Safety and Efficiency of Pyeloplasty in The First Six Weeks of Infants' Life.

PURPOSE: The aim of this study was to assess the safety and the efficiency of pyeloplasty in infants with ureteropelvic junction obstruction (UPJO) in the first six weeks of their life. MATERIALS AND METHODS: Clinical records of the patients who had surgery during first six weeks of life for UPJO between June 2009 and June 2014 were analysed retrospectively. RESULTS: In this period, twenty-six dismembered pyeloplasties were performed in twenty-four patients on mean operation age of 27.3 ± 10.2 days (range 8-42 days). On the first postnatal ultrasound all twenty-six renal units had SFU-4 hydronephrosis. Mean preoperative and postoperative anterior-posterior pelvic diameter and parenchymal thickness were 33.1 ± 8.9mm (range 14-49mm), 3.2 ± 1mm (range 1-4,6mm) and 14.7 ± 6.6mm (range 6-27mm) and 7.8 ± 1.9mm (range 3.0-10.4mm), respectively. The differences between preoperative and postoperative parenchymal thickness and anterior-posterior pelvic diameter were statistically significant (P ˂ 0.0001). Preoperative MAG3 dynamic renal scintigraphy showed obstructive pattern on the diuretic renogram in 26 units. Mean preoperative and postoperative differential renal function on dynamic renal scintigraphy of the affected renal units were 46 ± 15 and 44 ± 15, respectively. Postoperative drainage was normal on dynamic renal scintigraphy in 25 (96.2%) of the 26 units, redo-pyeloplasty was needed in only one unit (3.8%). CONCLUSION: In conclusion, patient selection and timing of surgery are very important in the protection of renal function in newborn with UPJO. In our opinion, if there is indication for surgery, early surgical intervention should not postpone in this period. Surgical treatment of UPJO during first six weeks of life is safe and effective.

Expression of androgen, estrogen, and progesterone hormone receptors in the penile tissues of children with different types of hypospadias.

OBJECTIVE: Androgen (AR), Estrogen (ER) and Progesterone (PR) hormones play an important role in the prenatal and postnatal development of urogenital tract and especially the penis. The expressions of AR, ER and PR receptors in penile tissues in children with hypospadiases had also been shown previously. In this leading study, to demonstrate of the sex hormone receptor expression in cases with different types of hypospadias were aimed. METHODS: This study was designed in children operated due to hypospadiases without DSD. Biopsy samples of 3 mm's were obtained from three different sytes as the lateral parameatal tissue and the anterior corner of the prepuce, and inner layer of posterior prepuce. The presence of AR, ER and PR receptors was investigated immunehistochemically. RESULTS: Mean age was 5.4 years in 18 children with hypospadiases; in totally 33 specimens were taken in 5 subcoronal as 5 specimens, and 7 penile as 15 specimens, and 6 penoscrotal as 13 specimens. According to sytes of samples; 13 samples were from lateral para-meatal tissues, and 13 were from anterior corners of prepuces, and 7 were from inner layers of posterior prepuces. In regard to receptor expression; ER and AR receptors were positive in 29 (87.8%) and 12 (36.4%) respectively; PR receptors were negative. CONCLUSION: This study emphasized the dominant expression of estrogen receptors in penile tissues of children with hypospadias. Although there was not a manifest correlation of androgen receptors absence in regard to the severity of hypospadias patients, there was a marked estrogen receptors presence in penile tissues. These findings suggest that the disrupted androgen and estrogen receptor interaction and/or balance could play a role during the development of external genitalia in hypospadias patients. Progesterone receptor was not present and therefore the active role in the postnatal development of hypospadias is still debatable.

Management of Transverse Testicular Ectopia with Persistent Mullerian Duct Syndrome.

According to additional anomalies, transverse testicular ectopia (TTE) is classified into three groups. Type-2 TTE, accompanied by persistent mullerian duct syndrome, constitutes approximately 20% of the patients. Surgical treatment should be planned after careful physical examination, ultrasonography, and genetic/endocrinologic evaluation. Herniorrhaphy, orchiopexy with testicular biopsy, and excision of the mullerian structures are the most appropriate surgical approaches in cases of TTE with persistent mullerian duct syndrome. We aimed to share our approach to the diagnosis and treatment of a patient with type-2 TTE. Possibility of TTE should be kept in mind in children with nonpalpable testis on one side and inguinal hernia on the other side.

Changes in cholinergic and nitrergic systems of defunctionalized colons after colostomy in rabbits.

BACKGROUND: This study was designed to assess smooth muscle function and motility in defunctionalized colonic segments and subsequent changes in pathways responsible for gastrointestinal motility. METHODS: Two-month-old New Zealand rabbits were randomly allocated into control and study groups. Sigmoid colostomies were performed in the study group. After a 2-month waiting period, colonic segments were harvested in both groups. For the in vitro experiment, the isolated circular muscle strips which were prepared from the harvested distal colon were used. First, contraction responses were detected using KCl and carbachol; relaxation responses were detected using papaverine, sodium nitroprusside, sildenafil, and l-arginine. The neurologic responses of muscle strips to electrical field stimulation (EFS) were evaluated in an environment with guanethidine and indomethacin. EFS studies were then repeated with atropine, Nω-nitro-l-arginine methyl ester, atropine, and Nω-nitro-l-arginine methyl ester-added environments. RESULTS: Although macroscopic atrophy had developed in the distal colonic segment of the colostomy, the contraction and relaxation capacity of the smooth muscle did not change. EFS-induced nitrergic-peptidergic, cholinergic-peptidergic, and noncholinergic nonnitrergic responses significantly decreased at all frequencies (0.5-32 Hz) in the study group compared with those in the control group (P < 0.05). CONCLUSIONS: Although the contraction capacity of the smooth muscle was not affected, the motility of the distal colon deteriorated owing to the defective secretion of presynaptic neurotransmitters such as acetylcholine, nitric oxide, and neuropeptides.

Occurrence of paratubal cysts in childhood: An analysis of 26 cases.

A retrospective analysis was conducted on the case files of patients operated on for adnexal pathology between January 2004 and December 2015 at our institution. The data of the patients with a diagnosis of paratubal cysts (PTCs) was extracted. A total of eighty-seven children were operated on for adnexal pathology. Twenty-six (29.9%) patients had PTCs. The patients were predominantly grouped as adolescents (n=22) and newborns (n=3). One of the patients was a pre-pubertal girl. Of the 26 PTC cases, 18 of them occurred on the contralateral side to ovarian pathology (OP) or physiologic ovarian changes (POC) such as corpus luteum cyst or follicular cyst which their final diagnosis confirmed by histopathology examination; in one case, the PTCs were bilateral; and in three cases, PTCs were ipsilateral with OP or POC. There were only 4 solitary PTC cases identified among the 26 cases. Paratubal cysts can be observed at every age, even in newborns. Their association with OP or POC in childhood cases needs to be clarified to further understand the hormonal mechanisms that are influencing the occurrence of these cysts.

The EXIT for Prenatally Diagnosed Cervical Cystic Teratoma: A Case Report.

The Ex-utero intrapartum treatment (EXIT) is a procedure performed during caesarean section while on fetal-placental circulation. We present a prenatally diagnosed cervical cystic mass causing tracheal compression which was managed successfully with the EXIT procedure.

Concurrence of Meningomyelocele and Salt-Wasting Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency.

Congenital adrenal hyperplasia (CAH) is a group of inherited defects of cortisol biosynthesis. A case of classical CAH due to 21-hydroxylase deficiency (21-OHD) with early onset of salt waste and concurrence of meningomyelocele (MMC) was presented here. The management of salt-wasting crisis which is complicated by a postrenal dysfunction due to neurogenic bladder was described. Possible reasons of growth retardation in the one-year follow-up period were discussed. A significant regression of the phallus with proper medical treatment was also mentioned.

Pure gonadal dysgenesis (Swyer syndrome) due to microdeletion in the SRY gene: a case report.

46,XY complete gonadal dysgenesis (Swyer syndrome) is a rare cause of disorder of sexual development. This syndrome is caused by a defect in the determination of sex during embryogenesis and is characterised with female external genitalia, normal or rudimentary uterus, and streak gonads, despite the presence of the 46,XY karyotype. Most of the studied cases presented with leak of secondary sex characteristics and primary amenorrhea during adolescence. Laboratory findings reveal hypergonadotropic hypogonadism. Herein we present the case of a female with a 46,XY karyotype who was admitted with delayed puberty and detected to have a microdeletion in the SRY gene and diagnosed to have Swyer syndrome. We highlight the importance of karyotype analysis in patients with delayed puberty and primary amenorrhea. Once the diagnosis of 46,XY complete gonadal dysgenesis is established, early laparoscopic removal of the dysgenetic gonads is crucial to prevent the development of gonadal malignancy.

Testicular cavernous hemangioma presenting as an ipsilateral lesion in a child with multicystic dysplastic kidney.

Childhood malignant testicle tumors are very rare. In the literature to date, it has been reported that there are only 24 cavernous hemangioma cases existing in English literature. In this study, we discuss a testicular mass case which was diagnosed as cavernous hemangioma. The patient, who followed for left multicystic dysplastic kidney since his birth, was admitted to our clinic with complaints of left testicular mass and pain at 1-year of age. Histopathological investigation revealed cavernous hemagioma. Even if it has its characteristic ultrasonographic findings, radiology is too far beyond to eliminate the malignancy, final diagnosis can only be made after orchiectomy.

Four-year retrospective look for acute scrotal pathologies.

OBJECTIVE: A group of diseases in the scrotum setting forth by the sudden swelling and redness and pain consist of acute scrotal pathologies. The most common causes of acute scrotum in children are epididymitis, epididymo-orchitis, orchitis, testicular torsion, torsion of the appendix testis, incarcerated inguinal hernia and traumatic hydro/hematocele. In this study; we aim to evaulate patients with acute scrotal pathologies who were interned in our department. METHODS: All hospital data of cases who were interned at our deparment due to acute scrotum in between June 2010-June 2014 were evaluated retrospectively. Cases with incarcerated inguinal herni were excluded in this study. RESULTS: In a 4-year-period 114 cases were interned in our department with acute scrotum. Mean age of the patients was 7.6±4.577 years (min: 1m-max: 18yrs). Doppler US was performed in 112 patients to evaluate the blood flow while in 2 patients applied after normal office hours were evaluated without Doppler US and operated under emergency conditions. The patients had received diagnosis of epididymitis/epididymo-orchitis/orchitis (n=83 cases; 72.8%), testicular torsion (n=24; 21.1%), torsion of the appendix testis (n=2; 1.8%) with and traumatic hydrocele/ hematocele (n=5; 4.4%). While detorsion was performed in 18 (75%) cases with testicular torsion and orchiectomy in 6 (25%) cases. Histopathological evaluation of orchiectomy specimens revealed hemorrhagic necrosis and hemorrhagic infarction or ischemic changes. Normal testicular size and vascularity were detected in 11 (61.1%) cases with detorsioned testis as detected by follow-up Doppler US. Late orchiectomy was performed in 7 cases (38.9%) with complete atrophy due to lack of blood supply. CONCLUSION: Although Doppler US is very helpful for differential diagnosis of patients with acute scrotum who applied early period, if Doppler US will lead to a waste of time, direct surgery without delay will reduce the risk of testicular loss.

Clinical features and long-term outcomes of idiopathic urethrorrhagia.

The aim of this study was to describe the clinical features and long-term outcome of the patients who were treated at our institution for idiopathic urethrorrhagia. The data of 10 male patients, who underwent cystoscopy between October 2010 and March 2013 due to urethrorrhagia, were evaluated retrospectively. Ten male patients aged between 8 and 16 years at first submission. Four patients (40%) had low voiding frequency (2-3 per day). Three of the four patients had abnormal uroflowmetry/EMG findings. Cystoscopy was done in all patients which revealed bulbar urethral inflammation and hemorrhage in all. Symptoms were not resolved on three of the patients who were under observation, having symptoms on average for 29.6±10.5 months. Complete resolution developed in the other seven patients. Six of the patients` symptoms were resolved soon after cystoscopy. In the patients' with or without normal uroflowmetry/EMG findings urethrorrhagia resolution rates were 86% and 33%, respectively. In the evaluation of urethrorrhagia; detailed history taking, basic laboratory investigation and cystoscopy are enough. The typical patients may be treated expectantly. In our opinion, it seems that dysfunctional voiding and infrequent voiding might cause delayed remission and/or recurrence of urethrorrhagia. Even though, it does not effect the treatment, in the persistent cases, confirmation of diagnosis by cystoscopy helps to lessen the anxiety of the family and might decrease the use of many unnecessary diagnostic tools in the long term follow ups.

Single center experience in patients with unilateral multicystic dysplastic kidney.

OBJECTIVE: To determine the clinical features, long-term outcomes, and additional urological anomalies of patients treated for multicystic dysplastic kidney (MDK). MATERIALS AND METHODS: Patients with MDK who were followed between January 2004 and October 2012 were reviewed retrospectively. Demographic, clinical, laboratory, and radiological data were evaluated. RESULTS: A total of 68 patients with MDK were followed for a mean period of 46.8 ± 32.4 months. MDK was detected by antenatal ultrasound in 64 (94.1%) of the patients. Ten patients had (14.7%) additional urological anomalies in contralateral kidney. Vesicoureteral reflux was found in five patients (7.3%). Other urological anomalies were megaureter (two), cortical renal cyst (two), ureteropelvic junction obstruction (one), and renal ectopy (one). Urinary tract infection was detected in 14 (20.5%) patients. Four (5.9%) patients had hypertension. Compensatory hypertrophy was detected in 29 (42.6%) patients. Eight (11.8%) of these 29 patients had glomerular hyperfiltration and three (4.4%) of these eight patients also had proteinuria. Follow-up ultrasound revealed complete involution in 19 (35.8%) patients. Nephrectomy was performed in 15 (22.0%) patients. Indications of nephrectomy were, recurrent urinary infection (four), hypertension (three), ureterocele (two), renal calculi (one), flank pain (one), hematuria (one), persistent large cystic kidney (three), and atypical US findings (two). CONCLUSION: In patients with MDK, depending on the clinical, laboratory, and radiological findings, appropriate timing of operation by multidisciplinary approach is important for early detection and treatment of any possible complications.

Continuous-type splenogonadal fusion: report of a rare case.

Splenogonadal fusion is a rare congenital anomaly. It has two subtypes, discontinuous and continuous. Up to one-third of continuous-type fusion is associated with other congenital anomalies. We present a continuous-type splenogonadal fusion case that was found incidentally during indirect hernia repair; the testicle was preserved during excision. Laparoscopic exploration was helpful in identifying the isolated polysplenia as the origin of continuous-type splenogonadal fusion, and in excising the cord-like attachment proximally. The patient had no other associated anomaly.

Paraurethral Skene's duct cyst in a newborn.

Paraurethral or Skene's duct cysts are rare causes of interlabial masses in neonates. The diagnosis of Skene's duct cysts in the neonatal period is based on its location, in relation to the urethra, and the demonstration of transitional epithelium in the cyst wall. The distinguishing features of paraurethral cysts are the displacement of urethral meatus by the mass and a cyst containing milky fluid. Thus, we report a case of a Skene's duct cyst in a newborn which was treated by incision and drainage.

Liver abscess: increasing occurrence in premature newborns.

Neonatal liver abscess is a very rare condition associated with high morbidity and mortality rates. There seems to be an increasing trend of this rare condition amongst the newborns admitted to neonatal intensive care units. We report a case of liver abscess in a premature newborn and briefly review the literature and discuss its management.