reason for angioembolization was preparation for surgery (3), treatment of a life-threatening event (1), or palliative care (3). Each case is presented and discussed. The outcome was subsequent complete surgical resection in 3 cases, tumor vanished in 1 case, symptom control was achieved in 1, and the other 2 patients improved their survival and quality of life, however, died of disease progression. CONCLUSIONS Tumor angioembolization may enter the treatment algorithm for selected patients who have to face difficult or unwarranted surgical procedures or have diseases where conventional therapies have failed.

Fibrolamellar hepatocellular carcinoma in an infant and literature review.

Hepatocellular carcinoma (HCC) is a rare pediatric neoplasm exceptionally reported in infants and fibrolamellar hepatocarcinoma (FLC) a HCC variant. Controversy exists whether FLC has a better prognosis than classic HCC, although recent studies of children and young adults with FLC did not report a better outcome. We present a 4-month-old male infant without any related metabolic or infectious disease who developed a metastatic and multifocal FLC. Serum alpha-fetoprotein determinations were always normal. Induction chemotherapy using cisplatin and Adriamycin resulted in a partial response, however, refractory disease developed and regional metastasis precluded surgical resection. The child died from tumoral progression.

Use of 3D Prototypes for Complex Surgical Oncologic Cases

IntroductionPhysical 3D models known by the industry as rapid prototyping involve the creation of a physical model from a 3D computer version. In recent years, there has been an increasing number of reports on the use of 3D models in medicine. Printing such 3D models with different materials integrating the many components of human anatomy is technically challenging. In this article, we report our technological developments along with our clinical implementation experience using high-fidelity 3D prototypes of tumors encasing major vessels in anatomically sensitive areas.MethodsThree patients with tumors encasing major vessels that implied complex surgery were selected for surgical planning using 3D prototypes. 3D virtual models were obtained from routine CT and MRI images. The models, with all their anatomical relations, were created by an expert pediatric radiologist and a surgeon, image by image, along with a computerized-aided design engineer.ResultsSurgeons had the opportunity to practice on the model before the surgery. This allowed questions regarding surgical approach; feasibility and potential complications to be raised in advance of the actual procedure. All patients then successfully underwent surgery as planned.ConclusionHaving a tumor physically printed in its different main component parts with its anatomical relationships is technically feasible. Since a gross total resection is prognostic in a significant percentage of tumor types, refinements in planning may help achieve greater and safer resections therefore contributing to improve surgical management of complex tumors. In this early experience, 3D prototyping helped significantly in the many aspects of surgical oncology planning.

Urinary and Gastrointestinal Malakoplakia in a 12-Year-Old Girl

Malakoplakia is a rare chronic inflammatory disease that was originally described in the urinary bladder but can involve many other organs and soft tissues. It is believed to be caused by an alteration in the bacterial phagocytic system. Clinically, it is described as single or multiple tumors that can appear in any part of the body. Histologically, the presence of Michaelis-Gutmann bodies is pathognomonic. Malakoplakia in children is rare. Few pediatric cases in the urinary tract, kidney, or gastrointestinal tract have been published. We present a case of urinary and gastrointestinal malakoplakia in a 12-year-old girl.

Laparoscopic pyeloplasty in pediatric patients. Our initial cases and lessons learned

OBJECTIVE To analyze the initial experience in our first patients with ureteropelvic junction obstruction (UPJO) treated by laparoscopic surgery. PATIENTS AND METHODS All laparoscopic Anderson-Hynes pyeloplasties performed from July 2007 to April 2009 were analyzed. Before surgery, patients underwent a renal ultrasound and isotope renogram. A double J catheter was left in place and subsequently removed. Patients were followed up by ultrasound and renography. RESULTS Fourteen patients with a median age of 8.6 years were analyzed. Preoperative ultrasound showed a median renal pelvis diameter of 34.5 mm. Median operating time was 235 min, and median hospital stay 5.5 days. Median renal pelvis diameter decreased in all patients (13.5 mm). CONCLUSIONS Laparoscopy is an effective procedure for UPJO correction in children, although operating times are still long.