Myointimoma (angiocentric myofibroblastic tumor) of the glans penis in an adolescent: a case report and review of the literature.

BACKGROUND: Soft tumors of the penis are extremely rare in childhood and adolescence. Because there are no guidelines, each such case raises embarrassment over the extent and degree of aggressiveness of the diagnostic and therapeutic procedures. Herein, we describe a case of a teenager with a penile myointimoma along with a review of the literature. So far, only 10 cases have been reported in this age group. CASE PRESENTATION: The 15-year-old boy presented with a 6-months history of a slowly growing, palpable firm nodule in glans penis. Physical examination revealed a palpable, well circumscribed, firm, whitish painless mass, around 1 cm in diameter within the glans. Ultrasound revealed hypoechogenic, hypoperfused poorly defined area inside the glans. MRI did not confirm any other pathologic mass inside the glans and corpora cavernosa. An excisional biopsy was performed; its immunohistological features indicated myointimoma. DISCUSSION AND CONCLUSION: Given the rarity of this benign entity, the main importance is to distinguish myointioma from more clinically aggressive neoplasms. The key to a correct diagnosis is a careful histological examination, including immunohistochemistry. Local excision is safe and effective treatment modality.

The safety of neoadjuvant hormonal treatment in infants with cryptorchidism.

BACKGROUND/PURPOSE: The standard treatment for boys with non-syndromic cryptorchidism is an early orchidopexy. It is unclear if surgical intervention alone is enough for future fertility. Recent studies show benefit of neoadjuvant or adjuvant hormonal treatment with gonadorelin (GnRH) for spermatogonia maturation based on testicular biopsy. The aim of this prospective study was to assess the safety of this treatment in infants with undescended testis at the recommended timing of early gonadorelin administration and timing of orchidopexy. METHODS: Unilateral cryptorchid full term boys were initially examined (including hormonal, physical and ultrasound examination) at the age of 2.5-3.5 months. At 6 months of age, cryptorchidism was confirmed. Those with non-syndromic cryptorchidism and palpable or sonographically detected testis were randomly assigned into two groups: with and without intranasal gonadorelin treatment. Inclusion criteria were met by 36 boys (21 in GNRH and 15 in the control groups). The following orchidopexy was performed before 12 months of age with repeated examination at time of surgery. Penile size and testicular volume (using ultrasound) and basal serum levels of LH, FSH, testosterone, Inhibin B and AMH were recorded at age of 3.0 (mean) months and 11.0 (mean) months (date of surgery). The stimulation hormonal levels were checked during GnRH administration. RESULTS: Between minipuberty (mean 3 months) and time of orchidopexy (mean 11 months of age) the penile size increased significantly and similarly in both groups. There was no significant difference in the change of the volume of descended testis between the groups nor of the volume of undescended testis. In addition, we did not find any significant difference in the change (drop) of hormonal levels of LH, FSH, Testosterone, Inhibin B and AMH (Table 1a) CONCLUSION: The neoadjuvant gonadorelin stimulation in infants with unilateral undescended testis has not shown any specific effect on the development of penile size, testicular volume and hormonal levels at time of orchidopexy in comparison with boys without stimulation, and in the mid-term, this treatment can be considered safe. Further follow-up is necessary to evaluate the long-term effect of this early treatment.

Laparoscopic lymphatic and artery sparing microsurgical varicocelectomy - technique, results and long-term outcomes.

INTRODUCTION AND OBJECTIVE: It is generally considered that artery sparing suprainguinal varicocelectomy is associated with a higher risk of persistence in comparison with the non-sparing (Palomo) procedure. Artery sparing is desirable in specific conditions. Based on our 21-year long experience, this study aims to describe technical details and standard steps of the procedure, leading to a comparatively low recurrence rate. MATERIAL AND METHOD: 336 patients, prospectively collected, who underwent laparoscopic lymphatic and artery-sparing microsurgical varicocelectomy as a primary operation between March 1999 and February 2020, were retrospectively evaluated. Patient age was 7-21.5 years (mean 15.4). The left side was involved in 313 (93.2%), both sides in 23 (6.8%) patients. In total 359 varicoceles were repaired, in which 281 cases were grade III, 65 grade II and 13 cases were grade I. The most common indications for surgery were left testicle hypotrophy, demonstrated in 167 (49.7%) patients, an abnormal spermiogram in 48 (14.2%), pain in 28 (8.3%) and bilateral involvement in 23 (6.8%) of patients. The technique has been standardized into four steps: early artery identification; peeling the network of small veins off the artery; peeling the lymphatic vessels off medium and large size veins and division of all veins; check of residual vascular bundle containing the artery and lymphatics only (video - Appendix A). Mean postoperative followup was 27.1 (range 0.5-174) months. Complications were recorded. Persistent varicocele was defined as clinically significant varicocele accompanied by renotesticular reflux on Doppler ultrasound. Ultrasound was used to rule out hydrocele formation and testicular atrophy. RESULTS: Persistent varicocele was recorded in 15 of 359 (4.2%) cases; secondary hydrocele was detected in 1 case (0.3%). Testicular atrophy was not detected in any of the operated patients. Most complications were recorded in the first 3 years after the introduction of the method; 5 recurrences of 290 (1.7%) cases were detected over the last 18 years (Table). DISCUSSION: The method meets all requirements of subinguinal microscopic repair. The artery preservation is desirable in previous (and for future) inguinal and subinguinal surgery cases where collaterals could be compromised. Artery sparing allows for a future vasectomy. Boys with a varicocele on a solitary testicle may be good candidates for this procedure as well. We consider the method as alternative for experienced laparoscopic surgeons. CONLUSION: The laparoscopic lymphatic and artery sparing microsurgical varicocelectomy is safe and effective method with a low recurrence rate like the non-sparing suprainguinal repairs.

Distal urethral plate adhesions: New anatomical perspective in hypospadias.

INTRODUCTION: We found midline epithelial adhesions in the glandar urethral plate in patients with hypospadias. After dissolution, a blind epithelized channel becomes visualized inside of the plate pointing to immature embryonic luminization. In addition it reveals that the epithelized surface of the distal urethral plate is larger than previously considered. OBJECTIVE: To determine the incidence and extent of these new anatomical details of urethral plate in hypospadias patients. METHODS: We prospectively assessed the detailed anatomy of the urethral plate in 72 consecutive patients with hypospadias. We recorded the presence of adhesions in the middle of the glandar urethral groove that can be easily dissoluted (dissolution line - D-line). We recorded the plate width before and after D-line dissolution, the presence of the hidden blind channel at continuation of D-line (channel type-A) and of the visible blind channel between D-line and urethral hypospadiac meatus (type-B) (Figure). In 62 patients, where the urethral plate tubularization was considered (Duplay, TIP), septs between channels were opened in the midline and a final width of the plate was measured by rolling the plate around a tube. RESULTS: Midline adhesions (D-line) were found in all 72 patients. Mean length of D-line was 5.13 ± O.17 mm. Mean plate width before dissolution was 5.9 ± 0.15 mm, and after dissolution 7.8 ± 0.16 mm. A blind channel of type A was detected in 22 patients (31%), type B in 24 (33%), type A and B in 16 (22%), and none in 10 patients (14%). Mean final plate width after D-line dissolution and opening of septs between channels in 62 patients with urethral plate tubularization was 8.7 ± 0.15 mm. DISCUSSION: The main contribution of our study is a new perspective of distal urethral plate anatomy that enables enlargement of the epithelized surface of the distal urethral plate by dissolution of the preexisting epithelized groove and opening of epithelized channels within the plate. To the best of our knowledge, this anatomical anomaly has not been described previously. CONCLUSIONS: The distal urethral plate of all hypospadias patients is partially "folded" in the midline by epithelial adhesions of different depth and extent that may be easily dissoluted. In half of the patients (53%) the "folded" part of the plate continues proximally as a blind channel inside the urethral plate (type A channel). Opening of these structures together with the well-known urethral plate pits (type B channel) helps augment the width and the overall epithelized surface of the distal urethral plate.

Isolated low initial differential renal function in patients with primary non-refluxing megaureter should not be considered an indication for early surgery: A multicentric study.

INTRODUCTION: Low initial differential renal function (DRF) in patients with primary non-refluxing megaureter (PNRM) is considered an indication for surgery as are an increase of dilatation and symptoms. OBJECTIVE: We hypothesized that low DRF is not necessarily a result of obstruction, but may be due to impaired development of the upper urinary tract. Thus, in the absence of symptoms, there is a low risk for further loss of renal function. This study aimed to assess whether initially low DRF is a reliable indicator of obstruction. STUDY DESIGN: We reviewed data from four university centers between 1995 and 2010. Patients under 12 months of age with unilateral primary non-refluxing megaureter (PNMR) and a DRF between 10% and 40%, and followed minimally 24 months, were included. Patients were placed in two groups based on management: group A, surgical; group B, conservative. The dynamics of DRF in relation to age and type of treatment was studied. In each patient we recorded the earliest (initial) DRF, the last known (final) DRF, the age when MAG-3 scans were performed and the type of treatment. RESULTS: From 25 patients, 16 were treated surgically (group A) and 9 followed conservatively (group B). The initial mean DRF in group A was 33.1% and in group B 34.5%, at a mean age 3.0 and 3.6 months, respectively. The final mean DRF in group A was 40.1% and in group B 43%, at a mean age 59.9 and 46.3 months, respectively. Using two-way repeated ANOVA (age [initial DRF, final DRF] vs. group [group A, group B]), we found non-significant difference between the groups in the DRF, F (1, 21) = 0.96, p = 0.338, while we observed statistically significant and similar increase from the initial to final DRF in both groups, F (1, 21) = 16.66, p = 0.001 (Figure). DISCUSSION: This is the first study focusing on the evolution of renal function in patients with PNRM and low initial DRF. Results suggest that the diagnosis of obstruction is inaccurate in most infants with unilateral PNRM if it is based on low initial DRF only. Renal deterioration rarely occurs in asymptomatic patients, and even profoundly impaired kidneys have potential for improvement. Limitations of our study include retrospective design and lack of standardization of treatment among the four centers. CONCLUSION: Low DRF in asymptomatic and anatomically stable patients with PNMR should not be considered an indication for early surgery. These findings challenge current practice and should be confirmed by a prospective study.

Unstented laparoscopic pyeloplasty in young children (1-5 years old): a comparison with a repair using double-J stent or transanastomotic externalized stent.

OBJECTIVE: To evaluate feasibility of unstented laparoscopic pyeloplasty in young children to prevent pyelonephritis and second anaesthesia. PATIENTS AND METHODS: During 2006-2013, 70 children (1-5 years old) underwent laparoscopic pyeloplasty for high grade hydronephrosis. Unstented repair was indicated in 34 children (GroupL1), double-J stent was placed in 21 patients (Group L2) and uretero-pyelostomy stent (Cook) in 15 patients (Group L3). Stenting was preferred in large thin-walled pelvis, thin ureter, kidney malrotation, and unfavourable course of crossing vessels. The outcome was compared with age-matched group of 52 children who had open surgery during 1996-2006 (Groups O1, O3). RESULTS: Operation times were significantly shorter in Groups L1 and L2 than in Group L3; the times were shorter in open repairs. Three patients with crossing vessels from Group L1 had urine leakage and one had obstruction (11.4%). In Group L2, one patient had obstruction, one incorrect placement of the stent, and one girl had serious pyelonephritis (14.3%). In Group L3, displacement of uretero-pyelostomy occurred in one patient (6.7%). There is no statistical difference between laparoscopic groups and between laparoscopic and open groups. CONCLUSION: Unstented laparoscopic pyeloplasty is a safe procedure in selected young children with favourable anatomical conditions preventing additional anaesthesia and stent-related complications.

Torsion of spermatic cord in children: a review.

The current opinion on spermatic cord torsion is discussed in this review, with special attention to natural history, value of diagnostic tools, evidence for surgical management, outcome and management of atypical forms of torsion.