Risk factors and predictors of severity score and complications of pediatric hemorrhagic cystitis.

PURPOSE: We retrospectively analyzed our institutional incidence of hemorrhagic cystitis, identified risk factors, and examined associations of risk factors with disease severity and genitourinary complication rates. MATERIALS AND METHODS: We reviewed charts of all consecutive pediatric patients treated from 1986 to 2010. We analyzed demographics, underlying diagnosis and treatment data to assess risk factors for hemorrhagic cystitis. We also correlated disease severity scores with clinical predisposing factors, and performed univariate and multivariate analyses to examine associations between risk factors and outcomes. RESULTS: Hemorrhagic cystitis was observed in 97 of 6,119 children (1.6%), most of whom (75%) had severity scores of II or III. Mean ± SD age was 12.2 ± 6.3 years for patients with hemorrhagic cystitis and 10.5 ± 7 years for patients without hemorrhagic cystitis (p = 0.017). On univariate analysis increased risk of hemorrhagic cystitis was significantly associated with age greater than 5 years, male gender, cyclophosphamide or busulfan chemotherapy, bone marrow or peripheral blood stem cell transplantation, pelvic radiotherapy and underlying diagnoses of rhabdomyosarcoma, acute leukemia and aplastic anemia. On multivariate analysis age greater than 5 years, allogeneic bone marrow or peripheral blood stem cell transplantation and pelvic radiotherapy were significantly associated with increased risk of hemorrhagic cystitis. Older age, late onset hemorrhagic cystitis, positive urine culture for BK virus and bone marrow or peripheral blood stem cell transplantation were associated with greater disease severity. Patients with higher severity scores more frequently experienced bladder perforation, hydronephrosis, overall hemorrhagic cystitis complications, and increased creatinine and blood urea nitrogen levels during followup. CONCLUSIONS: Older age, previous bone marrow or peripheral blood stem cell transplantation and BK virus in the urine are risk factors for hemorrhagic cystitis and are associated with a higher severity score. Higher severity scores are associated with increased rates of genitourinary complications and renal impairment.

Renal medullary carcinoma in a white adolescent with sickle cell trait.

Renal medullary carcinoma (RMC) is a rare neoplasm of the kidney that has been recently described. It is almost exclusive to young patients of African descent and associated with sickle cell hemoglobinopathy, mainly sickle cell trait and hemoglobin sickle cell disease. The prognosis of RMC is very poor because of the highly aggressive behavior of this neoplasm and its resistance to conventional chemotherapy. Metastatic disease is almost universal at the time of presentation, and the malignancy is minimally responsive to a variety of regimens and/or modalities, including surgery, radiotherapy, chemotherapy, and biological immune-modulation therapy. We report the seventh case of a left RMC occurring in a white child with sickle cell trait, but with a localization of the tumor in the left kidney, considered a nonpredominant side for this type of tumor.

Pediatric standard and robot-assisted laparoscopic pyeloplasty: a comparative single institution study.

PURPOSE: We report our experience and compare the outcomes between standard and robot-assisted laparoscopic pyeloplasty to treat ureteropelvic junction obstruction in children. MATERIALS AND METHODS: A retrospective cohort study was performed of all children who underwent standard or robot-assisted laparoscopic pyeloplasty for ureteropelvic junction obstruction at a single institution from October 2007 to January 2012. Indications for surgery included symptomatic obstruction and abnormal diuretic renal scan. A successful outcome was defined as resolution of clinical symptoms, improvement of hydronephrosis on ultrasound, stable ultrasound with resolution of symptoms or improvement of the drainage curve on diuretic renal scan. RESULTS: We reviewed 18 patients (median age 8.1 years) who underwent standard and 46 (8.8 years) who underwent robot-assisted laparoscopic pyeloplasty (p = 0.194). Median operative time was 298 minutes (range 145 to 387) for standard and 209 minutes (106 to 540) for robot-assisted laparoscopic pyeloplasty (p = 0.008). Mean hospitalization was similar between the groups (1 day for standard vs 2 days for robot-assisted laparoscopic pyeloplasty, p = 0.246). Narcotic use was similar between the groups. Median followup was 43 months for standard and 22 months for robot-assisted laparoscopic pyeloplasty (p <0.01). Renal ultrasound showed postoperative improvement of hydronephrosis in 85% and stable disease in 15% of patients following robot-assisted laparoscopic pyeloplasty, and improvement in 89.5% and stable disease in 10.5% after standard laparoscopic pyeloplasty. Symptoms resolved in 100% of patients (38 of 38) after robot-assisted laparoscopic pyeloplasty and 87.5% of patients (7 of 8) after standard laparoscopic pyeloplasty. CONCLUSIONS: Robot-assisted laparoscopic pyeloplasty and standard laparoscopic pyeloplasty are effective techniques to correct ureteropelvic junction obstruction, with similar outcomes. Robot-assisted laparoscopic pyeloplasty had a shorter operative time, and its success and complication rates are comparable to standard laparoscopic pyeloplasty.

Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns.

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung's disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.

The impact of obesity on surgical outcome after pancreaticoduodenectomy.

CONTEXT: The effect of obesity on surgical outcome is becoming an increasingly relevant issue given the growing rate of obesity worldwide. OBJECTIVE: To investigate the specific impact of obesity on pancreaticoduodenectomy. DESIGN: A retrospective comparative study of a prospectively maintained database was carried out to investigate the specific impact of obesity on the technical aspects and postoperative outcome of pancreaticoduodenectomy. PATIENTS: Between 1999 and 2006, 92 consecutive patients underwent pancreaticoduodenectomy using a standardized technique. The study population was subdivided according to the presence or absence of obesity. RESULTS: Nineteen (20.7%) patients were obese and 73 (79.3%) patients were non-obese. The two groups were comparable in terms of demographics, American Society of Anesthesiology (ASA) score as well as nature and type of pancreatico-digestive anastomosis. The rate of clinically relevant pancreatic fistula (36.8% vs. 15.1%; P=0.050) and hospital stay (23.1+/-13.9 vs. 17.0+/-8.0 days; P=0.015) were significantly increased in obese vs. non-obese patients, respectively. Pancreatic fistula was responsible for one-half of the deaths (2/4) and two ruptured pseudoaneurysms. The incidence of the other procedure-related and general postoperative complications were not significantly different between the two groups. Intrapancreatic fat was increased in 10 obese patients (52.6%) and correlated positively both with BMI (P=0.001) and with the occurrence of pancreatic fistula (P=0.003). CONCLUSION: Obese patients are at increased risk for developing pancreatic fistula after pancreaticoduodenectomy. Special surgical caution as well as vigilant postoperative monitoring are therefore recommended in obese patients.

Three-week or one-week bladder catheterization for hypospadias repair? A retrospective-prospective observational study of 189 patients.

PURPOSE: While there is little scientific evidence over the optimal duration for transurethral bladder catheterization after hypospadias repair, most surgeons leave the catheter for 7-10 days. We herein describe our experience with bladder catheterization for three weeks after hypospadias repair, an approach not previously described in the literature. PATIENTS AND METHODS: We reviewed the charts of 189 patients who underwent hypospadias repair by a single pediatric urologist. The study population was divided as follows: group 1 consisted of children operated between March 2007 and September 2010 and whose catheters were left for one week (n=95); group 2 consisted of those operated between September 2010 and July 2013 and whose catheters were left for three weeks (n=94). The primary objective of the study was to compare complication rates between the two groups. Secondary outcomes were evaluation of the effect of age, surgical technique, curvature, and hypospadias degree as potential factors for postoperative complications. RESULTS: Median age at hypospadias repair was 18 months (range, 3-100 months) in group 1, and 16 months (range, 2-96 months) in group 2, P=.209. The complication rate was 22.1% (n=21) for group 1 and 7.4% (n=7) for group 2, P=.005. Complications observed in group 1 and 2 were meatal stenosis (n=4 and 2, respectively) and urethro-cutaneous fistulas (n=17 and 5, respectively). Coronal fistulas manifested more frequently in patients in group 1 compared to those in group 2 (13.7% vs. 3.2%, P=.01). Complications were observed in 20 patients out of 139 (11.5%) after Duplay, and in 8 patients out of 15 (53.3%) after Duckett (P<.001). In Duplay cases, complications were significantly associated with one-week bladder catheterization (OR: 5.00; 95% CI: 1.53-16.32; P=.008) and higher age group at operation (OR: 1.88; 95% CI 1.07-3.28; P=.026). In Duckett cases, number of surgeries, age, severity, curvature and catheter duration were not found to be associated with complications. CONCLUSION: In cases of Duplay, a three-week instead of one-week catheterization and age below 6 months at hypospadias repair are associated with a better outcome and fewer complications.

Growing teratoma syndrome: first case report in a 4-year-old girl.

Growing teratoma syndrome (GTS) consists of germ cell tumors that grow following chemotherapy despite complete eradication of the malignant cells. They can metastasize to any site, particularly the retroperitoneum, mediastinum and cervical region. It typically affects young adults and adolescents. Here we describe the youngest case reported in a 4-year-old girl with an ovarian mixed germ cell tumor who underwent an oophorectomy. Her tumor markers normalized by the end of her chemotherapeutic treatment; however, she developed a retroperitoneal mass that was subsequently resected. Histopathology revealed a mature teratoma, consisting of a GTS. We stress the need for early recognition and treatment of GTS to avoid the subsequent morbidity and mortality associated with it. Although GTS has an excellent prognosis when completely resected, it is essential that the patient be regularly followed-up with serum tumor markers and imaging.

Endoscopic treatment with dextranomer/hyaluronic acid for persistent incontinence after continent urinary reconstruction.

PURPOSE: To determine the applicability and long-term outcome of endoscopic injection of dextranomer/hyaluronic acid (Dx/HA) to correct incontinence in patients who had previously undergone continent urinary reconstruction. PATIENTS AND METHODS: A retrospective cohort study was performed of all patients who underwent Dx/HA injection at our institution from January 2001 to June 2011. All patients had adequate bladder capacity and compliance on maximized medical therapy before injection. "Success" was defined as either "continence" (daytime dry interval >3 hours) or "improvement" (daytime dry interval >2 hours). RESULTS: A total of 22 children (16 females and 6 males) had Dx/HA injections for persistent incontinence from either bladder neck (7), Mitrofanoff (10), or both (5). Median age at injection was 13 years (range 4-21). Children underwent an average of 1.6 injection sessions per patient with an average of 2.6 mL of Dx/HA per session. At a median follow-up of 72 months (range 4-104), 19 (86.4%) patients had successful results (16 continent, 3 improved). For those incontinent from bladder neck, 42% became continent after 1, 75% after 2, and 83% after 3 injections, with a success rate of 91% (10 continent, 1 improved). For those incontinent from Mitrofanoff, 20% became continent after 1, and 73% after 2 injections, with a success rate of 86% (11 continent, 2 improved). CONCLUSIONS: Endoscopic injection of Dx/HA to correct residual incontinence in selected children after urinary reconstruction appears to be safe and effective, achieving a dry interval in more than two-thirds of patients.

Pediatric varicocelectomy: a comparative study of conventional laparoscopic and laparoendoscopic single-site approaches.

BACKGROUND AND PURPOSE: Laparoscopy is a common approach to manage varicoceles in both the adult and pediatric population. The purpose of this study is to report our experience and compare outcomes between conventional laparoscopy and laparoendoscopic single-site (LESS) surgery for varicocelectomy in the pediatric population. PATIENTS AND METHODS: A retrospective cohort study was performed of all patients who underwent conventional laparoscopic varicocelectomy (LV) and laparoendoscopic single-site varicocelectomy (LESSV) at a single pediatric institution from December 2007 to March 2012. Patient demographics, intraoperative details, narcotic use, and complications were reviewed. RESULTS: LV was performed in 32 patients and LESSV in 11 patients. None had conversion to open surgery. Median age was 16 years for LV (range 12-23) and 15 years for LESSV (range 12-20), P=0.061. Median operative time was 55 minutes for LV (range 28-90) and 46 minutes for LESSV (range 33-59), P=0.037. Nine (81.8%) patients in the LESSV group and 10 (31.2%) patients in the LV group were administered narcotics in the recovery room, P=0.005. One (3.1%) patient in the LV group was administered ketorolac in the recovery room, P=1. Five patients in each group, LESSV (45.5%) and LV (15.6%), received acetaminophen in the recovery room, P=0.092. All procedures were performed on an outpatient basis except for one because of a concomitant procedure. Median follow-up was 22 months in LV and 15 months in LESSV, P=0.015. One (3.1%) postoperative hydrocele was noted after LV and 1 (9.1%) after LESSV, P=0.451. All varicoceles were clinically resolved in both groups. CONCLUSIONS: LESSV is comparable to LV in the pediatric population. Our initial experience indicates that the LESS approach may be more painful in the immediate postoperative period than conventional laparoscopy. The LESS technique warrants further evaluation to determine if one approach is clearly more advantageous.

Comparison of pediatric robotic-assisted laparoscopic nephroureterectomy and laparoendoscopic single-site nephroureterectomy.

OBJECTIVE: To compare outcomes between pediatric robotic-assisted laparoscopic nephroureterectomy (RALNU) and laparoendoscopic single-site nephroureterectomy (LESSNU). METHODS: A retrospective cohort study was performed of all patients who underwent RALNU and LESSNU at a single pediatric institution from April 2009 to April 2013. Patient demographics, perioperative details, and outcomes were reviewed. RESULTS: Thirty-two patients (20 men, 12 women) were identified. Twenty-four patients underwent RALNU and 8 patients underwent LESSNU. There was no significant blood loss, intraoperative complication, or conversion to open or standard laparoscopy for either procedure. Median age was 55.1 months (range 4.5-171.8 months) for RALNU and 51.6 months (range 16.3-144.9 months) for LESSNU (P = .695). Median weight was 19.1 kg (range 7-55 kg) for RALNU and 16.9 kg (range 11-41 kg) for LESSNU (P = .727). Median operative time was 227 minutes (range 112-362 minutes) for RALNU and 174 minutes (range 74-288 minutes) for LESSNU (P = .028). Median length of hospital stay was 2 days (range 1-4 days) for RALNU and 1 day (range 0-6 days) for LESSNU (P = .134). Median in-patient postoperative narcotic use of morphine-equivalent was 0.03 mg/kg/day (range 0-0.2) for RALNU and <0.01 mg/kg/day (range 0-0.2) for LESSNU (P = .134). Median in-patient postoperative Ketorolac use was <0.01 mg/kg/day (range 0-0.8) for RALNU and <0.01 mg/kg/day (range 0-0.5) for LESSNU (P = .784). Median follow-up was 22 months (range 0.8-48.4 months) for RALNU and 18.8 months (range 0.3-29.4 months) for LESSNU (P = .361). We observed 2 complications (8.3%) in RALNU and 1 in LESSNU (P = 1). CONCLUSION: LESSNU has a significantly shorter operative time with comparable in-patient postoperative narcotics use as compared to RALNU.

Factors associated with relapse and survival in Wilms tumor: a multivariate analysis.

PURPOSE: Lymph node metastasis and anaplasia predict relapse-free survival in Wilms tumor. We performed a multivariate analysis of our institutional database to identify factors independently associated with relapse-free and overall survival. METHODS: We retrospectively reviewed cases of confirmed Wilms tumor diagnosed between 1990 and 2010 and treated at our institution. The log-rank test was used to screen variables for consideration in the proportional hazards model. RESULTS: A total of 95 patients were treated at our institution during the study period, with a median follow-up of 3.3 years. Factors correlated with overall survival in the univariate analysis were local disease, metastasis, tumor size, anaplasia, renal vein tumor thrombus, inferior vena cava tumor thrombus, lymph node positivity, and tumor rupture. On multivariate analysis, factors associated with increased risk of death were lymph node positivity and anaplasia. Factors correlated with probability of relapse in the univariate analysis were lymph node positivity, anaplasia, and female sex. All 3 of these factors were also independently significant on multivariate analysis. CONCLUSION: Lymph node involvement and anaplasia are significantly correlated with probability of relapse and overall survival, reemphasizing the strong recommendation to sample regional lymph nodes during Wilms tumor resection.

Ectopic gastric mucosa in the cervical esophagus presenting as a recurrent neck abscess: a case report.

We report a unique case of ectopic gastric mucosa (EGM) in the cervical esophagus. The patient presented with a recurrent cervical abscess communicating through a fistula with the EGM. Surgical treatment consisted of complete excision. The postoperative course was complicated by a breach in the hypopharynx, which was treated conservatively, and a stenosis of the esophagus requiring balloon dilation.

Is the Fowler-Stephens procedure still indicated for the treatment of nonpalpable intraabdominal testis?

PURPOSE: There are no formal guidelines for the management of boys with nonpalpable testis. In this article, we report our experience in treating all nonpalpable intraabdominal testis (NPIT) with standard inguinal orchidopexy without dividing the spermatic vessels stating that the Fowler-Stephens technique is no longer indicated for the treatment of the intraabdominal testis. METHODS: Between June 2003 and April 2008, we treated 23 boys with NPIT confirmed by ultrasound or laparoscopy. All cases were treated through an inguinal orchidopexy without division of the spermatic vessels by fixing the testis to the scrotum by 2 absorbable stitches even if there was an upward retraction of the scrotal skin. Location and size of testis were reported at 1 week, 1 month, 3 months, and 6 months through physical examination. RESULTS: Average age at presentation was 24 months. Ten patients (43%) had the NPIT on the right side, 8 (35%) on the left side, and 5 (22%) were bilateral. We had no intraoperative complications. All patients were discharged on the same day. In all cases, the testis was normal in size and found in the scrotum after 6 months of follow-up. CONCLUSION: On the basis of our experience, we believe that the Fowler-Stephens procedure is not indicated anymore in the management of NPIT. Orchidopexy without division of the spermatic vessels should be the treatment of choice even for the cases of very high intraabdominal testis because it does not affect normal testicular vascularization and is minimally invasive.

Prenatal diagnosis of an intrathoracic gastric duplication: a case report.

We report an intrathoracic gastric duplication cyst detected antenatally on obstetrical ultrasonography at 33 weeks of gestation. After birth, the newborn was directly intubated. At day 19, the intrathoracic cyst was removed, and the patient discharged on his 27th day of life. Histologic findings confirmed the diagnosis of a gastric duplication cyst. This appears to be the first reported case of an intrathoracic gastric duplication detected antenatally. Intrathoracic gastric duplication should be among the differential diagnosis of any mediastinal cyst discovered antenatally; its detection allows appropriate management at birth.

An unusual case of bilateral primitive obstructed megaureters due to ureteral valves presenting with anuria at the age of 1 month.

Ureteric valves represent a very rare etiology of ureteral obstruction. We experienced an unusual case of bilateral distal ureteric valves that presented as bilateral primitive obstructed megaureters with anuria at the age of 40 days. To our knowledge, this is the second case of bilateral involvement of distal ureteric valves reported in the literature. Bilateral ureteral valves should be included in the differential diagnosis of bladder outlet obstruction, as well as bilateral primitive obstructed megaureters in children. Excision and ureteral reimplantation is curative.

Topical application of mitomycin C in the treatment of esophageal and tracheobronchial stricture: a report of 2 cases.

We present 2 cases of successful treatment of recurrent anastomotic strictures using a topical application of mitomycin C. In the first case, a 4-year-old boy had a cervical cyst excised, which appeared to be an ectopic gastric mucosa. He consequently presented severe stenosis at the origin of the cervical esophagus that needed repeated balloon dilatations. The second case is about a 12-year-old girl who presented a traumatic complete rupture of the right mainstem bronchus managed by primary repair, with subsequent anastomotic stricture. Both patients were successfully managed with topical application of mitomycin C (1 mg/mL), and needed no more dilatations.

Upper airway obstructive symptoms because of ectopic gastric mucosa in a newborn: a case report.

An 8-month-old female infant was referred after unsuccessful attempts of extubation owing to upper airway obstruction by a pharyngeal soft tissue mass. Laryngoscopic examination revealed an obstructing mucosal fold originating from the posterior pharyngeal wall. The lesion was totally excised with remarkable improvement of the infant's deglutition and respiration. Histologic examination revealed an ectopic gastric mucosa. To our knowledge, this is the first report of ectopic gastric mucosa causing upper airway obstruction in a newborn.