Transient minimal hydronephrosis on contralateral kidney in infants with unilateral hydronephrosis: Is it an early sign of worsening of the affected kidney?

Background/aim: The criteria for surgical management of ureteropelvic junction obstruction are not well-defined, and there is a risk for loss of renal function before the operation. In this context, certain changes in contralateral kidney had been investigated in order to increase the sensitivity of diagnosis. In this study, we aimed to investigate whether contralateral transient minimal hydronephrosis (CTMH) can be considered as an "early alarm" sign for worsening of the affected kidney in infants with hydronephrosis. Materials and methods: A total of 182 infants (92 surgically treated and 90 conservatively followed-up) with unilateral hydronephrosis were retrospectively analyzed. Ultrasonography and renal scan findings were evaluated. Correlation between the appearance of CTMH, contralateral compensatory hypertrophy (CCH) on ultrasonography, and prognosis of the affected kidney were evaluated. Results: Among the surgically treated patients, 18 (19.6%) patients developed CTMH on average 7 months (0­13 months) before surgery. Among these 18 patients with CTMH, 12 patients (66.6%) had loss of renal function preoperatively, while this ratio was 29.7% on their counterparts (p = 0049). CCH was observed in 31 (33.7%) individuals in surgically treated patient group including all 18 patients with CTMH, while none of the conservatively followed-up patients developed CCH and/or CTMH. In the multiple logistic regression analysis, among the variables investigated, CTMH was found as an independent predictor of the deterioration in the affected kidney and of the poor prognosis (p = 0.011 and p = 0.0004, respectively). Conclusion: In our study, among the variables investigated, CTMH was found as an independent predictor of the deterioration in the affected kidney and poor prognosis in infants followed-up with isolated unilateral hydronephrosis. Additionally, CTMH can be considered as an "early alarm" sign for worsening of the affected kidney and the need for surgical intervention.

Surgery for the complex Wilms tumour.

The prognosis of children with Wilms tumour has greatly improved since the introduction of adjuvant radiotherapy and chemotherapy more than 70 years ago with a current overall long-term survival approaching 90%. Before this, surgery was the only option with around 20% survival, even in low-risk categories. The focus is now on management options for those patients in specific subgroups, who continue to have lower event-free survival and who suffer from the long-term effects of treatment. These include those with anaplastic histology, bilateral Wilms, biologically high-risk tumours and those that relapse (Aldrink et al. in J Pediatr Surg 54(3):390-397, 2019). The major advances that have already been made in risk assessment and tailoring the chemotherapy/radiotherapy to achieve maximum advantage with minimum toxicity and long-term morbidity have been predicated on safe and complete resection and staging of the tumour. There are, however, still surgical challenges faced with respect to resection of 'complex' tumours; thus, surgeons need to understand their role and advance their expertise in the overall treatment of children with Wilms tumour. These include very large tumours not responding to chemotherapy, ruptured tumours, tumours with intravascular invasion into the inferior vena cava (± 10%), heart and hepatic veins (± 4%) and Wilms tumours in horseshoe kidneys (< 1%) (Kieran and Ehrlich in Urol Oncol https://doi.org/10.1016/j.urolonc.2015.05.029, 2015). This review describes surgical strategies and techniques used in these situations, gleaned from the authors' experience in the surgical management of over 300 children with Wilms tumours in our centres over the last 30 years.

Ovary-sparing surgery for teratomas in children.

AIM: To share our experience in ovary-sparing surgery for teratomas in children. PATIENTS AND METHODS: The medical records of nine patients (mean age of 11.2 years, r 6-15 years) who had undergone ovary-sparing surgery for teratoma were analyzed retrospectively. Mean duration for follow-up was 29.5 months (r 15-75 months). RESULTS: Five patients suffered from chronic abdominal pain; two had acute colicky abdominal pain. In two patients, there was no presenting clinical symptom. Two patients were operated on emergency basis due to symptoms related with acute abdomen. On the other hand, seven were operated electively. Ultrasonography was performed in all patients. Additionally, MRI and tumor markers were performed in all but two. Main radiologic findings consisted of heterogenous cystic and solid ovarian masses predictive of teratoma. The definitive diagnosis in emergency cases were as follows: perforated appendicitis plus teratoma [1]; torsion of the ovarian mass with teratoma [1]. The final diagnosis in electively treated seven patients were: unilateral ovarian teratoma [4], bilateral ovarian teratoma [1], bilateral teratoma plus appendiceal inflammatory mass [1], unilateral teratoma and contralateral corpus hemorrhagicum cyst [1]. The operations were performed by open conventional surgery in six and laparoscopy in three patients. The procedures were ovary-sparing surgery in 12 ovaries, appendectomy in 2 patients and detorsion of ovary in 1 patient. The main indication for ovary-sparing surgery was the "existence of a perfect dissection plane between the tumor margins and healthy ovarian tissue". The remaining ovarian tissue was evaluated macroscopically for residual lesions. Frozen section was performed in three suspected patients and the ovarian margins were free of any tumor cell. The pathologic diagnosis was: mature cystic teratoma in 10, immature teratoma in 1 and corpus hemorrhagicum cyst in 1. The postoperative outcome and follow-up was uneventful. CONCLUSION: Heterogenous ovary mass containing solid and cystic portions with echogenic areas on ultrasound imaging is highly suggestive of ovarian teratomas. Emergent surgical intervention is indicated if there is any suspicion of ovarian torsion. Otherwise, MRI is performed for further radiological evaluation. Based on radiologic findings, ovary-sparing surgery can be safely performed if the preoperative diagnosis is teratoma and there is always a plane of dissection between the normal ovary and cyst wall.

The history of surgery in disorders of sexual development.

Disorders of sexual development (DSD) have been documented throughout human history with fascination. Healers of all cultures have struggled to explain, and later correct with surgery, the physical manifestations of DSD. DSD was portrayed in the mythology, legends, and art of the ancient Greeks, Romans, Sumerians, Babylonians, and Egyptians. Techniques of feminizing genitoplasties date to the time of Celsus in the time of Christ. Acceptable operative therapy for feminine phenotypes of DSD came in the 19th and 20th centuries. Masculinizing procedures, inherently more complex than feminizing genitoplasties, initially were variations of procedures for severe forms of hypospadias. Today most total penile reconstruction procedures use reconstructive and microvascular techniques invented in 20th century.

Resection of Inferior Vena Cava Without Reconstruction for Intravascular Intrusion of Wilms Tumor.

Persistent inferior vena cava (IVC) tumor thrombus in Wilms tumor patients represents a management challenge. We describe three pediatric cases with preoperative evaluation documenting complete IVC occlusion and well-developed collaterals. They underwent nephrectomy and tumor thrombus removal accomplished with circumferential resection of the retrohepatic IVC without vascular reconstruction. All patients are asymptomatic and disease-free at 9, 2.5, and 2 years after stopping therapy. Cavectomy without reconstruction is safe and well tolerated in Wilms tumor patients with completely occlusive IVC tumor thrombus. Additionally, when performed en bloc with nephrectomy and with clear margins, cavectomy obviates the need for radiotherapy per protocol.

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Does Voiding Cystourethrogram Exclude Posterior Urethral Valves in Late Presenting Cases?

INTRODUCTION: Delayed presentation of posterior urethral valves (PUVs) is a rare condition. Presentation and diagnosis of the patients with late PUVs are challenging. Voiding cystourethrogram (VCUG) is mainly practiced. In this study, we aimed to evaluate the children with late-presented PUVs, and the reliability of VCUG in this group. MATERIALS AND METHODS: Between January 2003 and December 2017 records of patients who were diagnosed with late-presented PUVs were analyzed. Delayed presentation of PUV was defined as patients who were diagnosed and treated after infancy. Cases were examined in terms of age at diagnosis, presenting symptoms, urinalysis, urinary ultrasound, urodynamic studies, VCUG, and dimercaptosuccinic acid scintigraphy findings. Postoperative follow-up conditions were also assessed. RESULTS: Seventeen boys were diagnosed with late-presented PUVs (mean age was 7.35 years). The most common symptoms at presentation were frequency (58.8%), day and nighttime incontinence (47%), and febrile urinary infection (41%). PUV was noted by VCUG in 10 patients alone. The classical sign of dilated posterior urethra was detected in 9 patients. The 10th patient had posterior urethral irregularity. Urethra could not be evaluated due to unsuccessful voiding in one patient. Six patients had normally appearing urethra on VCUG. Reflux was detected in nine (52.9%) patients. CONCLUSION: Late-presented PUVs may be missed on VCUG. Whether a PUV might be present is crucial in boys with a history of recurrent urinary infection, persistent reflux, and repetitive daytime incontinence. Based on our results, we conclude that cystoscopic examination should be preferred for those cases to diagnose PUVs regardless of VCUG results.

Testis sparing surgery for Leydig cell pathologies in children.

OBJECTIVE: The aim was to analyze testis-sparing surgical procedures in boys with Leydig cell pathologies. STUDY DESIGN: The hospital records of four boys with Leydig cell hyperplasia who underwent testis-sparing surgery for testicular masses between 2000 and 2012 were analyzed retrospectively. Tumor markers were evaluated and all boys underwent scrotal ultrasonography preoperatively. The hormonal profile was also analyzed for symptoms of precocious puberty. The testis was delivered through a high transverse inguinal incision and the tumor was excised by enucleation. After confirming the benign nature of the tumor with frozen-section examination, the testis was reinserted and fixed into the scrotum with absorbable sutures. All cases were followed-up with physical examination, scrotal ultrasonography, and measurement of ß-human chorionic gonadotropin (HCG), α-fetoprotein, and hormone levels. RESULTS: The mean age of the patients was 9.4 years (1.5-15 years). Testicular mass and scrotal asymmetry were detected in all cases. Ultrasonography was the main initial diagnostic modality for detecting testicular masses (Table). ß-HCG and α-fetoprotein levels were normal. Three cases had Leydig cell hyperplasia and one patient was diagnosed to have a Leydig cell tumor. Signs of precocious puberty were detected in the four patients. The mean follow-up period was 4.8 years (2-8 years). Neither recurrence nor testicular atrophy developed in the follow-up. Findings of precocious puberty continued in one patient with Leydig cell hyperplasia, in whom a 2-mm contralateral metachronous lesion was detected and enucleated successfully. DISCUSSION: Testis-sparing surgery with its potential long-term psychological, cosmetic, and functional advantages should be used in pediatric patients in whom a benign Leydig cell pathology is confirmed histopathologically. CONCLUSION: This intervention with good long-term results can easily be applied through a proper dissection plane in the testicle. Since testicular Leydig cell tumors in childhood have small rates of recurrence, this choice of treatment is efficient in patients with salvageable testicular tissues and normal levels of tumor markers.

A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation.

Altinay-Kirli E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

Testotoxicosis: Report of Two Cases, One with a Novel Mutation in LHCGR Gene.

Testotoxicosis is a rare disorder which presents as isosexual peripheral precocious puberty in males. Despite the pattern of autosomal dominant inheritance, sporadic cases also may occur. Due to activating mutation in luteinizing hormone (LH))/choriogonadotropin receptor (LHCGR) gene, early virilization and advancement in bone age are common with increased serum testosterone levels above adult ranges, despite low LH and follicular-stimulating hormone (FSH) levels. There are different treatment regimens, such as combination of bicalutamide (antiandrogen agent) and a third-generation aromatase inhibitor, that are reported to be well-tolerated and successful in slowing bone age advancement and preventing progression of virilization. We report here two patients who presented with peripheral precocious puberty and an activating mutation in the LHCGR gene: one with a family history and previously determined mutation and the other without family history and with a novel mutation (c.830G>T). Combination of bicalutamide+anastrozole was ineffective in slowing pubertal progression and bone age. Short-term results were better with ketoconazole.

Transvesicoscopic cross-trigonal ureteroneocystostomy in children: a single-center experience.

PURPOSE: To evaluate the safety and efficacy of transvesicoscopic ureteric reimplantation in children. PATIENTS AND METHODS: Seventeen ureteric units in 11 patients underwent a transvesicoscopic 'Cohen' ureteroneocystostomy in 2003-2007 and the results were retrospectively analyzed. There were four boys and seven girls. All patients had vesicoureteric reflux (VUR), except for one with paraostial diverticula. Six patients underwent bilateral and five unilateral transvesicoscopic reimplantation (a total of 17 units). RESULTS: The procedure was successfully completed in all patients. Mean operation time was 217 min in unilateral cases and 306 min in bilateral cases without perioperative complications, except for pneumoperitoneum development in two cases. In the early postoperative period, two patients developed macroscopic hematuria. Mean hospital stay was 3.8 days (3-5 days), except for one patient who suffered from urinary tract infection and needed longer hospitalization. Mean follow-up period was 4.5 years (3-7 years). One patient with bilateral VUR had passive unilateral grade I VUR on postoperative cystogram, giving a success rate of 91% (94% of ureters). This patient was followed conservatively. One patient had recurrent urinary tract infections without reflux. CONCLUSION: Transvesicoscopic cross-trigonal ureteroneocystostomy can be safely performed with a high success rate in children.

A short history of bladder exstrophy.

The first description of bladder exstrophy was noted on Assyrian tablets nearly 4000 years ago. Since then various papers has been published with an increasing rate. According to the available historical data, almost all operative techniques had been described during the last two centuries. We believe, the pioneers put a lot of work in this field and passed on their theoretic knowledge and surgical experience to the current era. Our duty is keep this treasure and add the benefits of recent new technological developments for the future care of our bladder exstrophy patients.

Histological evaluation of the testicular nubbins in patients with nonpalpable testis: assessment of etiology and surgical approach.

There is a controversy in the literature whether testicular nubbins carry malignancy risk and excision of the nubbin is necessary in patients with nonpalpable testis. It is also controversial whether vanishing testis has the same etiopathogenesis and risk with true undescended testis. The aim of this study is to investigate the histological findings of testicular nubbins in patients with nonpalpable testis and to question etiology and surgical indications for vanishing testis. We reviewed the histopathological results of 44 testicular nubbins in 40 patients (mean age: 4.1 years, range 1-13 years) with nonpalpable testis between 1992 and 2004, retrospectively. Exploration revealed 5 intraabdominal and 39 inguinal testicular nubbins. Of 44 specimens only 5 (11.3%) from inquinal testicular nubbins were found to have seminiferous tubules. Two of the five had seminiferous tubule structures with viable germ cells showing maturation correlating with age. The other two with scarce seminiferous tubules were seen on only a single area and one had Sertoli cells only. None of the excised tissue had malignant degeneration. The vas deferens was identified in 23 (52.2%), vessels in 26 (59%), calcification in 14 (31.8%) and hemosiderin in 12 (27.2%) of excised tissue. Presence of calcification in one-third of the nubbins supports vascular accident thesis in the etiopathogenesis of vanishing testis. The possibility for the presence of seminiferous tubules and viable germ cells in the testicular nubbin is low. These facts decrease theoritical risk of malingnancy. Therefore, an inguinal exploration for testicular nubbin in patients with vas deferens and vessels entering into the inquinal canal diagnosed at laparoscopy can be postponed untill testicular prosthesis implantation and the nubbin can be removed at this operation.

A therapeutic method for failed bladder augmentation in children: re-augmentation.

OBJECTIVE: To investigate the causes leading to the deterioration of previously successful bladder augmentation and to evaluate the efficacy of re-augmentation. PATIENTS AND METHODS: Between 1988 and 2004, 136 bladder augmentations were performed in two paediatric urological units in Hungary and Turkey. Re-augmentation was necessary in two patients after colocystoplasty and in three after gastrocystoplasty. A secondary augmentation was not required in any patients after ileocystoplasty. The clinical data of these five patients were evaluated. RESULTS: On the basis of the clinical signs and urodynamic studies, re-augmentation was performed 2-7 years after the initial augmentation cystoplasties. Anticholinergic therapy given before re-augmentation did not improve bladder capacity, intravesical pressure and/or bladder compliance. An ileal or sigmoid segment was used for the secondary augmentation. After re-augmentation, all five patients became continent, and showed marked improvement in their urodynamic parameters at a mean (range) follow-up of 6.8 (2-10) years. CONCLUSION: A decreased bladder capacity and/or compliance and increased bladder pressure after successful augmentation cystoplasty might be the result of: (i) impairment of the blood supply to the large bowel or gastric segment used for augmentation; or (ii) bowel mass contractions. Ileocystoplasty seems to be the "first-line" of choice for primary augmentation. Re-augmentation with a bowel segment is a suitable treatment if conservative treatment fails. Regular urodynamic investigations are needed for early detection of malfunction of the augmented bladder, and advising therapy.

Nonsurgical diagnosis and management of an inflammatory pseudotumor of the spleen in a child.

Primary splenic tumors in children are rare and usually benign. We report the case of a 7-year-old boy with a splenic mass that initially resembled an infectious process. Histopathologic examination of a specimen obtained using sonographically guided Tru-cut needle biopsy, performed after angiography, revealed an inflammatory pseudotumor of the spleen, an extremely rare benign lesion. The lesion has been managed conservatively with clinical and sonographic follow-up. This case shows that benign lesions of the spleen can easily be diagnosed using current radiologic and histopathologic techniques without the need for surgery, thus allowing total or even partial splenectomy to be reserved for patients in whom a mass cannot be diagnosed or in whom clinical progression is evident.