Results of varicocele treatment with laparoscopic Palomo technique in children and adolescents in years 2002-2012.

INTRODUCTION: Varicocele is caused by enlargement of internal testicular veins, including the pampiniform veno- us plexus. It is assumed that it occurs in 8% to 16% of the male population and predisposes to male infertility. The aim of our study was to assess laparoscopic treatment of the varicocele in children using the Palomo method. MATERIAL AND METHODS: In the years 2002-2012, our clinic treated 114 boys with the varicocele on the left side. In all patients the procedure involved ligation of veins, the testicular artery and lymphatic vessels with the Roeder knot. The average time of the procedure was 29 min. RESULTS: Varicocele recurrence was found in 2 out of 94 patients reporting for the follow-up visit, and this corresponds to 2.1% of the studied group. The postoperative hydrocele occurred in 20 patients, of which 5 (5.3% of the studied group) required surgical treatment with the Winkelmann technique, while in 15 (15.9% of the studied group) disappeared spontaneously during the average follow-up period of 1 year. No other intra- or postoperative complications occurred in any patient. Also no case of testicular atrophy was diagnosed. CONCLUSION: The results obtained from our laparoscopy patients using the Palomo method in treatment of varicocele can be considered satisfactory.

Clinical consequences of nephrectomy performed on medical grounds.

UNLABELLED: Epidemiological data show that chronic kidney disease (CKD) is a serious social problem and nowadays is included amongst civilisation diseases. Knowing the relation between decrease in the number of nephrons and progressing kidney damage caused by it, we can treated each nephrectomy from medical reasons as a risk factor for development of CKD. The aim of this study was a retrospective analysis of clinical effects of nephrectomy, taking into account evaluation of a functional status of the sole kidney. PATIENTS AND METHODS: The study covered 182 patients after nephrectomy in the period from 1979 to 2008. The morphological and functional status of the remaining kidney was evaluated using ultrasound scanning and on a basis of serum levels of creatinine, as well as the glomerular filtration rate. Occurrence of proteinuria and blood pressure values were also verified. RESULTS: The average creatinine serum levels increases after nephrectomy and is positively correlated with the age. The mean eGFR level is lower after nephrectomy and is correlated with kidney dimensions in the long-term control after nephrectomy, and with the age. The average systolic and diastolic blood pressure after nephrectomy in the long-term observation was higher as compare with values before procedure. Presence of protein in the urine of patients after nephrectomy correlated positively with increased blood pressure values. CONCLUSIONS: Nephrectomy constitute the risk factor of development of CKD and hypertension. Patients after nephrectomy needs systematic nephrological care to prevent CKD progression.

[Localization and functions of c-kit positive cells in the urinary tract]. / Rozmieszczenie i funkcje komórek cajala w drogach moczowych.

Interstitial cells of Cajal (ICCs) play an important role in the regulation of gut motility as they are responsible for the slow wave activity of smooth muscle. There is strong evidence that several subpopulations of ICC are present in the wall of the urinary tract. This review presents the currently available literature on the localization and proposed functions of interstitial cells of Cajal (ICC) in the urinary tract.

[True hermaphroditism in a 14-year-old boy--diagnostic and therapeutic difficulties]. / Obojnactwo prawdziwe u 14-letniego fenotypowego chlopca- trudnosci diagnostyczno-lecznicze.

The authors present a boy with a 46, XX/46, XY karyotype in whom true hermaphroditism was diagnosed, stressing the importance of establishing the cause of the condition and further management prior to discharging the neonate. The ultimate sex must allow the patient to best fulfill his/her psychosocial role in society.

[Partial gonadal dysgenesis in a 12-year-old girl--diagnostic and therapeutic difficulties]. / Czesciowa dysgenezja gonad u 12-letniej dziewczynki -- trudnosci diagnostyczno-lecznicze.

The authors present a 12-year-old girl who was seen in the out-patient Endocrinology Clinic, University Children's Hospital of Cracow, with the preliminary diagnosis of androgen insensitivity syndrome, presenting with progressing virilisation, breast underdevelopment and dysmorphy. Pubarche was normal. Gynecological examination revealed ambiguous external genitalia. On ultrasound examination homo genic gonad-like structures without follicles were seen, but no uterus was present. Basic hormonal studies indicated hypogonadotropic hypogonadism. The child was diagnosed as partial gonadal dysgenesis 46, XY. A gonadectomy was performed due to the 30-40% risk of gonadoblastoma development characteristic for such cases, and estrogen substitution followed after the surgery. The authors stress the necessity of including partial gonadal dysgenesis and partial androgen insensitivity syndrome in the differential diagnosis of delayed puberty. In both the above mentioned syndromes the clinical features are similar but the management differs. The authors stress the importance of establishing the cause of the condition and further management prior to discharging the neonate. The ultimate sex must allow the patient to best fulfill his/her psychosocial role in society and reduce the psychological trauma.

Suprasellar arachnoidal cyst as a cause of precocious puberty--report of three patients and literature overview.

The authors present three boys--3 years old, 5.8 years old and 10.4 years old--who were diagnosed with isosexual precocious puberty (IPP) triggered by a rare developmental disorder of suprasellar arachnoid cyst (SAC) accompanied by corpus callosum and fornix dysgenesis as well as anterior commissura magna agenesis (patient 1) and empty sella (patients 2, 3). The reason for diagnostic management recommendation was a rapid progression of IPP signs over one year (patients 1, 2) or 6 months (patient 3) prior to hospitalization, these signs having been present but less intense since infancy (patient 1), 4th year of life (patient 2) and approximately 8 years of age (patient 3). Neurological signs (spastic paresis in patient 1, postural tremor in patient 2 and head bobbing and behavioral changes in patient 3), as well as slowly progressing increased head circumference were observed since neonatal period (patient 1), 1 year old (patient 2) and approximately 4 years old (patient 3). None of the patients manifested hypophyseal-hypothalamic axis dysfunction other than IPP prior to and after surgical management. Shunt implantation resulted in gradual resolution of neurological signs in all patients and in patient 3 also in partial normalization of serum testosterone levels and growth rate. Regression of IPP in patients 1 and 2 was achieved by administration of a long-acting GnRH analogue. Our observations are in accord with data reported by other investigators and confirm the often slow, insidious development of subsequent SAC signs, the type and intensity of which differ from patient to patient. We suggest that some of the neuroanatomical anomalies coexisting with SAC may have a common genesis, or they could under certain conditions be an additional trigger for IPP and possibly other hypothalamopituitary dysfunction.

The retroperitoneal, inguinal approach to distal part of the ureter.

INTRODUCTION: The inguinal approach to the distal part of the ureter allows the surgeon to perform various types of procedures and is considered to be one of the minimally invasive techniques in pediatric surgery. We aim to describe our initial experience with the surgery of the distal ureter performed through an inguinal mini-incision. MATERIAL AND METHODS: Between March 2012 and June 2013, 8 patients were treated using a minimally invasive inguinal technique. The indications for surgical correction were single system primary obstructive megaureter, obstructive megaureter of the upper pole in a duplex kidney and distal ureteral stones. In all patients with single system obstructive megaureter and significant hydronephrosis, ureterocutaneostomy was performed. In one patient with duplex system primary obstructive megaureter and significant hydronephrosis of the upper pole, ureteroureterostomy of the dilated ureter to the normal caliber ureter in the distal part was performed. In the second patient with duplex system primary obstructive megaureter and reduced marker excretion of the upper pole in renal scintigraphy, ureterocutaneostomy was performed. In both cases of distal ureteral stones, deposits were removed by a simple incision. RESULTS: We did not observe any perioperative or postoperative complications. The imaging studies have shown that ureteral dilatation decreased in all but one child in whom the upper pole and the ureter were resected due to lack of function. CONCLUSIONS: The inguinal approach allows for the adequate visualization of the distal ureter, creating the conditions for implementation of the various procedures, reduces the risk of retrovesical plexus injury and minimizes visible scars.

Therapeutic results in stage I Wilms' tumors in children - 15 years of surgical experience.

INTRODUCTION: Progress in the therapeutic management of Wilms' tumors (WT) in children has resulted in the fact that presently, almost all children with stage I and favorable histology diseases have a chance for a cure. THE AIM OF THE STUDY: The objective of the report is an analysis of therapeutic results in children with unilateral stage I Wilms' tumors depending on the extent of tumor resection. MATERIAL AND METHODS: The analysis included children treated between 1993 and 2008 under the Polish Wilms' Tumor Study, initially following the protocol SIOP 93-01 (patients treated up to 2002) and subsequently SIOP 2001. Following neoadjuvant chemotherapy, depending on tumor size and tumor response to treatment, the children were qualified for a radical nephrectomy, simple nephrectomy, or nephron-sparing surgery. Following surgical treatment, all the children were subjected to adjuvant chemotherapy in keeping with the therapeutic protocol obligatory at the time. RESULTS: Within the 15 years, 111 children with Wilms' tumor were treated; the group included 43 children with stage I disease, which constituted 38.9% of the total number. Radical nephrectomies were performed in three (6.98%) children, simple nephrectomies in 32 (74.42%), and nephron-sparing surgery in eight (18.6%). Regardless of the employed surgical treatment modality, all the children are alive and none have demonstrated recurrent disease. The mean post-treatment completion follow-up period is 73 months (median value, 68 months). CONCLUSION: A simple nephrectomy and, whenever possible, nephron-sparing surgery represents sufficient treatment in 93% of children with stage I Wilms' tumor. To improve qualification for surgical treatment we propose the employment of NSS for stage Ia Wilms' tumors and SN for stage Ib Wilms' tumors.

Pediatric single port transumbilical nephrectomy and nephroureterectomy.

OBJECTIVE: To present seven cases of single incision laparoscopic nephrectomy and nephroureterectomy in children as a recent videoscopic innovation. PATIENTS AND METHODS: Seven children with nonfunctioning kidneys, three with multicystic dysplastic kidneys, two with end-stage renal nephropathy due to vesicoureteral reflux, and two with giant hydronephrosis were qualified to nephrectomy or nephroureterectomy. The surgery was performed transperitoneally using single incision access laparoscopy. The operative time was in the range of 50-90 min. RESULTS: There were no intraoperative or postoperative complications. The patients were discharged on the third postoperative day. The incision scars were hidden inside the umbilicus. CONCLUSIONS: Nephrectomy or nephroureterectomy using a single transumbilical port in children is a feasible and efficacious technique. The advantages are shortened convalescence, excellent cosmetic results, and reduction of potential wounds complications. However, clear indication of single site laparoscopic procedures in children remains to be clarified.

Retroperitoneoscopic nephrectomy in the prone position in children (point of technique).

We describe our initial experience with retroperitoneoscopic procedures in children in the prone position. Twelve children (five girls and seven boys; mean age 3.5 years) underwent nephrectomy, nephroureterectomy or heminephroureterectomy for obstruction or reflux within the non-functioning kidney, multicystic dysplastic kidney or upper pole dysplastic moiety. None of the operations were converted to an open procedure. The mean operative time for these procedures was 110 minutes. Blood loss was less than 20 ml.

Right atrial extension of Wilms' tumor.

BACKGROUND: Right atrial involvement in children with Wilms' tumor (nephroblastoma) is a very rare phenomenon. CASE REPORT: The authors present four children with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. In two children the intracardiac tumor extension was asymptomatic, while the other two had Budd-Chiari syndrome. Therapeutic management included initial three-drug chemotherapy in three patients, and primary tumor excision in one child, since the venous involvement had been not diagnosed. In each child, the therapeutic strategy was individualized and tailored to the course of the disease. Two patients were cured. The child with bilateral Wilms' tumor died due to disease progression. Another child died suddenly at home in the course of an intermission between consecutive cycles of successful chemotherapy. CONCLUSIONS: The extension of Wilms' tumor to the great vessels and the right atrium indirectly affects the final outcome. Preoperative chemotherapy in children with Wilms' tumor invading the inferior vena cava and the right atrium is the method of choice. The extent of surgery depends on the preliminary chemotherapy results.