Martin A. Koyle

CYSTIC DYSPLASIA OF THE RETE TESTIS: A BENIGN CONGENITAL LESION ASSOCIATED WITH IPSILATERAL UROLOGICAL ANOMALIES

PURPOSEnCystic dysplasia of the rete testis is a benign congenital lesion that can mimic testicular cancer. We report 6 cases, review the literature, discuss the embryological etiology and make management recommendations.nnnMATERIALS AND METHODSnThe records and pathology reports of 6 boys presenting with cystic dysplasia of the rete testis at 5 institutions were reviewed, as was the relevant literature.nnnRESULTSnOf the 6 cases 5 presented as scrotal masses in previously healthy boys and 1 as an abdominal mass in a newborn with multiple congenital anomalies. One patient had been followed from birth for a multicystic dysplastic kidney and 4 were found to have an ipsilateral absent kidney during evaluation. Development of the contralateral side was normal in most cases.nnnCONCLUSIONSnCystic dysplasia of the rete testis is an unusual, benign congenital lesion that can mimic testicular cancer in presentation. The presence of ipsilateral renal anomalies, particularly renal agenesis, can suggest cystic dysplasia of the rete testis in the differential diagnosis preoperatively. Even if cystic dysplasia of the rete testis is suspected, we recommend inguinal exploration and early control of the spermatic cord in the event that neoplasia is identified. If possible, the goal of preserving as much normal testicular parenchyma as possible is desirable. Long-term followup for possible recurrence is recommended, particularly after local excision.

Febrile urinary tract infection, vesicoureteral reflux, and renal scarring: current controversies in approach to evaluation

The ideal approach to the radiological evaluation of children with urinary tract infection (UTI) is in a state of confusion. The conventional bottom-up approach, with its focus on the detection of upper and lower urinary tract abnormalities, including vesicoureteral reflux, has been challenged by the top-down approach, which focuses on confirming the diagnosis of acute pyelonephritis before more invasive imaging is considered. Controversies abound regarding which approach may best assess the ultimate risk for reflux-related renal scarring. Evolving practices motivated by the emerging evidence, the desire to minimize unnecessary interventions, as well as improve compliance with recommended testing, have added to the current controversies. Recent guideline updates and ongoing clinical trials hopefully will help in addressing some of these concerns.

Congenital anterior urethrocutaneous fistula.

PURPOSEnCongenital anterior urethrocutaneous fistula is a rare anomaly that may present in an isolated fashion or in association with other penile abnormalities, such as chordee or hypospadias. There have been 18 cases of congenital anterior urethrocutaneous fistula reported in the literature. We present 14 additional cases of congenital anterior urethrocutaneous fistula.nnnMATERIALS AND METHODSnWe treated 14 patients with congenital anterior urethrocutaneous fistula, of whom 9 were uncircumcised at presentation. Two patients had evidence of chordee and 4 had distal hypospadias.nnnRESULTSnThe type of repair was determined by the anatomical variations of this anomaly. All cases were corrected electively by various techniques based on the degree of the defect, including primary closure via a Thiersch-Duplay urethroplasty, pedicle flap urethroplasty, hinged flap urethroplasty and interpositioned island pedicle tube or onlay urethroplasty.nnnCONCLUSIONSnTo our knowledge the embryological events that cause anterior urethrocutaneous fistula are unclear but they likely result from a defective urethral plate or an abnormality of the infolding of the urethral groove. Surgical technique must be individualized to fit the defect. While there has been considerable skepticism regarding the existence of congenital urethrocutaneous fistula, the fact that 9 of our 14 patients were uncircumcised confirms the congenital nature of this lesion.

A Comprehensive Review of Pediatric Urachal Anomalies and Predictive Analysis for Adult Urachal Adenocarcinoma

PURPOSEnWe examined the presentation, diagnosis and management of radiologically detected pediatric urachal anomalies and assessed the risk of malignant degeneration.nnnMATERIALS AND METHODSnOur radiology database (2000 to 2012) was queried for all children younger than 18 years who were diagnosed with a urachal anomaly radiographically, and the operative database was used to determine those who underwent excision. Data collected included demographics, presenting symptoms, imaging modality and indication for excision. These data were compared to the Ontario Cancer Registry to determine the risk of malignancy.nnnRESULTSnA total of 721 patients were radiographically diagnosed with a urachal anomaly (667 incidentally), yielding a prevalence of 1.03% of the general pediatric population. Diagnoses were urachal remnants (89% of cases), urachal cysts (9%) and patent urachus (1.5%). Ultrasonography was the most common imaging modality (92% of cases), followed by fluoroscopy/voiding cystourethrography (5%) and computerized tomography/magnetic resonance imaging (3%). A total of 61 patients (8.3%) underwent surgical excision. Indications for imaging and treatment were umbilical drainage (43% of patients), abdominal pain (28%), palpable mass (25%) and urinary tract infection (7%). Mean age at excision was 5.6 years and 64% of the patients were male. Based on provincial data, the number needed to be excised to prevent a single case of urachal adenocarcinoma was 5,721.nnnCONCLUSIONSnUrachal anomalies are more common than previously reported. Children with asymptomatic lesions do not appear to benefit from prophylactic excision, as the risk of malignancy later in life is remote and a large number of urachal anomalies would need to be removed to prevent a single case of urachal adenocarcinoma.

Failed Pyeloplasty in Children: Revisiting the Unknown

OBJECTIVEnTo perform a critical analysis of the management of the pediatric failed pyeloplasty in a large tertiary center. The ideal approach to this rare entity is not well established.nnnMETHODSnRetrospective record review of children undergoing pyeloplasty from 2000 to 2010. All cases that required any type of reintervention, excluding stent removal, were analyzed. Data collected included: demographics, indication for and modality of the initial surgery, presence of crossing vessels, mode of diagnosis of failure, and type(s) of reintervention with the correspondent success rate(s).nnnRESULTSnOverall, pyeloplasty failure rate was 27 per 455 patients (5.9%). Age, initial indication for pyeloplasty, and modality of surgery (open vs laparoscopic) yielded similar failure rates. Indications for reintervention were as follows: worsening asymptomatic hydronephrosis 16 of 27 (59%), pain 7 of 27 (26%), urosepsis 2 of 27 (7.5%), and others 2 of 27 (7.5%). Eight of 27 (30%) improved with 1, 14 of 27 (52%) had 2, and 5 of 27 (18%) required 3 reinterventions, respectively. Mean interval between the first operation and subsequent interventions was 19.3, 24.9, and 27 months for the first, second, and third reinterventions, respectively. Modalities of reintervention with respective success rates were as follows: double J stent insertion 16% (6%), endopyelotomy 18% (50%), redo pyeloplasty 12% (92%), and ureterocalicostomy 4% (100%). Only 1 patient (7%) was documented to have a missed crossing vessel. All patients were stable and doing well after a mean follow-up of 56 months after the first operation.nnnCONCLUSIONnAccording to this series, more invasive and definitive techniques, such as redo pyeloplasty and ureterocalicostomy, are more successful than minimally invasive ones to treat failed pyeloplasty and should probably be offered sooner rather than later.

Comparative Analysis of Detorsion Alone Versus Detorsion and Tunica Albuginea Decompression (Fasciotomy) with Tunica Vaginalis Flap Coverage in the Surgical Management of Prolonged Testicular Ischemia

PURPOSEnRecent data suggest that testicular torsion may include an element of the compartment syndrome that improves with decompression. In 2009 we instituted tunica albuginea incision with tunica vaginalis flap coverage as an alternative in cases in which the torsed testis continued to appear ischemic after detorsion.nnnMATERIALS AND METHODSnThe medical records of 65 boys who underwent scrotal exploration for testicular torsion between 2000 and 2010 were reviewed. There were 6 patients excluded from study due to lack of followup. Of the remaining 59 patients 31 (52.5%) showed improvement in testicular appearance after detorsion and underwent orchiopexy, whereas 28 (47.5%) did not show evidence of recovery after detorsion. Of these patients 11 underwent tunica albuginea incision with tunica vaginalis flap coverage and 17 underwent orchiectomy. Demographic data, duration of symptoms and rate of testicular salvage were analyzed.nnnRESULTSnMean patient age was 11.8 years (detorsion plus orchiopexy), 10.1 years (tunica albuginea incision plus tunica vaginalis flap coverage) and 10.1 years (detorsion plus orchiectomy). Average followup was greater than 6 months in all groups. Mean duration of torsion was 13.4 hours (detorsion plus orchiopexy), 31.2 hours (tunica albuginea incision plus tunica vaginalis flap coverage) and 67.5 hours (detorsion plus orchiectomy). Before tunica albuginea incision with tunica vaginalis flap coverage was offered, the rate of orchiectomy was 35.9% (14 of 39) vs 15% (3 of 20) after this technique was introduced (p <0.05). The rates of testicular salvage were 62.5% (detorsion plus orchiopexy), 54.6% (tunica albuginea incision plus tunica vaginalis flap coverage) and 0% (detorsion plus orchiectomy). Although the numbers are limited, it is likely that without tunica albuginea incision with tunica vaginalis flap coverage 6 of 11 testes would have been removed.nnnCONCLUSIONSnThis preliminary experience suggests that tunica albuginea incision with tunica vaginalis flap coverage is a promising option for the management of clinically marginal torsed testes, enhancing salvageability after prolonged ischemia. We recommend considering this maneuver before performing orchiectomy in selected cases of testicular torsion.

Antenatally diagnosed hydronephrosis: current postnatal management

The issue of antenatal hydronephrosis has become a routine component for the care of a pregnant woman despite limited evidence of a clinical benefit. The genitourinary tract represents the most commonly detected organ system with identified abnormalities, with antenatal hydronephrosis (ANH), being the most notable and common finding. ANH represents a spectrum, with most cases being a trivial and inconsequential finding on maternal fetal ultrasound. However, there is a correlation with increased grades of ANH being associated with increased severity of urinary tract pathology. Most patients can be managed expectantly with appropriate evaluation commenced postnatally based on severity of ANH and proper parental counseling and education. The purpose of this review was to assess current literature and guidelines pertaining to ANH and incorporate our practical interpretations of their significance.

Conservative management vs early surgery for high grade pediatric renal trauma - Do nephrectomy rates differ?

PURPOSEnGuidelines for management of pediatric high grade renal injuries are currently based on limited pediatric data and algorithms from adults, for whom initial nonoperative management is associated with decreased nephrectomy risk. Using a national database, we compared nephrectomy rates between children with high grade renal injury managed conservatively and those undergoing early surgical intervention.nnnMATERIALS AND METHODSnAll children with high grade renal injuries were identified in the National Trauma Data Bank®. High grade renal injuries were defined as American Association for the Surgery of Trauma grade IV or V renal injuries. After excluding fatalities within 24 hours of hospitalization, 419 pediatric patients comprised our study cohort. A total of 81 patients underwent early (within 24 hours of hospitalization) surgical intervention, while 338 were initially treated conservatively. Using stratified analysis with adjustment for relevant covariates, we compared nephrectomy rates between these groups.nnnRESULTSnNephrectomy was performed less often in patients treated conservatively (RR 0.24, 95% CI 0.16 to 0.36, adjusted for age, renal injury grade and injury mechanism). The decreased risk of nephrectomy was more marked among children with grade IV vs grade V renal injuries (adjusted RR 0.16, 95% CI 0.08 to 0.23). Multiple procedures were more common in patients initially observed. Of pediatric patients with grade IV and V renal injuries 11% still underwent nephrectomy.nnnCONCLUSIONSnConservative management of high grade renal injuries is common in children. Although mechanism of injury and renal injury grade impact initial clinical management decisions, the risk of nephrectomy was consistently decreased in children with high grade renal trauma managed conservatively regardless of injury characteristics.

Challenges in Childhood Urinary Tract Infection/Vesicoureteral Reflux Investigation and Management: Calming the Storm

The present report was initiated in 2010 by a panel of experts in pediatrics, nephrology, and urology at a round table meeting to review the management of vesicoureteral reflux (VUR) in children. Shortly thereafter, the American Urological Association published an update on VUR in 2010, and the American Academy of Pediatrics (AAP) released a new guideline in 2011, necessitating a reconsideration of our practice patterns. Although these updates would appear to clarify some of the issues surrounding the treatment of VUR in children, there is still ample confusion regarding who, when, and how to image febrile urinary tract infection (fUTI) and treat reflux in children. For purposes of our report, UTI refers to pediatric UTI. Febrile UTI (fUTI) represents the most common serious bacterial infection of childhood, affecting approximately 8% of children. Long-standing diagnostic and therapeutic protocols were predicated on the assumption that fUTI commonly led to chronic kidney disease and that VUR potentiated the risk of developing acute pyelonephritis and increased the potential for the long-term sequelae of renal scarring. This led to the concept of universal radiologic evaluation of children with fUTI, primarily searching for VUR. If VUR was uncovered, there were essentially only 2 therapeutic options: surgical correction of VUR (reimplantation/ureteroneocystostomy or endoscopic injection) or continuous antibiotic prophylaxis (CAP).

Innovations in the management of Wilms' tumor.

Advances in the management of Wilms’ tumor have been dramatic over the past half century, not in small part due to the institution of multimodal therapy and the formation of collaborative study groups. While different opinions exist in the management of Wilms’ tumors depending on where one lives and practices, survival rates have surpassed 90% across the board in Western societies. With more children surviving into adulthood, the concerns about morbidity have reached the forefront and now represent as much a consideration as oncologic outcomes these days. Innovations in treatment are on the horizon in the form of potential tumor markers, molecular biological means of testing for chemotherapeutic responsiveness, and advances in the delivery of chemotherapy for recurrent or recalcitrant tumors. Other technological innovations are being applied to childhood renal tumors, such as minimally invasive and nephron-sparing approaches. Risk stratification also allows for children to forego potentially unnecessary treatments and their associated morbidities. Wilms’ tumor stands as a great example of the gains that can be made through protocol-driven therapy with strenuous outcomes analyses. These gains continue to spark interest in minimization of morbidity, while avoiding any compromise in oncologic efficacy. While excitement and innovation are important in the advancement of treatment delivery, we must continue to temper this enthusiasm and carefully evaluate options in order to continue to provide the highest standard of care in the management of this now highly curable disease.