Abdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness.

INTRODUCTION: Abdominoplasty is an important component of the management of children with prune belly syndrome (PBS). While there are features of the abdominal defect in PBS which are common to all patients, there will be differences unique to each patient that should be taken into consideration in surgical planning. Specifically, we have come to realize that although the Monfort procedure assumes a symmetric pattern of abdominal wall laxity, this symmetry is rarely present. OBJECTIVE: The aim of this report is to describe our modifications and review our outcomes for the Monfort procedure which more completely address correction of the abdominal wall laxity including both common and uncommon features while positioning the umbilicus to a more anatomically correct position (Figure). STUDY DESIGN: Sixteen male patients with PBS and one female pseudoprune belly syndrome patient, aged 2-9 years, were treated at our institution between 2003 and 2014. Modifications incorporated into the abdominoplasty procedure for PBS applied to this study group included: 1) use of diagnostic laparoscopy to define the topography of the abdominal wall defect, 2) initial midline rather than elliptical skin incision to defer retailoring of the skin coverage until the final step of the procedure, 3) varying the width of the central plate to correct side to side asymmetry in redundancy, 4) plication of the central plate to reduce vertical redundancy and reposition the umbilicus, and 5) plication of focal areas of fascial weakness, most often in the flank region. RESULTS: All patients have improved abdominal wall contour with a more uniform correction of areas of weakness at a mean follow-up of 5.5 years (range 18 months-11.5 years). All patients and parents indicate that they are very satisfied with the outcome of their procedures without any revisions being performed. This study is descriptive in nature and retrospective, with the patient population treated in a relatively uniform fashion that does not allow direct comparison with other techniques. CONCLUSIONS: The modified Monfort procedure recognizes the pattern of abdominal muscular deficiency unique to each patient and incorporates this information into the surgical design.

High GMS score hypospadias: Outcomes after one- and two-stage operations.

INTRODUCTION: Established criteria to assist surgeons in deciding between a one- or two-stage operation for severe hypospadias are lacking. While anatomical features may preclude some surgical options, the decision to approach severe hypospadias in a one- or two-stage fashion is generally based on individual surgeon preference. This decision has been described as a dilemma as outcomes range widely and there is lack of evidence supporting the superiority of one approach over the other. OBJECTIVES: The aim of this study is to determine whether the GMS hypospadias score may provide some guidance in choosing the surgical approach used for correction of severe hypospadias. STUDY DESIGN: GMS scores were preoperatively assigned to patients having primary surgery for hypospadias. Those patients having surgery for the most severe hypospadias were selected and formed the study cohort. The records of these patients were reviewed and pertinent data collected. Complications requiring further surgery were assessed and correlated with the GMS score and the surgical technique used for repair (one-stage vs. two-stage). RESULTS: Eighty-seven boys were identified with a GMS score (range 3-12) of 10 or higher. At a mean follow-up of 22 months the overall complication rate for the cohort after final planned surgery was 39%. For intended one-stage procedures (n = 48) an acceptable result was achieved with one surgery for 28 patients (58%), with two surgeries for 14 (29%), and with three to five surgeries for six (13%). For intended two-stage procedures (n = 39) an acceptable result was achieved with two surgeries for 26 patients (67%), three surgeries for eight (21%), and four surgeries for three (8%). Two other patients having two-stage surgery required seven surgeries to achieve an acceptable result. Complication rates are summarized in the Table. The complication rates for GMS 10 patients were similar (27% and 33%, p = 0.28) for one- and two-stage repairs, respectively. GMS 11 patients having a one-stage repair had a significantly higher complication rate (69%) than those having a two-stage repair (29%) (p = 0.04). GMS 12 patients had the highest complication rate with a one-stage repair (80%) compared with a complication rate of 37% when a two-stage repair was used (p = 0.12). CONCLUSIONS: Guidelines to help standardize the surgical approach to severe hypospadias are needed. Staged surgery for GMS 11 and 12 patients may result in a lower complication rate but may not reduce the number of surgeries required for an acceptable result. Although further study is needed, the GMS score may be helpful for establishing such criteria.

Emergency Hospital Admissions for Initial Febrile Urinary Tract Infection: Do Patient Demographics Matter?

BACKGROUND: In 2011, the American Academy of Pediatrics revised practice parameters regarding febrile urinary tract infection (fUTI) in children aged 2-24 months. The Section on Urology opposed the omission of voiding cystourethrogram (VCUG), and expressed concern that potential untoward consequences of deferring VCUG may be most felt by children on Medicaid. OBJECTIVE: We ascertained imaging and characteristics of children presenting to the Emergency Department (ED) with initial fUTI to determine the impact of patient demographics on admissions for pyelonephritis. METHODS: Children aged 2-24 months presenting to the ED with initial fUTI were identified. Demographics, insurance status, laboratory studies, renal-bladder ultrasound (RBUS), VCUG, and hospital admission status were evaluated. RESULTS: Three-hundred fifty patients met inclusion criteria; 88 (25.1%) were admitted. Admitted patients were significantly (p < 0.001) younger (mean 0.31 ± 0.33 years) than those managed as outpatients (mean 0.91 ± 0.7 years). On univariate analysis, male gender (p < 0.001), Medicaid insurance (p < 0.05), and non-Hispanic race (p < 0.05) were associated with admission. Race retained significance on multivariate analysis; Caucasian children were 2.35 times (95% confidence interval [CI] 0.79-7.23) and African-American children 3.8 times more likely to be admitted than Hispanic patients (95% CI 1.88-7.63). Children with abnormal RBUS were 12.8 times more likely to require admission (95% CI 4.44-37.0). Medicaid was also independently predictive of admission; such patients were 2.6 times more likely to be admitted than those with private insurance (95% CI 1.15-5.88). CONCLUSIONS: Abnormal ultrasound, non-Hispanic race, and public insurance were strongly associated with hospital admission in children presenting to the ED with initial febrile urinary tract infection.

Further analysis of the Glans-Urethral Meatus-Shaft (GMS) hypospadias score: correlation with postoperative complications.

INTRODUCTION AND OBJECTIVE: The Glans-Urethral Meatus-Shaft (GMS) score is a concise and reproducible way to describe hypospadias severity. We classified boys undergoing primary hypospadias repair to determine the correlation between GMS score and postoperative complications. STUDY DESIGN: Between February 2011 and August 2013, patients undergoing primary hypospadias repair were prospectively scored using the GMS classification. GMS scoring included a 1-4 scale for each component: G - glans size/urethral plate quality, M - meatal location, and S - degree of shaft curvature, with more unfavorable characteristics assigned higher scores [Figure]. Demographics, repair type, and complications (urethrocutaneous fistula, meatal stenosis, glans dehiscence, phimosis, recurrent chordee and stricture) were assessed. Total and individual component scores were tested in uni- and multivariate analysis. RESULTS: Two-hundred and sixty-two boys (mean age 12.3 ± 13.7 months) undergoing primary hypospadias repair had a GMS score assigned. Mean GMS score was 7 ± 2.5 (G 2.1 ± 0.9, M 2.4 ± 1, S 2.4 ± 1). Mean clinical follow-up was 17.7 ± 9.3 months. Thirty-seven children (14.1%) had 45 complications. A significant relationship between the total GMS score and presence of any complication (p < 0.001) was observed; for every unit increase in GMS score the odds of any postoperative complication increased 1.44 times (95% CI, 1.24-1.68). Urethrocutaneuous fistula was the most common complication, occurring in 21 of 239 (8.8%) of single-stage repairs. Patients with mild hypospadias (GMS 3-6) had a 2.4% fistula rate vs. 11.1% for moderate (GMS 7-9) and 22.6% for severe (GMS 10-12) hypospadias (p < 0.001). Degree of chordee was an independent predictor of fistula on multivariate analysis; S4 (>60° ventral curvature) patients were 27 times more likely to develop a fistula than S1 (no curvature) boys (95% CI, 3.2-229). DISCUSSION: The GMS score is based on anatomic features (i.e. glans size/urethral plate quality, location of meatus, and degree of chordee) felt to most likely impact functional and cosmetic outcomes following hypospadias repair. We demonstrated a statistically significant increase in the likelihood of any postoperative complication with every unit increase in total GMS score. The concept that factors aside from meatal location affect hypospadias repair and outcomes is not novel, and degree of ventral curvature and urethral plate quality are often cited as important factors. In our series, boys with greater than 60° of ventral curvature undergoing a single-stage repair were 27 times more likely to develop a fistula than those without chordee on multivariate analysis, making severe curvature an independent predictor of urethrocutaneous fistula formation. That meatal location did not retain significance on multivariate analysis highlights the importance of considering the entire hypospadias complex when determining severity, rather than just evaluating the position of the meatus. Our study has several limitations that warrant consideration. While GMS scores were assigned prospectively, the data was collected retrospectively, subjecting it to flaws inherent with such study design. Furthermore, type of repair is influenced by surgeon preference and subjective assessment of hypospadias characteristics not incorporated in our scoring system (i.e. tissue quality, urethral hypoplasia, penoscrotal transposition). Despite these limitations, our study demonstrates a strong correlation between the GMS classification and surgical complications, furthering supporting its potential as a tool to standardize hypospadias severity and gauge postoperative complications. CONCLUSION: The Glans-Urethral Meatus-Shaft (GMS) classification provides a means by which hypospadias severity and reporting can be standardized, which may improve inter-study comparison of reconstructive outcomes. There is a strong correlation between complication risk and total GMS score. Degree of chordee (S score) is independently predictive of fistula rate.

Early effect of American Academy of Pediatrics Urinary Tract Infection Guidelines on radiographic imaging and diagnosis of vesicoureteral reflux in the emergency room setting.

PURPOSE: In 2011 the AAP revised practice parameters on febrile urinary tract infection in infants and children 2 to 24 months old. New imaging recommendations invigorated the ongoing debate regarding the diagnosis and management of vesicoureteral reflux. We compared evaluations in these patients with febrile urinary tract infection before and after guideline publication. MATERIALS AND METHODS: During 2 separate 6-month periods 350 patients 2 to 24 months old were evaluated in the emergency room setting. Demographics, urine culture, renal-bladder ultrasound, voiding cystourethrogram and admission status were assessed. RESULTS: A total of 172 patients presented with initial febrile urinary tract infection in 2011, of whom 47 (27.3%) required hospitalization, while 42 of 178 (23.6%) were admitted in 2012. Admission by year did not significantly differ (p = 0.423). After guideline revision 41.2% fewer voiding cystourethrograms were done (72.1% of cases in 2011 vs 30.9% in 2012, p <0.001). A 17.2% decrease in renal-bladder ultrasound was noted (75.6% in 2011 vs 58.4% in 2012, p <0.001). Of 55 voiding cystourethrograms 21 (38.2%) were positive in 2012 compared to 36.3% in 2011 (p = 0.809). Mean ± SD maximum vesicoureteral reflux grade was unchanged in 2011 and 2012 (2.9 ± 1.2 and 2.5 ± 0.93, respectively, p = 0.109). There was no association between abnormal renal-bladder ultrasound and voiding cystourethrogram positivity (p = 0.116). CONCLUSIONS: AAP guidelines impacted the treatment of infants and young children with febrile urinary tract infection. We found no relationship between renal-bladder ultrasound and abnormal voiding cystourethrogram, consistent with previous findings that call ultrasound into question as the determinant for additional imaging. Whether forgoing routine voiding cystourethrogram results in increased morbidity is the subject of ongoing study.

Emergency room visits and readmissions after pediatric urologic surgery.

OBJECTIVE: Reducing readmissions has become a focal point to increase quality of care while reducing costs. We report all-cause unplanned return visits following urologic surgery in children at our institution. MATERIALS AND METHODS: Children undergoing urology procedures with returns within 30 days of surgery were identified. Patient demographics, insurance status, type of surgery, and reason for return were assessed. RESULTS: Four thousand and ninety-seven pediatric urology surgeries were performed at our institution during 2012, with 106 documented unplanned returns (2.59%). Mean time from discharge to return was 5.9 ± 4.9 days (range, 0.3-24.8 days). Returns were classified by chief complaint, including pain (32), infection (30), volume status (14), bleeding (11), catheter concern (8), and other (11). Circumcision, hypospadias repair, and inguinal/scrotal procedures led to the majority of return visits, accounting for 21.7%, 20.7%, and 18.9% of returns, respectively. Twenty-two returns (20.75%) resulted in hospital readmission and five (4.72%) required a secondary procedure. Overall readmission rate was 0.54%, with a reoperation rate of 0.12%. CONCLUSIONS: The rate of unplanned postoperative returns in the pediatric population undergoing urologic surgery is low, further strengthening the argument that readmission rates in children are not necessarily a productive focal point for financial savings or quality control.

The GMS hypospadias score: assessment of inter-observer reliability and correlation with post-operative complications.

OBJECTIVE: An agreed upon method for describing the severity of hypospadias has not been established. Herein we assess the inter-observer reliability of the GMS hypospadias score and correlate it with the risk of a post-operative complication. METHODS: A 3-component method for grading the severity of hypospadias was developed (GMS). Eighty-five consecutive patients presenting for hypospadias repair were graded independently by at least 2 surgeons using the GMS criteria. Scores were compared statistically to determine agreement between the observers. The outcomes of these patients were then reviewed to determine how the GMS score correlates to the risk of a surgical complication. RESULTS: The G, M, and S scores had excellent agreement between observers. The GMS total score was exactly the same or differed by one point in 79/85 (93%) of patients. The complication rate was 5.6% for patients with a GMS score of 6 or less, but was 25.0% for patients with a GMS score greater than 6. CONCLUSIONS: The GMS score provides a concise method for describing the severity of hypospadias and appears to have high inter-observer reliability. The GMS score also appears to correlate with the risk of a surgical complication.

Pediatric chronic orchalgia.

INTRODUCTION: Chronic orchalgia, defined as testicular pain lasting > 3 months and interfering with normal activities, is neglected in the pediatric literature. We describe our experience with the evaluation and treatment of pediatric chronic orchalgia patients. MATERIALS AND METHODS: Charts were screened to identify patients meeting the criteria for chronic orchalgia. Charts were further reviewed to record the history and physical exam, diagnostic tests, treatment and outcomes. RESULTS: 65/982 patients met the criteria for chronic orchalgia. Mean age was 13 and mean duration of pain was 8.6 months. Physical exam findings were normal in 46 patients (70%). 59 patients were managed conservatively with resolution (10/59, 17%) or a single visit (36/59, 61%) in 78%. 13/59 (22%) patients showed either minor improvement or no change in symptoms. 5 non-responding patients were managed by the anesthesia pain service; 4 received epidurals with or without additional oral pain medications with 3 experiencing significant pain improvement. CONCLUSION: Conservative management of chronic orchalgia allowed symptoms to subside in the majority of cases. We recommend patients be treated with conservative measures for 1-2 months. If this fails, early involvement of the anesthesia pain service can offer treatment modalities such as epidural analgesia. Surgical management in the face of a normal physical exam does not seem to have a role.

Nephrogenic adenoma of the bladder in a prune belly syndrome patient: case report and review of the literature.

Nephrogenic adenoma (NA) is a rare lesion of the urinary tract widely considered to be a metaplastic response to urothelial injury. Herein, we present the case of an 8-year-old male with prune belly syndrome who presented with gross hematuria. Investigation revealed a bladder mass; however, upon cystoscopic examination, multiple polypoid lesions were identified. Microscopic examination revealed NA of the bladder. To our knowledge, this is the second reported case of NA of the bladder in association with prune belly syndrome.

Urological manifestations of Down syndrome.

PURPOSE: We identified the urological anomalies seen in patients with Down syndrome. MATERIALS AND METHODS: We reviewed the literature on Down syndrome and its associated genitourinary pathology. RESULTS: Associated defects have been reported throughout the genitourinary tract. The most common renal anomalies seen are renal hypoplasia, obstructive uropathy and glomerular microcysts. Decreased creatinine clearance has also been noted. Developmental anomalies, such as megaureter and vesicoureteral reflux, have been reported. Dysfunctional voiding and urinary retention have been found in these patients. Posterior urethral valves and hypospadias have also been seen. The testicular abnormalities most frequently reported are cryptorchidism, testicular cancer and infertility. CONCLUSIONS: Improved understanding and identification of the various urological anomalies associated with Down syndrome will aid in the care of these patients. In addition, an awareness of perioperative issues will further facilitate a smooth clinical course.

Efficacy of nephrectomy for the treatment of nephrogenic hypertension in a pediatric population.

PURPOSE: We evaluated the efficacy of nephrectomy for the management of nephrogenic hypertension in children. MATERIALS AND METHODS: We retrospectively reviewed the records of 320 children who underwent nephrectomy between 1991 and 2001, and 22 underwent nephrectomy for the management of hypertension. Of the 22 patients 16 (73%) had long-term followup, including 8 with end stage renal disease who were dialysis dependent, and 8 with normal renal function and unilateral parenchymal renal disease (UPRD). Within the UPRD group 3 patients had renovascular hypertension, 3 had reflux nephropathy, 1 had renal artery thrombosis and 1 had Page kidney. Age at hypertension onset, age at nephrectomy and elapsed time between diagnosis and intervention were studied. Antihypertensive medication requirements before surgery and after postoperative followup were evaluated to assess treatment efficacy. Complete success was defined as blood pressure normalization without antihypertensive requirements. Partial success was defined as decrease in medication requirements and/or discontinuation of minoxidil. Failure of treatment was defined as persistent hypertension, increased medication requirements or minoxidil dependence. RESULTS: In the end stage renal disease group mean age at diagnosis was 5.9 years (range 15 months to 10 years) and bilateral nephrectomy was performed at a mean age of 8.9 years (19 months to 15 years) with average elapsed time between diagnosis of hypertension and nephrectomy of 3 years. After a mean followup of 4.4 years (range 6 months to 8 years) 7 patients (88%) experienced complete or partial success and nephrectomy management failed in 1. In the UPRD group average elapsed time was 2.2 years (range 1 month to 10 years) between a mean age at diagnosis of 6.7 years (birth to 16 years) and a mean age at nephrectomy of 8.9 years (1 month to 17 years). After a mean followup of 1.6 years (range 1 month to 5 years) complete or partial success was experienced by all 8 patients (100%). All 8 UPRD group patients experienced adequate residual renal function. CONCLUSIONS: The vast majority of patients in both categories experienced complete or partial success from nephrectomy for the management of medication refractory hypertension. Nephrectomy for hypertension control is safe and effective, and obviates the need for morbid medications. We continue to accrue patients in a prospective manner.

Direct vision internal urethrotomy for the treatment of pediatric urethral strictures: analysis of 50 patients.

PURPOSE: In an attempt to evaluate our experience with the treatment of pediatric urethral stricture disease we performed a retrospective review of patients undergoing direct vision internal urethrotomy (DVIU). MATERIALS AND METHODS: The computerized surgical logs at 2 pediatric hospitals were reviewed to identify patients who underwent DVIU between 1992 and 2001. Hospital and clinical charts were then reviewed. Many variables were analyzed, including patient age, etiology of stricture, technique and clinical outcomes. Minimum followup to be included in clinical outcome analysis was 12 months. RESULTS: A total of 50 patients were identified (mean age 7.7 years, range 6 months to 17 years). The most common etiology for stricture formation was previous hypospadias repair (20 patients [40%]). Forty patients met the 12-month minimum followup requirement for clinical outcome analysis. Of these patients 20 (50%) had no symptoms to suggest recurrent stricture at a median of 2.0 years (mean 2.7 years, range 12 months to 7 years). Seventeen patients (42.5%) had symptoms of recurrent stricture at a median of 8 months (mean 13 months, range 2 months to 5 years). Technical factors did not influence the ultimate success or failure of the procedure. CONCLUSIONS: DVIU provides a therapeutic option that successfully treats approximately half of the patients with a reasonably low complication rate. Complications following DVIU should not preclude its use as a therapeutic modality for the treatment urethral strictures in children. If the child fails the initial DVIU, repeat attempts at endoscopic correction of urethral stricture should be abandoned in favor of definitive urethroplasty.

Role of surgery in children with rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma of childhood. Historically, surgery has played a central role in the management of children with this tumor, though with surgery alone survival rates were poor. With current multimodal (surgery, radiotherapy and chemotherapy) treatment of these patients, survival has dramatically improved and, with this improvement, there has been an evolution of the role of surgery in the management of this condition. MATERIAL AND METHOD: The contemporary published literature (English) regarding surgical aspects of pediatric rhabdomyosarcoma was reviewed and evaluated. RESULTS: Multimodal therapy has improved the survival of children with RMS from 25% in 1970 to greater than 70% today. Surgical procedures for childhood RMS today are less apt to be exenterative or mutilating than those employed thirty years ago. CONCLUSIONS: Surgery plays a vital role in the diagnosis and treatment of children with RMS. This role has evolved in the context of multimodal therapy and improved survival to an emphasis on less radical procedures with decreased morbidity.

MR imaging of kidneys: functional evaluation using F-15 perfusion imaging.

BACKGROUND: Children with hydronephrosis are typically investigated by a combination of diuretic renal scintigraphy, ultrasound, and voiding cystourethrography. Unfortunately, there is no gold standard to assess obstruction. PURPOSE: The purpose of our study was to evaluate the utility of dynamic contrast enhanced MR urography in the investigation of children with hydronephrosis to define urinary tract anatomy, to calculate differential renal function and to assess urinary tract obstruction. MATERIALS AND METHODS: Dynamic contrast-enhanced MR imaging was performed in 40 children with unilateral hydronephrosis. There were 14 girls and 26 boys with an age range of 1 month to 14 years (mean 1.4 years). The information from traditional imaging modalities was compared to the information obtained from the single MR study. RESULTS: The anatomic imaging with MR urography was superior to other modalities. The split renal function was estimated with MR urography by calculating the volume of enhancing renal parenchyma and was comparable to renal scintigraphy ( r=0.98). By using surgery versus non-surgery as the decision point, with MR urography the sensitivity was 100%, specificity 71%, positive predictive value 86%, negative predictive value 100%, and diagnostic efficiency 90%. For renal scintigraphy the sensitivity was 96%, the specificity 56%, positive predictive value 76%, negative predictive value 90%, and diagnostic efficiency 79%. CONCLUSIONS: Dynamic contrast-enhanced MR urography provides superior anatomic and functional information when compared with ultrasound and diuretic renal scintigraphy. The information is gathered in a single study that does not use ionizing radiation. It is likely that MR urography will replace renal scintigraphy in the evaluation of hydronephrosis in children.

Blunt traumatic hematuria in children. Is a simplified algorithm justified?

PURPOSE: We determined whether radiographic evaluation is indicated in all children with traumatic hematuria. MATERIALS AND METHODS: We retrospectively reviewed the records of 110 children from 1992 to 1999 diagnosed with blunt trauma and hematuria. It is routine practice at our emergency department to perform radiographic evaluation in all children with hematuria regardless of the degree. Each chart was evaluated for the mechanism of injury, degree of hematuria, hypotension, imaging studies, renal injury, renal anomalies, associated injuries and outcome. RESULTS: A total of 110 patients 1 to 18 years old (mean age 9) were identified. The most common mechanism of injury was motor vehicle accident in 37 children (34%), followed by a fall in 32 (29%). Grades I to V renal injury was present in 5, 6, 6, 6 and 1 cases, respectively (22%), while 1 (0.9%) involved ureteropelvic junction avulsion. No child had renal pedicle injury. In 9 patients renal anomalies were detected incidentally. Of the 110 patients 101 underwent radiographic evaluation, including computerized tomography in 97 (88%). The 24 patients (22%) with significant renal injury and all with incidentally diagnosed renal anomalies had 50 or greater red blood cells per high power field on urinalysis, while 1 with ureteropelvic junction avulsion presented without hematuria. Hypotension was present in only 3 patients (2.7%), who also had associated injuries, including 2 who presented with renal injury. All 3 with associated injuries. Associated injuries were identified in 11 of 25 patients (44%). The 9 patients (8%) who did not undergo radiographic imaging had negative results on repeat urinalysis with an excellent outcome. CONCLUSIONS: We recommend that radiological evaluation consisting of abdominal and pelvic computerized tomography should be performed only in patients with 50 or greater red blood cells on urinalysis, hypotension at presentation to the emergency room or based on the severity of mechanism of injury, for example high speed motor vehicle accident deceleration injuries. The patient who presented with ureteropelvic junction avulsion without hematuria would have undergone imaging considering the mechanism of injury and number of associated injuries.