Thoracoscopic repair of tracheoesophageal fistula and esophageal atresia.

PURPOSE: To evaluate the safety and efficacy of the thoracoscopic repair of esophageal atresia and tracheoesophageal fistula (EA/TEF). METHODS: From August 2005 to March 2008, 23 consecutive patients (16 boys, 7 girls) weighing 1,070 to 3,390 g underwent thoracoscopic EA/TEF repair. Nine cases had associated malformations. Two 5-mm and one 2.5-mm trocars were placed. The 5-mm 25- to 30-degree telescope was preferred. A pneumothorax was maintained with 5 to 6 mm Hg. The azygos vein was never divided. The TEF was closed with 5-mm titanic clips mainly.The esophageal anastomosis was made over 6-French nasogastric tube by three to seven simple stitches of 5-0Vicryl. The 8-French chest tube was left without suction. The enteral feeding was usually started on postoperative day 3 or 4. Barium swallow was performed on postoperative day 5 or 6, then the chest tube was removed. RESULTS: All procedures were successfully completed without conversion. The average operative time was 131 minutes (range, 55-245 minutes) with significant improvement after gaining experience (mean, 171 minutes for first 10 cases and 98 minutes for last 13 cases). There were two cases of accidental tracheal opening. The anastomotic leak rate was 13% (three cases), and all were healed on conservative treatment. Four cases required one to three courses of anastomotic stricture dilatations. There were three deaths (13%) of causes not related with performed operation. CONCLUSIONS: The thoracoscopic repair of EA/TEF is effective method, and based on our experience, it is the procedure of choice if performed by an experienced endoscopic pediatric surgeon. The intraoperative complications observed if properly managed have a good prognosis.

Laparoscopic splenectomy in children: experience in a single institution.

PURPOSE: To assess the safety and effectiveness of laparoscopic splenectomy in children. MATERIALS AND METHODS: Hospital records of 63 patients who underwent laparoscopic splenectomy between 1998 and 2005 were reviewed retrospectively. In 16 patients concomitant cholecystectomy was performed. All procedures were performed by the same surgeon. The indications for splenectomy were hereditary spherocytosis (n = 35), idiopathic thrombocytopenic purpura (n = 22), autoimmune hemolytic anemia (n = 3), and other diseases (n = 3). Details of operative technique were reviewed and their implications on intraoperative complications are analyzed. The postoperative course and long-term results were assessed. RESULTS: There were 35 girls and 28 boys, whose average age was 11.3 years (range, 3.9-19.5 years). There were 7 conversions, mainly at the beginning of the series. A mild degree of intraoperative bleeding was observed in 23 (36.5%) cases. In two cases (3%) severe bleeding led to conversion. Postoperatively, 1 patient required blood transfusion and 1 patient had signs of mild general infection that was treated conservatively. There was no mortality in this series. The mean operation time was 134 minutes for splenectomy and 174 minutes for splenectomy and cholecystectomy. Operative time did not significantly diminish at the end of the 7-year study period. CONCLUSION: Laparoscopic splenectomy in children performed by an experienced team proved to be safe and effective with minimal side effects and should be recommended as a procedure of choice in children who require splenectomy.

[Diagnostic difficulties in pediatric abdominal pain with potential appendicitis]. / Trudnosci diagnostyczne przyczyn bólów brzucha okolicy wyrostka robaczkowego u dzieci.

BACKGROUND: Pediatric abdominal pain is one of the most common symptom of children brought to attention of primary care physicians and pediatric surgeons. In many children clinical symptoms may be uncharacteristic and may lead to diagnostic difficulties. AIM: Clinical analysis of children with right lower quadrant abdominal pain suspected of appendicitis or children with periappendicular mass. MATERIALS AND METHODS: The histories of 14 children aged from 18 months to 17 years treated in Pediatric Clinic were analysed. All children were operated because of right lower quadrant abdominal pain or abdominal mass before admission to the Pediatric Clinic or during hospitalization in Pediatric Clinic. Intraoperatively in all children pathologies other than appendicitis were the cause of symptoms. RESULTS: The most often Crohn's disease were recognized (9 children), in 2 cases with concomitant other pathologies (fecal tumor of appendix in one case and with peritoneal abscess after perforation of intestinal wall). Sporadically the inflammation of the mesenterial lymph nodes caused by Yersinia infection suggested appendicitis. In one boy with ulcerative colitis, during exacerbation of the disease appendicitis complicated by rupture and peritonitis was observed. In 18-month old child with right lower quadrant abdominal mass invagination complicated by perforation of the ileum was recognized. In the case of 14-years old boy 6 months after appendectomy we observed mechanical intestinal obstruction complicated by perforation and peritonitis. Carcinoid of the appendix was the cause of abdominal pain in one child. CONCLUSIONS: 1. Appendicitis is the most frequent surgical etiology of the right lower quadrant pediatric abdominal pain. 2. Despite new diagnostic imagines there are no definite criteria to recognize appendicitis, in most cases physical examination and very carefull evaluation of abdominal pain are the most important. 3. All children with periappendipected of Crohn's disease. 4. All children with equivocal presentations of appendicitis and with normal appendix during operation should undergo further diagnostic evaluation.

Single nucleotide polymorphisms in the RET gene and their correlations with Hirschsprung disease phenotype.

Hirschsprung disease (HSCR) is a congenital, heterogeneous disorder, characterized by the absence of intestinal ganglion cells. Recent advances show that the RET gene is a major locus involved in the pathogenesis of HSCR. The aim of this study was to analyse if the HSCR phenotype in the Polish population is associated with the presence of polymorphisms in exons 2, 3, 7, 11, 13, 14 and 15 of the RET gene. Molecular results were compared with clinical and long-term follow-up data in 70 Polish patients with HSCR (84.3% with a short segment and 15.7% with a long segment of aganglionic gut). Single-nucleotide polymorphisms were analysed by using the minisequencing SNaPshot multiplex method. The 135G>A polymorphism in RET exon 2 was overrepresented in HSCR patients, compared with a healthy control group. Moreover, the 135G>A variant was shown to be associated with the severe HSCR phenotype. Two other polymorphisms, 2071G>A in exon 11 and 2712C>G in exon 15, were underrepresented in the patients. The results confirm that these RET polymorphisms play a role in the aetiology of HSCR.

[Prenatal diagnosis of congenital anomalies requiring surgical management in the Lower Silesia--fact or fiction?]. / Diagnostyka prenatalna wad wrodzonych wymagajacych leczenia chirurgicznego na terenie Dolnego Slaska--fakt czy fikcja?

In order to assess the current status of prenatal diagnosis of congenital anomalies requiring surgical management in the area of Lower Silesia, a retrospective study of medical charts of all neonates admitted to the Department of Paediatric Surgery in Wroclaw between 1997 and 2000 was undertaken. 218 babies affected by anomalies detectable antenatally by conventional ultrasound scan were subjected to the further study. Neural tube defects and abdominal wall defects were the most common and were diagnosed in 63 and 38 children respectively. The incidence of other congenital anomalies; hydrocephalus, oesophageal, duodenal and intestinal atresia, diaphragmatic hernia and urinary tract anomalies was similar in each group. The suspicion of anatomical defect or its precise recognition was made antenatally in 52 pregnancies (23.8%). The highest rate of positive prenatal diagnosis was noted among babies with hydrocephalus (50%) and urinary tract anomalies (42.8%). In other groups this rate was significantly less. Critical analysis of postnatal management in studied cases revealed that the babies with atresia of gastrointestinal tract, congenital diaphragmatic hernia and abdominal wall defects were negatively affected by a lack of prenatal recognition of the defect. Moreover, in more than 40% of all analysed pregnancies presence of risk factors (positive family history, course of pregnancy, mother's age, polyhydramnios, oligohydramnios, previous abnormal pregnancies) were identified that should have prompted an obstetrician to a through diagnostic evaluation of the foetus. The obtained results clearly indicate the necessity for the elaboration of a new protocol of antenatal care of pregnant women in the Lower Silesia. In case of suspected anomaly amenable to operative correction within the first days of life, paediatric surgeon should be an active member of a specialist team responsible for care of mother and her baby.

[Mediastinal tumors in children]. / Guzy sródpiersia u dzieci.

Mediastinal tumours are not very common among various tumours in children. The aim of our study was to evaluate the clinical features, diagnostic approaches and surgical management in 125 patients treated in our Department for 30 years. There were 39 lesions in the anterior, 21 in the medial and 49 in the posterior mediastinum. Solid tumours were diagnosed in 78% of patients. The range of age was between 4 days and 18 years of life. Patients below 5 years of life accounted for 68%. The most common were neurogenic tumours--35%, less frequently tumours of lymphatic origin were seen. Complete excision was possible in 66% of patients, in 26% open biopsy examinations were performed. Enlarged thymus was under control in our out-patient clinic. Combined treatment (radio-, chemotherapy and surgical treatment) was introduced to deal with neurogenic tumours in children. Complete remission lasting 3 years was achieved in 71% of patients in this group. Comparing two periods of time (1970-85 v 1986-2000) no significant differences were found in the prevalence of any kind of mediastinal tumours.

[Assessment of function of the only kidney in children after nephrectomy due to Wilms tumor]. / Ocena czynnosci jedynej nerki u dzieci po nefrektomii z powodu guza Wilmsa.

UNLABELLED: The aim of the study was to evaluate kidney function in children after unilateral nephrectomy caused by Wilms' tumour. The study included 40 former patients of the Department of Paediatric Surgery, who underwent complex therapy of Wilms' tumour at the age from 6 months to 8 years (mean 3.42 +/- 2.5 years). In all patients unilateral nephrectomy was performed and chemotherapy was administered. Additionally 24 out of 40 patients underwent radiotherapy. The time of follow-up was 5-26 years (mean 12.2 +/- 5 years). The patients were divided into 2 groups: la--observation time over 10 years, lb--observation time less than 10 years. Another division was made depending on radiotherapy factor: group R+ (24 patients) and group R-(16 patients). The control group consisted of age-matched 24 persons. In all patients the following was evaluated: endogenous creatinine clearance (Ckr), TRP, RH2O, serum and urine concentrations of electrolytes (Na, K, Ca, P), serum concentration of beta 2-microglobulin (beta 2-M) and urine excretion of beta 2-M and NAG. RESULTS: In all patients Ckr was significantly higher than in the controls. The highest values of Ckr were found in group la, and the difference in relation to group lb was significant (p < 0.02). No difference of Ckr was seen between groups R+ and R-. The mean urinary concentration of beta 2-M was similar in all groups of patients and significantly higher than in the controls. Serum concentration of beta 2-M and urine activity of NAG in all patients were within the normal range. Urine excretion of Na was significantly higher in all patients comparing to controls. CONCLUSIONS: 1. In children, who underwent Wilms' tumour therapy, glomerular hyperfiltration may occur even many years after nephrectomy. 2. Higher urinary excretion of beta 2-M and Na along with normal NAG activity and normal excretion of other electrolytes are the evidence of persistent although slight renal function impairment. 3. Children after complex therapy for Wilms' tumour should be under nephrological care for a long time.

[Renal neoplasms in children]. / Nowotwory nerek u dzieci.

UNLABELLED: Nephroblastoma is the most common kidney tumor in Polish children. OBJECTIVE: to present clinical material and outcome of 533 children with renal tumors. MATERIAL: 500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002. Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts. All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months. Pre-operative chemotherapy was done to 93.8% pts. RESULTS: Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma. Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%). In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed. Surgical complications were mild and occurred only in 8.9% pts. RESULTS: 5-years overall survival of CS I pts (favorable and standard histology) is 93.48%, CS II--96.8%, CS III--84.4%, CS IV--67%, CS V--58%. The results of treatment of 33 pts with non-Wilms renal tumors have improved lately. 78.7% of our pts achieved 5-years overall survival. CONCLUSIONS: The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children. The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.

Laparoscopic splenectomy in children--a multicenter experience.

PURPOSE: Splenectomy is frequently required in children for various hematologic pathologic findings. Because of progress in minimally invasive techniques, laparoscopic splenectomy (LS) has become feasible. The objective of this report is to present a multicenter experience of 159 laparoscopic splenectomies. METHODS: Records of 159 children, who underwent LS in 3 pediatric surgery centers between 1996 and 2006, were reviewed retrospectively with a special questionnaire. The indication for splenectomy were hematologic disorders (147), esophageal varices (6), splenic cyst (5), and tumor of the tail of the pancreas (1). The LS was performed using standard laparoscopic technique and instrumentation. RESULTS: There were 75 boys and 84 girls. Median age was 12.5 years (range, 2-19.4 years). Laparoscopic splenectomy alone was performed in 118 cases and LS with cholecystectomy or cholecystotomy in 36. In 5 cases, LS was performed together with fundoplication. Eight LS required conversion to an open procedure for following reasons: severe bleeding (3), massive splenomegaly (1), anatomical (2), and technical aspects (2). Accessory spleens were identified in 20 cases (12.6%). There was no mortality. Complications consisted of 8 conversions and postoperatively of mild generalized infection in 3 children and abdominal bleeding that resulted in re-laparoscopy in 1 case. CONCLUSIONS: Laparoscopic splenectomy can be safely and effectively performed in children.

Percutaneous internal ring suturing: a simple minimally invasive technique for inguinal hernia repair in children.

BACKGROUND: Laparoscopic herniorrhaphy in pediatric surgery is usually performed through three ports in the abdominal wall with intraperitoneal suturing. Our technique of percutaneous internal ring suturing (PIRS) requires only one umbilical port and needle puncture point. We describe the technique and evaluate the efficacy of percutaneous internal ring suturing for inguinal hernia repair in children. MATERIALS AND METHODS: We performed percutaneous internal ring suturing on 106 children (ages 28 days-14.5 years) with 140 hernias. The procedures were performed under general endotracheal anesthesia. Pneumoperitoneum was established with an open technique. Under laparoscopic-guided vision an 18-gauge injection needle with a nonabsorbable thread inside the barrel of the needle was placed through the abdominal wall into the peritoneal cavity. By moving the injection needle, the thread passed under the peritoneum around the entrance into the hernia sac. The knot was tightened from outside and placed in the subcutaneous space. The contralateral open inguinal ring was closed in the same procedure. RESULTS: The average operative time was 19 minutes for unilateral and 24 minutes for bilateral hernias. Follow-up ranged between 18 and 29 months. Cosmetic results are excellent with almost invisible scars. There were 3 cases of intraoperative complication: incidental puncture of the iliac vein that required no treatment. There were 4 cases of postoperative complications: one case of ileus adhesion with bowel strangulation, and hernia recurrence in 3 boys, one of whom was reoperated with percutaneous internal ring suturing. In 5 boys, transient hydroceles were observed that disappeared spontaneously after 3 to 5 months. CONCLUSION: The percutaneous internal ring suturing method seems to be a simple and effective minimally invasive procedure with excellent cosmetic results. The rate of complications is comparable to other laparoscopic techniques of inguinal hernia repair in children. According to our experience, percutaneous internal ring suturing should be considered as a treatment alternative.

Assessing bladder capacity in children: is it feasible at all?

AIM: The aim of the study was to assess correlations between body parameters and bladder capacity in healthy schoolboys and schoolgirls. On the basis of those anthropometric data functional bladder capacity (FBC) in children was estimated. MATERIALS AND METHODS: The questionnaires were sent to 1,055 parents of children aged from 6 to 14 years. Voided volumes were assessed during uroflowmetry, which was performed under typical conditions in separate rooms (Urodyn 1,000, Dantec, Skovlunde, Denmark). Residual volume was measured thereafter. Precise anthropometric parameters were obtained in standing the position. Children with dysfunctional problems, those who voided less than 100 ml and those with residual volumes > 5% of voided volume were excluded. Statistical analysis was done on the basis of the results gathered from 288 children. Pearson's correlation coefficient between body measurements and voided volume was calculated (P < 0.05). Regression modeling was used to propose the best formula for calculating FBC in children. RESULTS: Mean FBC is higher in girls than in boys (P < 0.05). The correlation between FBC and flow parameters was found (0.3 < "r" < 0.5). The value of the coefficient indicates loose relationship. We revealed correlation between weight, height, and length of the trunk, pelvic dimension and FBC. The best mathematical equation to estimate FBC in children based on the body measurements fits to 54% of population (FBC = -268.4 + 7.38 x flow time + 3.15 x suprasteral height + 1.25 x thickness of fat fold.

[Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL]. / Diagnostyka i leczenie raka tarczycy u dzieci w Polscew analizie wieloosrodkowej dla PPGGL.

INTRODUCTION: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children. MATERIAL AND METHODS: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children. RESULTS: Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations. CONCLUSIONS: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined.

[Increasing flow parameters and bladder capacity and its implication in maturing of lower urinary tract]. / Znaczenie wzrastania szybkosci przeplywu moczu i pojemnosci pecherza moczowego w dojrzewaniu funkcji dolnych dróg moczowych.

UNLABELLED: THE AIM of the study was to estimate changes in urethral flow parameters and bladder capacity in children. MATERIAL AND METHODS: Parents of 1055 children in the range of age 6-14 years were asked to fill a provided questionnaire. Parents of 466 children answered and agreed to have their child investigated. On the base of the questionnaire 103 children were excluded. Because of technical problems 33 children did not undergo the investigation. Voided volume less than 100 ml excluded farther 42 children. Uroflowmetric study was performed by means of Dantrec flowmeter (Urodyn 1000). Residual volume was assessed by means of ultrasonography. The results of 288 volunteers were analysed statistically (149 boys and 139 girls). Mean age was 10 yrs SD -- 2 yrs. RESULTS: Voided volume and flow parameters were higher in girls. Although voided volume and flow parameters increased and voiding time decreased in relation to age, only the differences in average and maximal flow were found to be significant (p< 0.05). CONCLUSION: The goal in development of lower urinary tract is to achieve the possibility to empty the bladder effectively. For this purpose increasing flow parameters seem to be most important.

Results of p53 analysis in pediatric malignancies in Poland.

BACKGROUND: Mutations of the p53 gene are thought to be causally associated with the development of various neoplasms. In tumors overexpressing the wild-form of p53, its functional inactivation has been suggested, and MDM2 seems to be important in this process. We analyzed p53 in childhood solid tumors, as data on pediatric malignancies are still limited. PROCEDURE: The p53 gene was screened for mutations by the PCR-S SCP method and sequencing. p53, p21, and MDM2 proteins were analyzed by Western blotting. RESULTS: Overall, p53 mutations were found at a low frequency, 7% (9/126); the frequency calculated for sarcomas was also low, 8.6%. Interestingly, three of the nine detected mutations were new ones. p53 protein was demonstrated in all tumor histotypes, overall, in 63% (43/68) of the tumors, with 18% showing marked overexpression. No p21 was found; and the 76 kDa MDM2 protein was demonstrated in 18% (6/33) of the sarcomas. CONCLUSIONS: In the series of pediatric malignancies studied, the frequency of p53 mutations was very low, whereas p53 protein was present in a high fraction of the tumors. Thus, in total, p53 abnormalities were frequent.

[The role of ret gene in the pathogenesis of Hirschsprung disease]. / Znaczenie genu RET w patogenezie choroby Hirschsprunga.

Hirschsprung disease is a congenital disorder with the incidence of 1 per 5000 live births, characterized by the absence of intestinal ganglion cells. In the etiology of Hirschsprung disease various genes play a role; these are: RET, EDNRB, GDNF, EDN3 and SOX10, NTN3, ECE1, Mutations in these genes may result in dominant, recessive or multifactorial patterns of inheritance. Diverse models of inheritance, co-existence of numerous genetic disorders and detection of numerous chromosomal aberrations together with involvement of various genes confirm the genetic heterogeneity of Hirschsprung disease. Hirschsprung disease might well serve as a model for many complex disorders in which the search for responsible genes has only just been initiated. It seems that the most important role in its genetic etiology plays the RET gene, which is involved in the etiology of at least four diseases. This review focuses on recent advances of the importance of RET gene in the etiology of Hirschsprung disease.