Pediatr Surg Research Articles

Are Abdominal X-Rays a Reliable Way to Assess for Constipation?

Currently to our knowledge no validated reliable tools are available to evaluate constipation in children. Abdominal x-rays are often done in clinical practice to evaluate patients with lower urinary tract symptoms. Although 3 previously published rating tools exist to score constipation based on x-ray, there is little information on their merits. We assessed these 3 tools for reliability among multiple practitioners. We retrospectively analyzed abdominal x-rays in a cohort of 80 patients between ages 4 and 12 years. X-rays were independently assessed by each of us using the previously published Barr, Leech and Blethyn scoring tools. Scores were analyzed for reliability using standard statistical methods. The range of weighted κ score, indicating reliability, were 0.0491 to 0.4809 for the Barr, 0.1195 to 0.2730 for the Leech and 0.0454 to 0.4514 for the Blethyn method. Guidelines for κ scores are greater than 0.75-excellent, 0.4 to 0.75-good and 0 to 0.4-marginal reproducibility. ICC, another reliability measure, was 0.02577 for the Barr, 0.3313 for the Leech and 0.201 for the Blethyn method. ICC interpretations are greater than 0.75-excellent, 0.4 to 0.75-good and 0 to 0.4-poor. There was a trend toward good interrater reliability between more experienced urology practitioners with the Barr and Blethyn tools (0.48 and 0.45, respectively) but not between less experienced raters or with the Leech tool. Currently available scoring tools to evaluate constipation by x-ray do not have good reliability among multiple examiners. Further research is needed to develop an alternate tool to increase the reliability of x-ray to assess constipation between multiple raters.

Use of Cutaneous Flap for Continent Cystostomy (Daoud Technique)

We present the results of a new technique using a pedicled cutaneous flap for continent cystostomy. A total of 15 boys and 8 girls (mean +/- SD age 13.4 +/- 6.4 years) underwent continent cystostomy for neurogenic bladder (20), bladder exstrophy (2) and sequelae of hypospadias (1) between 1999 and 2008. In this procedure a rectangular pedicled flap is surgically elevated from a hairless area on the abdomen. The flap is tubularized and passed through the anterior abdominal wall directly into the bladder. A submucosal detrusor incision is made to expose the bladder mucosa, and the distal part of the flap is anastomosed to the bladder mucosa in a circular manner. The tube is positioned along the incised detrusor, which is closed over. Viability of the flap, self-catheterization management and continence status are then evaluated. Mean +/- SD followup was 4.5 +/- 3.1 years. There was 1 case of distal necrosis of the flap, which required a secondary surgery using the Mitrofanoff technique. The 22 remaining flaps were initially viable, although 2 patients were eventually lost to followup and 3 subsequently presented with false-passage incidents requiring a few days of calibration using a balloon catheter. Dryness was achieved immediately in 73% of the cases. After adding a complementary bulking agent the dryness rate reached 77%. We present a novel approach to continent cystostomy that is safe and easy to perform. This technique is a less invasive and more efficient alternative to other commonly used approaches.

Total Endoscopic and Anal Irrigation Management Approach to Noncompliant Neuropathic Bladder in Children: A Good Alternative

We prospectively evaluated the efficacy and durability of combined intradetrusor botulinum-A toxin and endoscopic treatment for vesicoureteral reflux with anal irrigation as a total endoscopic and anal irrigation management approach. This minimally invasive protocol is used to manage myelomeningocele and noncompliant bladder in children who do not respond to standard conservative therapy and have urine and stool incontinence. Ten females and 3 males with a mean +/- SD age of 5.3 +/- 2.5 years with myelomeningocele and vesicoureteral reflux who did not respond to standard conservative treatment were prospectively included in this study. All had at least 1 year of followup. All patients received a cystoscopic intradetrusor injection of 12 U/kg (maximum 300 U) botulinum-A toxin into an infection-free bladder. Vesicoureteral reflux in a total of 20 refluxing ureters, including bilateral vesicoureteral reflux in 7 patients, showed no resolution on pretreatment voiding cystourethrogram. Thus, we administered a submucosal Deflux injection. Since most patients were still diaper dependent due to stool incontinence, we extended management to include complete bowel rehabilitation with the new Peristeen anal irrigation system to manage stool incontinence. Mean maximum bladder capacity increased significantly from 75 +/- 35 to 150 +/- 45 ml after 1 month (p <0.02), to 151 +/- 48 after 6 months (p <0.002) and to 136 +/- 32 after 1 year (p <000). Maximum detrusor pressure decreased significantly from 58 +/- 14 to 36 +/- 9 cm H(2)O after 1 month (p <0.001), to 39 +/- 9 after 6 months (p <0.001) and to 38 +/- 6 after 1 year (p = 000). Of 20 refluxing ureters (95%) completely resolved, including 1 after attempt 2, and 1 with grade V vesicoureteral reflux remained unchanged despite 2 attempts. Seven of 8 urinary incontinent patients (87.5%) attained complete dryness between catheterizations and 1 partially improved. Ten of 13 patients achieved stool dryness with anal irrigation 1 to 2 times weekly. Three patients who were stool continent on standard enemas did not require this irrigation system. This new total endoscopic and anal irrigation management approach is a comprehensive, minimally invasive, safe, simple, effective way to achieve most goals when treating these patients by protecting the upper tract, maintaining the bladder at safe pressure and providing a satisfactory social life with satisfactory urine and stool continence.

Cisplatin plus pirarubicin chemotherapy and combination ifomide, etoposide, pirarubicin, and carboplatin chemotherapy for hepatoblastoma.

9534 Background: The Japanese Study Group for Pediatric Liver Tumor is running cooperative treatment studies on hepatoblastoma (HB) since 1991. The main aim in JPLT-1 study was to evaluate the efficacy of cisplatin/pirarubicin. A total of 145 cases were registered and their 6-year overall survival (OS) and event-free survival (EFS) were 73.4% and 66%, respectively (J Pediatr Surg 37: 851-6, 2002). Since the outcomes of advanced HB cases remained poor, JPLT-2 protocol was launched in 1999 to evaluate the cure rate of risk-stratified HB: standard risk HB (a tumor involving three or fewer sectors of the liver) and high risk HB (a tumor involving all sectors of the liver or with metastasis). Methods: In JPLT-2, children with HB who were younger than 15 years of age were eligible for inclusion in the study. The cisplatin/pirarubicin regimen (CITA) is kept as the first line and the second line regimen consisting of ifomide, etoposide, pirarubicin, and carboplatin (ITEC) is recommended for the tumors resistant to CITA. For high risk HB cases, high dose chemotherapy with stem cell transplantation (SCT) was carried out. Results: Until 2007, 245 hepatoblastoma cases have been registered in JPLT-2, and 203 cases have completed the protocol including 69 cases (18%) with metastatic tumors. The 3-year OS and EFS of the cases with standard risk HB were 96% and 76%, while that of the cases with high risk HB was 54% and 34%. Among 34 high risk HB cases (30 with metastatic HB and 4 with unresectable HB) who underwent high dose regimen with SCT, only 21 cases (57%) were cured, indicating that high dose chemotherapy with SCT is not so effective in advanced tumors. With this regimen, 5 cases suffered treatment-related death. And the late phase complications were 3 cases with maldevelopment, 13 with cardiac complications, 20 with ototoxicity and 4 with second malignancies. Conclusions: As compared with other regimens, CITA regimens achieved similar or superior rates of survival among children with standard-risk HB. ITEC regimens and high dose chemotherapy with SCT is not so effective in high- risk HB. More promising strategies including liver transplantation and new targeting drugs should be developed for high risk HB. No significant financial relationships to disclose.

Spermatogenesis After 1-Stage Fowler-Stephens Orchiopexy in Experimental Cryptorchid Rat Model

To evaluate the validity of 1-stage Fowler-Stephens orchiopexy, we performed this procedure for intra-abdominal testes in an experimental cryptorchid rat model and assessed postoperative spermatogenesis. Cryptorchidism in rats was induced by injecting flutamide into the abdomen of pregnant Sprague Dawley rats for 7 days (days 14 to 20 of gestation). Four-week-old cryptorchid rats were divided into the 4 groups of sham operation (group 1, 7 rats), orchiopexy only (group 2, 7), spermatic vessel ligation only (group 3, 7), and spermatic vessel ligation and orchiopexy (1-stage Fowler-Stephens orchiopexy, group 4, 7). The testes were removed 12 weeks after birth in all groups, and testicular weight, testicular histopathological findings and presence of sperm in the epididymis were examined. All operated testes had reticulated vessels on the tunica albuginea. There were no statistically significant differences in the testicular weight between groups 1 (mean +/- SD 0.47 +/- 0.04 gm) and 4 (0.30 +/- 0.19), suggesting that Fowler-Stephens orchiopexy did not induce significant testicular atrophy. However, seminiferous tubule diameters in group 4 were significantly smaller than in group 1 (p <0.001), and no sperm was observed in the epididymis of group 4 rats, suggesting that Fowler-Stephens orchiopexy reduced seminiferous tubule diameters and did not improve spermatogenesis. Although Fowler-Stephens orchiopexy is a good procedure to maintain testicular size and correct cosmetic deformity of an absent scrotum, it may not significantly contribute to the improvement of spermatogenesis.

Hereditary multiple intestinal atresias: 2 new cases and review of the literature

Intestinal atresias are a common cause of newborn bowel obstruction (Dalla Vecchia LK, Grosfeld JL, West KW, et al, Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133[5]:490-496). Hereditary multiple intestinal atresias, first reported by Guttman et al in 1973, is the rarest form of multiple atresias (Guttman FM, Braun P, Garance PH, et al, Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg 1973;8:633-640; Bass J, Pyloric atresia associated with multiple intestinal atresias and immune deficiency. J Pediatr Surg 2002;37:941-942.). It has been proposed to be autosomal recessive, to involve atresias in a variable combination of sites from stomach to rectum, and to be universally fatal (Bilodeau A, Prasil P, Cloutier R, et al, Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004;39:726-730; Moreno LA, Gottrand F, Turck D, et al, Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet 1990;37:143-146). Patients have significant intestinal dysfunction and unrelenting sepsis stemming from a poorly defined, severe immunologic defect. Our case report presents 2 full siblings to nonconsanguineous parents with pyloric atresia, multiple small bowel and colonic atresias, and severe immune dysfunction. Care was withdrawn within 3 months of life on both siblings after multiple bouts of sepsis. Data suggest that the immune defect may not be primary, but in fact be secondary to intestinal dysfunction. Although the subjects in this article ultimately had fatal outcomes, a comprehensive immunologic/physiologic picture is presented in hopes of furthering the understanding of this grave disease.

Pediatric Melanoma: One Institution’s Experience With 150 Patients

Source: Aldrink JH, Selim MA, Diesen DL, et al. Pediatric melanoma: a single-institution experience. J Pediatr Surg. 2009; 44(8): 1514– 1521; doi: 10.1016/j.jpedsurg.2008.12.003Researchers from Duke University reviewed their experience with pediatric melanoma. The medical records of all children <20 years of age treated for melanoma at their institution between 1973 and 2007 were reviewed. A total of 150 subjects with a mean age of 15.1 years were identified. At the time of diagnosis, 11% were 0 to 11 years of age, 42% were 12 to 16 years, and 47% were 17 to 19 years. Only 2% of the patients were black, all in the youngest age group. Common locations for primary lesions included trunk (37.8% of study patients), extremity (36.5%), and head and neck (24.3%); two children had ocular lesions.Follow-up data were available on 140 patients, with a mean length of follow-up of 8.5 years (range 1 month to 34 years). Overall survival was 84%. Survival in those initially misdiagnosed, however, was 66%. A total of 41 patients developed recurrences (29%), with the highest rate of recurrence among those aged 17 to 19 years at diagnosis (31%). Primary location of the tumor, sex, and thickness of the tumor were not significantly associated with chance of recurrence. Patients initially misdiagnosed had the same risk of recurrence as those who were correctly diagnosed (28.4% and 18.8%, respectively; P=.56). Of the 29 patients who developed metastatic melanoma, 24 died.The authors conclude that pediatric melanoma requires increased awareness by physicians and pathologists to make a timely and accurate diagnosis.Melanoma remains an uncommon diagnosis in the pediatric age group, accounting for only 2% of all melanoma cases.1 Nevertheless, the incidence of melanoma is increasing worldwide and this change is expected to become manifest in children as well.2 Practicing pediatricians and surgeons must work closely with their pathologists to, first, identify those patients who actually require excisional biopsy, and then select those who should proceed with wide excision with lymph node biopsy and further therapy as dictated by depth of lesion and nodal staging.A few definitions are necessary to help clarify discussion of pediatric melanoma. Spitz nevus was originally described in 1948 as a benign juvenile melanoma, an unfortunate term as this lesion is benign yet histologically alarming. This entity continues to present a significant diagnostic challenge to both clinicians and pathologists.3 The pathologist must examine the entire lesion as these melanocytes are atypical, and vary from spindled to epithelioid, but unlike melanoma, are symmetrical, show maturation and junctional cleavage, and lack mitotic figures deep in the dermal melanocytes. Still, expert dermatopathologists can disagree in some cases.Historically, the vertical thickness of the primary tumor and the anatomic depth of invasion are the principle measures in local tumor classification of melanoma staging. Sentinel lymph node biopsy (SLNB) is a methodology increasingly used to stage melanoma. In the current study this procedure was performed in only 18 of the 150 patients, all since the year 2000.1 Blue dye is injected intradermally at the site of the primary melanoma and allowed to be taken up by the lymphatics which then show that the first blue node in the regional lymphatic basin is the node that should contain a metastasis if any tumor is present. This node is termed the sentinel lymph node. The regional lymphatic basin can also be identified by injecting technetium 99m-labeled colloid intradermally at the primary site and then performing lymphatic mapping with a hand-held gamma probe intraoperatively.With accurate diagnosis, the outcomes for pediatric melanoma are very good, with published five-year survival rates of 74% to 89%. Melanoma survival rates among children have continued to improve by 4% per year over the last 30 years. In addition, despite higher rates of positive SLNB, children and adolescents have a lower incidence of recurrence and improved disease-free survival when compared to adults.1,4

A practical approach to the management of pediatric fecal incontinence

We describe the rationale and key aspects for success of a bowel management program for the treatment of pediatric fecal incontinence. A retrospective review was done of the medical strategies used for the treatment of over a 1000 patients with fecal incontinent, combining the experience of the authors' previously published reports (Peña A, Guardino K, Tovilla JM, et al. J Pediatr Surg 1998; 33:133-137 and Bischoff A, Levitt MA, Bauer C, et al. J Pediatr Surg 2009; 44:1278-1284) and additional treated patients after those publications. Emphasis was placed on the review of the key factors needed to achieve success. Through the years, we have learned important lessons that resulted in our current strategy which allows us to obtain better results than earlier in our series. At present, the key aspects for success are (a) to distinguish between true fecal incontinence and pseudoincontinence; (b) to determine the characteristics of the colon (dilated or nondilated), ascertained by looking at the patient's contrast enema, and determine the treatment strategy from this; (c) to monitor the result of the enema (amount of stool left in the colon) with daily abdominal radiographs during a 1-week period; and (d) to thereby modify the type of enema daily, depending on the clinical result, and the abdominal radiograph. Following a systematic rationale in the classification of patients with fecal incontinence and applying selectively, individualized management, it is possible to achieve a 95% success rate in patients suffering from fecal incontinence.

Reply by Authors

No AccessJournal of UrologyLetter to the Editor/Errata1 Apr 2010Reply by Authors Emre Huri, Turgay Akgül, and Cankon Germiyanoğlu Emre HuriEmre Huri More articles by this author , Turgay AkgülTurgay Akgül More articles by this author , and Cankon GermiyanoğluCankon Germiyanoğlu More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2009.12.130AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Reply by Authors." The Journal of Urology, 183(4), p. 1650 References 1 : Investigation of factors affecting result of distal hypospadias repair: comparison of two techniques. Urology2006; 68: 182. Google Scholar 2 : Factors affecting outcome of one-stage anterior hypospadias repair: analysis of 422 cases. Pediatr Surg Int2002; 18: 142. Google Scholar Second Urology Clinic, Ankara Training and Research Hospital, Ankara, Turkey© 2010 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 183Issue 4April 2010Page: 1650 Advertisement Copyright & Permissions© 2010 by American Urological Association Education and Research, Inc.MetricsAuthor Information Emre Huri More articles by this author Turgay Akgül More articles by this author Cankon Germiyanoğlu More articles by this author Expand All Advertisement PDF DownloadLoading ...

Melamine Related Bilateral Renal Calculi in 50 Children: Single Center Experience in Clinical Diagnosis and Treatment

We investigated the clinical diagnosis and treatment features of bilateral renal calculi in young children who ingested melamine tainted infant milk formula. We retrospectively analyzed clinical data on 50 children (mean +/- SE age 23.4 +/- 3.1 months) with a history of ingesting melamine tainted infant milk formula and suffering from bilateral renal calculi. All patients underwent ultrasound and renal function evaluation. Treatment included cessation of melamine tainted formula consumption, hydration, urine basification and hemodialysis if necessary. Bilateral renal calculi peaked in 6 to 18-month-olds (58% of cases). The male-to-female ratio was 3.1:1.0. Calculi ranged in diameter from 4 to 10 mm in 33 patients (66%) and 2.5 to 4 mm in 17 (34%). Of the 11 patients with renal failure 8 underwent 1 to 4 sessions of hemodialysis. Of the 9 bilateral obstruction cases with renal failure 8 did not require surgical intervention but 1 required ureteral catheterization to drain the renal pelvis. All children experienced a good outcome and were discharged home after a mean +/- SE hospitalization of 8.1 +/- 0.7 days. Melamine related urinary calculi were most often seen in patients 6 to 18 months old. Conservative management has been sufficient in most cases. However, these children need to be monitored for long-term effects of melamine tainted milk formula consumption.

Spontaneous bladder perforation: a rare complication of tuberculosis

Spontaneous bladder perforation secondary to tuberculosis (TB) is very rare. Only three cases have been reported so far in the literature. Due to its rarity, the diagnosis of spontaneous bladder perforation is often missed. Confirmation of TB via culture takes a long time and starting empirical treatment for TB is necessary. We relate our experience with a young woman who presented with clinical features of a perforated appendix and was only diagnosed with bladder perforation during laparotomy. She also had distal right ureteral stricture and left infundibular stenosis. The provisional diagnosis of TB was attained via typical histopathological features and a positive Mantoux test. She was started empirically on anti-TB treatment and recovered without any complications. Urine culture after 6 weeks confirmed the diagnosis of TB.

Accessory Lobe of Right Liver Mimicking a Pulmonary Tumor in an Adult Male

We describe a rare case of ectopic liver tissue in the right thoracic cavity. A 39-year-old man with a suspected lung tumor underwent thoracotomy. The intraoperative finding revealed an accessory liver lobe connected to the right lobe of the liver by a small pedicle that pierced the diaphragm. A pathologic evaluation confirmed the specimen to be hepatic tissues. In retrospect, the correct diagnosis of accessory liver could have been achieved using appropriate radiologic investigations if this possibility had been considered.

Inflammatory Myofibroblastic Tumor of the Esophagus

Inflammatory myofibroblastic tumors are regarded as intermediate-grade tumors with a potential for recurrence. Although these lesions have been found in nearly every anatomic location, there are few documented cases of esophageal localization. The rare case reported here concerns a 55-year-old woman with an extremely large inflammatory myofibroblastic tumor of the esophagus; the tumor was 20 cm in length and 6 cm in diameter. Wide surgical excision was performed. Histopathologic and immunohistochemical analyses confirmed the diagnosis of an inflammatory myofibroblastic tumor. There was no recurrence or tumor metastasis 6 months after the operation.

The Outcome of Closing Gastroschisis: Two Case Reporta and Literature Review

Correction to: The Outcome of Closing Gastroschisis: Two Case Reports and Literature ReviewEur J Pediatr Surg 2010; 20(01): 65-66DOI: 10.1055/s-0029-1216380

Virtual bronchoscopy as a dynamic modality in the diagnosis and treatment of suspected foreign body aspiration

By this notice, the Editor and the Publisher of European Journal of Pediatric Surgery retract from publication the following article: Cevizci N, Dokucu A I, Baskin D, Karadağ C A, Sever N, Yalçin M, Bahadir E, Başak M. Virtual bronchoscopy as a dynamic modality in the diagnosis and treatment of suspected foreign body aspiration. Eur J Pediatr Surg 2008; 18: 398-401. Epub 2008 Nov 20. This retraction is due to substantial overlap of data, subject matter and illustrations with the following article: Ozkurt H, Bahadir E, Ucgul A, Altuna C, Basak M, Cevizci N M, Dokucu A I. Comparison of multidetector computed tomography-virtual bronchoscopy and conventional bronchoscopy in children with suspected foreign body aspiration. Emerg Radiol Epub 2008 Nov 19. We apologise to our readership.

Long-term Use of Antegrade Continence Enema

Source: Yardley IE, Pauniaho S, Baillie CT, et al. After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. J Pediatr Surg.2009;44(5):1274–1277; doi:10.1016/j.jpedsurg.2009.02.030The antegrade continence enema (ACE) procedure involves the construction of an abdominal wall stoma using the appendix as the conduit from the skin level in the right lower quadrant into the cecum; this allows access for antegrade irrigations on a regular and scheduled basis to provide effective colonic washout in patients with refractory chronic constipation. Investigators from Alder Hey Children’s Hospital in Liverpool, England, sought to determine the long-term use of ACE. All children who had an ACE at a single tertiary care center between 1993 and 1997 were surveyed to determine if they were still using the ACE and, if not, why use had been discontinued.Of the 84 eligible patients, 61 could be contacted. The mean age was 22.4 years (15–35 years) with an average follow-up time of 11 years (8–14 years). Their underlying diagnoses included spina bifida (27), anorectal malformation (18), intractable constipation (11), sacral agenesis (2), trauma or tumor (2), and Hirschsprung’s Disease (1). Thirty-six of the 61 patients (59%) were still using their ACE. In those patients continuing to use their ACE, the overall satisfaction score was 4 (on a scale of 1 to 5, with 5 being very satisfied).Among the 25 patients (41%) who reported discontinuing use of their ACE, 14 stated they had done so because the washouts had proven ineffective for prevention of soiling. Five others described significant pain with washouts, stomal stenosis, or difficulty in using the stoma during pregnancy. Two patients stopped using the ACE because of psychological issues, one because of being disturbed at the appearance of the stoma and one secondary to depression. Two additional patients were unable to comply with the washout regimen, rendering the ACE ineffective. There was no association between diagnosis and non-use. Several individuals reported feeling abandoned on becoming adults and losing the support they had in childhood.The authors conclude that patients discontinue use of the ACE for a variety of reasons and suggest that newer refinements of the surgical procedure may lead to its greater long-term successful use. Most concerning, however, is the high level of discontent with the transitional care arrangements on reaching adulthood.Dr Cavett has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.The ACE allows the patient to receive effective colonic irrigation via the right colon, thereby promoting cleanout of the entire colon at one time. The colon can then serve as a large reservoir for stool, permitting absence of soiling during the day. The usual regimen involves a mixture of water and either mineral oil or glycerin in volumes and concentrations necessary to obtain washout results.The principal complication of this procedure is painful catheter insertion as a result of stomal stenosis. Mucus leak can be a problem but this is usually temporary during the first several weeks following the surgery. Several changes in the washout regimen are usually necessary before a steady state is reached.2The operation is not terribly complicated and involves constructing a stoma via the tip of the still-attached appendix. An anti-refluxing wrap of cecal wall around the base of the appendix can usually prevent fecal reflux. Much attention must be paid to construction of the stoma itself, interdigitating skin and opened appendiceal lumen in such a way as to allow easy catheterization, yet it must remain fairly flat and dry of mucus.3This group report denotes two important points for surgeons and pediatricians alike. First, we have a good tool available to us when patients need this level of colon washout which cannot be managed with rectal irrigations, and would benefit from an intervention that offers some degree of social acceptance. Second, we must endeavor to provide seamless care as patients with lifelong problems move into the adult world of health care. These patients require ongoing support and care if maintaining continence is to be effective.

Pectus Excavatum With Compression of the Inferior Vena Cava

Pectus excavatum (PE) is a common disorder of the chest wall that affects 1 in 500 of the general population. Affected individuals have cosmetic and, less commonly, cardiopulmonary consequences that typically present during the adolescent years. The latter is appreciated only in those with severe phenotypes, especially if associated with a “straight back” that further limits the anterior-posterior thoracic dimensions. Although the cardiopulmonary complications of PE have been well described, we describe a unique presentation of a patient who had experienced years of intractable syncope before receiving corrective surgery for severe PE. The role of cardiovascular magnetic resonance imaging (CMR) in her diagnosis and subsequent treatment is also highlighted. A 22-year-old woman presented with a long-standing history of recurrent syncope, beginning in early adolescence. These episodes typically occurred after prolonged periods of standing or walking, beginning with a prodrome of diaphoresis and blurred vision and culminating in loss of consciousness. She described self-taught maneuvers that reduced these symptoms, including twisting her torso to the side and performing rapid, shallow breathing. In spite of these maneuvers, she continued to be limited by frequent syncope and ultimately stopped attending school at 16 years of age. On examination, she had a marked pectus deformity with a straight back appearance. Her heart sounds were normal, with a Grade II out of VI systolic ejection murmur at the left sternal border. There was no jugular venous distention and no peripheral edema. Following indentification of the chest wall deformity further questioning revealed that both her brother and paternal uncle had severe PE, suggesting an autosomal dominant inheritance …

A Gender Transformation in Urology

A Gender Transformation in Urology

Polyorchidism: A Meta-Analysis

Polyorchidism is an uncommon congenital anomaly. We systematically analyzed the literature due to the contradictory data regarding this condition. We conducted a database search and evaluated relevant articles for the appearance, comorbidities and complications of supernumerary testes. Only cases of polyorchidism confirmed by histological examination were included in the study. We found 140 cases of polyorchidism. Triorchidism was the most common type, and 6 cases of 4 testes (4.3%) were reported. Most supernumerary testes (64%, chi-square p <0.001) were drained by a vas deferens. Median patient age at detection was 17 years. Left side demonstrated predominance (64.5%, chi-square p <0.001). Most cases were found during surgery for other symptoms including inguinal hernia, undescended testicle, testicular torsion and scrotal pain. Only 16% of patients complained of an accessory mass without any symptoms. Neoplasms were found in 9 cases (6.4%), of which 8 were malignant and 1 was benign. Data available for 7 malignomas (88%) revealed that all were in cryptorchid supernumerary testes. Polyorchidism is rare and is generally found during evaluation for other conditions such as inguinal hernia, undescended testis and testicular torsion. Cryptorchidism appears to be the most important risk factor for malignancy in patients with supernumerary testes. Thus, patients with nonscrotal supernumerary testes require appropriate counseling. The supernumerary testis is frequently drained by a vas deferens, implying a probable reproductive function. Decision for surgery, biopsy or orchiectomy should be based on concomitant symptoms, cryptorchidism or suspected malignancy. We provide a guideline for management based on a functional classification of polyorchidism.

Acute Bilateral Renal Vein Thrombosis

Acute Bilateral Renal Vein Thrombosis