Successful surgical excision of the accessory urethra in a 13-year-old boy with Effmann Type IIA-2 urethral duplication. Case report and literature review.

INTRODUCTION AND CLINICAL IMPORTANCE: Urethral duplication is a congenital anomaly characterized by the partial or full development of an auxiliary urethra as a second urethral channel varying in extent and location. The course of treatment for urethral duplication should be individualized for each patient based on the type of anomaly and the existence of symptoms. In most cases, if the ventral orthotopic urethra is normal, excision of the dorsal auxiliary urethra is almost always curative. CASE PRESENTATION: We report a case of a 13-year-old boy who presented with recurrent urinary tract infection, occasionally having drops of urine at an external pin hole meatal opening on the dorsum of the penile shaft. Several investigations were performed to confirm the presence of the dorsal accessory urethra. The patient underwent complete excision of the accessory urethra, and 12 weeks post-surgery the patient was completely asymptomatic. DISCUSSION: Urethral duplications are rare anomalies. These anomalies have been classified in various ways in the literature, including sagittal and lateral duplications, dorsal and ventral duplications. The sagittal plane is where the majority of urethral duplications occur. Based on the anatomic variation, clinical presentation and severity of the anomaly, urethral duplication treatment should be customized for each patient. An asymptomatic child may require no specific treatment. Total surgical excision of the dorsal accessory urethra appears to offer the best curative solution. CONCLUSION: Excision of the dorsal accessory urethra is usually curative when the ventral orthotopic urethra is normal. Thorough investigation before surgery is mandatory to determine the type of urethral duplication. The most common diagnostic procedures for urethral duplications include cystoscopy, KUB ultrasonography, and micturating cystourethrography. These procedures can help plan surgery, prevent complications such as neurovascular bundle injuries and urine incontinence, and improve patient self-esteem and quality of life.

The antegrade continence enema procedure in the setting of acute appendicitis.

The antegrade continence enema (ACE), known as the ACE procedure, has gained popularity over the past two decades in the treatment of refractory fecal incontinence. This case report describes an acutely inflamed appendix being used for an ACE procedure. To my knowledge, this has not previously been documented in the literature.

Comparing the outcomes of tubularized incised plate urethroplasty and dorsal inlay graft urethroplasty in children with hypospadias: a systematic review and meta-analysis.

BACKGROUND: Excellent outcomes have been reported following the widely accepted tubularized incised plate urethroplasty (TIPU) and its relatively recent modification, the dorsal inlay graft urethroplasty (DIGU). However, there is a lack of consensus on which technique offers more favorable postoperative outcomes. AIMS: To systematically compare the reported outcomes of the TIPU and DIGU techniques in children undergoing primary hypospadias repair. DESIGN: A systematic review and meta-analysis of randomized and observational studies. METHODS: An electronic database search was conducted up to May 2018. Sources included Medline, Embase, Cochrane library, CINAHL, Web of Science, and Google Scholar as well as trial registries and grey literature sources. Studies were selected if they compared the postoperative complications of TIPU and DIGU in children. Secondary outcomes included standardized cosmetic scores and urinary flow studies. A meta-analysis of reported complications was performed using a random-effects model. RESULTS: Two randomized, two prospective, and two retrospective studies met the inclusion criteria. TIPU and DIGU were performed in 350 and 267 patients, respectively. Pooled analysis did not demonstrate a significant difference regarding postoperative urethrocutaneous fistula, meatal/urethral stenosis, wound dehiscence, or total complications. Subgroup analysis according to hypospadias severity did not alter initial findings. Statistical analysis of secondary outcomes was not feasible due to insufficient data. Most studies were of low methodological quality with a high risk of bias. CONCLUSIONS: There is no strong evidence to suggest that either technique offers more favorable outcomes. Until more robust randomized trials exist, decisions regarding the appropriate repair should be based on the surgeon's experience and outcomes.

Incidence of Low-Grade Testicular Injury in Orchidectomy Specimens Post-testicular Torsion.

INTRODUCTION: Following detorsion and orchidopexy for testicular torsion, predominantly animal studies have reported a risk of autoimmune and reperfusion injury to the contralateral testis. As a result, when testicular viability is compromised, orchidectomy is readily performed. This practice increases the likelihood of testes with potentially reversible injury being excised. We aim to determine the incidence of such occurrences and review the available evidence for and against early orchidectomy when testicular viability is doubtful. MATERIALS AND METHODS: Data for a 15-year period from two pediatric institutions on testicular torsion in children younger than 16 years were reviewed. Using a previously published grading system, the orchidectomy specimens in this cohort with early low-grade injury were analyzed. Low-grade injury suggests the possibility of restitutio ad integrum implying restoration of exocrine and endocrine function of the affected testes. RESULTS: Between both institutions, 222 scrotal explorations were performed for testicular torsion; 20 neonatal and 202 outside the neonatal period (age range [median]: 1-28 days [3 days] and 3 months-16 years [13 years], respectively). Of these scrotal explorations, 17 neonatal and 66 nonneonatal orchidectomies were required (85 vs. 33%, respectively; p < 0.0001). From these orchidectomy specimens, 5 (6%) were found to have low-grade injury. The ages of these five children ranged from 9 to 16 years (median 15, mean 13.6 years). Their symptom duration ranged from 8 to 37 hours (median 14, mean 18 hours) and two of these children had a preoperative ultrasound documenting no flow to the testis. CONCLUSION: The finding of histopathological features that may represent salvageability of a torted testis occurs relatively rarely. Because of this possibility, appropriate intraoperative steps to check for reperfusion must be undertaken prior to orchidectomy. More evidence for the use of antioxidants and tunica albuginea decompression to improve testes salvage rates is required. The potential for exocrine and endocrine function if partial testicular atrophy occurs and the evidence for contralateral autoimmune testicular damage in pre- and postpubertal males require further investigation.

Early Bilateral Gonadoblastoma in a Young Child with Mosaicism for Turner Syndrome and Trisomy 18 with Y Chromosome.

Mosaic Turner syndrome (TSM) commonly occurs in the form of 45,X/46,XX and 45,X/46,X,i(X)(q10). Mosaicism for a Y chromosome, 45,X/46,XY, has been well documented and is associated with increased risk of gonadoblastoma (GB). To date, there are only six reported cases of TSM with a trisomy 18 karyotype, and only two of these were phenotypically female with 45,X/47,XY,+18 karyotype. We present the case of a phenotypically female infant born with dysmorphic features. G-banded karyotype and interphase FISH of blood showed 45,X in 95% and 47,XY,+18 (trisomy 18) in 5% of cells analysed. However, interphase FISH of buccal cells showed only the presence of the 45,X cell line. Due to the presence of Y chromosome material, elective gonadectomy was performed at 13 months of age. There were bilateral streak ovaries with early evidence of GB bilaterally, a rudimentary uterus and bilateral fallopian tubes with unilateral ectopic adrenal tissue identified histologically. Interphase FISH of the gonadal tissue was similar to the blood findings with 45,X in 86% of cells and 47,XY,+18 in 14% of cells analysed. This case highlights a rare karyotype of TSM and trisomy 18 in the same patient and is the first reporting the associated finding of bilateral GB.

Gonadoblastoma in patients with 45,X/46,XY mosaicism: A 16-year experience.

BACKGROUND: It is recognised that individuals with a 45,X/46,XY karyotype, known as Turner mosaic syndrome with Y chromosome material (TMSY), have an increased risk of developing gonadoblastoma (GB), which may then devolve into one of a number of germ cell malignancies. Hence, children with TMSY are usually recommended to undergo prophylactic gonadectomy. OBJECTIVE: We designed this study to describe the phenotypic features of our series of children with TMSY who underwent prophylactic gonadectomy in order to evaluate the prevalence of GB and germ cell malignancies in their resected specimens. STUDY DESIGN: This is a retrospective case series wherein we comprehensively reviewed the clinical, histological, and cytogenetic features of all patients who underwent prophylactic gonadectomy at three tertiary paediatric referral centres over 16 years. Cases were identified from surgical logbooks and through the institutional histopathology database. Data were collected with particular reference to clinical phenotype, predominant karyotype cell line, operative management, anatomical findings and the presence of neoplastic changes. RESULTS: Fourteen children ranging in age at the time of surgery from 2 weeks to 17 years were included in the series. Eleven children were reared as females. The three children who were reared as males had severe penoscrotal hypospadias. The 46,XY cell line was the predominant cell line in seven (50%) cases in blood lymphocytes. The resected specimens from four patients (28.6%) contained GB, with three patients having bilateral GB. This sub-group of patients with GB were aged 5 months, 48 months, 71 months, and 13 years. GB arose in one patient with and three patients without genital virilisation. There was no focus of invasive germ cell tumour in any specimen. DISCUSSION: GB may be present in infants with TMSY as young as 5 months, even with low levels of Y chromosome material. The prevalence of GB in prophylactic gonadectomy specimens is similar to many previously reported series, although the absence of dysgerminoma in our series is reassuring. The exclusive presence of GB in intra-abdominal gonads is in keeping with the findings of several other series. CONCLUSION: Owing to the presence of gonadoblastoma in the gonads of children with TMSY as young as 5 months, we recommend that all patients with intra-abdominal gonads in the context of TMSY should duly undergo prophylactic gonadectomy, although the timing of such surgery can be discussed with parents during counselling regarding the risk of malignancy.

Does Functional Outcome Improve with Time Postsurgery for Hirschsprung Disease?

PURPOSE: Constipation and incontinence are significant problems following pull-through surgery for Hirschsprung disease (HD). There is evidence that these problems improve with time. However, there is also evidence showing no improvements and furthermore, significant long-term data are lacking for the newer endorectal pull-through. We aim to determine if there is clinical evidence that show improvements in functional outcomes with time after an endorectal pull-through surgery for HD. METHODS: We utilized the validated pediatric incontinence and constipation scoring system (PICSS) to score 51 consecutive children 3 months to 15 years posttransabdominal or transanal endorectal pull-through for HD. Cases of total colonic aganglionosis and Down syndrome were excluded. PICSS scores below the age-specific lower limit 95% confidence interval scores represent incomplete continence or constipation, respectively. We performed linear regression to analyze the relationship between PICSS scores and the follow-up duration and then compared the demographics of children with and without incomplete continence and constipation, respectively. Significance was set at p < 0.05. RESULTS: The median age at PICSS interview was 71 months (range, 6-191 months). Incontinence scores obtained from 42 children older than 35 months showed a positive relationship with the follow-up duration (p = 0.03). Constipation scores obtained from 51 children were unrelated to follow-up duration (p = 0.486). When demographics were compared, the continent children had longer follow-up than those with incomplete continence (mean, 111.64 vs. 69.19 months; p = 0.051), however follow-up duration did not differ in the group of constipated children compared with the nonconstipated group (mean, 61.88 vs. 71.80 months; p = 0.321). CONCLUSION: These findings suggest that after an endorectal pull-through, improved continence should be expected with time but constipation often continues to be an ongoing problem.

Laparoscopic Excision of Large Intra-Abdominal Cysts in Children: Needle Hitch Technique.

Laparoscopic surgery has both diagnostic and therapeutic advantages in the management of intra-abdominal cysts in children. Large cysts in small children pose technical challenges during laparoscopic surgery, requiring multiple incisions and advanced laparoscopic skills. This paper describes a novel laparoscopic technique using minimal manipulation for both aspiration and excision of the cyst. This simple, safe, and effective approach was used to achieve traction and facilitate excision of a large intra-abdominal cyst in a neonate and a young child.

Publication patterns on neonatal surgery over 65 years.

BACKGROUND/PURPOSE: Staying abreast of the literature in a given speciality is difficult. The aim of this study is to analyze the publication patterns of the neonatal surgical literature over the last six decades. MATERIALS: A search strategy for the Web of Science database was designed using MeSH defined terms for 10 index neonatal surgical conditions, with output analyzed over two time-periods. RESULTS: There were 6215 and 6144 publications for periods 1 (1945-1994) and 2 (1995-2010), respectively. There were 24 and 546 articles published in 1945 and 2010, respectively. The mean citation counts of the top 50 publications are 228 and 156 for periods 1 and 2, respectively. There were 6 and 11 authors with two or more publications in the top 50 list in periods 1 and 2, respectively. Three of the pediatric surgery journals cumulatively have published 30.9% of the total articles. CONCLUSIONS: Publication patterns for neonatal surgical conditions have changed significantly over time. The majority of articles are published outside of pediatric surgical journals. Pediatric surgeons should not limit their reading to these journals.

Cultural bias in the AAP's 2012 Technical Report and Policy Statement on male circumcision.

The American Academy of Pediatrics recently released its new Technical Report and Policy Statement on male circumcision, concluding that current evidence indicates that the health benefits of newborn male circumcision outweigh the risks. The technical report is based on the scrutiny of a large number of complex scientific articles. Therefore, while striving for objectivity, the conclusions drawn by the 8 task force members reflect what these individual physicians perceived as trustworthy evidence. Seen from the outside, cultural bias reflecting the normality of nontherapeutic male circumcision in the United States seems obvious, and the report's conclusions are different from those reached by physicians in other parts of the Western world, including Europe, Canada, and Australia. In this commentary, a different view is presented by non-US-based physicians and representatives of general medical associations and societies for pediatrics, pediatric surgery, and pediatric urology in Northern Europe. To these authors, only 1 of the arguments put forward by the American Academy of Pediatrics has some theoretical relevance in relation to infant male circumcision; namely, the possible protection against urinary tract infections in infant boys, which can easily be treated with antibiotics without tissue loss. The other claimed health benefits, including protection against HIV/AIDS, genital herpes, genital warts, and penile cancer, are questionable, weak, and likely to have little public health relevance in a Western context, and they do not represent compelling reasons for surgery before boys are old enough to decide for themselves.

Are routine dilatations necessary post pull-through surgery for Hirschsprung disease?

INTRODUCTION: We aim to compare the anastomotic stricture and enterocolitis rates between groups who either had or did not have anal dilatations (AD or NAD) prescribed routinely post pull-through surgery for Hirschsprung disease (HD); by this means, we will evaluate the benefit of routine dilatations. METHODS: A retrospective review of the records of all children operated on for HD between 1997 and 2010 was performed. Associated Down syndrome and total colonic aganglionosis were excluded. Two cohorts were identified; those who had anal dilatation prescribed routinely (AD) and those who did not (NAD). In the latter group, if an anastomotic stricture was subsequently diagnosed, anal dilatations were initiated. The anastomotic stricture and enterocolitis rates between groups were compared. Significance was set at p < 0.05. RESULTS: There were 73 children that met the inclusion criteria (30 AD and 43 NAD). The NAD group had the longer mean follow-up period of 91 versus 59 months (p = 0.026); however, follow-up duration was unrelated to the anastomotic stricture rates (p = 0.575) and enterocolitis rates (p = 0.150). The anastomotic stricture rates were 13% (n = 4) versus 14% (n = 6) (p = 1.000) for the AD and NAD groups, respectively (relative risk [95% confidence interval] RR [95% CI], 0.95 [0.29 to 3.09]; p = 0.94). The mean duration between surgery and stricture occurrence was 348 versus 74 days for the AD and NAD groups, respectively. The enterocolitis rates were 23% (n = 7) versus 28% (n = 12) (p = 0.788) for the AD and NAD groups, respectively (RR [95% CI], 0.84 [0.37 to 1.87]; p = 0.66). CONCLUSION: We have not shown a reduced risk of developing anastomotic strictures or enterocolitis if anal dilatations are prescribed routinely. However, when routine dilatations were prescribed, predominantly late onset strictures of perhaps a different etiology occurred.

Hydrocele on the web: an evaluation of Internet-based information.

OBJECTIVE: To evaluate the quality of health-based information available to patients and their parents on the Internet regarding hydrocele, a common paediatric condition, an Internet search was performed and a questionnaire distributed. MATERIAL AND METHODS: The top 100 websites from the five most accessed search engines were reviewed by entering the term "Hydrocele" into each search engine. Website authorship was determined by close examination of each website. Websites were assessed for accuracy and validity according to the Health On the Net Foundation Code (HONcode), DISCERN score and JAMA benchmark criteria, recognized scoring systems. A voluntary written questionnaire was distributed to parents of patients on two consecutive days outside a paediatric clinic. RESULTS: After duplicate and inaccessible sites had been excluded, 37 unique websites were identified: 11 were academic, eight were produced by physicians not affiliated with an academic institution, three were commercial, four were attached to discussion groups or social media sites, four were media related and seven were unspecified. There was a significant difference between the academic and the physician-related sites compared with the other categories, with academic and physician sites scoring highest on the recognized scoring systems (p = 0.0001). It was found that 56.7% of patients accessed the Internet regarding their condition and 33% believed this information to be accurate. CONCLUSION: The quality of health-related information overall is of a poor quality. Academic and physician-provided websites were shown to contain better quality information. Clinicians have a potential role not only to direct patients to appropriate sites, but also to help to develop content on the Internet.

Comparative review of functional outcomes post surgery for Hirschsprung's disease utilizing the paediatric incontinence and constipation scoring system.

PURPOSE: We aim to analyze differences in functional outcomes in children operated on for Hirschsprung's disease (HD) using the Paediatric incontinence/constipation scoring system (PICSS) validated in a normative group. METHODS: A retrospective review of the records of all children operated on for HD between 1997 and 2010 was performed. Patients had either a Soave or transanal endorectal pull-through. Children with total colonic aganglionosis and Down's syndrome were excluded. Utilizing the PICSS children who scored below their age-specific lower limit 95 % confidence interval PICSS scores were considered to have incomplete continence or constipation. The rates of incomplete continence and constipation were compared between groups. Significance was set at p < 0.05. RESULTS: PICSS analysis could be completed in 51 (Soave 35, transanal 16). The median age at interview was 71 months (range 6-191 months). The rate of incomplete continence was 75 % (n = 21) and 71 % (n = 10) for the Soave and transanal groups, respectively (p = 1.00). The constipation rate was 34 % (n = 12) and 25 % (n = 4) for the Soave and transanal groups, respectively (p = 0.74). The overall rates of incomplete continence and constipation rates were 74 and 31 %, respectively, compared with 14 and 10 %, respectively, when rates were calculated by review of records. CONCLUSION: The PICSS is a sensitive tool for assessing functional outcome post HD surgery. The Soave and transanal procedures have similar functional outcomes.

Urinary incontinence in children: botulinum toxin is a safe and effective treatment option.

PURPOSE: This study's aim was to assess the use of intravesical injection of botulinum neurotoxin type A (BoNT-A) as a treatment of overactive bladder (OAB) in children. METHODS: A 6-year retrospective study of children who received BoNT-A for OAB was performed. Treatment outcome was classified as complete success (CS), partial success (PS) or treatment failure (TF). RESULTS: Of the 57 patients who received BoNT-A treatment for OAB, 35 were males. CS occurred in 74.2% of males and 54.5% of females. PS was achieved in 20% of males and 18.2% of females. TF occurred in 2.9% of males and 22.7% of females. Anticholinergics had previously been used and had been effective in 58.6% and 83.3% of males and females. Significant side effects to medications were experienced in 12 (41.4%) males and 4 (22.2%) females. Of these, BoNT-A achieved CS in seven (53.3%) males and two (50%) females and PS in three (25%) males and one (25%) female. BoNT-A was successful in seven (58.3%) males and two (66.7%) females where anticholinergics were ineffective. CONCLUSIONS: BoNT-A has a role in a carefully selected subgroup of children with overactive bladder symptoms including those with medication side effects and treatment compliance issues. It may have a role in patients who do not respond to conventional therapy.

Neonatal scrotal wall necrotizing fasciitis (Fournier gangrene): a case report.

INTRODUCTION: Necrotizing fasciitis in neonates is rare and is associated with almost 50% mortality. Although more than 80 cases of neonates (under one month of age) with necrotizing fasciitis have been reported in the literature, only six of them are identified as originating in the scrotum. CASE PRESENTATION: We report the case of a four-week-old, full-term, otherwise-healthy Caucasian baby boy who presented with an ulcerating lesion of his scrotal wall. His scrotum was explored because of a provisional diagnosis of missed torsion of the testis. He was found to have necrotizing fasciitis of the scrotum. We were able to preserve the testis and excise the necrotic tissue, and with intravenous antibiotics there was a successful outcome. CONCLUSIONS: Fournier gangrene is rarely considered as part of the differential diagnosis in the clinical management of the acute scrotum. However, all doctors who care for small babies must be aware of this serious condition and, if it is suspected, should not hesitate in referring the babies to a specialist pediatric surgical center immediately.

Congenital fibrosarcoma of the ileum: case report with molecular confirmation and literature review.

Congenital fibrosarcoma is a rare, soft tissue malignancy of infancy, most commonly involving the distal extremities. We report a case of congenital fibrosarcoma of the ileum in a 5-day-old boy who presented with an acute abdomen due to ileal perforation. Partial ileal resection was carried out with successful anastomosis. Grossly, the resected small bowel showed focal luminal stenosis with a thickened, indurated wall. Histology showed a transmural primitive spindle cell proliferation with a morphology consistent with congenital fibrosarcoma. The associated hallmark chromosomal translocation t(12;15)(q13;q25) was demonstrated by reverse transcriptase polymerase chain reaction.